 
A LUPUS HANDBOOK: THESE ARE THE FACES OF LUPUS

With Updates for 2012, 2013 and 2017

Copyright ©2011 A. G. Moore Copyright ©2017 A. G. Moore

Web site: rhythmprismpublishing.com

Smashwords Edition

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All Rights Reserved. This book may not be reproduced in any form, in whole or in part (beyond that copying permitted by U. S. Copyright Law, section 107, "Fair Use" in teaching or research, Section 108, certain library copying, or in published media by reviewers in limited excerpts), without written permission by the author. Requests for permission should be sent to A. G. Moore, rhythmprismllc@gmail.com

Other books by this author:

A Book of Memories

Art Literacy

Florence Nightingale: Nurse, Pioneer

Jonas Salk: The Battle Against Polio

Rabindranath Tagore: Champion of a World Without Borders

Marie Curie: Pioneer in Science

The Modern British Empire: A Brief History

What Is Radioactivity? The Basics, With a Student Study Guide

Rabindranath Tagore and The Modern British Empire

Ernesto's Nose

Marie Curie: Radium, Polonium

Masks: A Hudson Valley Childhood

The Four Masters of Yuan and Literati Art: Tradition in China from Mongol Rule to Modern Times (in pre-publication)

These Are the Faces of Lupus

By A. G. Moore,

Editor: Mike Moore

Disclaimer:

Nothing in this book is meant to prescribe or endorse any treatment or medication. The opinions offered are those of the author and carry no kind of warranty as to accuracy or effectiveness. No source, website or otherwise, cited in this publication is endorsed by the author

This book is dedicated to my mother, and to my brother Everett.

### Acknowledgments

I thank my husband and my children for supporting me throughout the many edits of this book and throughout my "good" and "bad" days.

I thank my siblings who have traveled with me every day of my life.

I thank the members of my writing group: each one of them contributed significantly to this project.

I thank Maxwell Corydon Wheat, first Nobel Laureate of Nassau County, New York. His guidance and wisdom were invaluable influences on the development of this book.
Disclaimer

Nothing I say in this book is meant to suggest or recommend a treatment or remedy for any disease. I am not a doctor; I am a patient. Anyone who is sick, or suspects they might be sick, should see a doctor. What the book does suggest is that it makes a difference which doctor you see. And when you do receive advice, use the same critical faculties you would if you were buying a refrigerator or a new car. Inform yourself. Read everything you can. And it's usually a good idea to get at least a second opinion.

Keep in mind that any treatment plan, whether good or bad, impacts the patient more than the doctor. Mistakes made will be regretted by the doctor, but the consequences of those mistakes will be born most dramatically by the patient.

TABLE OF CONTENTS

Part I Profiles

Introduction to Profiles

Chapter 1\--Hannelore Kohl

Chapter 2\--Inday Ba

Chapter 3\--Seal

Chapter 4\--Flannery O'Connor

Chapter 5\--Ferdinand Marcos

Chapter 6\--Tim Raines

Chapter 7\--Barbara Enright

Chapter 8\--Ray Walston

Chapter 9\--J. Dilla

Chapter 10\--Elaine Paige

Chapter 11\--Michael Jackson

Chapter 12\--A. G. Moore

Part II The Mechanics of Lupus

Chapter 13\--Diagnosis

Chapter 14\--Physiology

Chapter 15\--History

Chapter 16\--Overlap Syndrome

Chapter 17\--Treatment

Chapter 18\--Prednisone

Chapter 19\--Causes, Correlations, Associations

Part III Lupus and the Patient

Chapter 20\--Photosensitivity

Chapter 21\--Finding a Doctor

Conclusion

Part IV Reference

Glossary

Laboratory Tests

Appendix

*Added in 2012, 2013 and 2017

*Electrical Stimulation of the Vagus Nerve (2017)

*NSAIDS (2017)

*Mitochondria May Hold a Key to Promising Therapy (2017)

*Lupus and Stress

*Plaquenil

*Azathioprine

*Methotrexate

*Lupus and Gender

Belly Fat and Stress

Cortisol and Insulin Resistance

Cortisol and Essential Hypertension

Footnotes

Photo Credits
2017 Introduction

There are many to whom I owe a debt for helping me with this book and for helping to bring it to the attention of the lupus community. I'd like to thank the Lupus Foundation for featuring the book on its Recommended Reading list. Jenny Thorn Palter, Editor of the Foundation's Lupus Now magazine, played a significant role. She was patient, helpful and kind as I sought review of the book.. Thank you, Jenny. I hope you are realizing your dreams, as you helped me to realize mine.

Since my book was published, a great deal has happened in lupus research. On area of investigation that appeals to me involves electrical stimulation of the vagus nerve. There is hope that electrical stimulation will become a relatively safe and effective treatment. A brief description of this research appears in the _Appendix_ of this book.

Another area of investigation involves interferon and mitochondria. As with electrical stimulation of the vagus nerve, this research holds promise for development of targeted, rather than global, treatment of lupus. A brief description, with references, is offered in the Appendix.

Introduction to Profiles

This was not an easy book to write. Culling through some of the personal stories was wrenching, though I found many of them, just about all of them, inspiring. While researching Flannery O'Connor's life I was so affected that I had to take a six month break. Upon completing Inday Ba's profile I tried, unsuccessfully, to contact her mother. And, after learning about Barbara Enright's precedent-setting successes, I shamelessly engineered a brief encounter with her at the 2009 World Series of Poker Tournament.

Over the last seventeen years I have read a great deal about lupus--books, medical monographs, online blogs. Some of the material was clinical, some subjective, some simply, heartbreakingly, plaintive. I intend my effort here to have a broader scope than any of these. I aim to be candid and not to disguise unpleasant truths. For example, a recent study suggests that more than 92% of lupus patients experience anxiety.1 This fact appears in a medical journal but is not likely be found in less scholarly publications, which tend to assume an encouraging, even solicitous, tone.

In my experience, selectively presenting, or sugar-coating information does not instill confidence. On the contrary, it leaves patients poorly equipped to participate in their care and to make important decisions. If people want to read up on a disease, then they deserve to understand the facts. It might be reassuring to learn that anxiety is not necessarily a personality flaw, or an indication that a patient is not adjusting well to being ill. Anxiety, they would discover, can be a symptom of lupus. I also think acknowledgment of this would encourage doctors to treat patients holistically, to address anxiety, gastrointestinal problems, arthritis, etc, as part of an organized disease process. When patient and doctor openly exchange information they can operate more cooperatively and successfully.

The first time a rheumatologist said the word "lupus" to me, I was going full steam in a new career and was finishing up the requirements for a masters degree. I was also married and raising two high school age children. There was no room for illness, but illness would not go away. Neither would any doctor say with certainty what it was that was messing up my life. Only once had the word "lupus" been mentioned as a possible explanation.

For more than four years I struggled through remissions and flares. Finally, physically and emotionally depleted, I stopped working. Several months later the lupus diagnosis was pronounced. It would take another ten years, however, for an effective treatment plan to be designed--one that would enable me to lead a somewhat manageable existence.

In all those four years, between the first suspicious symptoms and the eventual certitude of a diagnosis, I was sick. The absence of classification did not make me any less sick.

So many people are ill with this disease, or one like it, and they do not present with the clinical clarity that would induce a doctor to say, "Yes, you have lupus," or "No, you definitely do not have lupus." These people suffer, though no doctor has given their suffering a label. As hard as it is to be ill, it is harder to be ill and yet beyond the pale of medical treatment because nobody can figure out what is wrong with you.

_These Are The Faces of Lupus_ is for everyone: those who have been diagnosed, those who might be, those who are doing research because they don't feel well and want to know more. The book is also for family, friends and anyone who is simply curious.

My research is responsible, my sources legitimate. I do not attempt a detailed discussion of the biochemical aspects of this disease, though I refer the interested to literature that can examine such issues. There are appendices and glossaries, for people who love definition, as I do.

The reader will not find within this book an extended narrative of my experience with lupus, though I use my experience sometimes to illustrate a point. The personal profiles that are provided are of extraordinary people. I hope I do them justice, for they represent countless others who may not be well-known, but whose lives were touched, sometimes tragically, by a disease called lupus.
Part I

### The Profiles

### Introduction

It is not known how many people have lupus in the world. At best, only a rough estimate can be made because of the difficulty in diagnosing this chameleon-like illness. Whether the number is one in five hundred, one in five thousand or one in fifty thousand, this statistic represents individuals who deal with the challenges of illness every day of their lives.

As Flannery O'Connor once observed, illness is an isolating condition. The abundance of Internet forums dedicated to different diseases attests to this loneliness. Sick people reach out to others similarly affected. They are comforted by association and they learn by shared experience. The Internet phenomenon prompted me to include in my book profiles of personalities who have coped with lupus. Many of the names I mention will be easily recognized. Some are less well known. Every profile, however, is a story that I find instructive.

Lupus is a chronic disease, one of many autoimmune illnesses. It is not uniquely unpleasant or unfair, although it does have a unique set of symptoms. Everyone gets sick eventually. It is part of the human condition. Learning about lupus, or any chronic disease, has universal applicability. While the profiles in the book are not exactly parables, I hope they serve a similar function. Each person confronting this disease had life, family, ambition. Some lost their lives. Every one dealt with family and ambition differently. Whatever the manner and outcome of their struggle, at the end all shared one thing with each other and with us-their humanity.
Chapter 1

Hannelore Kohl

German Chancellor Helmut Kohl's Wife
It was about a quarter to nine in the morning. The pool at the Mirage Hotel opened at 9:00 sharp and my husband wanted to be there at exactly that moment so he could grab a prime chair. He tucked a water-warped potboiler under his arm and positioned a pair of impenetrable sunglasses on his nose, then bade me farewell. I strolled over to the window and looked below for his lanky form to appear on the patio. We always asked for a room with a pool view, so we could connect from a distance, each of us in our place, suiting ourselves with an activity that gave us pleasure.

The deck was not crowded: mornings tend to be quiet in Las Vegas. My husband, tall and square, moved languidly through the rows of chaise lounge and stopped at his preferred station. He stretched out indolently, his already tan skin inviting more unfiltered, cancer-inducing rays. I turned from the window and closed the curtains. My hot coffee had cooled to a comfortable temperature and the butter had melted invitingly on my over-sized roll. I unfolded the Las Vegas Review Journal and began to read, headlines first, then local news. On page two, an article about ex-Chancellor Helmut Kohl's wife caught my eye. She had committed suicide. I read on. She was depressed, the report said, because she had been forced by a severe sun allergy to lead a reclusive life in the country. The allergy was not only to sun, but to "bright lights"; exposure to light caused an extremely painful reaction. I put the paper down. It all sounded so familiar.

Some years before, when I was forty-five, a doctor raised the possibility that I might have lupus. Four years later, that possibility became a diagnosis. Many more years went by before a rheumatologist came up with a protocol for controlling my disease. This progression of illness and treatment is not unusual for lupus, a very idiosyncratic disease with a wide array of symptoms. Photosensitivity is just one of lupus' many clinical aspects, and affects as much as 50% of systemic lupus patients.1 I am part of that 50%.

I cannot tolerate sunlight and am sensitive even to certain types of artificial light. My reaction goes beyond rashes and skin discoloration;with sufficient exposure, it manifests with full-blown systemic inflammation. To complicate matters, I am also allergic to chemical sunscreens. Thus, I have learned to live an odd kind of life, out of the sun and away from direct UV radiation, such as is found in fluorescent lighting. I've accumulated a wardrobe of hats in different styles and colors. Most of my summer shirts are long sleeved and can be rolled down against UV exposure should I end up in an inhospitable environment. My husband has become expert at spotting the one table in a restaurant or chair in an airport that isn't bathed in overhead lighting. And when we go to Las Vegas, he spends mornings at the pool, within view, and I spend that time relaxing in our room--resting, reading and writing.

Maybe Mrs. Kohl didn't have lupus, but it sure sounds to me as though she did. And it sure sounds as though her disease had a devastating effect on her. She was alone when she died; her "allergy" separated her from her husband and the life she previously enjoyed. I learned later that her "light allergy" started as a reaction to penicillin, and I recalled my own allergy to penicillin, which first expressed as arthritis, and in a subsequent event as hives.

I surveyed the color-coordinated furnishings in my hotel room and savored the taste of my now-cool coffee. I felt a deep sympathy for Hannelore Kohl. Not only was her photosensitivity very possibly related to a systemic illness like lupus, but so, probably, was her depression. Something like 80 % of all lupus patients experience a disturbance in their emotional equilibrium as a direct result of the disease2. I could not help thinking that, with the proper medical and personal support, the outcome might have been very different for Mrs. Kohl.

I appreciated at that moment, as I have many times before, that despite the difficulties I have experienced because of lupus, I am very lucky. My lupus is "mild" and I receive excellent medical care. My husband is loving and supportive. He makes accommodations so that we are able to go away together, and so that I may live a happy, full life. Hannelore Kohl, and untold other patients, have not been so fortunate.

This book is for them. It is from a friend and a peer. I intend the book to be a resource for people seeking information and I would like it also to provide support for those who might not find it elsewhere. Lupus is not an abstract concept--it happens to people (and animals, too, it turns out, like President George H. W. Bush's dog, Millie).3 Consequently, I have written book not about statistics and laboratory tests (though these elements exist here, especially in part two) but about the way lupus affects lives.

I hope all of us, with lupus or without, will see something of ourselves in the individuals I describe, as I saw myself in the story of Hannelore Kohl.
Chapter 2

Inday Ba

Gothenberg, Sweden

Birthplace of Inday Ba

There's a scene at the beginning of the movie, _A Man Who Knew Too Little_ , in which a prostitute fights with her knife-wielding pimp. Police burst into the room and rescue the woman. We soon learn that the scene is a farce–the prostitute and her pimp are part of a TV audience participation show and the fight sequence is performed repeatedly, with different audience members. The role of the prostitute, Des, lasts for only a few minutes. It is played by a virtual unknown named Inday Ba, an actress who does not remain unknown for long.She hones her craft and in time is offered a contract with the Royal Shakespeare Company. She does not get a chance to fulfill the contract, however. Within days of signing, she falls ill and eventually loses her life to a determined enemy, systemic lupus erythematosus.

Inday Ba (N'Deaye), the daughter of a Senegalese father and Swedish mother, was born on August 10, 1972, in Gothenburg, Sweden.1 When she was an infant her parents divorced and Inday was raised by her mother.2 The impulse to act came early to the child, and endured even through the most ferocious stages of her disease.3

In 1991, at the age of 18, Inday graduated from what was the Swedish equivalent of an American high school4. With an eye to realizing her thespian ambitions, she moved to London and eventually enrolled in the Webber Douglas Academy of Dramatic Arts5. Webber Douglas was a venerable institution with a long list of notable alumni, including: Terence Stamp, Julia Ormond, and Angela Lansbury.

Shortly after graduating from Webber Douglas Inday Ba landed a variety of small roles; her IMDB filmography indicates that she performed professionally 22 times before finally getting a recurring part on the British TV medical drama, _Casualty_.6 The year was 2002 and it was a momentous time in Inday's life. Her career was taking off: she met and married her husband; she bought a home; she settled down in Somerset--and she learned she had lupus.By the end of 2002 Inday Ba was admitted to St. Thomas Hospital with acute kidney inflammation.7

Inday Ba continued to work despite her illness. She told no one outside her family about the lupus diagnosis. However, the disease took its toll. Inday said she could not be married with her illness, so she separated from her husband, sold her house, and moved to London.8

On the website established by her mother as a memorial, Inday is described as being very strong willed and motivated. She wanted, apparently, to combat her disease without resorting to more powerful medications than she already was taking. This underestimation of lupus' deadly potential very likely hastened Inday's death. While the medicines used to treat lupus are dangerous and often have unpleasant side effects, the choice to medicate or not can be a choice between life and death. It is the artful and precise application of powerful medicines, in the hands of skilled practitioners, which can tip the scales in favor of the patient over the disease.

The tragedy of Inday Ba's death had many authors. Stricken with a vicious form of lupus, she was under medicated and probably suffering from impaired decision-making. During her final crisis, when she was transferred to intensive care, it was determined that lupus was attacking her lungs, kidneys and brain.9

Further evidence of Inday Ba's mental deterioration can be gleaned from her memorial website which describes a notable transformation that had begun to take place in Inday's personality. Her mother paints a portrait of an exhausted, anxious young woman who had grave concerns about her future.10Inday's actions in the period preceding her death seem to confirm this observation. In December of 2005, already concerned about her declining health and feeling her mortality, she decided to travel to Senegal, where her grandmother lived.11The urge to connect with family is understandable–Inday Ba was not close to her father and had never met her grandmother. However, as strong as the impulse to bond might have been, it was clearly dangerous to undertake such a journey while she was evidently ill.

The final causative element in Inday Ba's premature death is the hardest for me to accept or to explain–medical misadventure.

On January 4, 2005, weeks after she returned from Senegal and one week before her final hospital admission, Inday's lip swelled. Alarmed, she sought treatment at St. Thomas Hospital. The doctor who saw her decided that her lip was swollen because of an undefined allergic reaction–this despite her history of lupus and associated kidney disease. He took no blood, ran no tests and sent her home.12

One (critical) week after the consult at St. Thomas, Inday Ba experienced severe shoulder and back pain. This time she turned to a chiropractor for help. He diagnosed muscle strain, worked what he believed to be the affected areas, and sent her home.13

The next day, feeling worse, Inday went back to St. Thomas. She was admitted immediately, with pneumonia. Lupus was raging in her body and her kidneys were in trouble. She never recovered from this episode. She spent the next three months in and out of the hospital and ultimately succumbed to the ravages of the disease.14

I first saw the name "Inday Ba" when I was searching through lupus websites, looking for material about well-known people who had the disease. That's all Inday Ba was to me, a name, material. Then I saw her picture and read her mother's sorrowful tribute. The swiftness and ferocity of the young woman's illness was startling, her determination to realize a dream, inspiring.

She was so close, when she died, to receiving the acclaim she had worked for. I went online again–not to look for a name this time, but to find a person. The movie she appeared in early in her career, _The Man_ _Who Knew Too Little_ , was on DVD and selling for about three dollars.

I waited to watch the film until I had time to concentrate. I didn't know what kind of part Inday Ba had, and I wasn't sure I'd recognize her. But once the credits rolled, and the action began, there she was, vivid, alive, sweet--the dynamic face her mother posted on the memorial website.

Inday Ba was a natural actress, convincing, beautiful.

Hello, Inday, I thought. Lupus cheated you of the recognition you earned. Well, here I am, and you live on, at least up there on the screen. I'm going to do whatever I can, in my small way, to let others know about you and your brave life.
Chapter 3

Seal

Paddington Basin

Seal's Hometown
*Since this book was first released in 2011, Seal and his wife have divorced. However, they assert that they intend to raise their children in an environment of mutual respect and cooperation.

Seal, the international recording star, understands that people react to the noticeable scars on his face. These distinctive marks are mistakenly taken for tribal wounds, a legacy perhaps of some ritual he endured as a child in Africa. But, Seal hastens to explain, the scars are not tribal; they are the result of a disease he has had since he was a teenager--discoid lupus. 1 At a 2008 benefit concert, Seal revealed that he had been suffering from the disease for more than twenty years.

Discoid lupus erythematosus is a disease in which skin lesions form, mostly on the face, neck and scalp. Though Seal has carried the mark of this illness since his youth, he accords it no more importance than he accords any other difficulty he has confronted. He has said that whatever happened in his childhood, as difficult as some of those things were, he has no regrets. All of it, the good and the bad, made him the man that he is today and so all of it was a necessary part of his growth2.

Seal Henry Olusegun Olumide Adeola Samuel, the second of six children, was born on February 16, 1963 in Paddington England. Not long after his birth he was sent to live in a foster home. The interlude with this foster family was apparently the happiest of his childhood. He remembers fondly that he was accepted by the foster parents as though he was their own child3.Unfortunately, the peaceful period came to an end when he was four years old. That is when his biological mother reappeared and took him to live with her4.

After two years she became ill and Seal changed hands once again as he went to live with his natural father and his stepmother. Seal characterizes the years that followed as"horrific"5Not only did the family live in great poverty but they also were constantly in tumult. Seal describes his father as being a violent man, who used whips and fists to enforce order. The father, Seal states, was the product of a home in which he also experienced violence. It was a cycle that was visited on succeeding generations. Understanding this, Seal determined that he would break the cycle and not repeat the mistakes of his forebears. When he left home, Seal explains, he left behind the violence. In order to become the man that he is today, Seal had to stand up to his father and strike out on his own6.

Seal was fifteen years old. He dropped out of school and worked at odd jobs to support himself. Eventually, he returned to school and earned an associate degree in architecture7. When he wasn't working, or in school, he focused on his true interest: music. He played in pubs, performing anywhere he could, before joining the band, _Push_ 8. With _Push_ he traveled through India where he experienced a kind of spiritual awakening9. Evidence of this new insight would appear later in his music. Finally, in 1990, his first big break came when he collaborated on a hit album with music producer Adamski10. The following year Seal produced his own album, and the smash single _Crazy_ was released11.

By 1996 Seal was an international brand.That year the music industry took note of his status by granting him its highest award: the Grammy. Seal received three of these awards: one for Best Male Pop Vocal, one for Record of the Year and one for Song of the Year12.

Although he enjoyed professional success and artistic fulfillment, the part of Seal's life that brought him most satisfaction was his family. In 2004 he married supermodel, Heidi Klum. Klum was expecting a child from a previous relationship when they married. Upon his marriage, Seal expressed a sense of fulfillment; he stated that with this new family he had finally achieved everything he always dreamed about. Seal adopted Klum's child, Leni, and the couple went on to have three more children together.

Being a father, for Seal, is the most important job in his life. It's important to him to do this job right, because he realizes that there are no second chances with his children 13

Seal today is a multimillionaire. He is a respected musician, with fans all over the world. At any point in his life he could have buckled to the challenges he encountered. Perhaps the few years he spent with a loving foster family gave him the personal resources to prevail over hardship. Or, perhaps he was gifted with an innate strength of character and that enabled him to overcome obstacles. Whatever the origin of his drive, however, it is inescapable that he has succeeded where many would have failed. He carried with grace and poise the evidence of disease; overcame a legacy of poverty; defied a childhood filled with violence; and contributed to the world a body of music that enriches and inspires.

Some people are defeated by adversity. Some succeed despite adversity. Seal seems to be one of those rare individuals who grow stronger because of adversity. He once said that his father's gift to him was to show him, by example, exactly what a father shouldn't do14.

Seal Henry Olusegun Olumide Adeola Samuel, whom the world knows as Seal, has an illness called lupus; he has transformed what could have been the stigma of disease into a kind of trademark. By any standard, this would be a significant achievement. However, for Seal, it is just one of many accomplishments in the life of a very remarkable man.
Chapter 4

Flannery O'Connor

Writer
Flannery O'Connor enjoyed in her lifetime the admiration of some of the most influential writers of her time--this despite the fact that her physical existence was defined, for most of her adult life, by illness.For her, sickness was a place and she described it in a letter to a friend as the only place she had ever been. In the letter she talks about the challenge of being ill as one of isolation. Sickness is a place without company; it is a place in which one must reside alone and in its solitary nature, it is "instructive" 1

Sickness may have had an enduring influence on Flannery O'Connor's life, but she did not allow it to determine her destiny. Mary Flannery O'Connor (she later dropped the Mary) was born on March 25, 1925 to Edward and Regina O'Connor in Savannah, Georgia2When Flannery was eleven Edward learned he had systemic lupus, a disease that was then shrouded in mystery3. Not until 1949–twelve years later–was a diagnostic tool (presence of LE cells in the blood) for the disease discovered and not until 1950 was an effective treatment (cortisone) available4. As Flannery so characteristically stated, when her father suffered from the disease the only remedy was the one offered by the mortician5.

From 1937 to 1941 the O'Connor family struggled to cope with Edward's disease; they moved first to Atlanta, where Edward sought work, then to Milledgeville, Ga. On February 1, 1941, Edward died. His death was widely reported as "sudden", largely because he and his wife had scrupulously concealed his illness6.

