Personal Stories

Holly

It took Tri-State member Holly Counihan over two years to receive a scleroderma diagnosis. Like so many others, Holly spent those years in excruciating pain and confusion. In addition to living with scleroderma, Holly is also an avid traveler and adventurer. Read our interview with Holly in the current issue of Scleroderma Exchange. Below you’ll find a short memoir Holly wrote about her journey towards finding a diagnosis.

Holly and her husband Rick in Petra, Jordan.

In August of 1999 I was diagnosed with systemic or ‘defused’ scleroderma.  I was overjoyed to finally have a diagnosis, any diagnosis, as it had been a long road to that moment, including ten misdiagnoses and untold pain and anguish. 

My story begins two years earlier, in early 1996. I began to notice that my hands would occasionally turn blue. I thought it was from holding a cold can of soda or water. Then I began to notice that my lower legs and ankles were turning yellow and the skin was shiny and tight. Next I noticed I could not walk as fast or as long as I had in the past.  My fingers were the size of large, fat sausages, the skin taut and shiny. 

Prior to this point I was a very physically active person and went to the gym at least four times a week. Now I could barely drive myself back and forth to work. My breathing had become more and more difficult. My husband and I are great travelers and adventurers. We had just planned a hunting and fishing trip to South America. As we would be in a malaria-infested area, we took an anti-malaria drug with us. It seemed to me that the only time I felt good on that trip was when I was taking these drugs. My energy level was almost non-existent by the time we returned home. 

One day while standing in front of my computer at work, a co-worker (who was also a nurse) confronted me. She asked me if I knew where I was. Apparently the look on my face was the answer she dreaded: I didn’t know where I was or who she was—and I had known her for 25 years. She called my husband and he came to collect me. After that, I had a difficult time going to work. Thank God I owned the business and my employee was a saint!

My husband and I began the rounds of doctors. First we went to my primary care physician. He thought I was a hypochondriac!  I complained of a constant pain in my left thigh and a pain in my left hip, as well as a shortness of breath. I then went to a teaching hospital in central Connecticut. Their rheumatologist told me I was “fat and lazy”. Next I went to see their infectious disease department. They told me I had Lyme’s Disease. It’s true, I did have it. My vet had diagnosed me a year before. They took so much blood from me that I passed out behind the wheel of my car on the interstate and ended up in a grocery store in a daze. The cashier was able to get me some orange juice and call my husband who once again came and got me. 

Now the trips to the emergency room began. I was having more and more trouble breathing. Back to the central Connecticut hospital emergency room. This time they said I had Syphilis! The rash that I had was symmetrical and the intern thought that Syphilis was the cause. In actuality, the real source of the rash was a reaction to the antibiotics I was taking for an oral infection! This was the lowest point in my life. I remember going into the garage and looking at the gas for the lawn mower. I wanted to drink it and get my life over with. I knew I could not go on. Not much longer.

My next doctor was a Lymes’ expert. He examined me and said that yes, I had contracted it, but it was gone. He thought that since I had been to so many foreign countries I had perhaps contracted some strange and exotic disease.  More blood work. No results. More titers.

I broke down and cried to my best friend Donna. Her husband had been very ill and they had gone to Boston for help. She told me I was not a hypochondriac and that she would ask her husband’s doctor for a referral to a doctor who might be able to help. Next, I went to see Dr. Susan Davidson at Brigham Women’s Hospital in Boston. She asked me to bring my file. I brought all of my test results.  I had 4 or 5 Lymes titers, blood work, stool samples—you name it. She spent 45 minutes reading my file and then entered the consulting room. She was wonderful. She told me that I was “grievously ill” and that my insurance was not taken at her hospital. We discussed the possibility of my having Lyme’s Disease again and she said “no”; but if I was insistent, she would get me an appointment with the man who isolated and discovered the disease! She was encouraging and told me to go to Yale, where my insurance would be taken. 

With the hope given to me by Dr. Davidson, I found a new primary care doctor.  He treated the tobacco workers from the fields and he knew Lymes’ very well. He said that I might have had it, but not anymore. He called Yale, faxed my records, and I was on my way to yet another doctor, more blood work, and more heartbreak. Or so I thought. 

