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Liz's Story: My Second Chance

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...

liz devivo and family
Liz Devivo and Family

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.

 

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