An only child, Flannery was strongly affected by her father's death. Consistent with what would become a lifelong inclination, she used religious symbolism to interpret the loss, comparing God's grace to a bullet in the side7. Years afterward she described her childhood as one of premature sophistication. She claimed that she was a very old twelve-year old. Only as an adult did she discover the levity that eluded her in her youth8. She demonstrated early in her life a tendency toward the peculiar. Fascinated by chickens, she made clothing for them and at six was filmed teaching a chicken to walk backwards9.

She pursued an MFA at the Iowa Writer's Workshop, and while there had her story, _The Geranium_ , published10. With the appearance of _The Geranium_ in a prestigious journal ( _Accent_ ), Flannery's acceptance into the literary community, and her future, seemed assured: she was shepherded around New York City by Robert Lowell; spent a year in residence at Yaddo (an artists' retreat); and sojourned with Robert Fitzgerald (a poet) and his family in Connecticut11. 11 She began to imagine for herself a certain kind of life. She wrote, in those early years when a bright future seemed within her grasp, that she could see herself never returning to Georgia and that if her mother did not live there, she probably never would12

In December of 1950, noting a debilitating soreness in her arms and generalized arthritis, she felt she should go home to Georgia for treatment. During the trip her symptoms worsened and by the time she arrived in Milledgeville she was terribly ill. Like her father before her, Flannery O'Connor learned she had lupus13.

Flannery weathered this crisis, but her plans for the future did not survive. Doctors warned that if she was to have a future at all, it would be in Milledgeville under her mother's care. Regina and Flannery O'Connor moved into a small farm, Andalusia, where Flannery spent the next thirteen years writing and indulging in a few favorite pastimes: raising peafowl, painting,shooting off volumes of correspondence. She directed her letters to friends, fans, literary colleagues and theologians. A significant number of her fans, she wryly admitted, were prison inmates, who identified strongly with the darkly drawn characters in her short stories.

Only occasionally did Flannery leave Andalusia--usually to give a lecture, and once, at her mother's insistence, to make a pilgrimage to Lourdes14.

Flannery's illness waxed and waned. From 1955 on she used crutches (steroid use often leads to osteoporosis), but whatever the state of her health, she continued to work. She early established the habit of writing for three hours a day, even when that writing seemed nonproductive15She described the dedicated labor of producing her first novel, _Wise Blood_. She was at it for five years and when it seemed she was nearly done, lupus struck. The disease not only robbed her of vigor, but it required her to take steroids, which took their own toll. High dose steroid therapy deprived her of sleep and put her in an almost manic state16

Though Flannery's physical existence was severely limited, her creative drive never diminished. Inspiration for most of her quirky characters came from observing relatives, neighbors and Andalusia's hired help. But her greatest source of inspiration, one overlooked by many of her more sophisticated readers, was religion. Flannery O'Connor was a committed and philosophically engaged Roman Catholic (Robert Lowell once wrote that he was dismayed by her preoccupation with religion and regarded those who encouraged her in this direction as "fanatics")17. She stated frankly that her writing served her religious convictions and that the writing could not be properly understood unless approached from this perspective18.

As Flannery neared her thirty-ninth birthday, doctors grew increasingly concerned about a large fibroid tumor, which they believed was causing severe anemia. While they acknowledged that any operation for Flannery would be risky, they decided the tumor had to be removed.

On February 25, 1964, a hysterectomy was performed19. Flannery was infused with cortisone before the procedure in order to combat potential kidney inflammation. The surgery was successful; unfortunately, the preoperative precautions were not. Flannery spent the next six months in and out of hospitals, battling inflammation and infection. Her lupus had flared with a vengeance and Flannery was fighting for her life.

Aware she was not likely to live for very long, Flannery nonetheless continued to work, completing two more stories20. One, _Parker's Back_ , she composed entirely in her head, only committing the final draft to paper, because she was too sick to sit at her desk. From her bed she also edited galleys of older stories, in preparation for a collection she hoped to release21.

Flannery O'Connor died on August 3, 1964, at the age of thirty-nine22. Seven years later she was awarded the National Book Award in Fiction for the posthumously published _The Complete Stories_ , the same book she had been working on in her last, sickest weeks22. During her brief career Flannery produced thirty-two stories, two novels, three story collections and over a hundred critical essays. Although she said she had always lived in sickness, in the years following her death the opposite has become true. Flannery O'Connor has lived, and will continue to live, virtually everywhere: on college campuses, in European salons, in the minds of people all over the world.
Chapter 5

Ferdinand Marcos

President of the Philippines
In February of 1986, President Ferdinand Marcos fled the Philippines with his wife, Imelda. When his residence, Malacanang Palace was opened, the opulence of the home became an international scandal. There were, among other personal possessions, 1000 ladies handbags; 508 ball gowns; 3000 thousand pairs of ladies shoes;1 a mural depicting Ferdinand as Adam and Imelda as Eve;2 and-a kidney dialysis machine3. The extravagance of the palace was taken as a testament to the corruption of the Marcos government and the existence of the dialysis machine as proof that Marcos was suffering from a life-threatening illness: systemic lupus erythematosus.

Reconstructing the story of Marcos' life requires separating the truth from an artfully crafted legacy. For example, it is a fact that as a youth Marcos spent four years in prison for murdering his father's political opponent, Julio Naludenson4. What is not clear, however, is that Marcos actually committed the murder, for the Philippine Supreme Court eventually exonerated him and released him from prison5. It is also a fact that Marcos received commendations after WWII for resisting the Japanese wartime occupation6 What is not clear is that he deserved these commendations: CIA records unsealed years later suggest that perhaps instead of being a hero, Marcos was a collaborator and engaged in black market trading with the Japanese7.

It is a fact that when Marcos left office in 1989 he was a very wealthy man. What is not clear is how he acquired this wealth. It has been suggested by many that the Marcos regime was a kleptocracy, that Marcos, his wife and his cronies enriched themselves at the expense of the Philippine people and economy. However, Mrs. Marcos has steadfastly explained that her husband's great wealth came from fortuitous gold trades and that he benefited greatly from a rise in the price of gold8.

It is a fact that Benigno Aquino, leader of the Philippine's strongest opposition party, was assassinated on the tarmac at Manila Airport9. What is not clear is that Marcos ordered the assassination, although Aquino's widow publicly blamed him for her husband's death.

Finally, it is a fact that Ferdinand Marcos died on September 28, 1989 of multiple organ failure after a lengthy and debilitating illness10. What is not clear is that he suffered from systemic lupus. His wife and representatives consistently reported, when he was president, that he was vigorous and in good health. However, during his third presidential term he was absent without explanation for long periods. And a palace physician who suggested that he had had a kidney transplant was later found murdered.12 Additionally, when the privacy of Malacanang Palace was breached, not only was a dialysis machine discovered, but also uncovered was a chamber equipped like a small hospital13.

The following biographical information seems to be reliable: Ferdinand Emmanuel Marcos was born on September 11, 1917 in Serrat, the Philippines14. His father, Mariano, was a local politician and was implicated, along with Marcos and two of his brothers, in the murder of Julio Naludansan15. In 1940, all four Marcoses were released from prison16. However, while in prison, Marcos did not waste time--he studied for the Philippine bar exam and passed with an almost perfect score. When this excellent result was challenged, Marcos retook the test orally and performed almost as well17.

In 1954 Marcos met Imelda Romueldez, a singer and beauty contest winner. After an eleven day courtship, the two were married. In honor of their eleven-day whirlwind relationship, Ferdinand gave Imelda an eleven carat ring18.

Marcos' first elective office was in the House of Representatives. He subsequently served in the Senate for three terms. In 1965 he was elected President of the Philippines and held that position until he was forced into exile19.

Some have characterized the Marcos government as a dictatorship. Certainly, Marcos eschewed constitutional restraints. Soon after his election he abolished the nation's 1937 constitution and dismissed the parliament. He imposed martial law during a time of unrest and kept it in place for seven years, lifting the police regimen only when a visit from Pope John Paul II was imminent20. During his twenty-one year presidency, Marcos managed to plaster his image just about everywhere. His picture appeared on billboards along the road, in the hallways of government buildings and beside blackboards in school classrooms21.

Although he served three presidential terms,questions were raised about the legitimacy of his last two elections. The perception of poll irregularities, a widespread belief that his administration was corrupt, and the Aquino assassination all contributed to the insurgency which forced Marcos from the country.

Opposition to Marcos was not unanimous. He had his supporters, not only the so-called cronies, but also those who feared communism and social disorder. These supporters point to his muscular response to the communist threat and his strict anti-crime measures. Also, he was a firm ally of the U.S, which reciprocated his friendship almost until the very end.

On their flight into exile, Ferdinand and Imelda traveled courtesy of the U.S. Air Force22 They left the Philippines as a very wealthy couple, though no one was ever been able to tally exactly how much they were worth. Most estimates ranged in the billions23. Some of their wealth they managed to bring with them on the two planes that transported them to the United States. On one plane Marcos assembled an entourage of family and close associates. On the second plane he stuffed as much of his treasure as he could physically fit24.

When news of the second treasure-laden plane got out, there was a public outcry and a formal protest from Corazon Aquino, the new President of the Philippines. The contents of that second plane were eventually confiscated by the U.S. and returned to the Philippines. The Aquino government continued its efforts to retrieve wealth appropriated by Marcos. In response to one suit, the Swiss government turned over 684 million (US) dollars that had been deposited in foreign accounts26.

Ferdinand Marcos spent his three-year exile in Hawaii, much of that time in the hospital. Because of his illness he did not have to answer charges of fraud and embezzlement that were pending against him in U.S. courts. His death came after a long hospital confinement, almost all of it in intensive care. Although details of the illness were never revealed by Marcos' family, the public assumption has been that he died of complications from lupus27.

The Aquino government would not allow Imelda Marcos to bring her husband's remains to the Philippines until 1992, when the family received permission to bury Marcos in his home province28His mummified corpse is on display there today, open for public viewing in a refrigerated crypt29.

In recent years the Marcos family has enjoyed a kind of political resurgence--two of the children have served in congress, numerous civil suits by the Philippine government to recover assets have been dismissed or defeated and Imelda, though eighty years old, expressed an interest in running for office31 . As a final act of Marcos brand rehabilitation, in 2007 Imelda sponsored the publication of seven books on the Marcos presidency32.

She explains her sponsorship of these books; she feels that it is time for the stories she has to tell to come out. She wants to add her own chapter to the history of the Marcos presidency as it currently exists33.
Chapter 6

Tim Raines

Sanford, Florida

Time Raines' Home Town
*Since this book was published, Tim Raines has received notice that he will be inducted into the Baseball Hall of Fame in Cooperstown New York on July 30, 2017.

Sometimes lupus appears with such swift intensity that it presents an immediate and critical danger to life. Such was the case with Tim Raines, major league baseball player, and Montreal Expos Hall of Famer.

It was 1999. Raines had been having an off year; mid-season he went on the disabled list and four days later underwent a kidney biopsy. His doctors were investigating the cause for a sudden fifteen-pound weight gain and blood pressure that was sky high. The biopsy revealed that Raines was suffering from acute kidney inflammation. His nephrologist determined, after further testing, that the inflammation was due to systemic lupus. Raines was hospitalized and given several rounds of high dose medication1.

However, Raines was down, not out. When asked what the side effects of the medical regimen were likely to be, all he had to say was that he expected to get "better"2.

Tim Raines was born on September16. 1959 in Sanford, Florida. In 1979 he married his high school sweetheart, Virginia Hilton. He began his professional baseball career when he was drafted by the Expos in 1977 and he played his first major league game with the Expos in 1979. He was early recognized as an aggressive player and over his career held records or near records for his base-stealing exploits3.

During his career he played for a number of teams--the Yankees, the White Sox, the Oakland Athletics, the Orioles and, most significantly, the Expos. It was with the Expos that he received the most recognition, garnering several MVP awards, as well as entry into the Expos Hall of Fame4

In 1982 Raines went into drug rehab; he acknowledged that he had been playing baseball while under the influence of cocaine and had actually carried drugs on him while on the field. In 1985 he and several other major league players testified (with immunity) in the trial of an accused drug distributor Curtis Strong5.

Today Raines has retired from major league baseball and works as manager for the Newark Bears, a minor league team in New Jersey. He has remarried and lives with his new wife in Arizona6.

Raines is perennially listed as a Cooperstown Hall of Fame candidate, but he has fallen short of election each year. There are some who believe that he will never be granted this honor, not necessarily because they don't see him as a Hall of Fame caliber player, but because the shadow of those years when he abused drugs hangs fatally over his Hall of Fame prospects7.

Some Tim Raines statistics:

*Only one of three players in major league baseball to steal a base in each of four decades.

*Second father and son pair (played with his son on October 4, 2001) to play on same team in major league game.

*Holds Expos/Washington Nationals Franchise record for steals, triples, singles walks and runs.

*Fourth highest number of stolen runs in major league history8.
Chapter 7

Barbara Enright

Marquis on Binion's Horseshoe Casino
Block 16 in Glitter Gulch, at the beginning of the twentieth century, was home to brothels and gambling parlors. Local residents recall that prostitutes in scanty attire sat on the brothel porches and waited for their clients to call1. Glitter Gulch and Block 16were both testaments to the fact that through much of Las Vegas' history, it at least partly owed its existence to the patronage of men. Men who worked the gold and silver mines in the 1800's; men who built Hoover Dam in the 1930's.

By the time Benny Binion built the Horseshoe Casino on a plot that abutted Block 16, seedy gaming parlors and brothels had been bulldozed and converted to parking lots. Not that Benny would have objected to the brothels, or any other income-producing enterprise. Legend has it that he had fled to Vegas from Dallas back in '46 because the Chicago mob and inhospitable local politicians muscled him out of town. There are some who claim that Benny left behind a legacy of multiple murders, bootlegging and organized gambling rackets2.

When Benny Binion set up business in Glitter Gulch, Vegas was a pretty open town, but not open enough for Benny. He didn't like the $50 limits that were traditionally placed on wagers, so he raised the limit to $500, and then he removed the limit altogether (on first bets). Binion's Horseshoe Casino became the first establishment to offer a no limit game. It was also the first casino to put down carpet (instead of sawdust) on its floors3.

If Benny was anything, he was an original. Thus, in 1970 he began another tradition, one which today has grown to be an international attraction that draws participants and spectators from around the globe: The World Series of Poker. While its million dollar pot was originally the main draw, now the prestige earned by winning the title and the tournament's golden bracelets are at least as coveted as the cash prize4.

Today, Benny Binion and the Horseshoe Casino no longer host the World Series of Poker. Benny died in 1989, and a few years later a corporate conglomerate (Harrah's) bought the rights to the tournament. Before Benny departed from the poker scene, however, he pulled off at least one more "first": the Women's Tournament in the World Series of Poker. In 1986, Barbara Enright became the first Women's Tournament champion and the first female to claim the tournament's coveted gold bracelet. Nine years later, she won her second Women's Tournament, and her second gold bracelet5.

Barbara did not rest on her laurels. She continued to enter open (mixed gender) competitions, until, in 1996 she won the World Series Pot Limit Hold 'em Tournament and, to the surprise of almost everyone (except herself), made it to the finals table in the Main Event--the only woman ever to do so6.

In the shadow of block 16, where women were once sold for as little as a dollar and where they whiled their days awaiting the pleasure of men, Barbara Enright played with the best of them--and won. By the time she received her third trophy bracelet in 1996, Barbara had been diagnosed with lupus for twenty years7

Barbara Enright was born on August 19, 1949 in Los Angeles California. In her youth she was licensed as a cosmetologist and worked at a number of jobs: cocktail waitress, bartender, and stylist for Hollywood celebrities. In the 1970's she tried her hand at poker in the Gardenia California card rooms, where she did so well that she eventually quit her other jobs and dedicated herself to poker8.

Barbara asserts that she has never played a "woman's" game. She comes at her opponents with decidedly "unfeminine" aggression. Enright explains her success in the traditionally male-dominated poker room: she simply states that she is aggressive and that if a woman is too "soft" in tournament play she cannot succeed. But if she asserts herself and defies the expectations of the men sitting at the table with her, she can do "very well"9.

On July 6, 2007, Barbara Enright was inducted into the Poker Hall of Fame, the first woman ever to be invited into that select academy. As of 2007, it was estimated that her tournament winnings exceeded $1,200,000 , more than any other woman in tournament history at that time10.

Today she lives in Hollywood California with Max Shapiro, a poker player and a columnist for Card Player Magazine12.

Barbara once said that her dream was for there to be world peace and free medical coverage for every person12Perhaps the content of this dream was influenced by her experience with lupus. While most of her bios refer to the fact that Barbara has lupus, her challenge with the disease has remained private. Some writers refer to the disease as almost a footnote in her life; many writers do not refer to it at all. It seems that the kind of lupus Barbara lives with and the treatment she receives for it, are very different from the lupus that took Inday Ba's life. Since her diagnosis in 1976, Barbara has raised a child, managed a successful career in an exotic profession and earned a reputation for being smart, friendly and focused.

In the storied saloons of Glitter Gulch, where ladies were historically dispatched like grains of sand from the vast and unforgiving Mojave desert, Barbara Enright made her mark. A combination of raw talent, hardscrabble determination and a dollop of luck helped her to secure a place in the history of Las Vegas and the world of professional poker.
Chapter 8

Ray Walston

The Mississippi River and New Orleans

Ray Walston's Birthplace
Ray Walston. Some may not recognize the name, but all will know the face of this accomplished actor. _From My Favorite_ _Martian_ to _Picket Fences_ , Walston displayed a knack for being in productions that had a lasting influence. He enjoyed a career that spanned seven decades and that adjusted nimbly to the dramatic technological and cultural changes that occurred in his lifetime. His first performance was in a minor stage production, his last in an independent film. Between these two seemingly insignificant events was a body of work that would have fulfilled the ambitions of several men.

Born in Mississippi on November 2, 1914, on the eve of World War I, and given the name Herman by his parents Mittie and Harry, Walston was witness to both the Great Depression and WWII1. He made his Broadway debut in 1945 in the play _G.I._ _Hamlet_. Something about the job must have impressed him, because for almost half a century, he never looked back2.

Throughout his life, Walston demonstrated an extraordinary work ethic and talent. His professional longevity was no doubt largely due to these qualities, which allowed him to move effortlessly from one medium to another and, periodically, to reinvent himself when it seemed to some that his career had peaked.

The peaks in Walston's career were many. In 1956, Broadway granted him its highest award when he won a Tony for his performance in the musical _Damn Yankees_ 3. In 1962 he became a television legend with his portrayal of space alien Uncle Martin in the series _My Favorite Martian_ \--a role Walston later would find hard to live down4. Although he worked successfully at many projects in the seventies, it wasn't until 1982 that Walston was able to overcome the "Martian" effect; in _Fast Times at_ _Ridgemont High_ , when he played the exasperated high school teacher, Mr. Hand, and once again captured the hearts of a generation of young Americans5. In what was billed as a supporting role, Walston managed to project a personality, as foil to Sean Penn's surfer dude Spicoli, that essentially stole the movie.

Besides _Fast Times_ , Walston appeared in a number of successful films and television shows--among them (for the big screen), _The Sting_ and _The Silver Streak_ 6. In 1992, at the tender age of seventy eight, Walston reinvented himself yet again. He agreed to play a judge in the television series _Picket_ _Fences_ and was so successful at fleshing out the judge's character that he was nominated for three Emmys--two of which he won. In his 1995 acceptance speech Walston said that he had been waiting sixty years to get up on the stage but that he only had thirty seconds to describe the experience that led him to that achievement7.

The wry intelligence reflected in his 1995 acceptance speech seems to have been a mainstay of his professional private personality. Not only was his characterization of Uncle Martin (from _My_ _Favorite Martian_ ) that of a cantankerous alien, but, in his own life, he once so got under his wife's skin that she suddenly sued him for divorce. According to reports, a stunned Walston worked quickly to reconcile with the woman he had been married to for fifty-one years8.

Ray Walston was eighty-six years old when he died. He is said to have struggled with lupus for six years before he succumbed to the disease. Which proves that there is nothing typical about a lupus patient9.

Lupus is overwhelmingly a woman's disease. It is largely (in the US) an African- American, and even more specifically, a Caribbean-American disease. The disease strikes the young much more frequently than the old. Walston was white, male and long past young. Lupus didn't ask his age, race or gender.

Some reports about Walston's death describe it as resulting from a sudden illness.Apparently, as with many people who have lupus, the fact and nature of his illness were not well known. There is no way to tell what form his illness took, or if his advanced age made him more susceptible to its complications.

One thing is certain, though, Walston was a first-rate American actor who made an indelible mark on American culture. He honed his craft all of his adult life and did not stop working until time, and lupus, overtook him.
Chapter 9 J. Dilla

An Open Hydrant on 7 Mile Road in Detroit
In 2006, comedian Dave Chapelle released a documentary film, B _lock Party_ , which introduced the world to Chapelle's Brooklyn neighborhood. The film contained a dedication to music producer, and performer, J. Dilla1. Many who heard the dedication likely could not connect Dilla's name to a particular piece of music, though Dilla has been acknowledged as one of the most important influences in the development of hip hop (Busta Rhymes)2.

J. Dilla, born James Dewitt Yancey on February 7, 1974, was the second of four children. As a child, James was surrounded by music--his mother was an opera singer, his father a jazz bassist3. When James was still an infant, he demonstrated a gift for music. His mother claimed that he had "perfect pitch" when he was only two months old4. This musical precocity developed into a passion for hip hop and eventually he associated himself with a group known as Slum Village5. He honed his skills in the basement studio of his family home, which was located just a short distance from Detroit's 7 Mile Road.

By the 1990's Jay Dee (the name he started to use professionally) had attracted the notice and respect of top-tier artists7. He worked with Janet Jackson, Pharcyde, De La Soul, Busta Rhymes, among others. However, none of these artists credited Jay Dee's early input on their albums. This lack of attribution would become typical of Jay Dee's collaborative efforts, so that the end-line consumer of music was unaware of his contributions and he remained largely unknown8.

In 2000, Dilla (he had changed his name once again) decided to strike out on his own9. He produced his first solo album, from which the single _Fuck the Police_ (a take on _Fuck Tha Police_ , a 1988 song by Gangsta Rap group _N.W.A_ ) was released. While the song carried a strong, and what some considered to be an offensive message, the record was representative of the political and social milieu in which Dilla lived. He explained that everyone knows that the police are corrupt and racist and that they regularly abuse people merely because of the color of their skin10.

In 2003 Dilla's solo career was gaining steam. As his professional fortunes improved, however, his health deteriorated. 2003 was the year in which Dilla started to show signs that he was ill. Throughout 2004 and 2005, rumors of his declining condition circulated. Word got out that he had been hospitalized. Despite this, his distinct talent was earning him a kind of cult status on the Internet and in the hip hop underground11.

When he went on a European tour in 2005 as a solo artist, he startled audiences by performing from a wheelchair12. Though he was unable to conceal his physical decline, he never admitted to friends and associates the extent of his illness. Thus the music world was stunned, on February 10, 2006, to learn that J Dilla had died, just three days after his thirty-second birthday13.

Reports of Dilla's death indicated that he had succumbed to TTP (Thrombotic Thrombocytopenic Purpura), a rare blood disorder in which clots occur throughout the body. Some of the reports also mention that he had lupus, as though his lupus and TTP were unrelated. This reporting reflected a common misunderstanding about lupus.TTP can occur on its own, and it is also a syndromes that can occur with lupus. An _Oxford Journal of Rheumatology_ article states that there are no precises statistics describing the exact concurrence of lupus and TTP, but that the the two probably occur together more than is commonly thought14. Treatment for both TTP and lupus (occurring together) is similar: cytotoxic drugs (chemo). Additionally, for TTP (and very uncommonly for lupus) plasmapheresis (removing and treating plasma) is undertaken15. The fact that the course of Dilla's disease was so swift–three years–is an indication of how serious TTP is, whether as a complication of lupus or as a disease in its own right.

