Shari's Story

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My Life With Scleroderma
January 15, 2001
1961 (Age 20-21)
It all started very simply, one day I was fine and the next I was having terrible swallowing problems, almost at every meal and they most always ended with me throwing up and then the discomfort disappeared until the next time I ate something. I had just begun married life and we were living in very small Grande Prairie, Alberta at the time. I was having a lot of heartburn as well and was self-medicating with Rolaids by the dozen. I was not diagnosed at this time as the doctor there had very few answers for me and thought it was just digestive problems.

1963-1964
We moved to Calgary and I was going on with my life, working and enjoying my life except for the swallowing difficulties that kept plaguing me and also increased heartburn. Trying to find myself a good family doctor was a bit difficult and then I developed large, open ulcers on my fingertips. They hurt so bad even to open and close my hands. I finally was having trouble working with these sores; they would ooze yellow liquid and throb all the time. I went to a doctor just to have them looked at and he informed me at length that I had Raynaud's Phenomenon and that it would most likely occur on my feet as well. There was, at that time, no reference at all that this was a side disease of scleroderma or any other illness. Just that I had to quit smoking as the nicotine constricts the blood vessels and was deadly to Raynaud's patients. He then lanced all my fingertips one by one and wrapped them up in huge bandages and told me to keep them clean and put ointment on them, similar to Polysporin and do not work at my job. Fortunately I have never had sores like these on my feet and after caring for my fingers for two months, the ulcers began to close and clear up but I have such sensitive fingertips now, I have great difficulty performing certain chores, etc. The ulcers have never returned on my hands.

1965 - 1966
Thank the Lord, I finally found a wonderful family doctor and when I went to him with my swallowing problems, the extreme heartburn and the episode with the ulcers, he felt that tests, AND I MEAN TESTS, were warranted, and so it began. Running all over for blood tests, heart tests, lung function tests, arthritis and rheumatism functions. All of these test results came back normal, with no sign of any disorder. My doctor felt we should take further steps and so he had me see an internal specialist and also a gastroenterologist. There again, came numerous tests from both these specialists, mainly for the swallowing difficulties which had escalated and I was beginning to lose weight from no nourishment. The compiled their test results and then had me come in to discuss them. As you can imagine, after all this time I was quite concerned that I had the worst illness ever and the news I received that day, was pretty devastating to me.

1967
I was given the diagnosis of "scleroderma" -(UGH) - the word alone to me conjured up horrible thoughts in my mind. It was explained as a degenerative tissue disease that affected the immune system, making it weak and there was no known cure for it and soberly,. They said 5 years was what I had unless research came up with the cure. My life, at 26 years old, flew before my eyes and I cried and cried with the doctors as they tried to encourage me, that was always hope. Of course, there were medications around to treat the many symptoms of scleroderma but nothing long term.

1968
If I thought I had been ill previously to this diagnosis, I had no idea what was in store for me in the next five to ten years.
I started having bloating sensations in my stomach and I would swell up until I couldn't bend over or barely walk. I went into hospital for these episodes, on and off for 5 years. No explanation was really given to me although the doctors agreed it was my swallowing problems getting worse as well as taking into much air all the time while sitting over my meals for 2 or 3 hours at a time, trying to get something in my stomach. In hospital, I was put on intravenous feeding and all sorts of tests performed but nothing came back unusual and so it continued. Then I had weight problems beginning with losing all the time and my strength was leaving me as well. Eventually, I had to have supplements to my diet, like Ensure, when I could get it down. I now weighed a mere 87 pounds and I was weak and drained and then in the midst all of this, my divorce came in 1978. The stress of it really kicked in the scleroderma and my immune system was barely functioning. This set off three bouts of pneumonia as well.

1978
As a single Mom with 2 children, I faced terrific financial constraints. I wound up in hospital again and more and more tests revealed not much. Most of my vital organs were fine, except the bowel, but I could handle that if I could just get back to eating.