My dear friend Michael took me to my first appointment at Yale.  By this time I was so ill I could not drive. I could not remember the names of things or the names of some of my closest friends. My first appointment at Yale was with the head of Rheumatology, Dr. Linda K. Bockenstedt. She had already read my file. 

She was prepared and she examined me from head to toe. I do remember this.  She asked me if I could walk backwards on my heels. I could not. She asked me if she could put water on my cuticles. She then looked at my fingers. “Umhum,” she said. Then we sat down and talked. She asked me hundreds of questions.  When she was done, she said that she had reviewed my titers for Lymes and that there must be a mistake in the test results. She said no one had as many something-or-others as I did. I said that I had had lots of titers done and that I was sure there were two different labs involved. Sure enough, two different labs provided the same exact results!  More “Umhums” and now she said we needed more blood tests. She also sent me to see the head of infectious disease, Dr. Frank Bia. Dr. Bia asked more questions and took more tests. Only three tubes of blood this time. Thank God for Mike, because of him I didn’t have to drive home. Dr. Bockenstedt said, “I’ll see you in a month”.

I knew that I couldn’t make it that long. I was sure I would be dead by then. It was so difficult to just exist. At this point in my life I was laying on the couch for 20 hours a day, trying to work 4. Even that was too much. I finally stopped working.  After two weeks, I called Dr. Bockenstedt and told her that I couldn’t wait any longer. She made an appointment immediately. When I arrived, I was with my friend Betty. My husband had taken so much time off from work that I had to ask friends to take me to my appointments. I was so grateful to have Betty there, knowing that if the diagnosis was bad I would stop listening somewhere along the line. Betty had her pen and paper ready. When we entered the examination room five students and Dr. Bockenstedt met me. I remember the first part of what she said: “It is our consensus that you have systemic scleroderma.”  I had no idea what that meant, but I was delighted that I had something that someone knew about!  She then explained what it meant in technical terms. She told me what could happen to me and what my life would be like in the future. She mentioned that one of the problems with systemic scleroderma was that it caused, in certain cases, a person to no longer be able to digest their food and the nutrients would not travel into the blood stream and a person could starve to death if they were not fed intravenously. I stopped her. I said, “Dr. B, you don’t know my family or me. My Grandfather, Michael, came from Russia in 1915.  In fact, he walked from Kiev, Ukraine all the way to St. Petersburg, Russia. He then signed up with the Canadian Railway to come to Canada to build the TransCan railway. Why? He was starving to death in the Ukraine. My son is one of the top graduates of the Cordon Bleu in Paris, how could I starve to death?  My Grandfather came to this country to survive. I will too.” I asked her what I needed to do to stay alive. She said she would give me the medicine, told me to “put my affairs in order”, and that if it did not work I would be dead in 6 months. 

It was the longest six months in my life. I did it one day at a time—sometimes, one minute at a time. Little by little, slowly but surely, I got better. It was an infinitesimal recovery: so very slow. I saw Linda once a week, then bi-monthly, then monthly, then every three months and now, 12 years later, twice a year.  She began my recovery with me. I wrote down my questions for her. I researched my medications so that I took them correctly—some with food, some at night, always the correct way. Anything she told me to do, I did it. I did not interpret her instructions; I implemented them. We became partners in my recovery. Yale is a teaching hospital and I am a study. I cannot speak highly enough about the fellows, the staff, and Dr. Bockenstedt. I know in my heart that, had I not arrived at the doors of this institution, I would not be writing this story today.

On the second anniversary of my diagnosis, I was sitting in the departure lounge of Johannesburg International Airport, heading for Mozambique with my husband to hunt wild game in a wild country. Whether or not you agree with hunting is irrelevant. What is relevant is the fact that I was off my couch, breathing and living life. 

When I finish this story, I will switch my computer over to the Internet to continue the plans for my 60th birthday celebration next year in Israel, followed by a month-long trip to South Africa and Namibia.