J. Dilla left behind a musical legacy that has grown larger since his death. He was posthumously given a Plug Award for Artist of the Year and one for Producer of the Year. Besides Dave Chapelle's cinematic tribute, he has received praise from countless music luminaries, including Erykah Badu and Busta Rhymes. However, in addition to his artistic legacy he also left behind a family devastated by loss and burdened by a mountain of debt16.

Dilla's mother revealed that the family home, the one in which Dilla practiced his craft in a basement workshop, had been sacrificed to pay off bills accumulated from Dilla's illness. While his music and personality remain marketable commodities, according to his mother, conflict between Dilla's business manager and family have separated his heirs from these assets. His children live with their separate mothers on social security and his own mother, who battles lupus herself, works as a Health Aide17.

J. Dilla was an accomplished musician with an impressive, though not popularly appreciated, body of work to his credit. Not only did his illness deprive him of the opportunity to realize the full bloom of his talent, but it took from his family the economic and emotional benefits that, without lupus, they certainly would have enjoyed.
Chapter 10 Elaine Paige

The Lyceum Theater in London, Where Elaine Paige Began Her 2006 Tour
Elaine Paige described her first bout with lupus as "devastating". The onset of the disease was classic in the presentation of polyarthritis. Not only did she experience stiffness and pain, but also the swelling of several joints, including her hands, knees, wrists and ankles1. It's never a good time to get sick, but for Paige that moment was particularly inconvenient, because she was in the middle of rehearsals for a West End (London) revival of the musical _Anything_ _Goes_. Although she consulted a number of doctors, Paige received an accurate diagnosis from none of them. Finally, a fellow cast member, who also had lupus, recognized the symptoms and directed her to a renowned rheumatologist2.

Page has always been grateful for that intervention and her experience sensitized her to the difficulty that most people have getting a diagnosis and receiving appropriate medical care.

In her autobiography, _Memoir_ , she describes the sometimes insurmountable hurdle some people encounter in seeing help for their disease. One woman became so distraught over her illness and the lack of diagnosis, Paige reports, that she ended up spending two weeks in a psychiatric hospital3.

The doctor who treated Paige was Dr. Graham Hughes, the director of the Lupus Trust in London. Dr. Hughes addressed Paige's symptoms with aggressive medication. The disease went into remission and Paige was able to go on with the play rehearsals4.

Elaine Paige has a reputation for being a perfectionist--so much so that some have labeled her "difficult". If she is indeed difficult, she is at least as hard on herself as she is on anyone else. Her expectations for herself are very high--she doesn't just want to do well, she wants to do so well that people rise from their seats at the end of a performance and give her a standing ovation. Almost unthinkable to her is the scenario where she doesn't turn up for a performance at all--this, for her is "heartbreaking"5. Paige's dedication to audience and skill has yielded professional dividends throughout her life. Nominated for five Oliviers (the British equivalent of the NY Tony), she won in 1978 for _Evita_. She has released numerous albums, eight of which went gold and four that went platinum6.

Born in Barnet, North London in 1948, she first thought she might be a tennis player, but was dissuaded from this course by her headmistress who suggested her height (under five feet) would be a disadvantage. When she was fourteen she heard the soundtrack for _West Side Story_ and changed her ambition. This time her teachers noted her talent and offered encouragement. Although both of her parents worked in non-musical fields, each had at least dabbled in music at some point in their lives7.

Paige's breakthrough came at the age of thirty when she was offered the role of Eva Peron in the West End production of _Evita._ For this performance she won not only the Olivier, but also a Variety Club Award and the Society of West End Theatre Award8.

After her first bout with lupus in 1989, Elaine Paige continued to perform successfully. In 1995 she faced another life-altering health crisis--breast cancer. She discovered a lump in her breast and wasn't properly diagnosed until nine months later, when surgery was performed. Today she is presumed to be cancer free, though she is aware that once you have had a cancer diagnosis, the possibility of a re-occurrence always lurks in the background.

In 1999 there was a recurrence of lupus, but this time, recognizing what was going on, she received prompt treatment and once again the disease went into remission.

Elaine Paige recognizes her good fortune--she has the kind of fame that for many would be only the stuff of dreams. When Susan Boyle, overnight success on a British talent show, was asked about her future ambition, she said she wanted to be like Elaine Paige. Despite her acclaim, Paige does not rest on her laurels. She has become an enthusiastic support of several charities and sits on the board of the Lupus Trust, which offers treatment for and research into this disease. She also helps to raise funds for cancer research and care.

Elaine Paige may be a perfectionist, and as such she may be "difficult", but it is perhaps these very qualities which allowed her to continue with her career despite illnesses that might have persuaded a less motivated person to give up.And the success she has enjoyed--winning awards, earning the reputation in many quarters for being "first lady of the British Theatre"10 **\--** has not diminished her capacity to sympathize with others who suffer.

Elaine Paige has prevailed over illness and she has learned from it. She seems to be one of those people who measure success not only by what they receive in their lives, but also by what they have given.
Chapter 11 Michael Jackson

Michael Jackson's Gary, Indiana Home
There's very little I can say about Michael Jackson that is not already widely known: he was an international music phenomenon; the course of his life was set at least as much by family and culture as it was by natural talent and choice; his death was premature and tragic. Although I can add nothing to the conversation on these points I can discuss with some clarity the way that lupus has affected his life.

It is likely that Michael Jackson had lupus; Dr. Arnie Klein, described by CNN as Jackson's personal physician, stated in 2009 that he hold told the singer he had lupus. So I will assume that Michael Jackson had lupus and that his conspicuous avoidance of the sun was possibly related to the disease.

From there, everything about Michael Jackson and lupus is conjecture–and some of the conjecture is irresponsible. I have read, for example, that lupus may have been the reason for his death; it is suggested that atherosclerosis, which occurs in lupus patients, may have at least contributed to, if not caused, a fatal heart attack. It is true that there is an established association between lupus and atherosclerosis; the connection is two-fold: 1)Lupus causes inflammation in the blood vessels and the inflammation causes stiffening of the vessel walls and the build-up of deposits; 2) The medications used to treat lupus also can cause atherosclerosis.

However, there is nothing in the record to suggest that this disease process was significantly present in Michael Jackson. Certainly, if Jackson had lupus he could have had atherosclerosis. It is also the case that Jackson's peculiar personality might have been caused by inflammation of the brain. Since lupus affects virtually every organ, brain and vessel diseases could have been present–as could any number of other diseases. However, no evidence has been brought to bear to show the Michael Jackson suffered from significant cardiovascular disease or that his eccentricity was a result of organic brain disease.

It is safe to guess that *Dr. Conrad Murray, the physician who administered what is presumed to be a fatal dose of propofol to the entertainer, would like to believe that lupus or another disease process contributed to Jackson's death.

However, there is a coroner's report. And the report clearly states that Jackson died of acute propofol intoxication. Coroners' reports are not always so unequivocal:Brittany Murphy, for example, died at the age of thirty two. The coroner determined that though medication played a role in her death, pneumonia and anemia played a larger role.

Another celebrity, Anna Nicole Smith, who died prematurely, likewise had a more complicated coroner's report than Michael Jackson's. According to the report, Smith died of an accidental overdose of a number of different medicines, though no medicine was taken in a high enough dose to cause her death. Also contributing to her death were two abscesses on her buttocks1.

If we compare the three coroners' reports:Smith's, Murphy's and Jackson's, it becomes almost a certainty that Jackson died from a drug administered by his doctor and from nothing else. Although the coroner had the opportunity to examine Jackson's coronary arteries and the other vessels surrounding the heart, he does not suggest that the state of Jackson's arteries had anything to do with the performer's death. And the coroner also does not suggest that lupus was a factor in the death. On the contrary, the medical examiner's report clearly and succinctly attributes Jackson's death to the administration of a very dangerous and potentially lethal drug.

While lupus can be a terrible disease and while it wreaks havoc on the lives of many, it is not in the service of lupus patients to create a sensational belief in the danger of the disease. People who have lupus have enough to deal with; they don't need imaginary victims and imaginary symptoms in order to be persuaded that the disease warrants the most careful and expert medical attention.

*Dr. Murray was later convicted of involuntary manslaughter.
Chapter 12 A Few Words About Me

A Picture Taken a Long Time Ago, While I Was at Work
There is nothing remarkable about my life except in the way all lives are remarkable. I was born in a rural hamlet just as spring was breaking, though a check of the _Farmer's_ _Almanac_ indicates there was likely snow on the ground the day I came home. A new baby is usually a joyous occasion, but I think the joy might have been muted in my case because my mother was ill with pneumonia and I was the fifth addition to a family that was already stretched beyond available resources. My older siblings included a brother who was severely disabled because of a brain injury and three other children who ranged in age from sixteen months to five years. Eighteen months after my arrival the youngest, and last, of our brood was born.

My father was probably absent for both homecomings, as he generally was through most of my childhood.

While the circumstances in which I grew up were difficult, they were not as difficult for anyone as they were for my mother, who strove to provide us with small pleasures. She sewed late into the night and made frocks out of scraps of cloth. She saved pennies so we could take a taxi to a nearby city and go to an occasional movie. She threw birthday parties for us--family affairs because no one was allowed to come to our ramshackle house. We were tucked in gently every night and dressed with care each morning. We lacked so much that an ordinary childhood might offer, but we knew love in such abundance that it has stayed with us all of our lives.

Life changed course dramatically one day as my mother bundled us up and moved to New York City. The sleepy, secluded existence was over and we were thrown into the bustle of a dynamic city. The most important opportunity offered to me by that city was the library and its unlimited supply of books. I virtually read my way through adolescence; books taught me that the universe is not defined by physical boundaries but by the imagination.

In the spring of my high school senior year I was extended a lifeline. New York University, and others, offered to pay my way through college. Though another income was sorely needed at home, my family insisted I accept the scholarships. Because of their encouragement I received an excellent education and it is that education which has enabled me to write this book.

Events followed fairly routinely after college, though there were minor detours because of my peculiar personality. I took a job in an office, looked around for something that offered more money and soon ended up with a civil service position. There I met my husband.

We married in the spring and both of my children were born in the spring. It seems so many of the landmark events in my life have occurred in springtime.

When my children were of a certain age I searched for employment that might be satisfying. I became a teacher, by accident, but I loved my accidental profession. I was given the chance to inspire the dispirited–for my students all had been diagnosed with one emotional difficulty or another.

I went back to school, as I had all my life, though this time it was for professional advancement and not personal enrichment. Life was full and a little stressful. Two teenagers at home, a full time job and matriculation for a masters degree. I fell ill.

At first it I suspected I might have strep throat. But the lab culture was negative and the effects of my illness, a few swollen joints, lingered. Then my blood pressure rose, not in a way that I noticed subjectively, but at my annual work-related check-up the reading topped 140/90. Finally, I visited my personal physician to find out what was happening. He took blood, sent the samples out and when the results came back referred me to a rheumatologist. And thus my long odyssey for diagnosis and appropriate treatment began.

It was spring again, when those early symptoms of lupus appeared. Something about the spring I think has governed the rhythm of my life. I like to believe that, anyway, because as hard as things did get over the next seventeen years, spring is after all a time of renewal. And that is how I have always insisted upon looking toward the future.
Part II

The Mechanics of Lupus

### Introduction: It's Personal

Lupus is not about a diagnosis. It is not about a set of symptoms, laboratory tests or medications. It is about hope and determination. It is a twelve-year-old who needs a kidney transplant but still wants to play softball. It is a fifty-year-old man who has a seizure and wants to drive, and it is a twenty-two-year-old woman who tries to have a child, despite her several miscarriages. It is Flannery O'Connor spending her remaining bit of life polishing stories, Barbara Enright breaking world records and Inday Ba signing a contract with the Royal Shakespeare Company though her disease is raging.

Lupus is these and other people, their family and friends. And it is me.

Lupus is personal.
Chapter 13

Lupus Diagnosis

Lupus has been called the disease with a thousand faces1.

When someone asks, "What is lupus?", they probably are not inquiring about the biochemical changes that occur with lupus. More likely, they want to understand the experience of lupus. And the answer to their question is, it's hard to say, because from person to person lupus is different. Some people get achy and tired. Some people get headaches. For some there's a stroke, or kidney failure, or muscle spasms. For some people, it's all of the above. The manifestations of lupus are as varied as the imagination allows. Even doctors who treat the disease are occasionally surprised by the way it behaves.

This variability of disease manifestation is one of the reasons managing lupus is so difficult--the doctor and the patient always have to ask, even in an established lupus patient, is this lupus acting up or is this something else? Determining what exactly is going on is critically important when considering treatment options.

Lupus is a chronic disease that, in most cases, waxes and wanes: there are periods of exacerbation and periods of remission. In very rare instances, an individual does not survive the first acute phase; usually, when this happens, there is a great mystery about what has made the person ill. The disease might have been preexisting but relatively quiescent. In such cases, lupus as the cause of death is sometimes overlooked. A sudden heart attack or stroke, caused by lupus, may simply be recorded as a cardiovascular or cerebral event. Most typically, however, the illness runs the course between active and inactive stages.

Generally, the disease is progressive: it may begin as a flu-like illness with vague arthritic symptoms and then over time evolve to include a number of organ systems.

Although George Costanza, of the Seinfeld situation comedy, was horrified at the prospect of having lupus, the disease is actually no more horrifying or mysterious than any other diseases for which there is no cure or known cause. Lupus is an autoimmune disease and as such has a lot of company.

Lupus can appear anywhere in the body, and once it appears in one place, it has the potential to manifest in another place. It does not metastasize like cancer, but since the disease is progressive, the tendency to have inflammation can grow and be experienced at different sites. In lupus, immune cells somehow get misprogrammed. These cells, which are the body's defense against invading microorganisms, mistake the body's own tissue for foreign matter. Instead of protecting the body, they attack it.

These immune cells on attack literally become enemies within as they produce inflammation. It is inflammation and the direct assault of immune cells on tissue that destroy healthy organs.

The root cause of lupus is a mystery. A number of factors are suspected in its development. Studies have shown a definite genetic association. In looking at the occurrence of lupus in identical (homozygous) twins, for example, researchers have determined that in an overwhelming number of cases, if one twin is stricken with the disease, then the other is affected. However, the concurrence of disease is not one hundred per cent. The _American Journal of Medicine_ published a study (Oct, 1975) which demonstrated that only fifty-seven percent of twins in the study group were both ill with the disease. Of the remaining twins in the study, twenty or thirty percent showed only one twin ill; the other carried traces of lupus antibodies in their blood but otherwise appeared to be healthy. Of the remaining ten or twenty percent of the twins in this study, one twin was ill and the other had absolutely no sign of disease, either serologically or clinically. Thus, in this small percentage, there was zero concurrence of disease2. As this study demonstrates, genetic heritage is strongly influential in the development of lupus but it is not determinative.

There is no cure for lupus, though there are treatments that are usually effective. For more than fifty years the first line of defense in lupus treatment has been corticosteroids (such as prednisone). Sometimes, in severe cases, chemotherapy is tried (for example, Cytoxan--usually, for nephrotic lupus).Although lupus is not cancer, lupus patients tend to get cancer (especially lymphoma) more often than the general population. In addition to corticosteroids and chemo, there are a number of treatment options. I discuss these in greater detail later in the book.

No single diagnostic test for lupus exists--there is no tissue exam, as with cancer--that can definitively identify the disease. While abnormal activity in lupus can sometimes be verified with a biopsy, this in itself is not sufficient to arrive at a diagnosis. The American College of Rheumatology has been trying to come up with a diagnostic tool for years, but has not yet succeeded. The time it takes for a patient to receive a diagnosis of lupus, from the first suspicion of disease, can be weeks or as long as ten years3. In my own experience, there was a four and a half year delay from the moment lupus was first suggested to the day when someone definitively and absolutely declared that I had the disease.

On the Lupus Foundation website I came across a question that poignantly expresses the fear and uncertainty with which an undiagnosed lupus patient lives. It is obvious from the question that the individual feels alone and without resources. Whoever is writing this question is "afraid". Perhaps the doctor will not correctly diagnose the illness, whatever it is. Perhaps, if the patient does have lupus, the doctor will diagnose it too late and the illness will already have done great damage. The individual wants a speedy diagnosis. How, the questioner, wants to know, can the process be made more efficient.

The answer provided by the Lupus Foundation is calm and as reassuring as reality allows: there is no way to speed up the diagnosis. This is an elusive disease and no one test exists that allows the physician, with certainty, to say, "yes, you have it", or "no, you don't". Diagnosis of lupus, the Foundation informs the individual, can take months or years, sometimes as long as ten years4.

In the past eighteen years, to the best of my recollection, I have consulted eight rheumatologists. Early in this eighteen-year journey I met one rheumatologist who said there was nothing wrong with me and that there was no need to see him again. ("Why do you want lupus," he asked. "Don't you know there's a stigma?"). Unfortunately, his advice to me was very persuasive. Not only did I not consult him again, but for three years, no matter how sick I felt, I did not consult anyone. Finally, in 1996 I hit the proverbial wall. Although I still refused to see a rheumatologist, I was motivated to make an appointment with a dermatologist because I had developed a rash on my face;it was through the dermatologist that I stumbled into a lupus diagnosis.

The rash, which was slightly raised and red, had at first seemed innocuous. I applied over-the-counter hydrocortisone cream to it; however, not only did the rash not go away, it got worse. I was working, so I waited till Saturday to see the doctor. Unfortunately, by Friday things took a rather strange turn. Not only was the rash conspicuous, but at about two o'clock in the afternoon I started to cry.This wasn't a boohoo out loud cry, but a soundless weeping. Tears welled up and poured down my cheeks. I was embarrassed, far more embarrassed by the tears than by the rash. Despite this overt sign of distress, I felt removed from the weeping. I did not summon the tears; they operated of their own volition. My co-workers commiserated and the more they commiserated, the more productive my tear glands became. I waited out the clock and tried to keep a low profile until it was time to go home.

I'm not sure what the record is for continuous crying, but I might have broken it that day. From whichever source these tears came, it was inexhaustible. The flow continued on and off through the night. The next morning I awoke to more moisture. I had a dilemma--go to the dermatologist and disgrace myself or forget about the rash and stay home. But all those salty tears had flowed across my already inflamed cheeks. The rash looked dreadful and my choices were reduced to one: get something that would make the rash go away.

I composed myself somewhat as I greeted the receptionist, though this respite from dampness was short lived. As she settled me in a treatment room, the tear duct seeping began again.

The doctor came in not long after--perhaps he was alerted by an alarmed receptionist. A youngish man with dark hair, the dermatologist regarded me soberly as I apologized for my inappropriate behavior. His first words were gentle, but direct: "Do you have lupus?" he asked.

The history poured out from me as fluently as the tears had. I had been sick, I explained,and at one point was suspected of having lupus, but no one would help me. I had given up and wasn't looking for help anymore, but this rash was embarrassing. I had been crying inexplicably since the day before. And then I asked him, this man I had just met, if he could help me.

The rash on my cheeks, he explained, was a classic example of the "wolf rash" that presents in active lupus. And the crying was very likely a manifestation of the disease. He told me he needed to order some tests. He wanted to be thorough and check everything. He wrote instructions to the lab and told me to go directly to have blood drawn.

The lab tests came back with a number of markers for lupus and from there the second part of my lupus odyssey began. I had been through a fallow period, when I lacked legitimacy and was basically labeled by at least one doctor as a neurotic and now I was in the second stage, where I had the endorsement of a doctor, though only a dermatologist, and I did not have the imprimatur of a rheumatologist. Without that imprimatur there was no way I would receive proper treatment.

I have been out of the wasteland (no appropriate treatment) for several years now. Before I discovered my current doctor there were rheumatologists who confirmed that I had lupus, but none of them seemed to get a handle on just how lupus was affecting my body. So effective treatment was not forthcoming. Not till I met my current doctor did I find the relief that I enjoy today.

The road to my doctor was paved with mishaps. One doctor confused my medical records with another of his patients who shared my name but was twenty years younger than I was. Another doctor suggested I might have MS. Yet another rheumatologist, concerned about the side effects of prednisone, recommended I go to bed for two weeks the next time I had a flare. Many doctors were compassionate and bright. While these doctors offered me the comfort of knowing I could go to someone, they were not able to significantly affect the quality of my life.

Lupus is considered a collagen disease and thus its treatment is assigned to the specialty of rheumatology. Unfortunately, as my experience illustrates, there is great variability in the skill of different rheumatologists. Medicine may be a science, but its clinical application is an art. With lupus this is even more true than with many other illnesses because there exists for lupus no definitive set of diagnostic criteria. The American College of Rheumatology has compiled eleven symptoms and signs, a kind of clinical guidebook for the assessment of disease status. This ACR list is regarded by many rheumatologists as a diagnostic bible, though the ACR cautions practitioners that the criteria are not to be used this way. The list is compiled to assess patient suitability for inclusion in research studies. The ACR advises that dependence on the eleven criteria by themselves would inevitably exclude from treatment patients who might have lupus. But, since doctors have little else to go on, they defer to the list.

When someone first explores the possibility that they might have lupus, one of the first things they come across is the ACR criteria. These criteria have changed several times over the years and will certainly change again. (For example, Reynaud's used to be on the list but is not included anymore). If a patient presents with four of the eleven criteria, then a lupus diagnosis is strongly suggested. Some of the criteria are considered to be more diagnostically compelling than others; Anti-Smith (which would be classified as an immunologic disorder) for example, does not appear in any disease but lupus. Also persuasive, but not determinative in a diagnosis, is the classic butterfly (malar) rash.

I have provided below the ACR list as it currently exists5.

*Malar rash, which can be raised or flat. This rash spreads over "the malar eminences"--cheekbones--and does not appear in the area that extends from side of the nose to the corners of the mouth (nasolabial folds).

*Discoid rash. This is different from the malar rash--it appears anywhere, but mostly on the head neck and scalp and where the skin is exposed to the sun. Discoid lupus lesions are oval shaped (discs), red and raised. Often, scarring occurs.

*Photosensitivity--skin rash that results from exposure to the sun, physician is supposed to observe this or learn it from the patient's history.

*Oral or nose ulcers. These are reported to be often painless

*Arthritis. This is supposed to occur in more than one joint, be demonstrably tender, red and/or swollen. This arthritis, unlike rheumatoid arthritis, does not cause joint destruction

*Serositis--Inflammation of the lining of the lungs (pleuritis), heart (pericarditis) or gut (peritonitis/ascites)

*Proteinuria--excess protein in the urine

*Neurological disorder

*Certain blood disorders: hemolytic anemia, leukopenia, lymphopenia, thrombocytopenia

*Antinuclear Antibodies

*Immunologic disorders--these are abnormal laboratory findings in different aspects of the body's immune system: Anti-Smith, Double Stranded DNA, Single Stranded DNA, False Positive Syphilis Test, antiphospholipid antibodies
Chapter 14

Lupus Physiology

Apoptic Cell, Indicated by the Arrow in a Mouse Liver
I am not a scientist. I took my last college biology course some forty years ago, so I will not pretend that I have a thorough understanding (as would a doctor) of the physical changes that occur in someone that has lupus. However, I grasp the basic principles of this process and will attempt to sum them up.

So far in this book I have discussed the fact that lupus is an autoimmune disease and I have explained that in autoimmune diseases the body is in essence at war with itself. Instead of attacking foreign organisms, the immune system becomes, in a sense, a traitor and goes on an internal assault, destroying healthy tissue and organs. I've explained that it is the inflammation caused by a hyperactive immune system which causes damage in lupus patients. A question I haven't addressed is, how exactly does the action of the immune system cause inflammation?

Apparently there are two kinds of cell death that occur within the body, apoptotic and traumatic. Apoptosis occurs routinely, when a cell dies a natural, programmed death. Traumatic death is, as the name implies, from injury. As apoptosis takes place, the body sloughs off the debris from the deceased cells, kind of the way a garbage truck carries away trash. In lupus, the mechanism for removing the trash, the bits of dead cells, works imperfectly. As these bits of cells deposit in blood vessels and other tissues, they cause inflammation. Failure to clear apoptic cells leads to more apoptosis and necrosis (pathological cell death)1.