1978 - 1980
A team of doctors finally decided to concentrate on my eating, with whatever drugs were out there. After numerous tries, we hit a few together that started to settle things down inside me. I was told I wouldn't work again and in the shape I was in at the time, I had to believe the doctors. So, I went on welfare to support us and I had a great deal of help and support from my Mom. The weight was very slow at returning and in 1980, doctors decided I should go on a Bougie Tube to help me swallow. In between, I would go into the hospital periodically to have my esophagus stretched open and we all hoped this would help the eating. During this time I used to talk to myself while in bed, sick, I decided I couldn't let this devour me as I was told it probably would. My children were my main drive to keep me hanging in there at the worst times.
The rubber tube they wanted me to use was fitted to my esophagus. I did NOT kept it down there while I tried to eat; it was literally swallowed down my throat to force open the esophagus before I ate. This procedure was horrible, to say the least, but you do what you have to do to stay alive. I was very adamant with the doctors that I would not, now or ever, have a stomach feeding tube inserted in my abdomen surgically.

1980 - 1990
The Bougie Tube is not made here and so it had to be ordered, made for me at a cost of $ 200.00. It had to be replaced with anew one every three years, because there was mercury inside the tube to keep it pliable to work with. The cost was not covered by welfare or Health Care so I was hard pressed, every time it needed to be replaced.
As unpleasant as all this was, I dutifully used that tube whenever I felt I couldn't swallow and many, many times I just threw up everything and then I had to start all over again.
Nevertheless, I kept at it, as I saw it, that tube was all that was keeping me from starving to death and I just was not going to give up and let that happen. So I pressed on, very slowly, every day, and eventually the weight came on and I started feeling stronger each day. I got so exhausted most days just trying to eat but my children were always there for me, and my Mom, and from them came strength to venture on. I was always trying new drugs and techniques that came out. My family doctor then took very good care of me
In 1988, I developed high blood pressure and it was not known if it could be caused from the scleroderma and it still is not known to this day. I take many medications for the blood pressure because it is called "uncontrolled".

1990
The miracle drug, Prepulsid, arrived on the scene and I was put on it immediately. I truly have had great success with this drug. At first it was a gradual improvement and I used my tube as well and my swallowing improved greatly

1995 - 1996
Eventually, I was able to stop using my Bougie Tube altogether and I just relied on the Prepulsid (one pill, four times a day). So the last 8 to 10 years have been heaven for me as far as no eating problems at all.

2000
The worst thing I could think of happening has happened: There have been reported cases of death in people who have been taking the drug Prepulsid. The Scleroderma support group which I belong to is so afraid we will be taken off the drug and what will happen then? Sure enough they stopped manufacturing the drug and it is pulled off the shelves in stores all over the world.
I half expected my swallowing problems to return that very day I took my last Prepulsid. I went to my gastroenterologist and he gave me the only other drug available which is supposed to have somewhat the same effect as Prepulsid but it is not as good. It is called Domperidone; I have been on it now for six months and my problems have not returned. There are many people in the support group who have been switched to it as well and it is NOT helping them at all.
I have been told that scleroderma symptoms can lay dormant, not a remission but just lie under the surface for many years and then they can, and often do, return. I am praying that isn't going to happen to me but it's always on my mind that I could revert back again.

2001
All in all, I consider myself very fortunate to have lived, pretty well, for 33 years with scleroderma. As I approach my 60th birthday, I am developing a few other problems that are related to scleroderma and Raynaud's Phenomenon but my doctors and I are coping as best we can with what's available out there right now.
We living with scleroderma have absolutely no guarantees as to what lies around the bend for us but even living disease-free in our society today, we have no guarantees either.
When I think back to my diagnosis 33 years ago and the 5 to 7 years I was given as a prognosis, I thank God for every day I have been given and for the good doctors who cared that I have had along the way.
In my heart, my most dear hope is that a cure is found very soon for this, oh so mysterious, disease called SCLERODERMA.