Have there been bad times? Sure, but nothing brings out the best in life than the threat of having that life removed. Hope is what keeps me alive and hope keeps me going. If I could say one thing, it would be:  “Don’t give up: NEVER, EVER GIVE UP.” 

Sincerely,

Holly F. Counihan

Holly offers her help and encouragement to anyone who wants it. You can email her at hollyfc@cox.net.

Lori, center, with fellow support group members Juanita, left, and “Wil”, right.

Writing Through Scleroderma

Lori Carey lives in Middletown, NY, and is the mother of two children. She began to notice symptoms in 2005 and was diagnosed with scleroderma in 2008.  Lori recently accepted the role of co-support group leader in our Orange County support group. She was kind enough to answer a few of our questions, which we’ve published here along with two of her journal entries and one of her poems.

Scleroderma Exchange: When did you start writing, and why?

Lori Carey: I first started writing when I became ill. They didn’t know what was wrong with me. I had a lot of pent up frustration over not knowing what was happening or why it was happening. Being able to express my thoughts and feelings in my writing provides a never-ending source of comfort for me.

SE: For you, what’s the most difficult aspect of living with scleroderma?

LC: I miss my life – my life as I knew it – the type of life that I will never know again.  Although it was fast paced, stressful, exciting, and at times consuming, it was “my life and scleroderma took it away!”  Although there were good times and bad times, they were my times, spent with people I love and cherish, and they provided me with memories that I’ll never forget. The people you work with become your extended family, and I miss that.

SE: What do you wish more people knew about living with scleroderma?

LC: I think people should realize that living with Scleroderma is no different than having a commonly known disease.  We encounter the same life-altering events. But unfortunately our disease does not receive the same attention.  I’d like the public to understand that although we may not look sick, we are sick. Some of us you can tell, others not.  Please don’t assume that when you see me out and think I look good, that I must feel good, because looks can be, and are, deceiving – especially in my case.

Some of Lori’s journal entries:

(* Note: these dates are almost 5 months after I had to stop working and 11 months after the death of my BFF.    – Lori Carey)

November 16, 2010
It’s now about 1:30AM and the demon that possesses my body has awakened me in pain. I thought of just taking more medicine and falling back to sleep but all I feel like doing is crying myself to sleep. Oh this pain when will you leave me alone!!!!!! I HATE THIS. Is this what Aunt Judy felt????? Oh how I wish she were here. Maybe we could then find some answers as to why we both got this??? The pain is horrific this a.m. Why does this have to happen to me???

It’s now 4:20AM only got 3 hrs more of sleep. Pain in foot and ulcers woke me up. Pain killers bothered my stomach, my legs feel like lead, have a hard time lifting them up just to get into the car. If only J.D. were around to help me through this, he had such a different and positive way of talking to me. I miss him still very much.

November 27, 2010
It’s early, about 4:30 AM. Had to get up because I couldn’t stand the pain anymore. I think sometimes they see my pain and despair – like cousin John did the other day, but then he dropped out of site, busy I guess with his own life … when I needed him in mine.

Yes, people, you’re right when you say, “What could I do?” A simple talk sitting around friends would be nice. But we all know once you get sick people want nothing to do with you. Oh I wish I could cry out in pain it hurts so much! Scleroderma sucks!!!

December 4, 2010
While sitting I was rubbing my neck and felt indentations in the back. When I pulled skin back I felt something change in my throat. Having trouble breathing but will not say anything because I don’t want to hear it. No compassion, no talking to me about it. No do = no this = no that. I’m 48 years old and have this terrible disease. Why doesn’t god help me????? I hate this existence and would like some happiness and joy.

“A Dream This is Not”
by Lori Carey

As this disease attempts to overtake
My mind, body, and soul,
I intend to fight everything it sends my way.

Although I may not have the same
strength or endurance as others,
what I do have cannot be measured.

For it is a light inside me, fed from a source up above,
from the one that will be waiting for me
at the gates with opened arms,  “Welcome home.”

My love,
I’ve missed you, your smile
and those captivating blue eyes –

“Ditto.”

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).