Why the process of normal cell clearance becomes aberrant is the mystery in lupus. It is certain that there is a trigger to set off this aberration and that the trigger is at least partly environmental--whether the offending environmental factor is viral or chemical or something else, no one knows. When the lupus trigger is discovered, and it is certain to be one day, the cure for lupus will likely not be far behind.

According to Dr. H. Michael Belmont, a prominent lupus specialist affiliated with the Hospital for Joint Diseases, disease manifestation in lupus occurs not only from the deposits of cell bits, but also from the direct action of antibodies. He cites the example of psychiatric lupus-- "neuropsychiatric SLE"--the activation of which he attributes to the assault of "anti-neuronal antibodies".As Dr. Belmont explains, the tension between organ-destroying lupus flares and the administration of toxic treatment options is the challenge confronting lupus patients and doctors. And, as he states, patients with lupus are best served when in the hands of physicians, experienced with lupus, practicing in first-rate medical centers that treat large numbers of lupus patients 2.
Chapter 15

The History of Lupus

Pachacamac Ruins From Pre-Columbian Peru
The child was approximately fourteen when she died. Her hair was short and patchy. Examination of the lungs suggested the presence of pneumonia or edema. Tissue analysis revealed fibrous thickening of the coronary arteries and of vessels in the skin, pericardium, esophagus, liver and kidneys. Carbon dating of her mummified remains determined that she was deceased in 890 AD. An almost perfectly preserved representative of the Huari culture, she became the earliest suspected case of systemic lupus erythematosus ever discovered1.

Despite the antiquity of the Huari mummy, and the evident long history of lupus, the disease was not identified as a systemic disorder until about 1900. Before then, lupus was thought to involve only the skin. As such, it was often confused with other diseases that affect the skin, including cancer, syphilis, tuberculosis and leprosy.

One writer from the late middle ages stated: "Leprosy is more clearly recognized in the nose, where it shows well-defined symptoms. Sometimes it is also called wolf because it can contaminate all of a man's limbs as does cancerous lupus"2. Even as late as 1911, conflation of leprosy and lupus occurred. An entry in the Encyclopedia Britannica from that year states: "The essential character of leprosy is a great multiplication of cells, resembling the 'granulation cells' of lupus and syphilis, in the tissues affected."3.

Today there is a cure for syphilis and leprosy, but lupus has not enjoyed similar progress. This disease, though better understood today than ever before, remains a conundrum for researchers.

Lupus, like syphilis and leprosy, carried with it significant social stigma. Not only was lupus confused with these other diseases, but superstition surrounded lupus because of its name, which means wolf in Latin (wolves, in pre-modern times, had a mystic, sinister quality attached to them).

While evidence of lupus dates back to the ninth century, leprosy has a much older pedigree and its victims have suffered even greater isolation. The first descriptions of leprosy are found in the _Atharava Veda_ , an Indian text from about 2500 B.C.4. India is thought to be the country of origin for leprosy. From India it spread throughout Asia, the Middle East and the world. The disease was greatly feared, and proscriptions were written into law to protect the public from contagion. The _Law of Manu_ (another Indian text from about 1500 BC) prohibited contact with lepers and marriage into a leper's family. And while suicide is forbidden to most Hindus, lepers were encouraged to commit ritual suicide. Additionally, archaeological evidence indicates that upon death, lepers were forbidden the cleansing ceremony of cremation. Their remains can be found in Indian burial grounds along with those of others who were considered unworthy of cremation5.

In 1873, Gerhard Hanson identified the organism, bacterium Mycobacterium leprae, which causes leprosy6. Though the agent of transmission had been discovered, the mode of transmission had not, and no cure was offered. Not until 1983, with the introduction of multi drug therapy, was an effective cure for leprosy widely available7.Today leprosy (known as Hanson's disease) is not merely treatable; it is curable, though difficulties persist for those who contract it. There is no vaccine, it cannot be detected in those who are asymptomatic (as many who contract leprosy are), and the mode of transmission is still not clearly understood. In certain parts of the world leprosy remains a devastating diagnosis to the patient and the patient's family8.

Martin Pinzon was captain of the Pinta, one of the three ships that sailed with Columbus on his first voyage to the Americas. Conflicting stories about this troubled sailing cloud the record, but the goal of the voyagers is not in doubt. They wanted riches for themselves and the Spanish crown. The "conquistadors" brought back gifts, including several of the indigenous people they encountered. They also brought back, many believe, another gift--though this surely was not their intention--syphilis. The malady was said to have afflicted and ultimately killed Martin Pinzon himself9.

However, neither the cause of Pinzon's death nor the origin of syphilis has ever been proven. There is some indication that syphilis in Europe predated the Spanish conquests. There are descriptions of syphilis-type disease by Hippocrates and suggestions that an Augustinian friar in the 13th century suffered from it10. It has been proposed that in some instances illness diagnosed as leprosy was actually syphilis11. In any event, the type of syphilis introduced into Europe after the Spanish conquests was vicious (possibly because the Europeans had no immunity)12. Its victims, upon infection, often suffered pustules from their head to their knees and in many instances were dead within a few months13. Its reach was indiscriminate, affecting those of elevated and low circumstances alike. Among the more famous victims who succumbed to syphilis were: Lord Darnley, the second husband of Mary Queen of Scots; Heinrich Heine; and Franz Schubert14.

In nineteenth century France, infection with syphilis was an imprisonable offense. When the Societe Francais de Prophylaxie Sanitaire et Morale met, it discussed the problem of syphilis contagion. At the meeting, it was unanimously decided that imprisonment was the best option and that the length of incarceration should be as long as life (!!) or as little as several days15.

Treatments for syphilis included mercury and arsenic. In 1908 an antibiotic, Salvasan, was discovered16. Salvasan was not effective in late stage syphilis, but high fevers were. So a patient suffering from late stage syphilis was deliberately infected with malaria, in the hope that the malarial fever would cure the syphilis. Finally, an effective cure for syphilis was introduced after WWII with the discovery and production of penicillin17.

Lupus has a history even more muddled than that of syphilis. While syphilis had Ehrlich and Fleming (whose combined efforts resulted in the discovery of penicillin), for lupus there was no breakthrough moment, no flash of inspiration that revealed the disease's mysteries and yielded a cure. Rather, after a long dark age when virtually nothing was clear, gradual advances were made in the nineteenth and twentieth centuries.

There is no agreement among historians about where the term lupus came from. Some believe that Rogerius Frugardi, a thirteenth century physician, was the first to use the word. He referenced a lupoid rash, which, as he described it, had a butterfly configuration (raised and reddened skin that flared across the cheeks and nose in a butterfly pattern). Rogerius is said to have compared the rash to the bites and scratches inflicted by a wolf attack18.

Other historians place the first reference to lupus much earlier, asserting that Hippocrates described the disease when he noted a cutaneous ulceration19. Another school of thought attributes the term to Giovani Manardi (1462-1536), who used the word lupus to describe ulcers on the legs which, like the bite of a ravening wolf, ravaged the surrounding area20. It is also suggested that the word is derived from the wolf-like appearance of the face when the butterfly rash of lupus is present21.

Finally, in a completely divergent opinion, some suggest that the word lupus has nothing to do with any of the above, but originated from the French word loup, for the mask used by ladies to cover the face22.

Pierre Louis Alphee Cazenave, in 1851, was the first person to definitively describe lupus--though, again, he dealt only with the dermatologic manifestation. He wrote:

"In some circumstances (lupus) manifests itself at first as a violet rubefaction on this or that part of the face, and over many months the colour rises little by little; this surface becomes animated; a small ulcer forms and on tip of it, a scab, which then thickens and covers the ulcer, which becomes progressively deeper. Lastly, the skin may get thinner in imperceptible stages and adopt the appearance of a scar, without there being tubercles or ulcers, and without displaying worse injures than a livid color and from time to time a barely perceptible peeling23.

Although Cazenave described discoid lupus, the term did not come into use until Moritz Kaposi diagnosed the disease (DLE) in twenty-two patients. He noted that female patients not only suffered the ailment in higher numbers but the manifestation of the disease in females was more severe than it was in males. Further, Kaposi used the term disseminated lupus erythematosus to describe disease in which lesions were dispersed24.

Kaposi (after whom Kaposi's sarcoma is named) noted a coincidental occurrence of systemic symptoms with skin lesions (in 1872), although he did not see the visceral manifestations in his patients as an expression of lupus. He remarked that some of his patients who had been diagnosed with discoid (skin) lupus also had fever, weight loss, anemia, lymphoadenopathy and arthritis25.

While Cazenave and Kaposi lent definition and terminology to lupus of the skin, William Osler provided an understanding of systemic lupus. Between 1894 and 1903, he noted twenty-nine patients in whom there were "erythema (skin lesions) and visceral injuries". Osler was the first to observe renal and nervous system complications in lupus patients26.

Later physicians (Kraus, Bohac, Limand and Sacks) found endocarditis, pneumonia, and photosensitivity27. Until 1937, it was believed that without the skin lesions, a lupus diagnosis could not be considered. However, after 1937, it was determined that visceral injury (systemic disease) could occur in the absence of skin lesions28.

Slowly the definition of lupus was evolving: an inflammatory disease which expresses sometimes only as a dermatologic disorder and sometimes as a systemic disorder, with or without lesions.

After 1900 steady progress was made toward reaching a clear definition of the disease. However, advances in treatment and diagnosis were slow in coming.

In 1948, Malcolm Hargraves discovered a cell he called the LE cell, so named because he found it in the bone marrow of lupus patients (LE for lupus erythematosus)29. In 1949, Philip Hench discovered the anti-inflammatory properties of cortisone. For the first time, a widely accessible, effective treatment for lupus was available (effective at controlling symptoms in some, but not all). Hench and his colleagues at the Mayo Clinic received the Nobel Prize in 1950 because of this work.30 Today, prednisone, a derivative of cortisone, remains a cornerstone of lupus treatment.

After Hargraves' discovery, there existed a good tool for treating lupus, but significant obstacles remained in identifying the disease. Finally, in 1958, George Friou developed a means of identifying antibodies in the blood by using indirect immunofluorescence. The "anti-nuclear" antibodies he identified are at the core of the disease process in lupus.

Lupus is essentially a disease of inflammation--the immune system somehow goes haywire and cells start attacking the body. There is, in essence, an internal war: the body fails to recognize its own cells, attacks those cells as it would attack an invading microorganism, and essential wages war against itself. Thus, the use of the term anti-nuclear antibodies. When Fiou visualized these antibodies, he was able to identify people in whom the autoimmune process was active31.

It is understood that many people who have ANA's (antinuclear antibodies) do not necessarily have lupus. However, it is not at all likely that someone who has lupus does not have ANA's. Thus, indirect immunofluorescence became the threshold test for lupus (although, over a course of many years, someone with lupus, especially someone in remission, might test negative for the antibodies, eventually, with exacerbation of disease, the antibodies will re-appear.)

Since 1958 there have been refinements in the definition, diagnosis and treatment of lupus. Despite this, the disease remains chronic, disabling and fatal for many (especially children)32. With all of the tools at their disposal, rheumatologists still lack a foolproof methodology for diagnosing lupus. Some diagnostic criteria are considered compelling. Anti-Smith antibodies, for example, appear in no disease other than lupus (they are named after a lupus patient who had them circulating in her blood). However, only a small percentage of lupus patients test positive for Anti-Smith antibodies.

There are treatments. The treatments do not work for everyone and are themselves sometimes so toxic (recall Flannery O'Connor's terminal battle, when she was infused alternately with high-dose prednisone and antibiotics) that the patient succumbs to the side effects of a drug rather than to the ravages of disease itself.

Today, the search for understanding continues, and a cure remains elusive. Though survival rates for lupus patients have greatly improved, for certain subsets of patients (those with kidney involvement, for example), the ten-year survival rate has been estimated as high as 79% and as low as41%33.
Chapter 16

Lupus Overlap Syndrome

Direct Immunofluorescence of a Lupus Lesion
Some nine or ten years ago I noticed a scaly area about 3/4 of an inch below my left ear. This was more like an irritation than anything else. I didn't pay this curiosity any mind, because it looked insignificant. Months passed. From time to time the section of skin would become inflamed, but it was so inconspicuous, under the shadow of my jawbone, that I continued to ignore it.

Two years went by and the area of interest grew larger. Now it was red and irregularly shaped. Not painful, but not exactly without sensation either. It was a definite presence on my neck.

I knew that by ignoring that lesion (for it certainly had become a lesion) I was being irresponsible. So one day during a routine visit to my rheumatologist, I lifted my chin, and revealed my secret. The doctor leaned forward in her seat, and, with a look of consternation exclaimed, "What is that?"

She referred me to a dermatologist, who assumed a thoughtful pose and performed a biopsy. During the follow-up telephone call he was elliptical in his description of the condition, declining to provide information except to describe my irritation as a "process". Reluctantly, after I pressured him to be more forthcoming, he gave it a name: pemphigus.

Pemphigus? That was a new one and he essentially refused to provide more information about the condition. So of course I turned on my computer and looked up pemphigus on the Internet.

No wonder the dermatologist didn't want to give me that diagnosis over the telephone.Pemphigus is a truly horrible disease. The skin blisters. The blisters spread into each other and open when disturbed. The blisters occur when the body decides that one layer of skin is foreign and must be destroyed. Thus, it is, like lupus, an autoimmune disease, the body turning against itself. Pemphigus strikes at any age, but more likely affects people between the ages of forty and sixty.

Well, I decided, I simply refuse to have pemphigus. The dermatologist was wrong. For heavens sake, it was just a spot on my neck, a small benign area with which I had coexisted for years. I was going to prove the dermatologist wrong, so I called the Pemphigus Foundation for a referral to a doctor who specialized in this rare disease. I was given a name: Dr. Joyce Rico, who was a member of the dermatology department at NYU Medical Center in Manhattan.

Dr. Rico was skeptical of the pemphigus diagnoses; she saw what I saw--a small, scaly, unimportant lesion that was not intruding at all into my life.

The biopsy results came back--pemphigus. Laboratory examination revealed that auto antibodies, that is cells generated by my own immune system, were attacking a substance in my body called Desmoglein 3. Desmoglein 3 is the glue that holds epidermal cells together. When the glue breaks down the cells separate and blistering occurs. The results of the biopsy indicated that I had a particular kind of pemphigus (there are several types of pemphigus): pemphigus vulgaris1.

I was devastated by the news and expected my world to fall apart. The graphic pictures of pemphigus vulgaris patients that I had seen on the Internet showed that the disease with which I had been diagnosed was painful and disfiguring. Doctor Rico was very sanguine about my prognosis.

Most of those people in the waiting room have the same diagnosis," she said reassuringly. Well, they look normal, I thought, not sick at all.

Dr. Rico recommended a powerful topical steroid, one more potent than what my first dermatologist had prescribed. The pemphigus lesion on my neck cleared up and I have never had a sign of this disease again. Treating pemphigus aggressively, at its first appearance, is apparently protocol. The longer lesions remain, the more likely is the possibility that the disease will take hold.

As I have reviewed material about lupus and pemphigus I have learned that it is not surprising I have two autoimmune diseases. An estimated thirty percent of lupus patients have at least one other autoimmune disease. This concurrence of autoimmune syndromes is called overlap syndrome. Some of the common overlap diseases are scleroderma, myositis, Sjrogren's, Reynaud's and mixed connective tissue.

The rheumatologist who was treating me at the time of my pemphigus diagnosis said that her lupus patients did not get pemphigus. However, I have since learned that there is something called pemphigus erythematosus, also known as Senear-Usher syndrome, which is a combination of pemphigus foliaceus (a type of pemphigus) and lupus2.

Researchers disagree as to whether these two diseases can technically be termed "overlap" conditions or whether they occur simultaneously at all. Pemphigus is an extraordinarily rare condition. Some doctors never see a case. I hesitate to disagree with researchers, but I do have pemphigus and I do have lupus.

Some overlap diseases:

Scleroderma:There are an estimated 300,000 people in the United States who have scleroderma, and one third of whom have the systemic form of scleroderma3. Scleroderma is characterized by fibrosis (or hardening). As with lupus, diagnosis is difficult, because its symptoms are often confused with those of other autoimmune diseases4.

Two kinds of scleroderma are localized (affects only the skin, related tissue and muscles beneath the skin )5, and systemic (affects the skin and internal organs)6. Unlike lupus, scleroderma cannot be treated successfully with steroids or other immunosuppressive drugs. According to a study published in the _New England Journal_ _of Medicine_ in 2006, there is "limited evidence" that the use of corticosteroids and immunosuppressive alters the course of disease for patients with Scleroderma. However, doctors "frequently" prescribe the drugs to patients who have "all forms of Ssc" (Scleroderma)7. Symptoms of Scleroderma are addressed by treating the affected organs individually8

Rheumatoid arthritis, Sjogren's Syndrome, Reynaud's and, more rarely, systemic lupus can overlap with scleroderma. According to the Lupus Foundation, the primary treatment for scleroderma is different from that for lupus: the Foundation suggests that the patient with both scleroderma and lupus be treated for individual symptoms as they present9 .
Chapter 17

Treatment

Image of Methotrexate-induced Lung Disease
The range of lupus treatments is as broad as is the range of lupus manifestations. Treatment options have expanded in the last fifty years so that most patients achieve disease management. However, no treatment offers a cure, all treatments fail some patients, and the treatments themselves can have serious and even fatal side effects. The first time I consulted a rheumatologist he began me on a course of Trilisate, which is essentially high-dose aspirin. The nature of my illness was poorly understood at the time and for years was under treated. While the dictum in medicine is "do no harm" and the rheumatologists I consulted early on probably felt they were following that dictum by withholding more heavy-duty medication from me, what they actually did was bequeath to me years of avoidable pain and illness. I would not want to relive that time.

I hate being medicated. I have earned the displeasure of more than one doctor because of my resistance to medication. However, I would not want to live if I could not fall back on prednisone, or a substitute if prednisone fails me. It is not merely that diffuse pain, fatigue and migraines plague me. It is also mental anguish that besets me when a flare is in full swing. I am not myself and the person that I become is tormented.

The Trilisate therapy that my first doctor recommended lasted for only a month or two. not only experienced a distressing ringing in my ears while on it but I was also afflicted with multi-day headaches, headaches like none I had never experienced in my life. And my mood would suddenly decline so that I sometimes wanted to cry for no reason at all.

Ibuprofen was the next medication prescribed, in really high, prescription-strength doses. The ibuprofen worked well, not as well as the prednisone eventually would, but it quieted the head and soothed the muscle and joint pain. However, within a year or so I developed an allergy to ibuprofen.

After that, it was heating pads and pain patches for me. Tylenol was somewhat helpful at masking pain, but I developed a rash with that also. There came a time when prednisone was prescribed, in higher doses than I am taking now. That is, until I suffered an acute attack of pancreatitis. After that no doctor would prescribe prednisone (because pancreatitis, which can be fatal, is sometimes caused by prednisone.)

Several years passed. Terrible years. I took Ativan to soothe the mind, calm the gut and suppress muscle spasms. Ativan was a weak and largely ineffective band-aid.

A few years ago I was referred to my current rheumatologist. He tried first putting me on planquenil. Severe headache followed.Plaquenil would not be an appropriate medication for me I was told--headache is one of the possible side effects and once it appears it will always appear with plaquenil.

After the first run with plaquenil, the rheumatologist tried a "teaser" dose of prednisone--five mg. "I'm not worried about pancreatitis," he said. Well, I was, but I felt so miserable I was willing to run the risk just to get relief. Within twenty-four hours of that first five milligram dose my headache disappeared. Peace reigned in my troubled mind. My joints and muscles were more pain free.

It has taken several years for me to tweak the dose of prednisone to the lowest effective level. I no longer take five milligrams, ever, though five would probably yield greater relief, faster. I start now with 3 3/4, because my doctor told me that with a dosage under four milligrams I could avoid most side effects Sometimes the 3 3/4 is not enough and I have to increase the dose temporarily, but I always come down as quickly as possible and wean off as soon as my body can tolerate it.

I realize that I may not be able to get relief from low dose prednisone forever--as they say, lupus is a progressive disease and it may evolve to something that needs more aggressive treatment or something that cannot be treated. But nothing is forever and there are no guarantees in life for anyone. Whatever the future brings, it will bring. I have a great doctor. I trust him and I live my life today grateful that I can write this book, babysit for my granddaughter, enjoy my family. These are gifts I do not take for granted.

Lupus Treatment Options

Though there may not be a cure for lupus, the range of treatment options is quite wide. I start with the easiest and most obvious "treatment": rest and good nutrition. In the early days of my experience with lupus, whenever the disease flared up, my rheumatologist would say, "What have you been doing?" It was a good way of focusing my attention on the role that I play in the state of my health. As the years passed I was able to see a rhythm in the appearance of symptoms. December was (and still is) a bad month. I am very child-like in that I love holidays and work myself into a tizzy getting ready for them. When I was teaching school, the end of each semester was predictably the setting for a flare, because of all the test preparation and end-of-term paperwork. Anyone who is diagnosed with lupus is well advised to know their own body and to know the situations that will be tiring or stressful for them.

Good nutrition is a wise recommendation for anyone, but particularly for someone dealing with chronic illness. In the case of lupus, there are specific dietary considerations. Anyone with kidney involvement (and that's about 40% of lupus patients) has to avoid sodium. Everyone who takes steroids is going to have weight issues--the weight gain occasioned by steroid therapy is often compounded by inactivity. It is very hard to exercise when you are tired and in pain. Also, exercise is not a practical recommendation for someone having an active flare--exhaustion and pain preclude any serious exercise (unlike osteoarthritis or rhreumatoid arthritis, exercise in lupus often exacerbates already inflamed muscles). According to the Lupus Foundation of America, exercise which stresses joints should be avoided if those joints are inflamed. For example, weight lifting and jogging, or aerobic workouts that are high-impact, can do more harm than good if they increase inflammation. Anyone who experiences a lupus flare should also be certain to rest more than is their custom: not only do inflamed muscles need rest to recuperate, but the bone-wearing fatigue of a lupus flare is lessened by rest periods1.

Weight becomes a significant issue for anyone who is sedentary or on steroid therapy. Not only does an inactive person burn fewer calories, but steroids stimulate the appetite and also increase the tendency for fluid to build up in the tissue. Thus, diet becomes of critical importance for anyone who has a lupus flare and is on steroid therapy.

Finally, intake of vitamin D must be monitored to keep levels up, though obviously not into the toxic range. Apparently (according to my physician), low vitamin D levels are associated with increased rheumatologic symptomology. Also, adequate vitamin D is required to combat osteoporosis, which is a side effect of steroid therapy and is abetted by inactivity.

Another benign line of defense in the fight against lupus is to avoid UV radiation. There is a separate section in this book on UV and UV exposure. This seemingly insignificant aspect of the disease can become very intrusive. In the warm months of the year I find that I am out very little while the sun is shining. This eliminates a whole range of activities (and vacation choices). It is not obvious, until you have to avoid sunlight, how much people are drawn to it. Also, UV does not stop with sunlight--it can be found in certain computer screens ( I had one once that was making me ill and found out that it wasn't the screen itself that was the problem, but the back light behind the screen, which was fluorescent) and in ambient lighting. Apparently not all people who have lupus are photosensitive, but it is so prevalent that it is on the ACR(American College of Rheumatology) list of diagnostic criteria.

Something else everyone one can do for themselves is to be up to date on immunizations, because people with lupus have an increased susceptibility to infectious diseases. People who have lupus and are on immunosuppressive drugs have an even greater susceptibility to infectious diseases. According to the Lupus Foundation of America, the disease by itself compromises the body's ability to fight disease. Added to this, immunosuppressive drugs are by design agents that limit a bodies ability to fight infection. Thus, the lupus patient on steroids has a highly increased susceptibility to infectious diseases. Also, partly because of their compromised immune function, once a person lupus becomes ill, they tend to become more seriously ill than someone who is not similarly compromised. The Foundation recommends, because of this dynamic, that everyone with lupus take advantage of immunizations available in the community2

However, the Foundation cautions _that a person with lupus should never be immunized with a live virus. Live viruses, unlike killed virus, have the potential to infect, rather than protect, someone with lupus_ 3.

