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20 Years of FSGS in 2500 Words
February, 1986. It was a couple of months before my 15th birthday. During a school physical, I noticed I had gained about 10 pounds. I figured this was a good thing – since I could never seem to gain weight no matter what I did or ate. Soon after, I started to notice some other things as well. I was feeling a bit run down, which I chalked up to all of the running around I did. I was an active teenager, involved in BMX, skiing and martial arts.
Then one morning I woke up with my eyes practically swollen shut. My parents thought that maybe I was developing allergies, but I had a feeling that something wasn’t right. A few days later, after having trouble putting on my sneakers because my feet and ankles were swollen, my parents realized that something definitely wasn’t right.
We called a family friend who was a doctor, and he came over and had a look at me. He told my parents that he thought that there might be something going on with my kidneys and that I should probably go over to Children’s Hospital in Philadelphia and have some blood tests done.
I didn’t think much of it. I was more relieved that I got out of the long lecture I was supposed to be getting from my parents about my less-than-stellar grades as of late. I figured a couple of hours at the hospital and a quick needle stick was better than getting the, “straighten up and fly right” talk. In retrospect, I would have taken the lecture.
I spent about a week in the hospital getting labwork and a kidney biopsy. It turned out I had something called nephrotic syndrome, more specifically FSGS. My family and I had a lot of questions, but unfortunately there weren’t many answers. Nephrologists told us that my kidneys were inflamed and leaking protein, which caused my body to retain water, and that this inflammation was causing scarring in my kidneys. As those of us who have this condition know, that is pretty much all that is known about FSGS today.
When I asked how I got it, I learned a new word. Idiopathic. The medical term for “we don’t know how you got it.” When I asked if it goes away, the doctors said that it sometimes goes away but not usually. They said that they were going to treat me with a steroid called prednisone.
The next few months were extremely difficult for me. On the prednisone, I was sick all the time, once even getting pneumonia from a flu virus. I gained about 20 lbs. of water weight in just a few weeks. My skin stretched like a balloon. None of my clothes fit any longer, and my face blew up and broke out terribly. This was not the way I wanted to look when returning to school. As if I wasn’t self-conscious enough with the typical teenage adolescence stuff, I now had curious people coming up and asking me what happened to my face.
I began holing up in the house. I took long bike rides to clear my head, but with all of the extra water weight from the prednisone, I no longer felt comfortable riding. I needed something new to do. My older brother had been playing guitar for a couple of years and was getting quite good at it. When he first started, he told me that I should learn to play bass so we could start a band. I had a bass that I had borrowed from my grandfather, which, until that time, spent most of its time gathering dust. So I picked it up and started to play.
After about three or four months of treatment, the doctors decided that the prednisone wasn’t really working. Since that was the only course of treatment at the time, there were no other options – except wait it out and, if need be, get a transplant when the time comes.
I was just happy to be off of the prednisone. The other stuff I could put off in my head, because it was down the road at least for a while. Things pretty much went back to normal. With the help of some diuretics, I lost all of the extra weight in about five days and my complexion began to clear up. I even resumed some of my outdoor activities for a while.
Over the next couple of years my kidney function slowly deteriorated, and by the middle of my junior year of high school, my blood creatinine was up to about 10. I’m sure most of you who read this know, that means it’s time for either a transplant or dialysis. My parents, brother and aunt were tested and they were all sufficient matches. My dad, who was a 3-out-of-6 match, decided that he would donate a kidney.
I would say that this was the most successful of my transplants. I remember the date: November 15, 1988. I had a problem with an initial rejection that doctors treated with a drug called OKT3. If you’ve ever had it, you know that it’s a rough ride. For about five days, it seemed like every dormant bacterial cell and virus in my body went on the offensive. I had a sore throat, terrible bronchitis and a 104 ° fever. I was given alcohol sponge baths and had to sleep on a cold pad, which was a rubber mat with cold water being pumped through it. When I think about it, I remember lying on that mat with a pounding headache. The sound of that compressor was like a jackhammer in my skull.
Finally on the sixth day of the 10-day treatment, the sickness vanished and my new kidney was functioning well. In retrospect, I credit the OKT3 for the success of my first transplant. Although the FSGS did eventually start to come back, it was very mild and didn’t really affect my kidney function. This was probably the most normal time of my life. I eventually had to see my nephrologist only once or twice a year.
I graduated high school. I played in several bands. I even went skiing almost every winter. I began working full time. After several years of go-nowhere jobs, I decided I should probably go to college. I went to a community college for a couple of years (because of those grades), and then transferred to Rutgers in 1995.
All good things, however, do end and in 1997, after once again coming down with pneumonia, I found out that I was starting to lose kidney function again. I was living in New Brunswick, New Jersey, at the time, and going to Cook College at Rutgers. At the end of the spring semester, I moved back to southern New Jersey and attended Rutgers in Camden. By early 1998, I had lost enough function that it was time to put my name on a donor list. My family also started planning to donate. On December 8, 1998, I came home from school at around 6 p.m. I hit the button on my answering machine and listened to several messages from the University of Pennsylvania Hospital. A kidney was available for me, but if I didn’t call back within the hour, it would go to someone else. I checked the time. I had 15 minutes to call them back. I took the kidney.
