Personal Stories

Amy Gagner with her son and husband

My Life With Scleroderma

By Amy Gagner

December 1997 was the month and the year that changed my life forever. I was thirty-seven years old and I thought I was as healthy as could be. The first hint that something was wrong was when my mother-in-law Erika stared at my legs and asked me why my legs were so shiny and swollen. I started wondering myself; I had noticed I really hadn’t been feeling well. I called my primary doctor and made an appointment. During my visit, I told him what my symptoms were and he immediately put me on water pills. I took the water pills for about a week. The symptoms seemed to go away, but then came back shortly after. I then asked my primary what was wrong with me; but he had no answers for me. We were both baffled and not sure what was wrong. He sent me to a rheumatologist that was in the same group. By the time I saw the rheumatologist it was April 30, 1998, it had taken four months for him to see me, due to the overwhelming amount of patients he had. By the time I saw him my legs had swelled up to the size of tree stumps and were very hard. This also made it very difficult, almost impossible to walk. I had to wear slippers to keep the appointment. By this time it had affected my hands, arms, elbows, and face, which were now very hard as well. The doctor gave me a full examination; he ordered a bunch of tests. He also took an x-ray of my hands because they were curled up to the palms. Both of my doctors consulted one another that very day, and diagnosed me with Scleroderma. At that time I had no idea what Scleroderma was. The rheumatologist told me to get dressed and to meet him back in his office. He then proceeded to tell me that I was going to be sick of him and seeing his face. I started to laugh not sure at that point, if I should have laughed or cried. During my time at his office he said I would have to come back three times a week. He wanted to keep a close watch on me.

I left his office about three hours later; I sat in my car and cried my eyes out. I didn’t know how I was going to tell my family, I really didn’t know what to tell them.   It took me a good ten minutes to gather my thoughts, just so I could drive home. Thoughts were going through my head of what I would say to my family. When I got home I had to break the news to my husband Peter and son Derrick. Then I proceeded to call my father and broke the news to him as well. Next on my list were my two sisters and brother. When they all found out they were completely and utterly devastated. I then had to tell my mother, which was the hardest thing I had to do. My mother who had had a stroke at the age of sixty-seven was in a nursing home. I asked my sister Donna to please come with me, so we could tell mom together.  When I told my mother she cried, but I don’t think she really understood.

My doctor then told me that I would have to take an injection once a week, which I ended up taking for the three years. The injected drug was called Methotrexate. Methotrexate played a huge role in the success of softening up my skin. To this day some of my skin is still tight, especially my hands. In the beginning I had no flexibility in my hands at all. I found I had a difficult time doing things I once took for granted like driving, holding things, tying my shoes, holding a cup, holding a fork, just to name a few. Now those tasks were next to impossible. I was forced to drive with the palm of my hands using a rubber finger so I could grip the wheel. I found out that Scleroderma can take away your independence. The disease was making me more and more handicapped. My doctor wanted me to see a Scleroderma specialist in New Brunswick, New Jersey just to confirm that I did in fact definitely have scleroderma.

At this point I was working full time and still trying to deal with this disease. It was very hard for me to do my job. I was a computer biller, and had to use my hands all the time. I had to type on the computer and input numbers, which slowed me down since I was used to typing fast. During the first year and a half, I really didn’t know what was going to happen to me. I was very sick and not sure if I was going to make it. I was also in and out of several hospitals during that time.    Around June 2002 I started to get more and more tired.  I noticed that I was out of breath a lot. That month was very rough for me. One Saturday, I was at my father’s house and sat down on his couch, because I felt as if I was getting worse. I couldn’t get any air into my lungs. My father, who had oxygen in the house actually had to give me some. At that point he called 9-1-1 because I actually couldn’t breathe at all. They rushed me to the hospital that Saturday afternoon.  They ran more tests, and then they sent me home later that night. The next business day I went back to my rheumatologist, and he then referred me to the pulmonary doctor in his group.

The "A" Team, Amy's family, at the Northern NJ walk, 2007

The pulmonologist ordered a CAT scan of my lungs to see what was wrong. He said he thought the Scleroderma had gone into my lungs. He also ordered a biopsy of my lungs. The test results came back and showed that both lungs were scarred. The right lung was worse than the left. I had to stay in the hospital for four days. From that point on I have had to sleep with oxygen and also when I’m active. I have to nebulize three to four times a day and use my inhaler to help open up my lungs. My pulmonary doctor decided in December 2001 to order a test to see if there was any hypertension in my four arteries; the test came back clear. Thank God, that’s all I would have needed. I wasn’t so lucky in November 2005 because I was diagnosed with Pulmonary Hypertension in my lungs, on top of having Pulmonary Fibrosis. In November 2002 I left work on disability. I’m currently involved with a local Pulmonary Arterial Hypertension (PAH) Support Group. I also attend Tri-State’s educational seminars in New York City when I can. My large family has attended the Tri-State walkathon in Leonia, New Jersey since 2006.  I cannot walk myself but, watching them walk for me has meant so much!