The first medications most lupus patients are likely to be prescribed are NASIDs, non steroidal anti-inflammatory drugs. These are chosen for patients in whom doctors believe there is no organ damage occurring. Though these drugs are less dangerous than steroids, studies have shown that they are not as benign as once thought and thus should be used judiciously. Not only have they been implicated in ulcers and bleeding, but it is now known that the COX-2 inhibitors (ibuprofen, Celebrex and Naproxen among them) are also implicated in heart disease.

The next medication often prescribed for lupus patients is an antimalarial. Antimalarials have a long pedigree in lupus treatment. Although most people are referring to plaquenil when they speak of antimalarials, the first anti malarial used was quinine, as far back as 18944. Today, many people take plaquenil with little difficulty and with good results, though it is not recommended for severe lupus and there are a few provisos associated with this drug. The greatest risk is to the eyes, so it is recommended that anyone taking plaquenil get an eye exam every six months.

If none of the therapies listed above are effective, more powerful remedies exist. One is a class of drugs are known as cytotoxins (toxic to the cells), or in more common parlance, chemotherapy. I spoke with at least one person whose recalcitrant lupus was thrown into remission (so that they didn't need any more drugs for a long time) by Azathioprine, brand name Imuran.Other drugs in this class include: Methotrexate, CellCept and Cytoxan. While these drugs are used for lupus, they are also used to suppress tissue rejection in organ transplant patients5.

A newer therapy that is used in the rare lupus patient (as in J. Dilla's TTP, for example) is plasmapheresis, where the patient's blood is cycled through a machine and then returned to the body. The old plasma is discarded and the rest of the cells, along with replacement fluid, is returned. This is an extreme and very high risk procedure, though it can be effective in derailing lupus which has severe organ involvement. Like the other measures listed, it is not a cure but a reprieve.

All of the modalities described above are included in established protocols for lupus treatment. However, they are only as good as the doctor who employs them. There are recommendations and procedures in rheumatology and one certainly does not want to deal with a doctor who eschews science in favor of instinct. But instinct matters. Every case of lupus is unique. The ideal doctor uses the tools that medicine offers and tailors them to the patient at hand.I do not want my doctor to use unproven techniques, but I do want my doctor to have the courage to make a decision that does not rigidly adhere to a model that he learned about in a medical text.

Custom treatment has been a factor in my recent run of relatively good health. I really don't know how healthy I am. All I know is I can enjoy my life now and I look forward to many peaceful and comparatively pain-free tomorrows.
Chapter 18

Prednisone

The Image of a Fatty Liver, the Possible Consequence of Long-term Prednisone Use
I don't know if all people who are actively dependent on medicine for their well being have a love-hate relationship with it--I know Inday Ba (see _Profiles_ ) did, and so did Flannery O'Connor (see _Profiles_ ). And I know for sure that I do.

Doctors have a term for patients who do not obey: non-compliant. I think I would be in danger of earning this label from my rheumatologist if he were more paternalistic and inflexible. As it is, we talk over my choices. I often disagree with his recommendations, but eventually we compromise, even sometimes shake on a "deal". This rheumatologist has changed my life, partly because he has let me employ the tools that medicine offers in a way that I find personally palatable.

My medicine of choice, my poison, my ally, my temptation, is prednisone.

I have never been addicted to anything in my life, except maybe food, and love. But this is different--well, maybe it is a little like love because it pains me when it is gone. When I think of my need for prednisone and the hold that it has on my body once I take it, I think, "this is what a heroin addict in need of a fix must feel like." The lure of the substance is unremitting, its sweet succor irresistible.

Of course, from what I've read, a heroin addict wants a "fix" all the time, and never completely loses that longing, but I only crave my poison when I am not feeling well. And happily, that is not all the time. Between flares, the lightly tinted bottle sits in my kitchen cabinet, charmless. But when the lupus gets started, when the aching, disquiet, twitching, headaches, insomnia, gut volatility, etc. set in, then the bottle beckons.

How long can I wait?

My husband is always the first to crack.

"I think it's time," he'll say, without finishing the sentence. He doesn't have to finish it, because by the time he ventures the suggestion, the house has been pregnant with my illness for days, maybe weeks. Though I do my best to hide the symptoms of a flare, the signs are inescapable: midnight wandering, decreased consumption of food, Gatorade, heating pads, restlessness, irritability.

Sometimes I know right away that waiting is a game of chicken I cannot win. My husband and I hate, and fear, those midnight runs to the emergency room.

My resistance to taking prednisone in the last couple of years has diminished, as I have come to know the rhythm of my illness and the wages of prednisone better. I have been able to calibrate my intake so that I consume less, but do it earlier, before the flare has taken root and stubbornly resists intervention.

Of course, I am lucky, lucky in that so far a small amount of prednisone does the trick, that a doctor exists who is comfortable with a laissez faire doctor/patient relationship.He is available any time I need him. He would like to see me take more medicine, medicine that would make me feel well all the time. But this is the course I have chosen. I have read the research and believe I understand the course of my illness.

So, prednisone. What is it? What does it do that brings such relief? What are the consequences of prednisone therapy that persuade me to resist its charms?

Prednisone is a drug synthesized by Arthur Nobile in 1955 from a precursor drug, cortisone (also called hydrocortisone)1. Cortisone was developed in 1950 by Edwin Calvin Kendall and Phillip S. Hench. The drug was formulated to mimic a hormone that occurs naturally in the body, cortisol. Cortisol is designed to bond with cells throughout the body. It is secreted by the adrenal gland (the adrenal cortex) and is regulated by an interaction between the hypothalamus and the pituitary gland. Thus, the presence of cortisol in the blood is the consequence of a delicate interaction between several glands, and the influence of cortisol is diffuse in the body2.

Known as the stress hormone, cortisol is a part of the flight and fright response. While there is always a certain amount of cortisol circulating in the bloodstream, the hypothalamus and pituitary gland act together in an emergency to stimulate the adrenal cortex to release more in order to marshal the body's defenses. The effects of increased cortisol are manifold--some activities are suppressed (like the immune response) and some are increased (like heart rate and blood pressure)3. Obviously, it would not be a good thing for the body constantly to be in a state of high alert (stress), with a consequent disruption of systemic homeostasis.

Prednisone is so effective because it, like cortisol, suppresses the immune response. Thus inflammation, which is the destructive agent in lupus and which is a by-product of an immune reaction, is suppressed.

In addition to reducing inflammation, prednisone also suppresses the release of histamines4. The histamine and inflammation reducing properties of prednisone are essential for the treatment of lupus. Unfortunately the downside of prednisone use can be quite significant.

Besides the potentially life-threatening side effects that result from the use of synthetic corticosteroids (such as prednisone), there exists the possibility that prolonged therapy will result in adrenal insufficiency. In prednisone-induced adrenal insufficiency the hypothalamus forgets to signal the pituitary gland to release ACTH (adrenocorticotropic hormone). Without ACTH, the adrenal cortex does not produce cortisol, which is essential to maintain life. When adrenal insufficiency is present, steroid supplementation becomes necessary and in some cases must be continued for life5.

Although steroid therapy has been used for decades, research continues on cortisol and its synthetic replacements. For example, while it is understood that prednisone use can lead to organ damage, researchers are still establishing the dose level at which that damage occurs. In one study published in the _Journal of Rheumatology_ , it is stated that "irreversible" damage to the organs is not any more likely with a regimen of low-dose prednisone than without it6. My rheumatologist has told me, as mentioned earlier, that if I can keep the daily dose under four milligrams I can avoid most of the side effects, except for osteoporosis. The study ( above) is less definitive than that, but seems to confirm the general principle.

Other studies on serum cortisol levels address the issue of weight. A 2004 article in the _Journal of Clinical Endocrinology and_ _Metabolism_ , for instance, discusses the relationship between natural production of cortisol and weight gain. This article does not examine the effect of synthetic cortisone on weight gain (or weight distribution), but merely questions if there is a relationship between the amount of cortisol naturally secreted and the comparative weight of groups of people: men and women, the obese and the lean, the young and the old, etc. The conclusion of the article's authors is that increased levels of secreted cortisol do not correlate to increased weight. However, there was suggestive evidence that the distribution of weight was influenced by increased serum (bloodstream) cortisol. (Increased secretion does not necessarily result in increased serum levels because different groups clear the cortisol from their blood at different rates). Thus, the article explains, men, who on the average have higher levels, tend to gain weight in their abdomens, as do older people, who also on average have higher levels of serum cortisol. The authors conclude that the differences in fat distribution (favoring the abdomen) observed between aging men and women may be influenced by a stimulation of the hypothalamic/ pituitary/adrenal axis. This stimulation and increased abdominal fat is also associated with a "disturbance" in lipid and glucose metabolism7.

My personal experience with regard to weight gain has been that if I am on prednisone for a week or more (even under four milligrams), I put on weight and that weight is evident especially in the abdomen. My rheumatologist has assured me that weight gain will not occur at such low doses, but I respectfully disagree with him--and the _Journal_ article cited above seems to support my subjective observation.

Another factoid that I have picked up about cortisol: because the body produces a certain amount of cortisol on its own, complete replacement of the hormone does not occur unless daily dose approaches somewhere near seven milligrams. Apparently the body will usually continue to produce cortisol on its own until the amount of synthetic hormone introduced exceeds the typical production. However, since there is such variability in cortisol production among individuals, it is probably difficult to determine at what dose complete replacement occurs. The variability is illustrated in an article on _ehow.com,_ which discusses normal levels of cortisol in an adult. These levels vary not only by individual but also by time of day.Ordinarily, in the a.m. an adult will have between 5 and 31 micrograms of cortisol per deciliter. In the p.m., the levels usually vary between 3 and 13. These values are valid only for adults. Lower levels are detected in other age groups8.

While tapering, or weaning, off high doses of prednisone it is recommended always to do so gradually (to prevent adrenal insufficiency). Even at small doses, I have noticed that weaning is necessary. I usually dose down at the rate of half a milligram a day.

As discussed, steroid therapy carries a number of well-established risks. Some of the well-known side effects of steroid therapy (which can occur but do not necessarily occur) are listed below:9

cataracts

diabetes

pancreatitis

acne

bone loss (osteoporosis)

bone necrosis

hair loss

hair growth

adrenal insufficiency

high blood pressure

weight gain, especially trunk

psychosis

insomnia

blood clots

immune deficiency

glaucoma

ulcer

long-term migraines

mouth sores

joint pain

facial swelling

gastric bleeding

atherosclerosis

Some other uses of prednisone:

Prednisone is truly a wonder drug. Doctors and patients no longer think of it that way because it has been around for a while (widely available since the 60's) and is thus mostly taken for granted. Besides being the cornerstone of treatment for many lupus patients, it is used to treat any kind of inflammation, a variety of autoimmune diseases, and certain cancers, including acute lymphoblastic leukemia, Non-Hodgkins lymphoma, and multiple myeloma. Although it has been reported to cause long-term migraine, it is also used to treat migraine.
Chapter 19

Lupus Correlations and Associations

Not Always the Usual Suspects

Pallet of silica
I have touched briefly in previous chapters on the apparent genetic link to lupus; I have also discussed, in general terms, the likely association between some environmental factors and lupus. It is obvious that if the cause, or multiple causes, for lupus can be discovered then prevention of this, and possibly other autoimmune diseases, may be found. In this chapter I will consider the several genetic and environmental links to lupus that have been strongly indicated by responsible research. Some of the genetic associations are rather well known--among them the link between twins. One link that has received relatively little press is the one that exists between autism and autoimmune disease.

I became interested in the autism/ autoimmunity connection after I was diagnosed with Asperger's Syndrome. I am a little long in the tooth to benefit much from this information; nonetheless, the news was liberating. I understand more now about my core personality than I ever did and this understanding has allowed me to be more accepting of certain of my eccentricities. Having an inquiring nature, I began to ask questions about Asperger's--causes, manifestations, coping strategies, etc. However, it was a comment from my rheumatologist that led me to explore a possible link between Asperger's (or any condition on the autism spectrum) and autoimmune disorders. The rheumatologist asked, when learning of my Asperger's diagnosis, how likely it was that I would have both lupus and Asperger's.

That question got me wondering. So I tried to find out just how likely it was that I would have both conditions. I began my search (thank you, Google) and discovered that much work had been done in this area and that the results led most researchers to the conclusion that there is indeed a link between familial autoimmune disease and autism ( that is,autism spectrum disorders).

The studies I have read that link autism (and thus, Asperger's) to familial autoimmune disease are persuasive. From these studies I learned, for example, that there is a correlation between maternal rheumatoid arthritis and offspring with conditions that are on the autism spectrum. Also, there appears to be a link between rheumatic fever, in either parent, and offspring who have some form of autism. Interesting to me was the following: my mother had rheumatoid arthritis; my brother had rheumatic fever; several paternal uncles had rheumatic fever.

While it is difficult (perhaps not even valid) to extrapolate from the general to the specific, with my research I have come closer to getting an answer to my rheumatologist's question: how likely is it that I have both Asperger's Syndrome and lupus?

The most recent study that I discuss in this essay appeared in the medical journal _Epidemiology_ in November of2010. This study was rather large--it included 1227 cases of autism and 30,693 control subjects. The results revealed what the authors characterize as a "weak" link between autism and parental autoimmune disease. A slightly stronger maternal (rather than paternal) link was demonstrated. The authors conclude that maternal autoimmunity is definitely correlated with increased incidence of autism in offspring.If both parents have an autoimmune disease, the correlation with autism in offspring is even stronger, especially if the parents have had rheumatic fever. This association between rheumatic fever holds true even if only one parent, maternal or paternal, has had the disease.

While the authors of this study are not prepared to give a rousing endorsement to a strong association between familial autoimmune disease and autism, they nonetheless observe that the association between autism spectrum disorder and certain autoimmune diseases has been observed in earlier studies and that their analysis bears out the findings of those studies. More importantly, the authors suggest that these findings open up a significant area of research for understanding the causes of autism. They assert that by examining the link between parental autoimmune disease and the occurrence of autism, researchers may have a valuable tool with which to explore the root causes of autism1.

Another study, discussed in _Psychiatric_ _News_ on August 21, 2009, describes more definitively the link between specific autoimmune diseases and autism. This study included 689,196 children, of whom 325 were found to be on the autism spectrum. The results of this study also posit the existence of a link between maternal RA and autism spectrum disorders. The article describes a 70 percent increase in the risk for offspring to have autism if the mother is suffering from rheumatoid arthritis. Further, the authors of the study conclude that there is apparently a strong connection between maternal celiac disease and the incidence of autism. As stated in _Psychiatric News,_ there is a three fold risk of offspring developing autism if the mother has celiac disease.

Not all autoimmune diseases were associated with an increased incidence of autism. According to this study, three diseases--multiple sclerosis, psoriasis, and Crohn's disease--were not associated with autism.

In addition to finding a correlation between autism and autoimmune disorders, the authors of the _Psychiatric News_ article attempt to discover whether the perceived correlation reflects an environmental or a genetic influence. The researchers posit that evidence exists for the existence of both a genetic and a gestational connection between the two conditions. They arrive at this conclusion by focusing on those births which occurred without incident--that is, the infants were full term, full-weight and had Apgar scores of 6 or above. By limiting themselves to a consideration of only these "healthy" children, the authors of the study suggests they had reduced the chance of prenatal injury as a cause of the children's autism. Even with the cohort limited to uneventful births, the "risk associations"between autoimmune disease and autism "remained statistically significant". Thus, the authors of the _Psychiatric News_ article conclude that the association between autoimmune disease and autism held up even when difficult births were removed from the cohort. None of the children included in the study were underweight, or per-term and had no other noticeable medical issues. Indeed, to be included in the study, the infants had to have an Apgar score that was higher than 6. The authors conclude that the findings of the study strongly suggest that both autism spectrum disorders and certain autoimmune diseases have a common genetic factor2.

Finally, in a 1999 study from Johns Hopkins, researchers conclude that it is a disturbed immune system combined with environmental influences that contribute to the development of autism. In the Hopkins' study, 61 families with a family member diagnosed with autism, and 46 without, were examined. The study revealed that in the families where autism had been diagnosed, there was a 46% increase in the incidence of autoimmune disease. Further, the authors of the study noticed an increase in the incidence of autism that correlated with an increase in the number of family members who had an autoimmune disorder. The researchers report that with an increase in family members who had an autoimmune disorder, there was a significant increase in the occurrence of autistic offspring. When the number of family members affected by autoimmune disease increased to three, the odds of having an autistic child increased "from 1.9 to 5.5"3.

The studies I have cited are just a small sample of the work that has been done in the area of research that examines an autism/autoimmune link. Although there is certainly cause for reasonable skepticism, it is clear that the association between the two conditions demands further examination. As our understanding of the genetic basis for disease increases, so too may our insight into the correlation between autoimmune disease and autism spectrum disorders.

Environmental Suspects

As the earlier discussion of twin studies indicated, an environmental antagonist is implicated in the occurrence of many, if not all, cases of lupus. The evidence for the action of these antagonists are derived from animal and retrospective studies. The results are less than compelling for most environmental factors, but some of these factors demonstrate a stronger association than mere coincidence would allow.

Silica: As one study concludes, there is a "growing body" of evidence that links the occupational exposure to silica with the development of lupus4. While the silica exposure in this study was in the farming environment, and other occupation risks include those in sand blasting and ship building, there are residential uses of silica.Household abrasives, for one, contain silica.Latex paint also contains silica. Given the proven, though low association, between silica exposure and later lupus development, it would probably be wise to limit any exposure to this material. Silica is absorbed through the lungs; therefore, according to OSH, wearing a respirator while using silica offers at least some protection. These respirators can be purchased easily on line or in local stores, such as Home Depot. Just make certain that they are graded for industrial use and offer sufficient protection.This information is readily available on the packaging of the respirator.

Sun: A Canadian study concluded that individuals who worked in the sun in the twelve-month period prior to diagnosis of lupus had a significantly increased rate of disease5. This was especially true for those who reported having had a "blistering" reaction to the exposure.

Paints, Dyes, Nail Polish:

In the Canadian study, it was concluded that there exists a weak occupational association between artists (who work with dyes and solvents) and the development of lupus. Likewise, there was a suggestive association between workers who applied nail polish or nail applications and the development of lupus. Recreationally, those who spent 26 or longer days working with ceramics also demonstrated an increased occurrence of the disease.

As for pesticides, the NIH issued a report in March of 2011 that described a 60% increase in the incidence of rheumatoid arthritis and lupus among women who had repeated exposure to pesticides6.

Smoking In an April 2006 article, the Arthritis Foundation discusses the association between smoking and the development of lupus. The article states that while cigarette smoking has been linked to the development of cirrhosis, Graves disease and rheumatoid arthritis, the association between lupus and exposure to smoke is less clear. The evidence suggests that if there is an association, it is probably between current, and not past, smokers and the disease7.

This list of environmental antagonists for lupus is hardly exhaustive. One of the difficulties in doing a quality study is that information used in the study is often derived retrospectively--from the recollections of people as to their exposure.This is obviously not entirely reliable. Another source of information is from animal studies, which can only extrapolate to the human experience but are obviously not as accurate.

Viral: A viral link to lupus is well established. Two relatively common diseases--Epstein-Barr and parvovirus--are associated with lupus. Repeated bouts with Epstein-Barr is considered a risk factor8 and parvovirus cannot only precipitate the disease but can also mimic it9.

Breast Implants: It is impossible to discuss the relationship between breast implants and autoimmune disease with discussing the relationship between silicone and implants. While the discussion surrounding implants and autoimmune disease is often characterized as "controversial", the U. S. Food and Drug Administration has not given give a ringing endorse to silicone breast implants. The most recent FDA statement on the matter** (see note below) is that studies conducted by implant manufacturers show the implants have a "reasonable assurance of safety and effectiveness" when they they are used in the way the manufacturer recommends. Although many women seek saline implants as a safer alternative, the saline are not silicone free but are encased in a silicone shell. So the patient seeking breast augmentation is still left with the question of whether or not breast implants are safe.

There are many strongly suggestive studies which implicate silicone in any number of illnesses, most especially in autoimmune syndromes such as lupus. In 2004, for example, Bianca Phillips, in the _Memphis_ F _lyer_ , summarized research on implants. She cites in her article an opinion by the National Center for Policy Research for Women and Families, a non-profit public interest organization, which sees a significant flaw in the FDA findings. The CPR states that the FDA does not take into account the length of time that it takes autoimmune disorders to develop. Thus, the CPR concludes, the time between implantation and onset of disease may be outside the time parameters of many of the reviewed studies.

Phillips also refers to a study by Drs. Douglas Shanklin and David Smalley. published in the _Journal of Nutritional and_ _Environmental Medicine_ , in which the conclusion is offered that not only breast implantation, but other forms of silicone introduction adversely affect the immune system.

In a 1994 study, published in _Seminars in_ _Arthritis and Rheumatism_ , Dr. Gary Solomon examined 176 individuals who had silicone breast implants. He saw evidence of siliconosis in a significant number of these patients; his conclusion was that disease tends to occur in patients who have had silicone implants for many years and who have had some localized problem associated with them, such as contracture or rupture.

More recently, the non-profit CPR refers to the FDA's 2006 decision to allow the use of silicone breast implants by plastic surgeons after a 14-year moratorium. The CPR points out that though the FDA allows the use of silicone breast implants, it warns patients to get MRI's every two years to make certain that there are no leaks of silicone from the implants. Additionally, the FDA cites evidence that there may exist an association between anaplastic large cell lymphoma and any kind of breast implant.

I am not in a position to render a judgment on this subject; on the other hand, I do have an opinion and I believe that silicone in any form is not a good thing to put in the body. An individual wishing to have silicone breast implants should be fully informed about possible reactions to silicone and about silicone leaks. Additionally, there should be full disclosure about the statistical probability of a "leak" and silicone migration. I think, considering the number of responsible researchers who have raised questions about this substance, that a warning, similar to the ones that appear on liquor bottles or cigarette cartons, is warranted.

**Since I wrote this section, the FDA has come out with a statement that silicone breast implants are "safe". While there is no further clinical data that invalidates earlier studies which have suggested problems with the implants, the FDA explains it position:MRI's will no longer be necessary to track patient's progress because MRI's are so unaffordable that few patients follow this recommendation. Additionally, since after a discovery of silicone migration the options are very limited, the FDA has decided that MRI monitoring is not helpful. In response to this FDA declaration, the CPR has counseled caution in changing the protocol Obviously, the controversy over this often elective procedure continues.

PART III

### The Patient and Lupus
Chapter 20

Photosensitivity

Far-off Ultra Violet Light in Space

Until you have had to design a life in the shade, you don't realize how automatic, even natural, natural, sun-seeking is. It is quite ordinary for advertisements to feature pictures of vacationers basking in brilliant sunshine. The human race, it seems, does not crave the shade. And yet, most of my life is spent avoiding direct sunlight.

Ultraviolet light--such as that emitted by the sun or fluorescent light bulbs–is an acknowledged lupus antagonist. Sensitivity to UV light is so common in lupus patients that the American College of Rheumatologyincludes it on the list of diagnostic criteria. Estimates for occurrence of photosensitivity as a symptom of lupus vary. An article in a Maryland Lupus Foundation newsletter by Dr. Lenny Tuffanelli suggests that photosensitivity is reported in approximately 30 or 40 percent of people who have lupus. Dr. Tuffanelli cautions that in addition to this subset of photosensitive lupus patients,all people who have lupus should exert caution when exposed to UV radiation1.

Another lupus publication, _Freedom From_ _Lupus_ , estimates the number of lupus patients who are photosensitive is between 50 and 75 percent. This estimate is higher for those individuals who are diagnosed with discoid lupus. According to this publication, the occurrence of photosensitivity in lupus patients is higher for those suffering from "subacute cutaneous lupus"2.

