Lupus Erythematosus means 'red wolf' in Latin. A French dermatologist gave this name to patients who had a rash on the bridge of the nose and across the cheeks in a mask-like shape, like that on the face of a wolf.
I am one of the 50,000 Canadians known to have lupus erythematosus, an immune system disorder that causes the body to attack itself. It can hit like a ton of bricks, or it can simmer for a long time, whittling away its victim's joy of living.
Although it is more prevalent than muscular dystrophy, multiple sclerosis, and leukemia, many doctors still know little about it. They often refer to lupus as 'the great imposter' because its symptoms mimic other diseases and make it difficult to diagnose. It may begin with vague symptoms that come and go - fever, rash, loss of weight, loss of hair, sensitivity to cold and sunlight, fatigue, or joint aches - or it may start with major system involvement.
No one knows why lupus affects women, nine times more often than men, or why it occurs most frequently between the ages of 20 and 40.
I first got lupus when I was nine years old when The Mickey Mouse Club, crinolines and bobby socks were in vogue, and lupus was considered a rare and fatal disease of the blood. Children who got it usually died. This is my personal story of my ongoing battle with this disease that has so far spanned four decades.
My younger sister, Lynn, and I often were taken for twins since we were the same size although we were a year apart in age, and my mother dressed us alike. During our bi-yearly health checkups, our pediatrician would sit us together on the examining table and stand us side-by-side in the x-ray machine. Eventually he got us confused. He treated me for Lynn's sinus condition, and her for my kidney problem - a mistake which nearly cost me my life.
My kidneys became unable to filter body wastes and fluids. This made me so bloated and drowsy - it was like being slowly poisoned - that I barely could drag myself to school. One day, I put my head down on my desk and drifted into a deep sleep. The next thing I knew, my teacher who was calling me 'lazy' was awakening me.
Although I was hurt by his remark, in retrospect, this incident may have been a blessing in disguise. It led to my mother questioning me a lunchtime when she saw my eyes were puffy from crying. Later that night my parents discussed their fear that there was something very wrong with me. They had noticed a drastic change in me; I was no longer the energetic child they were used to. I had put on a lot of weight quickly, and my feet were so swollen that I could not get my shoes on.
Although I was seeing our family doctor regularly, I was not getting any better, so my parents followed their instincts and found another doctor for me.
Dr. White was a pathologist, nearing 70-years-of-age, who came highly recommended. He seemed to know immediately what was wrong. He pressed a deep indentation in the putty-like skin of my leg, shook a knowing head, and left the room. Within minutes he was back with a colleague of his, a nephrologist, a specialist in kidney disorders
A couple of hours later the results of urine and blood tests confirmed their suspicion that I had nephritis, inflammation of the kidneys. It had caused all my vital organs to become like sponges soaking up bodily fluids. Lupus was not considered at the underlying cause of my kidney trouble at that time.
My mother told me many years later that Dr. White had held very little hope for my survival. He said that "nature will take its course." My parents felt helpless because there was no cure for nephritis, and instead of medication I was given a diet of nothing but par-boiled rice. It was supposed to absorb the excess fluid in my body, and sure enough when I was discharged from hospital three weeks later, I was thirty pounds lighter.
Because I felt well, I was surprised at my doctor's prescription, which was an entire year of bed rest. I had just turned 10, and as a mother now myself, I understand that persuading me to stay in bed was a monumental task for my parents. A year passed and my doctor finally gave in to my pleas to go outside.
One day in July, I convinced my mother to let me accompany friends to a community swimming pool. The mile-long trek in the blistering sun tired us, and we were looking forward to the cooling water. But at the pool, an inhospitable attendant shouted at me, "you've got some nerve expecting me to let you swim here. Go home until that rash clears." The mirror in the change room revealed a mask-like, scaly rawness on the bridge of my nose and across my cheeks. (I now know that this butterfly configuration which itches and burns is a symptom of lupus caused by the sun. It meant that my lupus was not under control.
My parents phoned the doctor immediately. He referred me to a dermatologist who discovered that I was anemic and arranged for me to have a bone marrow test. We went back to the dermatologist's office a few days later to hear the results of the test. He was convinced that my rash and kidney problems were caused by systemic lupus erythematosus.
There was no known treatment for lupus, or information for patients. Dr. White could only hope that in time my body would recover on its own, and he prescribed what he thought best - another year of complete bed rest.
