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Lori: Surviving Daughter of Father who had Scleroderma

A Tribute to My Dad, Leo

Do you ever wonder what makes a good dad? Well, let me tell you about mine. Since the time I was a very little girl, I thought my dad was the greatest. He would play with me whenever I asked, whether it be a board game, baseball, fishing or anything. One of the things I remember most was when he would fly me around like an airplane. He lay on his back with his feet in the air, and my little body on top of his legs and he would 'fly' me and my brother around. I remember it being so much fun!

As we got older, my brother and I were involved in many sports and each time we looked up into the stands, there he was, even when it was an away game. We were not the best athletes, but we tried hard and he never criticized us whatsoever. He just gave us the encouragement we needed. He continued to follow this pattern with his four grandchildren: Ryan, Jeff, Allison and Nicole. They loved him so very much and he just adored them all, for they were his pride and joy! He was such a devoted, active participant in their lives as a 'Papa' as he was a father to myself and my brother, Jim and as husband to my mother, Carol.

My dad also was a very hard worker. He worked at Texas Instruments for thirty- nine years, and retired almost two years ago now. Little did he know, he would never get to enjoy it. He did not even live five months past his sixtieth birthday.

Now, let me tell you about his history with the horrible disease, scleroderma. He began to notice symptoms in 1989, the year my brother and I both got married. He was working in the yard one day, pulling up weeds and when he came in the house, he complained of his hands being puffy but he thought it was from pulling the weeds and such. When the puffiness did not go away, he went to his doctor. His doctor said he might have Raynaud's phenomenon, but never gave him any information regarding scleroderma and how this could be related.

He continued on, not worrying too much about his hands, and we made it through the weddings in June and then again in October. We had made plans to go to Atlantic City that April, but we never got there. My father had hypertension for many years, but had been doing so well, that his doctor took him off of his medications.

Well, one day while at work, he did not feel right and went to the nurse who took his blood pressure and it was sky-high. She sent him immediately to his doctor, who actually asked if it could be due to stress! That night, he got into bed early and still felt horrible. We called his doctor and they said to get him to the hospital as soon as possible. Come to find out, the blood pressure was due to a renal crisis from the scleroderma. We found this out after a few days in the hospital, and many tests. When the doctor told us it was scleroderma, we had no idea what he was talking about. They said it was a rare disorder, and that there was no cure, but we had no idea how bad it could be. My mom and I did research and were horrified to learn what a devastating disease this could be. Our family was in shock.

After this renal crisis, my dad's looks changed. He had been very robust and healthy-looking, with so much energy and zest for life. He changed by looking a bit more wasted, his neck skin tightened a bit as did his face, but not so much that people thought he was ill; they just thought he had lost some weight. He did not have as much energy any more, but over time, his kidney function had stabilized and he had regular check-ups and was told he was doing fantastic. The doctors told him the renal crisis was probably the worst that would happen to him. He did remain stable and did great for about ten years, though his hands were a bit worse, more hardened and such, making it more difficult for him to do some things.

Time had gone on and he now had four beautiful grandchildren. Ryan was born in 1992 and was the apple of my dad's eye, and Ryan just adored him. They had so much fun together, they would just play games, go places together, they had developed a great bond. Ryan really looked forward to seeing him, and we saw him almost every day since we lived so close by. Then my brother and his wife had Jeff two years after and then came along Allison and then, Nicole. All the kids loved playing with Papa. He taught them all to swim in their in-ground pool.

As I look back now on all the times we spent together, whether it just was hanging out, or vacationing or at holidays, it makes me very happy and proud to have such a close-knit family. We truly enjoyed being together and had a lot of fun, and I find this comforting.

In May of 2001, we had a big retirement party for my dad. He had worked so hard, he deserved a nice party with his family and friends. But after having had a routine CAT scan to see how his lungs were doing, the doctors discovered a huge kidney tumor and again, things took a turn for the worse. This was in June of 2001.

In August of 2001, after an agonizing two months of waiting, the surgeons finally removed what turned out to be renal cell carcinoma. The good news was it was totally contained and my father would not need to undergo chemotherapy or radiation. We hoped his other kidney would be able to handle the workload, and were so happy that he seemed he would be able to recover and get on with his retirement!

He recovered pretty nicely with no major complications, but he seemed to be getting very short of breath on exertion, especially with climbing stairs. He thought it was his heart as he also had a stent placed due to a blockage a few years prior. So he reluctantly went back to his doctor again. They ruled out any type of blockage or heart problem, and after undergoing some more testing (an echocardiogram and an MRI), they sent him to a pulmonologist as they believed this time it was his lungs that were getting progressively worse. It was true, they were worse. He had moderate pulmonary hypertension along with pulmonary fibrosis and inflammation in his lungs from the scleroderma.

I have read many articles that state people with scleroderma have progression after undergoing surgery and I truly believe this is what happened to my father as after he had his kidney out he seemed to go progressively downhill.

