My Scleroderma story begins in the early fall of 2004. I started experiencing some stiffness in my hips and legs and some very slight swelling in my fingers. I had been taking HRT for several years and had decided to go off of it and at the same time my cholesterol level was a bit high so my doctor had started me on Lipitor. By October I was starting to experience quite astonishing spells of Raynaud's. I had no pain and it was quite amazing to watch my hands lose all colour and resemble white candlesticks rather than fingers. I thought that perhaps this change in medications was the reason. My doctor didn't think so but after a few months I decided to find out by stopping the Lipitor and going back on HRT. Unfortunately, the symptoms didn't cease.
At that time I was working full time, usually 50 hours a week, in a demanding and exciting job. Additionally I volunteered every Friday night at our local hospice providing palliative care to our residents. Life was progressing as planned, and in my late fifties I was thinking ahead to the many things I would do upon retirement. In between these activities I was either out digging up and planting new gardens or painting and decorating my house. With many good friends and visits out of town to see family I certainly didn't have time for illness. In fact, I probably had taken 2 or 3 days off due to illness during the past 20 years.
The swelling continued but now in addition to my fingers, my feet and legs were getting larger daily. At this point I still had no pain but was experiencing increasing stiffness. Rising from a sitting position, getting in and out of a car, getting up in the morning and even lifting my leg over the side of the bathtub to get in the shower was increasingly difficult. By November I was starting to experience some breathlessness. And it all came to a head for me the day I took my box of recyclables to the curb and felt so breathless and weak I wasn't sure I could make it back to my house - approximately 40 steps. Although I had contacted my doctor when the symptoms began, this was really the beginning of a closer relationship with him. Previously I saw him once a year for my annual check-up and only in between if I needed to get an antibiotic or something. Now I was seeing him every 2-3 weeks.
In late December I was working a shift at the hospice and had to sit for a minute as I was so breathless. The on-duty nurse noticed my swollen feet (at this point I was having to wear shoes one size larger, and when I could get away with it I only wore some large, soft boots) and said that combined with my breathlessness this could be very serious and I should go to ER. I didn't do so at that time, however, on December 27th as I began vacuuming my living room breathing became very laboured and I felt as though my heart was beating so hard it would explode. At that point I got in my car and drove to the ER. They kept me until late that night doing all sorts of tests including a CT scan with dye as they thought I might have a blood clot in my lung. They released me with a clean bill of health but some water pills to reduce the swelling. So, although I knew I wasn't actually ready to die at that minute, I also knew I wasn't well and this left me feeling quite frustrated.
My doctor was quite tenacious, continuing to order other blood and urine tests, but all reports showed me as healthy. He told me that although the tests were not showing anything he knew from my history that this was something significant. I had not been in the habit in the past of calling him for any minor health issue that arose. He sent me to an Internal Medicine specialist and that doctor also ran lots of blood and urine tests and declared me well but with “a tiny amount of inflammation, which is not unusual for someone your age”. I asked how he would explain my swollen hands and feet and my breathlessness and he couldn't. So he then sent me for a pulmonary function test and an echocardiogram and both were normal.
So now it was early spring and at this point my family doctor thought that perhaps what I had was Lymphedema. He sent me for an ultrasound of my legs to check that there were no clots and of course there weren't, so then I began a series of massages specifically for lymphedema. It was at the third session that the masseuse pointed to a diagram on her wall of the lymphatic system. She informed me that in her experience patients did not present with all four limbs involved and showed me in the diagram why that was the case. So it was back to the drawing board!
Meanwhile, at work I was incredibly busy, juggling several high profile projects with very large budgets. I needed to be at my best but I wasn't sleeping (as now I was experiencing pain as well as stiffness), I felt really awful all the time and so was stressed to the maximum. One day I walked to the cafeteria, probably two hundred steps or so from my office and once there I didn't feel I had the strength to walk back. A colleague spotting me holding on to a railing and knew by looking at me something was wrong. She helped me back to my desk and from then on I only left my office when it was necessary to attend important meetings in other parts of the building.
By now it was the first week of May. My doctor had received back yet another series of blood tests and this one showed a slightly increased ANA. That was the first time I heard the word Scleroderma. He said that he had not seen a lot of cases but this test made him think that it might be so. He put me on 50mg of prednisone for 10 days to see if it would help. Help? Why I felt like Wonder Woman! The swelling went down, the pain disappeared, and I had energy to burn and figured that this was a miracle drug that I would like to take forever. I had out of town visitors for the last weekend I was on the drug and we had a great time as I really felt well and on top of my game. Then I finished the 10 days. The first few days off the drug I felt fine, almost as though a cure had taken place. By the third day I was surprised to feel just somewhat under the weather and blamed it on allergies. On the fourth day I was in an all-day important business event which had been planned for months. As I sat in the room I could feel myself coming down with something like a bad cold or flu. I was getting very warm and I could actually feel my body swelling. By the end of the meeting the buttons on my blouse were strained because I had swollen so much. One of my colleagues leaned over to me mid-way through and asked if I was alright. I realized once the event was over why she had asked; my face was so swollen you would have thought I had been stung by a swarm of bees.