(Long-term bed rest is no longer viewed as the cure-all that it was believed to be in the 1950s and earlier. In fact, 'weightlessness' is a factor in slowing growth and bone development in children.)
The following September I was able to return to school. I was excited but also nervous at the prospect of entering Grade Eight, a long way from Grade Five where I had left off.
I looked so healthy that my family and doctor began to think that I might be over my kidney problems - and as for lupus, it was never mentioned. My parents later said that they thought the diagnosis had been wrong. Nobody mentioned the probability of lupus being in remission, which actually was the case. As for me, now in high school, I simply reveled in the wonderful feeling of being back to normal.
However, soon I began to find the pace of school exhausting. My eyes and ankles were puffy in the morning, symptoms of kidney problems that I tried to conceal from my parents. The thought that I might have to put my life 'on hold' again hung over me like a dark cloud, and I ignored the fact that I was in trouble again until I had no choice. One night, I awoke to find my parents standing over me. They were startled when I opened my eyes, and their reaction made me run to the mirror.
It was clear that my pretense of being well was over - the swollen face with mere slits for eyes signaled kidney problems - the lupus was back! We called Dr. White's office but he was out of town. This turn of fate brought us to meet Dr. Ronald Elliott and, in retrospect, I doubt that I would be here today if I had not. Dr. Elliott became my doctor, friend, and confidante for many years.
After he examined me, he told my mother privately that I was close to death. He said if I were to recover from the near heart failure I was experiencing (from kidney damage), it would be the beginning of a long, difficult road ahead for all of us. Sympathetic to my fear of being hospitalized and away from my family, Dr. Elliott visited me at home instead. In fact, he kept me out of hospital for the next 20 years, although I had several severe flare-ups of lupus to contend with.
Cortisone was hailed as a miracle drug
Dr. Elliott knew about cortisone from current literature, and he read everything he could find on lupus. Rather than 'non-treatment,' his approach was aggressive intervention. He used cortisone, antimalarials and A.C.T.H. hormones, which stimulated the adrenal gland's production of natural cortisone. In the late 1950s, cortisone was being hailed as a miracle drug, but not much was known about the side effects it produced.
I was 13 years old, again feeling well, but embarrassed about a huge and rapid weight gain, a side effect of the medications, and which created what was called 'moon face.' My facial features changed so much that no one who had not seen me for a while recognized me. I was very sensitive about my appearance, which I thought was freakish, and I dreaded having to pass other children on the street because they would make fun of me.
Finally, after a year and a half, I was 'weaned off' cortisone and most of the side effects disappeared. I was well enough to return to high school, graduating without any further interruptions. I had learned to avoid demanding situations whenever I could and I decided to put off university for a few years. Instead I accepted a secretarial position at a company in Hamilton. I entered this stage of my life full of hope.
However, one year later, the joints in my legs and arms were stiff and painful again. My sister, Lynn, had to help me get into and out of my clothes. Although a hot bath and some aspirin in the morning got me through the workday, by early evening I was in agony again. I generally went to bed very early for some relief from the pain.
My hair started falling out too. It was a shock to find clumps of it on my pillow in the morning. Although I did not actually go bald, my normal abundance of long, think hair thinned out to barely cover my scalp. Of course, this completely sabotaged my attempt to hide my illness. I felt I had no choice but to quit my job to recuperate. After just six months, my hair returned to its natural state, and once again I looked the picture of health. I found a new job and returned to work.
In 1970 I met my husband, Ian. When our daughter Jennifer was born in 1973, I was happier than I had ever dreamed I could be. I thanked God for a healthy child, and we felt truly blessed. I knew that I was lucky to have come through this pregnancy without any problems in view of the long-standing and severe episodes of lupus I had endured in the past. I was less fortunate two years later with a second pregnancy.
I had a miscarriage, something that occurs frequently in women who have lupus and who have an anti-coagulant factor in their blood. Such women may also have problems after a successful pregnancy due to hormonal changes. Up until the 1970s, women with lupus, not in remission, were advised against becoming pregnant. (There are now obstetricians who specialize in 'high-risk' pregnancies and specialists in lupus to monitor a woman throughout her pregnancy and afterwards.)