There was really no treatment for this, except for some therapies that were believed might help. This included a daily dose of prednisone, Coumadin and Cyclophosphamide. Cyclophosphamide is a very powerful drug that suppresses the immune system; it is an oral chemotherapy treatment. When you are on this medication, you are supposed to be on a prophylactic antibiotic also so you will not get a dreaded pneumonia called Pneumocystis carinii pneumonia (PCP). This is seen in many AIDS patients when their immune system is very compromised and sometimes in cancer patients who are on chemotherapy.

Unfortunately, my parents were not told of the great risks of being on Cyclophosphamide. They were told his biggest worry would be possible blood in his urine and maybe bladder cancer on down the road. He was never told about this PCP. Also, he was intolerant to the sulfa antibiotics, so his doctor decided to hold the antibiotics for the time being, again never telling him of the risk of this pneumonitis! He never would have decided to take this medication if he truly knew all of the risks involved.

My father also had a great deal of difficulty on the Coumadin. They just could not get the right dosage for him. His blood was either way to thin, or not thin enough to be doing him any good. This was a major source of anxiety and frustration for everyone, but especially for my father, as he was at the hospital every other day having blood draws.

By the summer of 2002, although he had many problems, he was trying to adjust and enjoy the summer, but because of his physical limitations due to the shortness of breath and the aggravation with the many blood draws, he was becoming very discouraged. I believe he knew he was getting worse, but really never complained how bad he really was feeling, and although we were worried, we thought it was just progression of his disease, not pneumonia.

It was the beginning of August, 2002, when suddenly my maternal grandmother, dropped dead at age ninety-one. My parents were busy caring for my elderly grandfather and although my father never complained, he himself was close to death.

The night of my grandmother's funeral, dad had the chills so bad that he could not get warm. He was sweating and becoming more short of breath, but did not complain so much that he needed to go to the hospital. The next morning I stopped in to see him and he looked terrible and was shaking and was very short of breath. I called his doctor immediately as my mother was at church with my grandfather. His doctor said to bring him into the hospital. He warned me on the phone, only then, that because my father was on Cyclophosphamide, he was at risk for very rare infections. This was the first I heard of this. We got him to the hospital and they placed him on supplemental oxygen (50%) and did a lot of tests. When his doctor saw him, he said he would put him on Augmentin, hoping it was bronchitis, and actually released him from the hospital. PCP cannot be diagnosed without a bronchoscopy.

He went home, but he did not feel any better, and in fact, he felt much worse. He had severe shortness of breath and my mother drove him to the hospital in the wee hours of the morning, only one and a half days after being sent home on the Augmentin. At the hospital, he was immediately placed on oxygen and they again ran a battery of tests and blood work. They also had to do the bronchoscopy to rule out the PCP. After the bronchoscopy, he needed to be placed on more oxygen and his sats were still dropping. We were encouraged by the doctors as they had him on every antibiotic imaginable to cover any type of infection he might have and were told it might be several days before he would begin to improve. The doctors at this point did suspect PCP, but the bronchoscopy report never grew out anything, probably because they had placed him on some antibiotics in the meantime. But he was septic, having the disease throughout his body now and he never did improve. In fact that night, after being placed on oxygen and his sats still dropping, they dropped even further and he could not catch his breath.

He had to be rushed to the intensive care unit (ICU) and intubated, which must have been even more horrific for him than it was for us having to watch this and not being able to help him. This was the last time we saw him awake as after they did this procedure, he had to be very heavily sedated, so as not to fight the respirator, which now took over for his diseased lungs. He was in the ICU for many days and got progressively worse, now needing dialysis and so many other things, I cannot even remember as I was in such shock seeing him like that. He did not look like the same person that I knew and loved. He was now bloated and had tubes sticking out of him everywhere. To this day, I still cannot believe that was my father laying in that hospital bed. My only comfort is hoping that he felt no pain as he was heavily sedated.

We are sure there were many things he would have liked to have said to his family before he died. He was such a family man and truly enjoyed all the little things in life such as taking a walk, taking pride in his house and yard and cherishing his family.

As there was to be no hope for my father as his lungs were severely infected at this point, we had to take him off the respirator. They took out all the tubes and we gathered around him at his bedside. We held his hands and told him how much we loved him and watched him take his last breath at 5:50 p.m. on September 6, 2002.

It has been a rough year for our family, but I wanted to write this to possibly help any one else out there going through this. Take every precaution, educate yourself and above all, look out for yourself as the doctors may tell you one thing, but do what is right for you. You, above all, know how you are feeling and if it is not right, you must insist on further treatment.

Thank you for taking the time to read this story and take care of yourselves.

To Contact the Author

ISN Artist: Sherrill Knaggs (In Loving Memory)

Sherrill Knaggs, ISN Artist, created the digital photo to illustrate the story on this page. Sherrill lived in New Zealand. Her story was featured in ISN's book, Voices of Scleroderma Volume 2.

ISN Story Editor: Judith Thompson Devlin

Judith Thompson Devlin is the ISN Story Editor for this story. She is also lead editor of the ISN's wonderful Voices of Scleroderma book series!

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