I figured that the first transplant was successful, so the second was bound to work just as well, right? This kidney was even a 4-out-of-6 match, which was better than the first one. Unfortunately, that wasn’t the case. After quashing the initial rejection, with a new drug that wasn’t nearly as bad as OKT3, things were looking up. There was only one problem. I was spilling protein, a lot of it.
There were also problems with the great new medications that I’d read so much about. Cellcept tore up my digestive system, and Prograf gave me awful tremors and made my hair fall out. My kidney function went up and down, but I was able to have some semblance of normalcy for a while. I finally finished college in 2000, and got a job with a medical publication company in the Internet department.
Unfortunately, after about a year and a half, the kidney began to fail. My family and I started looking, once again, at transplant options. This time my brother volunteered. He was a 5-out-of-6 match, the best yet. We planned the transplant for August 29, 2001. About a month before the transplant I went on hemodialysis for the first time.
The best part of this transplant was that my brother Marc had his kidney removed lapyroscopically. This brand-new surgical method was much more advanced that the traditional method. In fact, Marc was the first person at Jefferson Hospital to have it done this way. Back when my dad had donated, doctors had to practically cut him in half to take out his kidney. Then his wound became infected and they had to reopen it. It was a mess. And I felt awful about it. With the new method, my brother was home in two days.
Unfortunately, the FSGS quickly returned. I had learned that there was some buzz lately about treating FSGS with plasmapheresis. I read some of the research and it sounded promising, so I tried it. Initially, it worked. I even completely stopped spilling protein for a while. The problem was that every time I would stop the treatments, the proteinurea would return. My kidney function once again began to deteriorate. After a year and a half, I was back on dialysis.
Although it may sound strange this wasn’t a particularly bad time in my life. My employer was very understanding through all of my surgeries and treatments and held my position for me. Once I started on peritoneal dialysis, I was able to focus on my work without interruption for a while. I also got engaged to a wonderful woman whom I’d met while in college, and we moved to Philadelphia together.
That’s right, four. It’s hard to believe that I’ve had my mid-section cut open that many times without completely falling apart. (I’ve also had two of my transplanted kidneys surgically removed.) I honestly wasn’t sure if I wanted to get another transplant. The emotional roller coaster of getting my hopes up and then having them dashed was becoming too much. I kind of preferred the regularity of dialysis. I was also worried about my mom. It had been 15 years since my dad had donated, and although all of my family is still in perfect health, I was worried that my mother may be too old to donate.
She was ready to donate, though, and she and the doctors convinced me that it was the better thing to do. They had a “theory” that because each of my successive transplants were better matches than the last, and that each transplant didn’t last as long as the previous one, a lower match may be better for someone like me with FSGS. Therefore, my mom’s match of 3 out of 6, which was the same match as my dad, may work. So, on January 21, 2004, my mom had her kidney lapyroscopically removed and donated it to me. Once again, though, the disease quickly returned.
I actually began plasmapheresis before the surgery this time, but even that didn’t help much. Then, IVIG was added to my treatment regiment. I was spending between 16 and 24 hours a week at the hospital, which was about twice as long as I spent on hemodialysis. The plasmapheresis treatments had some limited success, just as they did previously. But as soon as we cut them a little bit, the disease immediately returned.
During this time my fiancée and I were planning our wedding, which was to he held on June 5, 2004. After several months of treatment, we decided that whatever was going to happen was going to happen, and I didn’t want to go on my honeymoon completely drained from the treatments, so we stopped the treatments for a few weeks.
By the time I returned from our honeymoon, I had lost a significant amount of function and I was spilling a lot of protein. We tried going back on plasmapheresis, and it worked for a while, but the FSGS eventually won out. In December 2004, I went back on dialysis. A month later my mother’s kidney was removed.
I went on peritoneal dialysis for a few months but had some trouble with hernias along the incision where I had the previous nephrectomy. I’m on hemodialysis while I heal and should be back on peritoneal dialysis soon.
I’m also taking some time off from work. Although my employer was still sending me freelance work, I had too much going on medically for me to be reliable. I decided it would be better for me to go on disability for a while. I’m still playing music, though. My brother Marc and I have a band that plays clubs around Philly every now and then.
So that’s it …19 years of dealing with the ups and downs of FSGS. I’m sure that there are some things that you can relate to – and other things that you may have to look forward to. The one constant about this disease is that it’s rarely the same for any two people.
I’ll never say that I’ve given up on a successful treatment for the disease, but my decision-making process has definitely changed. I’m not on any list right now for a transplant. Unless something definitively new and/or truly revolutionary comes out, I’m going to wait on the sidelines for a bit.
No matter what’s happened with the diminishing success of my transplant attempts, nothing could ever discount the sacrifices of my mother, father, brother and an anonymous donor. Without them, every important part of my life – graduating high school and college, being successful at work and getting married– may never have happened.
For years I thought that I was alone in this struggle and that no one could possibly understand what it’s like to live with FSGS. But after reading so many of these personal stories, it’s truly comforting to know that that are others who understand my life. I’m thrilled that NephCure is finally getting a disease that is increasingly more common the attention it deserves.
Finally, I have to say that more than anything else, I feel lucky. I have a fantastic family, a beautiful, supportive wife and great friends. You’ve probably heard the cliché “At least you have your health,” I feel like I may not have that, but I have just about everything else.
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