Finally, the Stanford University School of Medicine published a study which revealed that 73% of SLE patients experienced photosensitivity3 and the University of Tampere, Finland showed that 70% of SLE patients experienced photosensitivity4. While there is obvious variability in the estimates of photosensitivity occurrence in lupus patients, there is little argument that the phenomenon is widespread. Most of the estimates about occurrence are derived from ACR studies, and implicit in the results reported are issues of observation and interpretation. As with the general difficulty in lupus diagnosis and treatment, the physician is often dependent upon a patient's experience; added to this potentially fallible source is the physician's own clinical impression. It is hardly surprising, therefore, that the results of this mostly subjective process are essentially unreliable. How precise is an estimate, after all, which starts at 30% (the bottom of Dr. Tuffanelli's range) and goes up to 75% percent (upper limit of ACR estimate)? How can a doctor, or patient, rely on such vague numbers?

I once visited a dermatologist so that I could obtain objective verification of my UV sensitivity. The dermatologist was one in whom I had great confidence--not only did I respect him, but he was well regarded by other physicians. I asked the dermatologist to test me for photosensitivity, so I would have documentary proof that I suffered from this problem. Such proof would come in handy, I explained, in emergency rooms where the tendency is to place a patient under bright fluorescent lights for hours on end. More than once I have remarked to my family, as I lay on a gurney under unshielded lights, that if I wasn't sick when I got to the hospital, I surely would be by the time I left.

"You see how red my cheeks are," I said to the dermatologist that afternoon, after I had spent some time in his waiting room, under his fluorescent lights. "That's what happens when I sit under fluorescent lights for any amount of time". The doctor's response was exquisite in its simplicity.

"A lot of people get rosy cheeks," he said. "Maybe because they're warm, or maybe because they are excited. Let's turn off the fluorescent light and see what happens." He flipped a switch and the room was illuminated only by a small light in the corner. We sat for a while--not long enough, I thought, to make the rosy color disappear. My doctor turned the lights on. He held up a mirror so I could look at my face when he did. Lo and behold, the flush had subsided, though not completely.

"You're right--you are sensitive to fluorescent lights," he declared, and he affirmed what my family and I had learned long before through hard experience. Unfortunately, the doctor's confirmation didn't make much difference to anyone else.

It is still the rare physician who takes my concern seriously. The last time I was in an emergency room, I was moved out of the light only after my daughter started tenting a sheet over my head. If medical professionals display such indifference (or ignorance) what kind of consideration can I expect in other public areas?

Dr. Victoria Werth, of the University of Pennsylvania Medical School, explains in a landmark study the process that causes photosensitivity. Dr. Werth and her research team at Penn believe that the key to photosensitivity in lupus can be found in the gene for tumor necrosis factor. She and her team have identified a variant of this gene which is associated with this particular manifestation of lupus. Dr. Werth asserts that identifying the gene variant that causes photosensitivity can help doctors to identify people who are likely to get lupus and such identification can also contribute to an understanding of why lupus develops.

Dr. Werth discovered that in a those lupus patients who have one or two copies of the TNF variant gene, exposure to sunlight stimulates the gene and this in turn causes skin cells to undergo apoptosis (cell death).The occurrence of cell death is then a trigger for immune system activation5.

People who have a pathological reaction to UV radiation (photosensitivity) must alter their lifestyles, sometimes in significant ways. Photosensitivity does not occur only in lupus patients; there is a surprisingly wide range of conditions which have associated with them UV intolerance. The New Zealand Dermatologic Society has compiled a list of these conditions. Some of them, like lupus, are diseases: Darier's disease, Rosacea, Pemphigus, Atopic dermatitis, Psoriasis. Some are genetic disorders: xeroderma pigmentosum, Bloom syndrome, Rothmund Thomson syndrome. Some are metabolic in origin: porphyria cutanea tarda, erythropoeitic protoporphyria, variegate porphyria, Gunther's disease. Some are idiopathic (unknown origin): Polymorphic light eruption, juvenile spring eruption, actinic prurigo, solar urticaria, chronic actinic dermatitis, hydroa vacciniforme, pseudoporphyria6. And some are simply caused by contact with the environment.

The most common recommendation for dealing with photosensitivity is to wear sunscreen. Unfortunately, the remedy is not that simple. For one thing, sunscreens can be treacherous. My last experiment with one that I bought in Walgreens caused my face to swell so that I looked as though I was suffering from dropsy. Not only did my face swell, but my whole system was put into overdrive and I was ill for about a week.

One well-intentioned website recommends patch testing a new product. I suggest that you patch test with extreme caution and maybe even medical supervision. I patch tested my new Walgreen's sunscreen twice, once on the inside of my arm and once on the outside. I waited at least 12 hours between tests and another twenty four before putting the lotion (liberally) on my face. Approximately eighteen hours after the last application I started to scratch. First the chin, then the arm. One side of the arm never reacted and the other showed a moderate reaction. It was just my face which really could not tolerate this chemical sunscreen.

Not only can sunscreens be harmful, but they also are only partially effective. If I wear sunscreen, long sleeves and a hat, I am pretty well protected from the sun. But this get-up does not render me so protected that I can wander around in full sun on a summer afternoon.

As for dealing with photosensitivity in a public facility, this for me is a very difficult issue. I generally do not expect people to make exceptions for me or put themselves out because of lupus or any other problem I might have. However, I think that so many people are affected by their sensitivity to UV radiation that simple structural modifications can be routinely written into building design. For example, inexpensive filters that block the harmful radiation can be placed over fluorescent lights. Certainly in a medical setting this modification should be mandatory.

The Americans with Disabilities Act requires that reasonable accommodations be made for people with disabilities. I cite below the passage from the ADA website:

Title III requires that a wide range of public accommodations in the private sector remove physical, communications and procedural barriers to access by people with disabilities. Title III addresses the widespread exclusion of people with disabilities from the routine activities of everyday life which most Americans take for granted. Title III covers sales, rental and service establishments, as well as educational institutions, recreation facilities and service centers. It also covers commercial facilities and private entities that offer examinations or courses related to licensing or certification, and transportation provided to the public by private agencies. These provisions are enforced by the United States Department of Justice7.

I don't think of myself as having a disability, but in the sense that light sensitivity is limiting I guess, according to ADA standards, I do have one. If, when new construction is undertaken in areas that serve the public, the installation of ramps and hand grips is required as a reasonable accommodation for the handicapped, why can there not also be a requirement that inexpensive filters be placed over fluorescent bulbs? Such a minor alteration would allow me to shop longer, walk through an airplane terminal with greater ease and eat in a restaurant without endangering my health.
Chapter 21

Finding A Doctor

Dr. Jonas Salk, Who Invented the Salk Vaccine and Then Gave Away the Patent
This may be one of the most significant challenges for some lupus patients. Any time someone seeks medical care, the quality of care is only as good as the treatment provider. Lists of rheumatologists can be obtained from local medical associations.Lupus support groups also generally provide a list. However, there is no procedure for ranking these doctors in order of effectiveness or experience with lupus.Prospective patients can do a few things to narrow the search:

1. Become familiar with a lupus support organization. Often, other patients will provide recommendations that the organization cannot officially offer. Word of mouth is probably one of the most telling appraisals of a doctor's effectiveness. The first doctor that helped me was discovered just this way. A volunteer at a local organization murmured a name and suggested the doctor might be a good fit.She was.

2. Check out credentials. Only go to board certified rheumatologists--this is a bare minimum. Hematologists who wish to be board certified must first get certified in internal medicine. Rheumatology is considered a sub specialty of internal medicine. The American Board of Internal Medicine (ABIM) has a website which allows you to check on the current status of a doctor.

3. Choose a physician who is affiliated with a well-respected hospital, usually a teaching hospital or research facility. By doing this you accomplish a couple of things: usually the best hospitals select the best doctors to work in them and, if you get sick, you don't want to get stuck in a hospital that has little experience with lupus patients. Outcomes for lupus patients improve in facilities that treat large numbers of lupus patients.

4. Decide if you are comfortable with the doctor. Try to visit when you are feeling relatively well. When you are sick, you may not be in a position to advocate for yourself or to make good decisions.

5. At some point, you have to put yourself in the hands of a physician. Most patients do not have the background to second guess their doctor on every move. That doesn't mean you blindly follow all suggestions, but in the end, she or he has training and experience you do not have. I believe doctors have an obligation to explain the rationale for a course of treatment and to offer the patient access to information so the patient can participate in decision-making. I believe the patient, if in good enough health, has an obligation to use that information to help steer the course of their care.
Conclusion

I am a patient advocate, not a doctor, biologist or psychologist. I write this book because I believe every life has value. My belief is not based in any particular religion or philosophy; it is based on experience--not mine but my brother Everett's.

Everett lived for fifty-nine years and he lived all of those years as an invalid. He never had the pleasure of chewing a piece of food, of reading a book, or of walking in the sand with his bare feet. But Everett was a whole person, as precious to me as anyone I ever loved. He was not diminished by his disability, except in the eyes of the ignorant.

My years with lupus certainly have presented me with challenges, but I have never felt that I suffered particular misfortune. At each stage of lupus I have tried to extract from life that which was possible and not dwell on that which was not. Of course, I had Everett as a model: he remained through his life sweet and engaging.

I know that the course of my illness has been mild and I do not presume to understand the distress of another who is confronted with a raging and intractable case of this or any disease. All I know is that whatever the situation, the only control I may have is over my response to that situation. And I refuse to relinquish that control to despair or despondency.

Which brings me to the issue of medical care, once again. The only times my self control is threatened is when my mental health is compromised. And since lupus, in my case, does affect mood it is essential that my disease be adequately addressed for everything else to fall into place. A watershed period, which drove home this truth to me, occurred about seven years ago.

Lupus was active and the medical care I was receiving did not manage its symptoms. As hard as this period in my life was, out of it came my search for a new doctor and my association with my current rheumatologist.

The year began badly--I experienced an acute episode of abdominal pain in February and lost several pounds as a result. Psychologically I was overwrought much of the time. Finally, my psychiatrist, who was convinced of the relationship between my disease and my mood, suggested that I go to an associate of hers at Beth Israel Hospital in New York for diagnostic testing.

My husband drove me to Manhattan. During my consult with the specialist, I became emotional and despite my best efforts started to cry. The doctor concluded his tests and scheduled me for a second round the following week. But that's not how things turned out.

As it turned out, I was beset that night with muscle spasms, with a twisting of my feet and a jerking of my hand. The spasms spread through my body.

I was frightened. I called my rheumatologist in the morning, but could not get through.So I went to my internist, who, also unable to reach the rheumatologist, decided to treat me with a course of prednisone.

It worked. By night peace reigned in my body. Unfortunately, the course she prescribed was to dose down daily, which in my state was apparently too precipitous.The spasms returned, although they were not as severe as they had been. I saw my rheumatologist and she upped the dose. I was feeling great after two weeks and she decided to wean me off the prednisone. Just as I was at the end of this weaning period I had another acute abdominal attack. This time I was so depleted by the loss of fluids that my husband called 911.

In the emergency room they decided I had pancreatitis. they encouraged me to check into the hospital but I decline their invitation; it seemed to me they were perplexed about how to proceed with both lupus and pancreatitis. (In a bizarrely amusing aside, the surgeon who was assigned to treat me held his hand to his mouth and pantomimed a drinking motion, thus suggesting that my current situation might have been caused by excess alcohol consumption.) I signed myself out of the hospital and went home.

About a month passed between my first visit with the specialist at Beth Israel and my follow-up consult. This doctor, whose field of study is organic brain disease, did not wait to speak to my referring psychiatrist;he gave me his impression as our session concluded. He said that I was a different person from the woman who had seen him a month before. The prednisone had apparently quieted my mind and my body. I was not only calm and collected at this second meeting, but I also performed better on cognitive tests. The doctor said there was no question in his mind that lupus instigated the mood disturbances I experienced and that I could treat these disturbances by treating the lupus.

Although I am not comfortable writing about my mood disturbances, I believe this aspect of my illness is essential to the message of this book. Without an excellent psychiatrist, who listened to me and didn't treat me like a text-book case, without an excellent rheumatologist who was willing to look at me and my symptoms as particular and not general, I could very possibly not be here today. I certainly wouldn't be writing books.

During my rough patch seven years ago I consulted: a nephrologist; a neurologist; a hematologist; two psychiatrists; an internist; a dermatologist; a radiologist (for an MRI of the brain); three rheumatologists. I was in the emergency room several times and only by determined defiance (I signed myself out AMA--against medical advice) avoided a hospital admission. It was a doctor, the rheumatologist who oversees my care now, who put an end to the medical round-robin.

Not that he understood everything right away--but he listened to me and he targeted his approach to fit my unique case.

Although my doctor is extremely confident--justifiably so--he would be the first to admit that doctors make mistakes. We have to place ourselves in their hands, but that doesn't mean we have to do so blindly.

A good doctor does not mind being questioned and indeed offers information about the patient's illness and treatment plan. My doctor not only explains what he is doing, but he refers me to research so that I can be comfortable with the choice that we both arrive at together.

If you have a doctor who is not responding to your concerns, is not forthcoming, or does not encourage you to learn more about your illness and treatment plan, then I suggest you look elsewhere. Either the doctor lacks confidence or suffers from arrogance. In either case, this cannot be an ideal situation for you.

Because of excellent medical care, I get up each morning and expect to have a good day. If the day isn't good, then I know I can go somewhere and have my problem competently and compassionately addressed. I wish everyone who reads this book, whether they have lupus or have no illness at all, the very same good fortune.

### Part IV

### Reference

Glossary and Lab Tests
Glossary

Alopecia--hair loss, can be from the scalp or the whole body

Analgesic--something that suppresses or lessens pain

Anemia--deficiency in the number of red blood cells or the amount of hemoglobin in the blood

Antibodies--An essential part of the immune process. These cells are produced by the immune system to attack foreign substances

Apgar Score--evaluates (on a scale of 1-10, where ten is a perfect score) the alertness of an infant immediately after birth. This test primarily is an indication or whether or not an infant needs immediate medical attention. However, repeated low values after a lapse of 15 to 30 minutes raise suspicion of long-term health issues

Autoantibodies--cells produced by the immune system that attack cells of the producing organism (against self)

Anti nuclear antibodies--antibodies that attack the nucleus of cells

Antiphospholipid Syndrome--when antibodies attack phospholipid, which is in the cell membrane.Antiphospholipid Syndrome leads to blood clots

Anti-RNP--antibodies against ribonuclear protein, which is located in the nucleus of a cell

Anti-RNP--occurs in lupus and other connective tissue diseases

Apoptosis--death of a cell

Ascites--fluid accumulation as a by product of serositis in the abdomen

Atherosclerosis--build up of fats in artery walls leading to thickening and stiffness

Cardiolipin--protein that exists in the mitochondrial lining of cells. Anti cardiolipin Syndrome can lead to blood clots and increased incidence of miscarriage

Cataracts--an opacity in the lens of the eye. Occurs often with age and sometimes as a side effect of steroid therapy

Central Nervous System--comprised of the brain and spinal chord

Cerebrovascular disease--disease of the blood vessels in the brain

Clearance Deficiency--a deficiency in the ability to clear cell debris from the system--found often in lupus

Collagen--the protein that makes up the majority of connective tissue in the body

Connective Tissue--as the name suggests, connective tissue connects and supports all the organs in the body

Complement--an essential component of the immune system which acts to specifically attack certain antigens or as a more general element in the overall immune response

Complement Deficiency--a reduction in the activity of the complement system

Creatine--substance present in the muscles

Creatinine--a substance found in the blood that is released as a by product of creatine. Elevated levels of creatinine are one indication of kidney disease

Differential Diagnosis--method of arriving at a diagnosis used in lupus.This is a process by which doctors evaluate a patient's complaint (reason for going to the doctor in the first place) and start with the most obvious explanation and through a process of elimination, aided by diagnostic tests, arrives at a conclusion. Differential diagnosis can thus take a very long time and involves interpretation and insight

Discoid Lupus--kind of lupus in which lesions, shaped approximately like discs, appear on the skin.Can also appear in systemic lupus.About ten percent of the people with discoid lupus go on to develop systemic lupus

Drug Induced Lupus--certain drugs precipitate lupus. However, with a discontinuation of the drug, drug-induced lupus goes away

Enzyme--a protein that acts as a catalyst on other substances in the body

Erythema--sometimes a symptom of inflammation, the skin becomes red as a consequence of underlying blood vessels dilating

Erythematosus--a redness of the skin caused by inflammation of the blood vessels beneath the surface

Glomeruli--small blood vessels in the kidneys which filter waste from the rest of the body

Glomerulonephritis--inflammation of the glomeruli

Hematologic--having to do with the blood

Hematuria--blood in the Urine (often detected only by laboratory analysis)

Hemolytic Anemia--an anemia that occurs from the premature destruction of red blood cell. If the bone marrow is not able to produce enough red blood cells to replace those lost, that is called hemolytic anemia

Hepatic--having to do with the liver

Immunofluorescence--a laboratory process in which the specimen is stained with a fluorescent dye

Immunosuppressive--something, such as a drug, which suppresses the activity of the immune system

Keratosis--overgrowth and thickening of skin tissue--usually discolored

Lymph--occurring throughout the body, it acts as a kind of transportation system. It carries fats, bacteria and other material to the lymph nodes where these impurities are filtered out. It carries lymphocytes, which are white blood cells that are part of the bodies disease fighting mechanism

Lymphoma--cancer that effects the lymph system. Occurs more commonly in lupus patients than in the general population

Lymph nodes--masses of tissue that act as filters for the lymph system

Malaise--a non-specific feeling of being unwell

Microphage--a large, fixed phagocyte

Myalgia--muscle pain

Myopathy--any disease of the muscle

NSAIDs--non steroidal anti inflammatories, such as aspirin

Necrosis--death of cells or tissues

Neonatal Lupus--when the mother transfers autoantibodies to the fetus in the womb

Nephritis--inflammation of the Kidneys

Neuropsychiatric--psychiatric condition arising from a neurological disturbance

Osteoporosis--thinning and weakening of the bones, often leading to fracture. Can be caused by steroid therapy

Pathology--sickness

Pemphigus--bullous autoimmune disease of the skin and mucous membranes

Peripheral Nervous System--connects the central nervous system to the rest of the body. Consists of a nerves and ganglia

Phagocyte--any cell that ingests invading organisms and helps clear cell debris

Phospholipids--are derived from fat and are in all cell membranes

Photosensitivity--demonstration of reaction to exposure to sun or UV radiation

Platelets--essential to clotting, they are irregularly shaped bodies that are in the blood. Their sticky surface allows them to adhere to each other and thus form clots

Pleuritis--serositis of the lungs

Pleural Effustion--fluid accumulates in the tissue between the lung and the chest wall

Pulmonary Emboli--blood clot in the lung

Red Blood cells--produced in bone marrow, red blood cells are the oxygen delivery system in the body.They pick up oxygen from the lungs and carry it to other cells

Renal--having to do with the kidneys

Renal Failure--when the kidneys no longer filter the blood and toxins build up in the blood stream

Sensitivity--measures the degree (statistically) to which a test identifies correctly people who have a condition

Serologic--having to do with the blood

Serositis --accumulation of fluid in the lung, heart or abdomen

Specificity--the degree (statistically)to which people are correctly identified as not having a condition

Steroid--naturally occurring or synthetic hormone that helps regulate different systems and maintain homeostasis

Subclinical--a disease process that exists but has not yet manifested symptoms. In this case, perhaps a laboratory test will reveal the existence of an abnormality that could become significant in the future or under certain conditions

Titer--level, or measurement of a substance in the blood

TTP (Thrombotic thrombocytopenic purpura)-a clotting disease in which tiny clots occur throughout the body

Urea Nitrogen--when protein is metabolized, it produces this waste product. Too much urea nitrogen in the blood is an indication of kidney stress

White Blood Cells--also called leukocytes, white blood cells are the units in the blood that fight invading organisms (infection)

### Laboratory Tests

Activated Partial Thromboplastin Time (APTT)--partial Thromboplastin Time. Measures the rate at which blood coagulates (clots). Tell is there is a clotting problem or if certain anti-coagulant rugs (like Heparin) are working properly

Albumin--tests for the amount of albumin the the blood--normal range is between 3.5 and .4 grams per liter. Less than that can indicate liver malfunction or kidney disease

Alkaline Phosphatase (ALP)--since alkaline phasphatase is made in the liver and the bones, an ALP test is used as an indicator of liver or bone abnormalities

ALT(SGPT)(alanine aminotransferase)--Tests for injury to the liver. This test, in conjunction with others (ex., bilirubin) can help pinpoint the kind of injury or disease that the elevated levels are indicating.The higher the ALT level, the more acute the problem

Amylase--elevated amylase indicates a problem with the pancreas. The higher the level, the more acute the problem

Antinuclear antibody--blood is tested either by using indirect immunofluorescence or Elisa (Enzyme-linked immunosorbent assay). The Elisa is less accurate but also less expensive. This test detects autoantibodies. If titer if 1:40 or less, this is considered a normal reading.

AST (SGOT)--this test is conducted to discover if liver disease is present. Elevated levels are an indication that the liver has been stressed

Anti dsDNA--several methods are used to detect the presence of anti-- dsDNA. EIR is the more accurate and thus the preferred test. IFA and RIA are also done. If these

tests reveal the presence of anti-ds DNA, this is almost diagnostic for lupus. This antibody is found almost exclusively in lupus patients and about 50-70 percent of them have it

Ant-ENA (anti Extractable Nuclear Antigens)--Several antigens are included in this test: RNP, SM SSA, SSB. Of these, only SM is found almost exclusively in lupus patients.

Anti-Ro--this is a test for an antibody that tends to appear when Sjogren's Syndrome is present

Anti SM--this tests looks for the presence of the Anti-Smith antibody.The antibody was found in the blood of a lupus patient named Smith and was named after her. Presence of this antibody is almost diagnostic for lupus

Band Cells--mature neutrophils ( a kind of white blood cell) that are found in large numbers in the presence of bacterial infection.

Basophils--a less common type of white blood cells that is found in elevated numbers when infection is present

bilirubin--produced by the liver, it is a byproduct of the breakdown of red blood cells. Elevated bilirubin can indicate liver problems

Biopsy--a laboratory sample, excised from an organ

Calcium--the most common mineral in the body, essential to normal function. 99% of it is found in the teeth and bones. Levels of calcium are strictly regulated by the interaction between the parathyroid, kidney, small intestines and skeleton.

Carbon Dioxide--levels of Carbon dioxide are looked for in blood gas tests.Different levels of carbon dioxide are reflect lung and kidney function

CBC--blood test that determines serum levels of red blood cells, white blood cells platelets and hemoglobin

C-Reactive Protein--a marker for inflammation in the body, not specific.Elevated levels of C Reactive is thought to be associated with an increased risk for heart attack.

Creatinine (CR)--high levels of creatinine indicate a problem with the kidneys

Eosinophils--a type of white blood cell. Elevated serum values typically are found in allergic reactions and parasitic infections

ESR--erythrocyte sedimentation rate.Elevated levels of ESR is an indication of inflammation.

Fibrinogen--a blood protein necessary for clotting. High or low levels can indicate clotting problems.

