Medical experiences: Scleroderma...

[fajrdrako]

Some years ago walkingowl gave me The Scleroderma Book: A Guide for Patients and Families by Maureen D. Mayes. I'd been avoiding reading it, on the grounds that ignorance is bliss: I've been living with scleroderma since I was four years old, and I was afraid to hear the medical details about it.

Time for some attack-escape. The only way to deal with fear is by facing it.

Scleroderma is a disease that causes scarring on the body - or at least, that's what I would have said before reading this book. Having read it, I can tell you more specifically that scleroderma is an autoimmune connective tissue disease affecting blood vessels and collagen. The body starts building scar tissue on healthy skin and doesn't know where to stop.

I don't remember when I first came down with scleroderma symptoms, though there are pictures of me at three looking sturdy and normal, so it must have been soon after that. I know I had it by four, because that's when the scars became visible - "scars" is what I've always called them, though it appears that isn't the proper medical term, which would be morphea or fibrosis. From that point I saw a lot of doctors, none of which could do anything about it - the treatment I got was for the scars to be rubbed nightly with vitamin E cream. I disliked it, because the cream was sticky and smelled bad, but it felt pretty good and I enjoyed the time with my parents. I had a bit of a medical phobia already, and I hated being poked and prodded and photographed by the doctors, who were not unkind, but who were more curious than sympathetic. I don't know when the diagnosis was made, or how; I think perhaps they didn't want to tell me, because the diagnosis came with the warning to my parents that I wouldn't live past seventeen. Not knowing this, I blithely lived on anyway, despite being extremely underweight and often ill. And the word "scleroderma" stuck in my brain at some point, as the probable name for my mysterious scars.

My parents handled it philosophically. "Everyone has something," my mother told me, and I believe that to this day. Not necessarily a disease, but an oddity, a handicap, a problem. You don't get through life without problems.

I didn't know until I was in my forties that when I was six, the doctors told my parents I'd die in my teens. "That must have worried you," I said, when my father told me.

"Didn't worry us at all," he said. "We just didn't believe them."

Smart people, my parents.

Scleroderma is said to be rare, but according to Wikipedia, it affects one person in 1,000. That isn't so rare. Further web-browsing shows that this statistic is for all types of scleroderma or related conditions; systemic scleroderma is more like 1 in 10,000, but no one seems sure. They aren't sure whether the incidence of scleroderma is rising, or just that there are more diagnoses than there used to be. Sometimes it's benign, sometimes it's fatal. Sometimes it's painful and bothersome, sometimes it's not. Sometimes it goes away after it appears, sometimes it doesn't. The closer I look, the more it all looks like total medical guesswork.

There are two main types of scleroderma: localized and systemic. I had heard about this and assumed I had the localized kind, because that's the kind that usually affects children. But no: turns out that with localized scleroderma, the scarring only affects one limb, and when the disease runs its course, the scarring goes away. My scarring never went away. It was at its worst when I was about eight, and about ninety percent of the scarring remains. Scarring marked my left arm and shoulder, part of my torso, the outer side of my left hip, leg, and foot and my right hip and thigh. It didn't affect just the skin, but the tissue under the skin, and the bone under that. It all makes me a little lop-sided, but I like to think that isn't generally obvious to onlookers.

So reading this book, it seems I have the worst kind of scleroderma - diffuse systemic. But my condition doesn't seem to be typical of systemic scleroderma, though it (not surprisingly) explains why I spent most of my chidlhood coming down with every virus in sight - my autoimmune system must have been a mess. Being tired all the time, lacking normal energy, needing more sleep than most people - yes, that's there. Being cold all the time is part of the condition, though I don't think I have Renaud's syndrome or the finger lesions - usually the first symptom, and 98% of cases have it. I do frequently get tingling in my fingers, though, as if there's a circulation problem. I don't have Sjogren's Syndrome, or sexual troubles, though I suspect it has affected my reproductive system. Over the past five years I've been suffering from esophageal reflux, which is another typical symptom, and it seems that the problems I had with candidiasis a decade ago are identical to typical scleroderma problems - including depression. My inexplicable high blood pressure, which didn't respond to diet control, is part of the pattern

Othe whole, I feel pretty good. So far, touch wood, none of the symptoms have been life-threatening, or resistent to treatment. In many ways I think I'm generally healthier than most people I know. I've had to work at it - careful about diet, doing yoga, exercising, making sure I get as much sleep as possible - but it's mostly paid off.

When I was in my twenties, a yoga teacher said she could make my scars go away with yoga. I turned down her offer, not because I thought she couldn't do it, but because I thought it would involve more effort, time and focus than I was ready to give it. I was a student at the time; life was busy enough. I also thought that the problem was mainly cosmetic, and wanting rid of them was sheer vanity. I don't believe that now - ever since an acupuncturist pointed out that the scars blocked the flow of chi. And since I realized that some of my ongoing problems are part of the disease.

Yoga should help even now.

The book didn't say much that I hadn't guessed already: that my main lifelong health abnormalities (like fatigue) are due to scleroderma, that there's no known cause or cure, and that it's difficult to pin down the effects of the disease, which are varied - and clearly my case isn't the most typical. Or the best. Or the worst. Just another example of a rather bizarre disease.

A character in the novel The Keep by F. Paul Wilson had scleroderma - the only time I've come across it in fiction.