As a young woman my passion and hobby for needlepoint was taken away from me, when I got this disease. Then in 2007, I started to needlepoint again!  It does hurt my fingers, but I look at it this way, it is good therapy for me. I just finished making needlepoint letters for my family, friends and for the hospital I currently go for tests. My disease has affected my family but also in a good way. My son Derrick especially has changed for the best. He has learned to not let anything stop him from living his dreams. One of his quotes that he loves to use is “The Tassel Was Worth the Hassle”. With this great attitude, he was Class President two years in a row and he also graduated Class Valedictorian from his high school in 2007. I was so proud to hear my son give his Valedictorian speech and so glad to see him graduate! He is now attending college and doing very well.

I have used my mother as an example to push myself. I have it in my head that I’m not going to give up. I am a wife and a mother. I have so much going for me; my family has been behind me 100%. After this whole ordeal I have told myself over and over again, not to give up. I have always kept a positive attitude, even to this day. I believe with any situation you must keep a positive attitude to keep going and to move forward. After all of this, I still have a smile on my face. I hope after reading this, you will also be inspired to never give up. Take it from me it can be done.  I have done it and so can you!

Amy Gagner is a Scleroderma survivor who lives in Lodi, New Jersey with her husband Peter and son Derrick, who both appear with her in the accompanying photo taken at Derrick’s graduation. The other photo is Amy’s family of walkers at the 2007 Leonia, NJ Scleroderma Walk.

Liz Devivo and Family

My Second Chance

by Liz DeVivo

The first indicator of scleroderma for me was Raynaud's when I was 30 years old and pregnant with my second child in 1996. At that time, I was diagnosed with mixed connective tissue disease, since there were no other symptoms other than an elevated ANA level.

The next few years brought a few more changes, including Telangiectasia, and changes to the skin on my hands and face. By 1999, I was officially diagnosed with CREST, and as I understood it from my doctor and all the literature I could get my hands on, it was the relatively mild form of scleroderma.

I was thankful to have the ‘milder’ form of the disease. I did not feel that any of the symptoms where hindering me in any sort of way either, in fact I finished two masters degrees during that time.

My rheumatologist suggested I take some tests to monitor all the internal processes that could be affected by the scleroderma, to create a baseline to monitor over time. In February of 2000, all of my tests (echo, pft’s, swallowing tests) came out normal.

By May of 2000, I found myself short of breath, having tremendous difficulty even negotiating the front steps to my home (I blamed myself for being lazy and not doing enough exercise.)

Further tests showed my blood pressure had sky rocketed. An x-ray showed that my heart was enlarged and surrounded by fluid.

They removed the fluid from me, and reassured me that they believed it was a one-time event.

Unfortunately it wasn’t, the pericarditis, began again, and this time they were able to confirm that it was in fact Pulmonary Hypertension.

The Pulmonary Hypertension was severe; oddly enough it did not progress as it usually does in most cases.

Four months later I started Flolan, an IV therapy that is helpful to many patients with Pulmonary Hypertension.

Unfortunately it did not slow the progression of the PH in me. My heart was failing rapidly. I had three more events with fluid around my heart and finally I had heart surgery where they opened a pericardial window, in an effort to enable the fluid to drain out on its own.

The next year brought more treatments and hospitalizations, including IV cytoxyn to stop the hardening of my lungs, and Viagra for my PH, but the deterioration continued. I knew transplantation had become my only hope.

Officially, the journey for my transplant started at Mount Sinai hospital in New York City where my pulmonary hypertension specialist referred me to their transplant program.

I went to meet with the Dr. for an initial interview.

He listened to my medical history while I coughed and coughed, unable to catch my breath. He told me right then and there that I was not a candidate given my history of scleroderma and my current symptoms. As he listed all the reasons why I wasn’t a viable candidate, the tears ran down my face. By the time I shook his hand, I had my next plan unraveling.

I went home and spoke with my sister-in-law, who had already been busy calling for information at other transplant centers.

At that time Pittsburgh, Texas and California where the only places that would even consider me as a candidate, given my scleroderma. (You see people with incurable diseases are not generally considered ideal transplant candidates for obvious reasons—I guess) at any rate, I set up an evaluation with Pittsburgh.

When I was evaluated in August, Pittsburgh determined I needed two lungs. At that time, they were concerned about my weight and wanted to try another medication for my heart failure. When I returned a few months later, my status was changed. Apparently the damage that occurred to my heart in the few months had been severe. They no longer thought that the lung transplant would be enough to heal the heart, now I needed a heart as well.

When I returned to the hospital, they set me up with another IV medication for the heart failure and insisted that I begin stomach tube feedings. I agreed to the IV, but asked to wait on the stomach tube.