Glucose--blood sugar. This test is performed after fasting. Higher than 127 (after a fast) indicates diabetes

Hemoglobin--a blood protein that carries oxygen throughout the body.Low hemoglobin counts indicate anemia, which can be caused by any number of factors. Excess hemoglobin is also a red flag

HDL Cholesterol--known as the "good" cholesterol because it clears cholesterol from the blood. High levels of HDL are associated with a reduced risk of heart disease

Hct (hematocrit--This test is performed to discover what percent of the blood is comprised of red blood cells. Low hematocrit is an indication of anemia

Hgb--another term for hemoglobin

Ketones--test is conducted after a fast (at least four hours). The presence of ketones is an abnormality. It can indicated diabetes or advanced alcoholism

LDL Cholesterol--the "bad" cholesterol. Instead of removing cholesterol it carries the substance to the cells

Lipase--high amounts of lipase in the blood is an indication of a problem with the pancreas

Liver Enzymes--the liver secrets a number of enzymes. Elevated levels of these indicate liver stress and warrant further examination

Lymphocytes--white blood cells. In the presence of infection, these numbers are elevated

Magnesium--an essential mineral in the body. A variety of conditions are associated with high or low magnesium levels

MCV (Mean Corpuscular Volume)-- This test measures the size of red blood cells

MCH (Mean Corpuscular Hemoglobin)--This test reveals how much hemoglobin each red blood cell carries

Macrophage--a phagocyte. Like sentinels, they stand guard at specific sites in the body ready to defend against invading organisms. Detection of an increase in the number of macrophages indicates an elevated immune response

Microphage--a phagocyte, like a macrophage, except more numerous, less long lived, and less specific in activity

Monoocytes--a kind of red blood cell.Elevated monocyte levels can be an indication of an infection and/or of inflammation

Neutrophils--white blood cells. This is the type of white blood cell most commonly found in the body. A variety of conditions lead to high neutrophil counts. Reduced counts indicate increased susceptibility to infection

Occult Blood--this test looks for blood in the stool that is not visible to the naked eye. Any number of conditions can be the cause of blood in the stool. Some, like hemorrhoids, are relatively benign, and others, like intestinal lesions, are not

pH--this test checks the acidity of the blood. The normal range for blood is slightly over seven. If the ph falls under 7.35 the condition is known as acidosis. If the ph falls over 7.45 the condition is known as alkalosis. The body keeps tight control over ph levels. Either acidosis or alkalosis are dangerous

Phagocyte--these are an essential part of the immune system. These exist to identify, surround and destroy invading microorganisms. Play a role in development of immunity and also are thought to play a role in the development of inflammation from autoimmunity

Phosphates--an essential mineral filtered by the kidneys. High levels of phosphates can indicate that the kidneys are not functioning properly

Platelet Count--platelets are essential for clotting. The number of platelets may increase or decrease with infection. Sticky platelets lead to clotting; a deficiency leads to bleeding

Potassium--an essential mineral and electrolyte. Testing for potassium is usually part of a routine work-up, but specifically targets problems with the kidney and heart function. Levels of potassium are closely associated with levels of sodium

Protein--test is ordered to look for a number of conditions, usually those of the liver and kidneys

RBC (Red Blood Count)--Usually conducted as part of a CBC (Complete Blood Count) RBC determines the number of red blood cells

Sodium--test conducted to determine the amount of sodium in the blood. A number of medications can disrupt the level of sodium and the results of this test would be one indication

Specific Gravity--part of urinalysis. Tests to see the water to urine concentrations

SS-A--a specific antibody test. Tends to appear in patients with Sjogren's and lupus/Sjogren's overlap.Important for an expectant mother to find out if she has this antibody because it can lead to serious complications for the baby

Triglyceride--a kind of fat in the body.An important source of ready energy. Triglycerides are formed when the body does not use calories you consume, especially calories that come from carbohydrates. Elevated triglyceride levels are implicated in heart disease and diabetes

TSH (Thyroid Stimulating Hormone)--Secreted by the pituitary gland, it controls the secretion of hormones from the thyroid gland

Thyroxine (T4)--one of the two hormones released by the thyroid gland. The other is thyroxin (T3)

Uric Acid--excess uric acid in the blood can be an indication of kidney disease

Urobilinogen--A product of the breakdown of bile by the liver. Abnormal levels can indicate liver problems

WBC--white Blood Count. Tests for the number of white blood cells. Normal range is approximately 4,000 to 10,000 per mcL. Too many can indicate infection. Too low can indicate immune deficiency
Appendix
May 2017

Electrical Stimulation of Vagus Nerve Shows Promise as Possible Treatment for a Range of Autoimmune Diseases

In December of 2016 the Feinstein Insitute (Manhasset, NY) published a report on the possibility of a new, targeted treatment for lupus. This treatment involves stimulation of the vagus nerve (see diagram below).

The Feinstein researchers built their theory on earlier studies that showed electrical stimulation of the vagus nerve was effective in treating rheumatoid arthritis. The vagus nerve is the largest cranial nerve. It runs from the medulla (at the base of the brain) down to the bowel. It therefore offers a direct path to nerve impulses that affect different organ systems throughout the body.

What the researchers of this study discovered was that if they blocked specific neurological signaling from the vagus nerve with electrical stimulation, they could tamp down an inflammatory response. The full article explaining the study may be found here:

http://static.smallworldlabs.com/bioelecmed/content/pdfstore/16_007_Steinberg.pdf

The encouraging aspect of this study is that it is a targeted approach. Unlike immune-suppressive drugs, electrostimulation would not suppress the entire immune system but would simply target specific areas in the central nervous system.

At the link below this paragraph, a detailed description of the vagus nerve pathway is described:

http://emedicine.medscape.com/article/1875813-overview

NSAIDS

5/10/2017

I opened my email this morning and discovered a note that had been forwarded to me about NSAIDS. I had written about this class of medication on my blog in 2012 and so was interested to read an update from the National Institutes of Health. In this update the NIH warns that use of NSAIDS is associated with an increased risk of heart attack. The report is careful to emphasize that an association is just that. It doesn't necessarily suggest a causative link but it does raise serious questions about the use of NSAIDS and the risk of heart attack. The NIH report may be found at this web address: https://medlineplus.gov/druginfo/meds/a682159.html

My blog contained information along the same lines. It was offered as a piece of information that is publicly available and it featured advice from the American Heart Association. While I cannot evaluate either the NIH report or the American Heart Association recommendations, I do believe every consumer who is using NSAIDS or considering using them should have this information at hand. The information may not change decisions but at least these can be made with a clear eye to possible consequences. Here's the blog:

12/28/2012

A Handful of Pills  
 _  
_If you have systemic lupus, there's a pretty good chance you have been prescribed a _NSAID_ (example: aspirin, ibuprofen, Naproxen, etc.) over the course of your illness. Given that likelihood, I have dedicated some time to seeking an understanding of this broad class of drugs. The following essay is one of several I hope to post on the subject.

In writing this post, I have tried to be very careful in my use of language; I draw no conclusions. The available information about NSAIDs indicates that caution should be exercised in their use. Some of the reasons for the caution are explained below. Since research on these drugs continues, the recommendations of the American Heart Association and the FDA for their use reflect only what is currently known. Recommendations of both organizations will likely change over time.

The American Heart Association has issued repeated advisories about the use of NSAIDs; while the Association allows that NSAIDS may still be prescribed under certain circumstances in clinical practice, caution is required. The Association recommends that NSAID use be tempered by an awareness of associated risks. These are risks about which the patient should be informed if NSAID therapy is decided upon. As the Heart Association explains in its report, _An Update for Clinicians: A Scientific Statement from the American Heart Association (_ web address: (http://circ.ahajournals.org/content/115/12/1634.full _)_ , the issues surrounding NSAIDS "highlight" the obligation of patients and doctors to balance "the risks and benefits of medications for pain relief".

So, what are these risks? This is a question that still has not been completely answered. Part of the answer is certainly that there is evidence of increased heart attack, heart failure, stroke and vascular events. The most recent advisory I found from the Heart Association (Sept, 2012) is entitled, _"Taking Painkillers Increases Death Risk, Second Heart Attack in Survivors",_ which may be found at: _http://formularyjournal.modernmedicine.com/formulary-journal/news/clinical/clinical-pharmacology/high-dose-non-cox-2-nsaids-associated-increase_ ). The article explains that the painkillers in question are NSAIDs. Anne-Marie Schjerning Olsen, the physician who led the relevant study, states: "It is important to get the message out to clinicians taking care of patients with cardiovascular disease that NSAIDs are harmful, even several years after a heart attack".

According to the Heart Association's 2007 study (cited above), not all painkillers appear to carry an equal risk of potentially lethal side effects. The Association cautions that results are suggestive and more information needs to be collected about the relative risks of each drug. With this cautionary note, they publish a chart that is meant to guide physicians in their use of NSAIDs. The chart describes a "stepped approach" to prescribing painkillers. In this approach, the least dangerous (according to Heart Association information) should be prescribed before moving on to those drugs that are seen to carry greatest risk. The Heart Association chart can be found at: Figure 7, web address http://circ.ahajournals.org/content/115/12/1634.full

Studies seem to suggest that NSAID risk is dose dependent, though what a safe dose is for an individual has not been established. The risk to individuals from NSAIDs (available over the counter in many cases) is considered so serious that the U. S. FDA has mandated inclusion of black box warnings in their packaging. A description of this black box warning can be found at the NIH website; _APPENDIX BLACK BOX WARNINGS OF INCLUDED DRUGS ._ Web address: https://www.ncbi.nlm.nih.gov/books/NBK53952/

As I repeat throughout my posts on this website: I am not a doctor or a researcher. I am a patient who reads and who seeks to understand. What I get from the research about NSAIDs is that these readily available drugs are serious medicine. The fact that they are over-the-counter and easily accessed belies the danger they may present to the medical consumer. Before taking any one of these, think for a moment. Be sure that the drug is important to your care. And, consult your doctor. Between the two of you, an informed decision about taking NSAIDs can be made.

Mitochondria May Hold a Key to Promising Therapy

5/14/2017

One area of investigation that seems promising comes out of the University of Vermont. Researchers there are collaborating with other institutions (Wellcome Trust, University of Glasgow, SUNY Upstate Medical Center, and Weill Cornell Medical College) to discover the role of "stressed out" mitochondria in the production of interferon. Interferon plays an important role in combating infection. Release of interferon also correlates with activation of lupus flares.

The researchers in Vermont are looking for a trigger that causes the mitochondria to release the interferon in the absence of infection. This aberrant immune response may hold the key to a targeted lupus treatment. If the mechanism for the mitochondrial misfiring can be discovered, then this one aspect of the immune response may be addressed. Researchers believe this would be a far less devastating approach to tamping down the immune response than the administration of drugs (steroids, for example) that affect the immune system globally.

An article that describes this new avenue of investigation may be found at:

###

### Stressed Out Interferons Reveal Potential Key to Alternative Lupus Treatment

https://www.med.uvm.edu/research/news/2017/02/13/stressed_out_interferons_reveal_potential_key_to_alternative_lupus_treatment

And an article describing the role of interferon in lupus activation may be found at:

Detection of interferon alpha protein reveals differential levels and cellular sources in disease

http://jem.rupress.org/content/early/2017/04/17/jem.20161451

Plaquenil

One of the first medications a lupus patient may be offered is Plaquenil. While every drug has side effects, Plaquenil is generally considered to be "safer" than many other medicines prescribed to treat lupus.

Researchers are finding out more about this drug all the time; the current state of science indicates that Plaquenil is effective against lupus, and other inflammatory diseases, because it creates an inhospitable environment for some actors in the immune process. In this way Plaquenil inhibits the immune response and thus also inhibits inflammation.

Though Plaquenil is considered to be relatively "safe", there are several populations in whom great care should be taken before medicine is prescribed. If you are over the age of 60, the chance for retinal damage increases. If you have psoriasis or porphyria, taking this drug may exacerbate your condition. Anyone with reduced liver or kidney function should also consider carefully if the risks of the drugs outweigh its benefits. Plaquenil can be extremely dangerous for children, especially children under the age of 6. People who have a metabolic disorder called G6PD run the risk of developing severe anemia on Plaquenil therapy.

The most commonly addressed side effect Plaquenil therapy is retinal damage. Every responsible opinion I have read prescribes a visual screening before therapy begins and then a re-check several weeks later. While it is rare for eye damage to occur, especially if therapy lasts less than five years, it is important to keep in mind that once damage occurs, it is most likely irreversible. And the changes in the retina may not be noticed subjectively but can be detected by sophisticated testing in the ophthalmologist's office. Many doctors recommend a yearly check-up; some recommend six months. If I were on Plaquenil I would go for six months because I'd like to detect damage before it goes very far.

With all of the warnings listed above (this is just a partial list of possible side effects) it would seem that Plaquenil is a dreadful drug. It really isn't for most people. Lupus is a dreadful disease and sometimes you have to take out a big gun to control it. Plaquenil is actually one of the smaller guns in the arsenal against lupus.

Plaquenil is supposed to be particularly effective at treating discoid lupus.

All of the sources I consulted implicate higher-dose and longer-term Plaquenil treatment in increased risk of eye damage. So if you are on Plaquenil for a many years, certainly as many as 8, your doctor might begin a conversation with you about switching to an alternative treatment.

Azathioprine

In my brief article on Plaquenil, I mention that there are bigger guns than this medicine in the rheumatologist's lupus arsenal. One of the bigger guns is azathioprine. Azathioprine is an immunosuppressant which was designed in 1957 for use in chemotherapy; however, the drug was discovered to be useful in the treatment of some autoimmune diseases, including lupus.

Although today azathioprine is widely accepted as a lupus treatment, the FDA has approved the drug for only two uses: kidney transplants and rhuematoid arthritis. All other uses are considered "off-label".

Nonetheless, azathioprine can be a powerful ally in the battle against lupus. The drug acts to inhibit cell reproduction, especially reproduction of T and B cells, two lymphocytes implicated in inflammation. Often, azathioprine is given in addition to a corticosteroid. When azathioprine starts to take effect (which may not be for months) steroid use may be reduced or even eliminated.

People taking allopuranol, niacin, warfarin and certain muscle relaxants should be aware that potentially dangerous drug interactions have been observed between when these medicines are taken in conjunction with azathioprine. There is also some controversy about whether a statistical increase in fetal abnormalities is due to azathioprine use or to the underlying conditions for which azathioprine was prescribed. According to the Mayo Clinic: "Studies in pregnant women have demonstrated a risk to the fetus". The Mayo site goes on to say that even though this risk exists, in some cases there has to be balanced the risk to the fetus against the risk to the mother's life.

The chances of developing skin cancer rises with azathioprine use, as does the likelihood of developing a rare type of lymphoma. There is also the potential to experience bone marrow suppression and leukopenia. These are just some, not all, of the potential side effects of azathioprine therapy. That said, this drug can be very useful and effective; it is taken successfully by many people. I came across the abstract of an article which describes the success rates for patients who were treated with azathioprine (see Arthritis and Rheumatism, below). People who had begun the drug course with a poor prognosis showed a dramatic increase in survival rates and those with a good prognosis showed a dramatic decrease in hospitalizations.

Although reading about the side effects of a medicine may be upsetting, that shouldn't dissuade anyone from accepting proper, sometimes aggressive treatment for their disease. Appropriate medical interventions, including powerful drugs, may be all that stands between a lupus patient and good health.
Methotrexate

Methotrexate is an immunosuppressive drug—which means it interferes with the production of certain kinds of immune cells. Back in the 1950s doctors noticed that patients who had certain types of cancer did better if they were deficient in folic acid. This observation led to the development of methotrexate, which interferes with the action of folic acid. Treatment with methotrexate became common for selected cancers because the medicine was found to have less side effects than some other preparations.

Eventually, methotrexate was discovered to be useful in autoimmune diseases, especially rheumatoid arthritis, but also lupus and Crohn's disease, among others. Doses necessary to achieve a therapeutic effect in lupus are much lower than those administered in the treatment of cancer. Consequently, side effects may not be as severe.

However, methotrexate is a powerful drug and can have very serious side effects. It's use is absolutely prohibited if there is a chance of pregnancy. Even after discontinuing the drug, pregnancy must be delayed until all of the medicine has cleared from the system. There is an increased risk of liver and lung damage while on methotrexate therapy. Also, nervous system complications have been noticed in some cases.

Drug interactions include penicillins, probenecid, phenobarbitol, carbamazepine, Bactrim and NSAIDs (although NSAIDs may sometimes be prescribed in combination with methotrexate). It is recommended that while on Methotrexate the patient abstain from drinking because of the potential to damage to the liver. It is essential that the patient be monitored (blood tests) regularly. Any sign of shortness of breath or chest discomfort should be reported immediately to the doctor.

Patients taking methotrexate often are prescribed a folic acid supplement to avoid deficiency.

Though Methotrexate is widely used for the treatment of lupus, it is generally not the medicine of choice for those who have lupus nephritis or organ involvement. According to the Mayo Clinic, methotrexate should be used with caution in lupus patients with these symptoms.

While methotrexate is a powerful drug and can have potentially serious side effects, the fact remains that it has been very useful in the treatment of a variety of diseases. As with every medicine, risk has to be weighed against benefits. For many patients, the benefits are greater than the risk.
Lupus and Gender

Most of us know that lupus affects females more commonly than it does males. The ratio most often reported is 1 to 9. That is, for every male that has lupus there are supposed to be about nine females. Generally, it has been considered that lupus in males and females is much the same disease. However, when it comes to lupus, nothing can be taken for granted. In a recent article, Julie Schwartzman and Chaim Putterman, of the Albert Einstein College of Medicine, explore the expression of SLE in males and females. Their findings are based on cross-cultural and cross-national studies and indicate a possibly clinically significant difference in the way lupus affects females and males.

The Schwartzman/Putterman article considers studies in China, Latin America, Greece and the U.S, among other ethnic groups. The findings almost uniformly indicate a more severe course of illness for males than for females (except for one contradictory study in Hong Kong). This less favorable outcome for males was linked, in most cases, to an increase in the incidence and severity of lupus nephritis.

There were other differences detected between males and females who had lupus. One of the differences was that it took less time for a male to be diagnosed with the disease than for a female to be diagnosed. Another difference was that males seemed to experience more seizures than females but reported less arthralgia.

Whenever a well-sourced, responsible study is released–and the Morris/Putterman paper is responsible in the extreme—caution should be exercised in its interpretation. In considering gender differences and healthcare, there may be issues of reporting and physician interpretation.

Speedier diagnosis, for example, could be due to the fact that many doctors are more likely to take seriously a man reporting symptoms than a woman reporting symptoms. Also influencing the speed of diagnosis could be the fact that, if indeed males experience higher incidence of lupus nephritis, then this symptom would likely lead to earlier diagnosis—since nephritis is almost a hallmark symptom of systemic lupus.

The implications of a possible gender difference in lupus manifestation are significant. It has been suggested that drug protocols might be established that target the particular susceptibility of male patients to severe lupus nephritis. Also, an awareness on the part of physicians and patients that this risk seems to exist, would suggest a more vigilant approach toward monitoring kidney function in male lupus patients
Prednisone And Belly Fat--Self-Perpetuating Cycle

Researchers at the University of California San Francisco examined the relationship between belly fat and stress adaptation.They separated women into groups according to their WHR (waist hip ratio) and subjected them to a series of stress tests.The study was premised on two facts: 1.That during periods of stress, cortisol is released into the blood stream and, 2. That the greater the WHR the greater the amount of cortisol released. The authors conclusions were somewhat surprising: they found that those with the greatest WHR demonstrated the poorest ability to adapt to stress. Even when identical environmental stressors were introduced over time, those with the highest WHR did not show a decrease in the stress response: that is, they were unable to adapt to the stress.

An interesting aspect of the study's findings was that even in lean women, this poor adaption was evident as long as the WHR was high. As a matter of fact, the leaner group with high WHR fared worse on this test than the more obese participants. The authors' conclude that individuals who have abdominal fat have an associated increase in susceptibility to psychological stress. They also demonstrate an increased sensitivity to serum levels of cortisol. The study has another interesting finding: while it is acknowledged that medicinally administered cortisol results in increased belly fat, cortisol increases which occur naturally, because of stress, also increase belly fat. This increase in belly fat, though induced by the body's own secretions, is also linked to an increase in psychological stress and disease vulnerability. Thus, the accumulation of belly fat, whether as a result of prednisone therapy, life style or genetics, has negative implications for the psychological health of the individual. This belly fat/psychological vulnerability link might be an element considered in the mental health treatment of the lupus patient--who already runs a high risk of psychiatric impairment as a direct result of disease activity and as an indirect result of physical limitations.
Cortisol and Insulin Resistance

An increase in cortisol production leads to insulin resistance and thus diabetes.Increased levels of cortisol, as a result of steroid therapy, leads to an increase in the release of glucose from the liver. Increased glucose in the blood leads to insulin resistance. Insulin resistance leads to the development of type II diabetes.

As described in the _Journal of Hypertension_ , insulin resistance is also strongly implicated in the development ofhypertension.1
Cortisol and Essential Hypertension

Cortisol suppresses the passage of salt out of the bloodstream. With more salt, there is increased blood volume and resulting higher blood pressure. Higher blood pressure leads to damage of the arteries which cascades into a number of symptoms including, greater likelihood of cerebral and cardiac events, and kidney damage. As described in the _The Journal of Clinical Endocrinology & Metabolism_, with elevated salt sensitivity comes an increased probability of developing essential hypertension. The association between salt sensitivity and increased occurrence of essential hypertension has been observed over different population groups: those who already have hypertension, normal subjects,African American subjects and caucasian subjects.

Not only does cortisol suppress the passage of salt from the blood stream, and thus potentially lead to arterial damage, but the _Journal_ article further suggests that increased concentrations of cortisol in the muscles of the arteries is itself damaging.The authors describe a process in which the influence of increased serum levels of steroids negatively impacts on the smooth muscles of the vascular system. And thus, the chances for developing hypertension are increased. This damage appears to be the result of increased sensitivity to"vasoconstrictors, angiotensin-II and phenylephrine."

Not only do increased concentrations of cortisol in the blood indirectly affect arterial integrity by limiting salt elimination, and directly by compromising smooth muscle response to vasoconstrictors, but the authors of the _Journal_ article also suggest that cortisol directly acts on the brain to induce hypertension. In their research they find the appetite for salt is strongly influenced by the central nervous system and that this appetite is increased by elevated levels of cortisol.1

### Footnotes

Introduction

1.The study was presented at European League Against Rheumatism at the annual congress of the European League Against Rheumatism in Copenhagen, Denmark. from UPI.com on June 15, 2009

Hannelore Kohl

1.In people with cutaneous lupus, photosensitivity affects 50 percent of those with discoid lupus and 70-90 percent of those with subacute cutaneous lupus.Lupus Foundation

2.The results from the performed examinations showed a high percentage (64.44%) of neuropsychiatric lesions in the patients with SLE. Hippokratia. 2008 Apr--Jun; 12(2): 103.Classification criteria for neuropsychiatric systemic lupus erythematosus: Do they need a discussion? S Monov and D Monova Department of Internal Diseases, Medical University, Sofia, Bulgaria Corresponding author: Monov S, 24, Veliko Turnovo st., 3.Clue to Bushes' Disease Sought in Water By Lawrence K. Altman, May 29, 1991

Inday Ba

1.In Memoriam Website by Copyright: Christina Johansson

1 Ibid

1.Ibid

2.Ibid

3.Ibid

4.Wikipedia

5.In Memoriam Website by Copyright: Christina Johansson

6.Ibid

7.Ibid

8.Ibid

9.Ibid

10.Ibid

11.Ibid

12.Ibid

Seal

1.Celebrities Who Have Overcome Obstacles, Jet, June 10, 1996

2.Contact Music.com January 4, 2007

3.MAIL online. Seal and Heidi: the couple who can't keep their hands off each other. Craig McLean. Nov 7, 2007

4.Ibid

5.Ibid

6.Ibid

7.Answers.com, Simon Glickman

8.Ibid

9.Ibid

10.Ibid

11.Ibid

12.Wikipedia

13.Film.com, Top 10 Celebrity Dads. Brad Witter Jan 19, 2009

14.MAIL online. Seal and Heidi: the couple who can't keep their hands off each other. Craig McLean. Nov 7, 2007

**Flannery O'Conno** r

1.Barnes and Noble Review. Amy Benfer February 25, 2009

2 Wikipedia

3.Brad Gooch, Flannery: A Life of Flannery O'Connor Little Brown

4.Lupus Foundation

5.Fitzgerald, Sally, Letters of Flannery O'Connor: The Habit of Being p. 57

6.Gooch 69

7.Guardian.co.uk Churchwell, Sarah\Grace Like A Bullet in the Side April 25, 2009

8.Gooch 46

9.Wikipedia

10.Wikipedia

11.Habit of Being xii

12.Gooch 156

13.Gooch 185

14.Flannery O'Connor, Everything that Rises Must Converge xxiv Farrar, Straus and Geroux

15.Gooch 225

16.Gooch 193

17.Gooch 267

18.Gooch

19.Gooch 358

20.Gooch 366

21.Gooch

22.Gooch 368

Ferdinand Marcos

1.The Shoes of Imelda Marcos by Lance Morrow Time.com Monday March 31, 1986

2.The Anti Marcos Struggle: personalistic rule and democratic transition in the Philippines. page 54 by Mark R. Thompson Yale university press 1995

3.Wikipedia

4.Wikipedia

5.The Philippines a Singular and a Plural Place by Joel Steinberg p116 Westview Press

6.Marcos Counterattacks in War Record Dispute January 25986 From Times Wire Services

7.Gale Encyclopedia of Biography on Answers.com

8.Wikipedia

9.Ferdinand Marcos, Ousted Leader of Philippines, Dies at 72 in Exile By Jane Gross, Special to The New York Times Sept 29, 1989

10.Wikipedia

11.Wikipedia

12.Bobwurth.com

13.Britannica Concise Encyclopedia on Answers.com

14.Wikipedia

15.Wikipedia on Answers.com

16.Wikipedia on Answers.com

17.AbouTime.co Story & Pix Annamarie Hoeve/TCS and Eric Pasquier/TCS

18.Wikipedia

19.Wikipedia

20.About.com Biography of Ferdinand Marcos By Kallie Szczepanski.