Jennifer was just over two years old when my world began to fall apart. Once again, I became victim to a long and vicious assault of lupus. Physical exhaustion was so great that I had no energy to look after her, and managing my home became an insurmountable task.
I went to bed as early as I could, and got up as late as I could. In between I was a zombie. It was all I could do to get through to 5:30 p.m. when Ian returned from work to take over. He became surrogate mother, cook, cleaner, nurse, as well as father in our home.
Once again I was prescribed high doses of prednisone, a derivative of cortisone, which causes 'cushingoid syndrome,' a factor in the development of early osteoporosis. I was in a catch 22 situation. I needed the prednisone to hold back my immune system's attack on my own cells, but the drug had devastating side effects.
As the months passed and I didn't improve, I feared I might never regain my energy and feel well again. Dr. Elliott knew that my lupus had gotten terribly out of control, and he located a team of specialists at McMaster Medical Centre that was interested in lupus.
I hope I never have to go through anything like that traumatic time from 1975 to 1981 when I was rushed to the Emergency Department time after time in virtually life-threatening situations.
The strain of those stressful years on my husband was evident for years. He carried a heavy load and was too proud, I suppose, to accept 'home care' and he turned down the offer to talk with a social worker or have our daughter counseled. I believe this was a mistake.
In 1978, I heard for the first time about other people in Hamilton who have lupus and I joined with them to become a founding member of The Lupus Society of Hamilton. Later, I served as the society's president and spoke to groups about lupus. As I began to learn more about the disease, I had to come to terms with its full impact: I have an illness with no known cause and for which there is no cure. I have learned much about lupus from experience, from literature and from my doctors, and I know the symptoms that signal a flare-up for me.
I'm often asked, "What is lupus?" to which I say think of your immune system as your personal internal army, constantly on the lookout and ready to fight off foreign invaders (colds, viruses and germs, for example). If you have lupus, your army might mistake the self as foreign and send the troops to attack it. Or it might issue an order to do battle with something that truly is foreign, but continue to send soldiers to the scene long after they are needed. In lupus, this surplus of antibodies causes damage.
Dr. Elliott died in 1989. I had the chance to tell him before he died how much I appreciated having his care and encouragement over the years. He was ahead of his time in understanding the importance of educating patients and making them part of the team treating their illness.
In 1998, I have been in remission for more that 16 years, thanks to the dedicated scientist/clinician who manages my adult lupus. I met Dr. Judah Denburg when he was a resident in the Lupus Clinic at McMaster in 1975, and he has gotten me through several bouts of severe lupus. Over the past 20 years, Dr. Denburg's career interest and research work in lupus has made him a world known expert in the field of central nervous system involvement in lupus. I am fortunate to know firsthand that having a knowledgeable, caring physician, whom you can talk openly with, and who knows your case, is of key importance to keeping well..
I have survived lupus for four decades. I have come through a single doctor's care and the care of a medical team through a time when there was a lack of prescribed medications, to an era where there was an overuse of so-called miracle drugs. You could say I'm living in harmony with an enemy I've learned to respect. I'm aware that lupus is unpredictable and recurrence is a constant threat. But I choose to be optimistic and enjoy life to the fullest, though there are bound to be some hurdles in my path.
Now most of my problems are related to long-term lupus and a result of past use of high doses of prednisone. Osteonecrosis (dead bone) caused by insufficient circulation of blood to the joints in my hips led to bilateral hip replacements a few years ago. Those operations in 1993 and 1994 have enabled me to work full-time, and play golf again.
It seems like a world ago when my only child, who was five years old, asked in a worried voice, "Will I ever get lupus, Mom?" I answered what I hoped and wished for. "You shouldn't worry about that Jennifer; there will be a cure for lupus soon."
Researchers worldwide continue to investigate hormonal, environmental, and viral factors in search of answers. This work has resulted in better management and an improved outcome, and enables people with lupus to lead productive lives. But twenty years later, there is still no cure for lupus.
Today, Jennifer, now a Nurse, so far has not been one of the 5% of offspring who develops lupus. She and husband, Jeff, have three healthy daughters, Lexi, 8 years, and Katelyn, 6 and Kiera, 1 pictured below.
Ian and I have a deeply ingrained fear, from my troubles as a young Mom with lupus because we know that until a cure is found, there is a real and frightening potential for lupus to severely disrupt our family again.