I cried, I could not bear the thought of another tube coming out of my body. How could I parent (my girls who were 5 and 8 at that time) physically with tubes and cords all getting tangled up all around me, all the time? I begged the dr. to let me try to gain weight on my own. I was weighing in at 98 pounds with fluid. He shook his head told me not too hold out too much hope, he had seen many others before me that were unable to keep the weight on and then became too ill for transplant. He was clear with me, their objective while I waited was to simply keep me well enough to transplant. He then suggested that I stay in the hospital till the transplant. I couldn’t do it, not yet anyway. It was a tortuous decision. I went home. I knew I needed to be home with my family and take care of my children, even though I was spending most of my days in bed, I knew I could not survive the wait in a hospital bed. I needed to see my daughters’ smiling faces everyday in order to have the will and strength to hang on.

I waited, and waited and waited. The physical, emotional and financial toll was immense on my family. I was fortunate and blessed then and now, and with support from family, friends, neighbors, doctors, nurses, the Scleroderma Foundation, transplant support groups, and complete strangers, I was able to hold on through the wait.

On July 29, 2003, 23 months and one false alarm later, I received my call, the real deal. My phone rang at 4:45 that morning and when my transplant coordinator offered me my heart and lungs, I was never so happy or at peace.

There was no fear that day, (the 23 months prior brought plenty of fear and anxiety, but on that particular day there was no fear—zero, just joy that just perhaps, I could have a life again.

We flew to Pittsburgh (I live in upstate N.Y.) and that day I received the most extraordinary gift anybody could ever give another—the gift of life.

The surgery was not without complications, and my hospitalization was longer than usual (four months). I also stayed in Pittsburgh at their family house for six weeks more before I was allowed to return home. If this all sounds challenging, difficult and downright tortuous it’s because it was all those things and more. But when compared to the alternative…

The reality was that it took many months before I could honestly say to myself, I feel better.

Every day since then gets better and better. I can’t believe how good life can be. I have no physical limitations. My scleroderma is milder than it ever has been; in fact my rheumatologist said at my last visit that if he didn’t know that I had scleroderma, he would never be able to tell from my physical examination. My hands are no longer curved and swollen, my circulation is much better, the Raynaud's is not gone, but much more manageable and my skin is not nearly as tight, (I can wear rings again), and most importantly, my pulmonary pressures are in the normal range!

I was blessed on the day of my transplants and every day since then, by a miraculous gift given to me by a complete stranger. I just am thrilled to be able to take care of my children, my family, friends, and myself.

And I can say without an ounce of hesitation or doubt, that it I would do it all again in a heart beat.

You can read more about my transplant journey at www.Lizfund.org (a website that was set up for me to help fundraise for the transplant).

The Gift Of Scleroderma

Betzi Sheff
Cooperstown Scleroderma Support Group

I know what you’re thinking! How can this terrible disease be considered a gift?

Please hear me out; as I think we forget some of the people we have met and things we have done on this journey with Scleroderma

When I first was diagnosed or should I say, finally diagnosed with Scleroderma, I like thousands had no inkling of what I was in for. I had never heard of Scleroderma and when the doctors presented it to me, I wish I had never heard of it! I was scared silly and thought I should start getting my life in order as soon as possible. I went from book to book and what I read was extremely scary. We’ve come a long way!

When I started the support group in Cooperstown with 2 other gals from Cooperstown, I had no idea who would come and who I would meet. I can say that our group is like a second family to me. I feel comfortable discussing Scleroderma and really enjoy meeting new people. Our primary goal is to get newly diagnosed patients through that first year of all the unknowns with this disease. It’s amazing when a new patient comes for the first time, and as time goes by, they start to relax and realize we’re all in the same boat together.

Another gift I have received by starting a support group is that I have come to grips with my diagnosis. For years, I pretty much ignored it and didn’t go for regular check ups. I felt I wasn’t symptomatic, so why go? My new friends in the group convinced me that I needed to see my rheumatologist on a regular basis and follow up with any tests that I might need. Knowledge is key and there are so many new treatments out there and more everyday. It’s an exciting time as all the research is really paying off.

The best gift is that I’m not afraid anymore. I have Scleroderma but it doesn’t have me. I will live my life to the fullest everyday and take time to appreciate how lucky I am. It has become a passion of mine. To seek out people who need help and to do my level best to help them understand the disease and to live the best they can with it.

We all have something to deal with in life. Scleroderma is my something. I wouldn’t have met as many special people with it and get to know their loved ones. There is nothing sweeter than helping someone get over those first few months of fear and see him or her evolve through the process. It empowers me and I am truly lucky!

Betzi Sheff is our SF Tri-State Support Group Leader for Cooperstown that meets at Bassett Health Care Facility in Cooperstown, N.Y.

Go to top.