21.About.com Guide Marcos required every business and classroom in the country to display his official presidential portrait. He also posted giant billboards bearing propagandistic messages across the country.

22.USAtoday.com Former Philippines president dies at 76 July 31, 2009

23.Wikipedia

24.Wikipedia

25.Resource.org 803 F.2d 993 Ramon AZURIN; Gregorio Araneta, Plaintiffs-Appellees, v.William VON RAAB, in his capacity as Commissioner of Customs of the United States Customs Service, Defendant-Appellant.No. 86-2154.United States Court of Appeals Ninth Circuit. Argued and Submitted Sept. 8, 1986. Decided Oct 26, Accompanying Marcos and his entourage was a second plane loaded with currency, jewelry, and other valuables.

26.Wikipedia

27.Ferdinand Marcos, Ousted Leader of Philippines, Dies at 72 in Exile By Jane Gross, Special to The New York TimesSept 29, 1989

28.Los Angeles Times September 28, 1989Marcos, 72, Dies in Hawaii Exile Aquino Forbids Return of Body to Philippines

29.Findagrave.com Ferdinand Marcos by Donald Greyfield. Ferdinand Marcos' body lies in a glass viewing case inside a refrigerated crypt. He is on perpetual view and a constant entourage of mourners and viewers come to pay their respects including Imelda. His family refuses to bury him until he is allowed full military honors provided by the government.

30.New York Times December 2, 2009 Imelda Marcos Opens Run for Philippine Congress by Carlos H. Conde

31.Foxnews.com 3 wins in Philippine elections mark Marcos family's highest rise since dictator ousted in 1986 May 12, 2010

32.The New York Times Marcos family returning to the limelight in the Philippines By Carlos H. Conde Sunday, July 8, 2007

33.Ibid

Tim Raines

1.Raines diagnosis: Lupus Veteran unlikely to return this year while treatment continues Ross McKeon Of The Examiner Staff SFGate August 7, 1999 04:00 AM

2.sfgate.com

3.Wikipedia Tim Raines

4.smmedia.com Newark Bears Professional Baseball Club Bears and Eagles RiverfrontStadium 450 Broad Street Newark, New Jersey 07102 NewarkBears.com

5.Wikipedia Pittsburgh Drug Trials

6.smmedia.com Newark Bears Professional Baseball Club Bears and Eagles RiverfrontStadium 450 Broad Street Newark, New Jersey 07102

7.The Juice Blog How Tim Raines' Eligibility Opens Bigger Questions on Hall of Fame Inductment: by Scott Long

8.smmedia.com Newark Bears Professional Baseball Club Bears and Eagles RiverfrontStadium 450 Broad Street Newark, New Jersey 07102 NewarkBears.com

Barbara Enright

1.Las Vegas Review Journal YOUNG LAS VEGAS, 1905-1931: BEFORE THE FUTURE FOUND US Sunday, May 01, 2005 City boasted active night life from an early age by Joan Whitely

2.Wikipedia Benny Binion

3.Ibid

4.Ibid

5.pokernews.com News Poker ProfilesWomen's Poker Spotlight: Barbara Enright, Poker Icon June 5, 2008 Tina Bergstrom

6.Wikipedia Barbara Enright

7.Lupus MCT Foundation Famous People With Lupus

8.Wikipedia Barbara Enright

9.Pokercurry.com WSOP Circuit KickoffFriday, October 24th, 2008

10.Wikipedia Barbara Enright

11.Gambling PhD Online Gambling Casinos with Online Casino Reviews Barbara Enright

12.PokerPages.com Barbara Enright

Ray Walston

1.Wikipedia Ray Walston

2.TCM Online

3.Ibid

4.Biography.com

5.Ibid

6.Wikipedia

7.IMDB

8.thestraightdope.com

9.Wikipedia

J.Dilla

1.Wikipedia J. Dilla This film is dedicated to the life and memory of Music Producer J Dilla, aka Jay Dee (James D. Yancey).

2.Wikipedia

3.Wikipedia

4.Wikipedia

5.Rolling Stone Influential Detroit producer worked with Common, D'Angelo,Tribe Alex Msr Posted Feb 13, 2006 12:06 PM

6.Wikipedia

7.Wikipedia

8.Wikipedia

9.Wikipedia

10 Wikipedia

11.Wikipedia

12.About.com J Dilla/Jay Dee By Henry Adaso About.com Guide

13.Ibid

14.Oxford Journals Rheumatology Thrombotic thrombocytopenic purpura insystemic lupus erythematosus: a frequent and severe consequence of active disease V. Majithia and V. Harisdangkul

15.Ibid

16.Wikipedia

17.Wikipedia

**Elaine Paig** e

1.The Times June 7, 2004 Living with lupus the singer Elaine Paige is one of the many who have this baffling disease By Jerome Burne

2.Ibid

3.Ibid

4.Ibid

5.Wikipedia

6.Ibid

7.Wikipedia

8.Mailonline.Elaine's close curtain call by Rebecca Hardy, DAILY Mail May 8, 2004

9.The Times June 7, 2004 Living with lupus the singer Elaine Paige is one of the many who have this baffling disease By Jerome Burne

10.Wikipedia

Part II Lupus Diagnosis

1.LUPUS: The Disease with a Thousand Faces by Jean-Luc Senécal, MD, RCPCCopyright Second Edition 1991, 4th printing 1998 Lupus Canada.

2.Volume 59, Issue 4 Pages 533-552 October 1975 of 31 Bottomorm 1Studies of twins with systemic lupus erythematosus: A review of the literature and presentation of 12 additional sets Top of Form S.R. S.S.R. Block; Lockshin; J.B. Winfield; W.A. D'Angelo; C.L. Christian.

3.Lupus Foundation of America

4.Ibid

5.Wikipedia

6.Ibid

Physiology of lupus

1.Wiley Online Research Article Increased apoptotic neutrophils and macrophages and impaired macrophage phagocytic clearance of apoptotic neutrophils in systemic lupus erythematosusYi Ren1,Jinling Tang2,M. Y. Mok1,Albert W. K. Chan1,Adrian Wu1,C. S. Lau1,*Article first published online: 7 OCT 2003 DOI: 10.1002/art.11237

Also: Nature 407, 784-788 (12 October 2000) doi:10.038/35037722 Corpse clearance defines the meaning of cell deathJohn Savill & Valerie Fadoka1AFF2FN1 Also: Wikipedia

2.Cerebel.com H. Michael Belmont, M.D

History of Lupus

1.nih.gov Generalized Connective Tissue Disease in a Mummy from the Huari Culture (Peru) Marvin J. Allison, PHD, Enrique Gerszten, MD, A. Julio Martinez, MD, and David M. Klurfeld, MS. Department of Pathology Medical College of Virginia Health Sciences Division Virginia Commonwealth University

2.chez.com The History of Lupus Erythematosus X. Sierra, M.D. (Terrassa, Barcelona-Spain)

3.Encyclopedia.org lovetoknow Leprosy

4.Sciencedaily.com Oldest Evidence Of Leprosy Found In India (May 27, 2009)

5.nih.gov The Stigmatization of Leprosy in India and Its Impact on Future Approaches to Elimination and Control Jesse T. Jacob1*and Carlos Franco-Paredes1,2 PLoS Negl Trop Dis.2008 January; 2(1): e113.Published online 2008 January 30. doi: 1371/journal.pntd.

6.Ibid

7.Ibid

8.Sciencedaily.com Oldest Evidence of Leprosy Found in India ScienceDaily

9.Wikipedia

10.factindex.com Syphilis

11.Wikipedia Leprosy

12.Ibid

13.Ibid

14.Ibid

15.univ.paris5 Fr Stigma of Syphilis in the 19th Century France Gérard Tilles M.D.

16.Wikipedia

17.Wikipedia

18.medscape.com The History of LupusAlso: cure4lupus.org History of Lupus

19.nih.gov The history of lupus erythematosus. From Hippocrates to Osler. Smith CD, Cyr M. Department of Medicine, University of Ottawa, Ottawa General Hospital, Canada.

20.medscape.com The History of Lupus Erythematosus Ravi K. Mallavarapu, MD, Edwin W. Grimsley, MD, FACP Department of Internal Medicine, Mercer University School of Medicine, Macon, Georgia

21.ning.com Vampires, Werewolves and Lupus Posted by Angie on October 30, 2010 at 12:00pm Also: Wikipedia

22.Wikipedia

23.univ.paris5 Fr Stigma of Syphilis in the 19th Century France Gérard Tilles M.D.

24.Ibid

25.cure4lupus.org History of Lupus

26.nih.gov The place of William Osler in the description of systemic lupus erythematosus R. Hal Scofield, MD and James C. Oates, MD

27.cure4lupus.org History of Lupus

28.chez.com The History of Lupus Erythematosus X. Sierra, M.D. (Terrassa, Barcelona--Spain)

29.Ibid

30.Ibid

31.wiley.com Fluorescent spot test for antinuclear antibodies by George J. Friou

32.hopkinschildrens.org Children with Lupus Have More Lethal Form of Kidney Disease Ekaterina Pesheva December 10, 2010

33.ispub.com The Internet Journal of Rheumatology Median Survival Rate In Patients With Systemic Lupus Erythematous Relative To Some Demographic Varieties S. Shamsadini & M. R. Shakibi

Lupus treatment

1.lupus.org Lupus Foundation of AmericaExercise, Fatigue and Photosensitivity

2.Ibid

3.Ibid

4.nature.com Past, present and future drug treatment for rheumatoid arthritis and systemic lupus erythematosus Patricia L Mottramaff1 17 June 2003.

5.lupusmd.org Immunosuppressive and Cytotoxic Drugs in the Management of Lupus John H. Klippel, MD Clinical Director, National Institute of Arthritis and Musculoskeletal and Skin Diseases

Prednisone

1.Wikipedia

2.Ibid

3.Ibid

4.jimmunol.org Prednisone inhibits the appearance of inflammatory mediators and the influx of eosinophils and basophils associated with the cutaneous late-phase response to allergen allergen Charlesworth, A Kagey-Sobotka, RP Schleimer, S Norman and LM Lichtenstein Johns Hopkins Asthma and Allergy Center, Division of Clinical Immunology, Baltimore, MD 21224.The Journal of Immunology, Vol 146, Issue 2 671-Copyright © 1991 by American Association of Immunologists

5.Wikipedia

6.Journal of Rheumatology Prednisone, Lupus Activity, and Permanent Organ Damage Mae Thamer, Miguel A. Hernan, YiZhang, Dennis Cotter and Michelle Petri 12/20/2010

7.Stress and Body Shape: Stress-Induced Cortisol Secretion Is Consistently Greater Among Women With Central Fat S. Epel, PHD, Bruce McEwen, PHD, Teresa Seeman, PHD, Karen Matthews, PHD, Grace Castellazzo, RN, BXH, Kelly D. Brownell, PHD, Jennifer Bell, BA, and Jeannette R. Ickovics, PHD

8.ehow.com Normal Cortisol RangeRebecca Laskowitz

9.Wikipedia

10.Ibid

Lupus Overlap

1.The Pemphigus Foundation

2.Senear-Usher Syndrome Pemphigus Erythematosus: Immunofluorescent Studies in a Patient Samuel F. Bean, MD; Francis W. Lynch, MD Arch Dermatol. 1970;101

3.Wikipedia

4.Scleroderma Foundation

5.Med TV

6.Mayo Clinic

7.New England Journal of Medicine 2006

8.Wikipedia

9.Lupus Foundation

Lupus: Associations and Correlations

1.(Epidemiology: November 2010-Volume 21-Issue 6-pp 805-808 Parental. Autoimmune Diseases Associated With Autism Spectrum Disorders in Offspring Keil,Alexandera; Daniels, Julie L.a,b; Forssen, Ullac,d; Hultman, Christinae; Cnattingius, Svenf; Söderberg, Karin C.g; Feychting, Mariad; Sparen, Pare)

2.(Psychiatric News August 21, 2009 Volume 44 Number 16 Page 25 © American Psychiatric Association Clinical & Research News Relationship Found Between Autism and Autoimmune Disorders Jun Yan)

3.(J Child Neurol June 1999 vol. 14 no. 6 388-394 Familial Clustering of Autoimmune Disorders and Evaluation of Medical Risk Factors in Autism Anne M. Comi, MD, Johns Hopkins Hospital, Division of Pediatric Neurology, and Kennedy Krieger Institute, Baltimore, MD , University of Tennessee, Developmental and Genetic Center, Knoxville, TN, Et.al

4.National Institute of Environmental Health Sciences, Epidemiology Branch, MDA3-05, PO Box 12233, Durham, NC27709, USA Biostatistics and Epidemiology Branch, Health Effects Laboratory Division, National Institute for Occupational Safety and Health, Morgantown, West Virginia, 26505, USA Christine G. Parks and Glinda S. Cooper National Institute for Occupational Safety and Health, Biostatistics and Epidemiology Branch, Health Effects Laboratory Division, 1095 Willowdale Road, Morgantown, WV26505, USA

5.From Rheumatology Occupational and Environmental Exposures and Risk of Systemic Lupus Erythematosus: Silica, Sunlight, Solvents Glinda S. Cooper; Joan Wither; Sasha Bernatsky; Jaime O. Claudio; Ann Clarke; John D. Rioux; CaNIOS GenES Investigators; Paul R. Fortin Authors and Disclosures Posted: 01/02/2011; Rheumatology. 2010;49(11):2172-2180. © 2010 Oxford University Press

6.Vol. LXIII, No. 6 NIH RECORD HOME NIH RECORD ARCHIVES NIH HOME PAGE March 18, 2011 Pesticide Use Linked to Lupus, Rheumatoid Arthritis By Jan Ehrman, Dr. Rajesh Ranganathan Dr. Christine G. Parks of NIEHS led a study showing that frequent or extended exposure to pesticides may increase the risk of developing such autoimmune diseases as lupus and rheumatoid arthritis

7.Research Update Cigarette Smoking and Systemic Lupus Erythematosus: A Smoking Gun? Arthritis Foundation-funded researcher Karen H. Costenbader, MD, and Elizabeth W. Karlson, MD, of Brigham and Women's Hospital in Boston, say there are wide variations among the studies that make such an analysis Source: Autoimminuty, Vol. 38, No. 7

8.Epstein-Barr Mayo Clinic DS00115 March 26, 2011©1998-2011 Mayo Foundation for Medical Education and Research (MFMER). All rights reserved

9.2nd Propedeutic Department of Internal Medicine, Hippokration General Hospital, 546 31 Thessaloniki, Greece. Abstract: Parvovirus B19 infection has been associated with a variety of rheumatic manifestations/diseases, mainly rheumatoid arthritis, vasculitis and systemic lupus erythematosus (SLE). PMID: 18549931 [PubMed-indexed for MEDLINE] Review Human parvovirus B19 infection: its relationship with systemic lupus erythematosus. [Semin Arthritis Rheum. 1999]

10.Gabriel J Kaufman, Rita A Sakr, Cyrille Inguenault, Isabelle Sarfati, Claude Nos and Krishna B Clough: _Silicone migration to the contralateral axillary lymph nodes and breast after highly cohesive silicone gel implant failure: a case report._ In: _Cases J_ 2, 2009, 420
Part III

Photosensitivity

1.Maryland Lupus Foundation, Photosensitivity and Lupus Erythematosus By: Denny Tuffanelli, MD Summer 2004

2.Freedom From Lupus By Health Research Today 2007

3.awaradspace.com Prevalence and expression of photosensitivity in systemic lupus erythematosus. Wysenbeek AJ, Block DA, Fries JF., Ann Rheum Dis. 1989 Jun;48(6): Department of Medicine, Stanford University School of Medicine, California

4.awardspace.com P _hotosensitivity in lupus erythematosus, UV photoprovocation_ _results_ compared with history of photosensitivity and clinical findings.Hasan T, Nyberg F, Stephansson E, Puska P, Hakkinen M, Sarna S, Ros AM, Ranki A., Br J Dermatol. 1997 May;136(5):699-705.Department of Dermatology, University of Tampere, Finland.

5.Autoimmune Skin Diseases Journal of Investigative Dermatology SymposiumProceedings (2004) 9,57; doi: 10.1111/j. 1087-0024.2004.00839.x Photosensitivity in Rheumatic Diseases Victoria P Werth, Muhammad Bashir and Wei Zhang

6.dermnetnz.org Photosensitivity New Zealand Dermatological Society Incorporated Dec 18, 2009

7.ada.gov Part 36--Nondiscrimination on the basis of Disability by public Accommodations and in commercial facilities

Sources for glossary and lab tests

1.labtestsonline.org

2.Wikipedia

3.fda, gov

4.medlineplus

5.Medicinenet.com

6.Medical Glossary.org

Appendix

Prednisone And Belly Fat--A Self-perpetuating Cycle

1.Stress an Belly Fat Journal of Psychosomatic Medicine 2003 Elissa S. Epel, PhD, Bruce McEwen, PhD, Teresa Seeman, PhD, Karen Matthews, PhD,Grace Castellazzo, RN, BSN, Kelly D. Brownell, PhD, Jennifer Bell, BA and Jeannette R. Ickovics, PhD From the Health Psychology Program, University of California, San Francisco (E.S.E.), San Francisco, CA; Rockefeller University (B.M.), New York, NY;University ofCalifornia, Los Angeles (T.S.), Los Angeles, CA; University of Pittsburgh (K.M.), Pittsburgh, PA; and Yale University (G.C., K.D.B., J.B., J.R.I.), New Haven, CT.

Cortisol and Insulin Resistance

1.Journal of Hypertension 1991, Hemodynamic Link between Insulin Resistance and Hypertension, Insulin resistance, Stevo Julius, et al)

Cortisol and Essential Hypertension

1.The Journal of Clinical Endocrinology & Metabolism Vol. 88, No. 6 384-92 Copyright © 2003 by The Endocrine Society Hypertension and the Cortisol-Cortisone Shuttle Marcus Quinkler and Paul M. Stewart Division of Medical Sciences, U. of Birmingham, Queen Elizabeth Hospital, Edgbaston, Birmingham B15 2TH, United Kingdom
I would like to thank those listed below for sharing their photos. All images, except pictures of the author's family, are in the public domain and were obtained from Wikimedia Commons.com:

Bundesarchiv, B 145 Bild-F074160-0035 / Wegmann, Ludwig / CC-BY-SA; Cmacauley; Erik of Gothenberg; Rhaworth; Bluemask; Larry D. Moore, Image © 1986; M. Lamar Griffin Sr.; Mitchellandness1; Ebyabe; Volkan Yüksel; Mikael Haggstrom; National Institute of Arthritis and Muscoskeletal Diseases; Dana Weidner; Roar Johansen; National Library of Medicine; Teamsuperawesome; Emmanuelm at en.wikipedia; Mluisamtz11; Nephron; Richard 2902 at en.wikipedia; Cjp24; David M. Jones, geograph.org.uk 913501 Copyright share and share alike; ASA/JPL Caltech; Fastily; Robert Whitaker, Anatomy of an Epidemic: Psychiatric Drugs and the Astonishing Rise of Mental Illness in America in Ethical Human Psychology and Psychiatry; U.S. Federal Information, Annual Statistical Report on the Social Security Disability Insurance Program, 2003; and SSI Annual Statistical Report, 2003; CDC; Gabriel J Kaufman, Rita A Sakr, Cyrille Inguenault, Isabelle Sarfati, Claude Nos and Krishna B Clough: Silicone migration to the contralateral axillary lymph nodes and breast after highly cohesive silicone gel implant failure: a case report. In: Cases J 2, 2009, 420

Photo Credits
Part I

Hannelore Kohl--Attribution: Bundesarchiv, B 145 Bild-F074160-0035 / Wegmann, Ludwig / CC-BY-SA; Wikimedia Commons

Flannery O.Connor--Chapter 4 Cmacauley, Wikimedia Commons

Gothenberg, Sweden–Chapter 2, Erik of Gothenberg, Wikimedia

Paddington, England–Chapter 3 Rhaworth,Wikimedia Commons

Ferdinand Marcos–Bluemask,Wikimedia Commons

Sanford, Florida–Chapter 6, Ebyabe, Wikimedia Commons

Binions Horseshoe Casino–Chapter 7, Image © 1986 Larry D. Moore Used under a Creative Commons ShareAlike License

New Orleans Panoramic–Chapter 8 M. Lamar Griffin Sr., Wikimedia Commons

7 Mile Road Hydrant–Chapter 9 Mitchellandness1,Wikimedia Commons

Lyceum Theater, London–Chapter 10 The Lud, Wikimedia Commons

Michael Jackson's Home–Chapter11, Volkan Yüksel, Wikimedia Commons
Part II

Lupus Diagnosis Chart–Chapter 1, Mikael Haggstrom, Wikimedia Commons

Malar Rash–Chapter 1National Institute of Arthritis and Muscoskeletal Diseases

Apoptotic Cell Chart–Chapter2 Dana Weidner, Wikimedia Commons

Pachacamac Ruins–Chapter 3, Roar Johansen, Wikimedia Commons

Kaposi's and Cazenave's–Chapter 3 National Library of Medicinemmunofluorescence Stain–Chapter 3 Teamsuperawesome, Wikimedia Commons

Pemphigus Stain–Chapter 4 Emmanuelm at en.wikipedia

Image of Methotrexate-Induced Lung Disease–Chapter 5 Mluisamtz11, Wikimedia Commons

Fatty Liver Micrograph–Chapter 6 Nephron, Wikimedia Commons

Figure of Women–Chapter 6 Richard 2902 at en.wikipedia Commons

Pallet of Silica Bags–Chapter 7 Cjp24, Wikimedia Commons
Part III

Kwin Yin geograph.org.uk 913501 David M. Jones Copyright share and share alike Wikimedia Commons

Far-Off Ultra-Violet Light in Space--NASA/JPL Caltech Wikimedia Commons

Blacklight Fluorescent Tubes Chapter 1 Fastily, Wikimedia Commons

Disability Chart\--Page 4 of "Anatomy of an Epidemic: Psychiatric Drugs and the Astonishing Rise of Mental Illness in America" by Robert Whitaker, in Ethical Human Psychology and Psychiatry, Volume 7, Number I, Spring 2005. Original source is U.S. federal information, Annual Statistical Report on the Social Security Disability Insurance Program, 2003; and SSI Annual Statistical Report, 2003. Wikimedia Commons

Senator Edward Kennedy–Chapter 2 Official Portrait, Wikipedia

Jonas Salk–Chapter 3 CDC