The next day I decided to work from home as this was an acceptable practice with my employer. It was the Friday before the long weekend in May and I had planned to take the following week off so needed to get this work done and off to my colleagues. I managed what I could but then, with my work unfinished, I crawled into bed and stayed there until mid-Saturday. My sister called from Ottawa to chat but I told her I was too sick to do so. At this point I thought perhaps I had pneumonia. I had tried to shower but had so little strength that I got out of the shower and got right back into bed just barely dried off. Sunday I was still in bed when my sister arrived. She was quite concerned after speaking with me so decided to drive down. She got me dressed and being the long weekend with no doctor available she took me in to ER. The kept me for a week. They ran all sorts of tests, blood and urine, x-rays, ultrasounds of my other organs, and even an arterial blood gas. Almost everything was “normal” except for a CT scan with showed something going on in my lungs - ground glass opacities which indicate alveolitis and pulmonary fibrosis. I was just beginning to show some skin thickening so they said that yes, it probably was scleroderma and advised me to get a referral to the Firestone Clinic at St. Joseph's Hospital in Hamilton for my lungs.
Of course, by this time I had done some research on Scleroderma and didn't like what I was finding out. So, hoping to overturn the diagnosis I made a chart. First, using my symptoms I searched the Internet for any diseases that matched. Then I weeded some out because other symptoms did not match. I then listed in columns all the symptoms I could find from various sources for scleroderma, lupus, rheumatoid arthritis, lymphedema, polymyositis, myxedema, and mixed connective tissue disease. Unfortunately, I had many more “x”s in the Scleroderma column than the others.
Between that May and the following September and my first visit to the Firestone Clinic my disease had progressed to the point where it was very evident I had scleroderma. Although on prednisone again I was experiencing shortness of breath at the least physical activity and my skin had tightened to the point that every time I gave my health card I received comments that I did not look my age. That would have been great for the ego except that I knew the real reason for it. I just kept that as my little joke. Unfortunately, I never did experience that initial elation I felt on the prednisone.
So, as I write this it is summer of 2008. I've been through the first year when I felt pain and discomfort, followed by what resembled a flu that wouldn't leave for seven months and resulted in a depression. I think that although some of that terrible feeling was due to the disease, some I am convinced was due to a medication that I was on that did not agree with me. When it started to cause problems with my liver I was taken off and I started to feel better. I then began a 17 month period of another medication, a mild chemotherapy which I had feared due to the possible side effects. However, I felt really well while taking this drug and it seems to have slowed the progress of my lungs. Hopefully, the effect will be long-lasting.
At present, I am in kidney failure. It's a question of which came first - the uncontrollable high blood pressure which led to kidney failure, or, the kidney failure which led to uncontrollable high blood pressure. I've been managing to keep dialysis at bay but with less than 12% of my kidney function I'm on borrowed time. A year and a half ago, in December 2006, I was prescribed oxygen full-time as my oxygen saturation levels and diffusion capacity are quite low. It's a mixed blessing. No one wants to be tethered to a tank wherever they go, however, without it I wouldn't be going anywhere.
We really don't know what life holds for us. I look around and feel fortunate as it could be much worse. I'm still able to live independently and have a wide support network. I have a wonderful family doctor whom I see monthly. We review my monthly blood tests and see how my haemoglobin and kidneys are faring and just really touch base. I know there is nothing else he can do for me but I'm so thankful to have him in my corner. Being able to talk to him helps me maintain my stability, especially when I'm feeling emotional. I see several specialists on a regular basis, all of whom I have complete confidence in. They are interested in and take care of the effects of this disease on various organs. But it is my family doctor that provides the emotional support that I also need and perhaps is most important to keep the total “me” going.
I haven't been able to return to my career since that long weekend in May 2005. But perhaps all of those years at work have prepared me to take on other challenges, so I'll do what I can as long as I'm able. The next milestone for me may be a lung and kidney transplant. I'm just starting my round of appointments to explore this and although I'm not ready yet I'd like to think if I get to that point it may be an option. So for now, I'm just trying to take care of myself as best I can, and keep on keeping on!
