by Barry Kratz
My name is Barry, and I was diagnosed with PV in June 1992 at the age of 22. I have been asked to share my story and give some insight to help overcome this sometimes-debilitating disease. I want to emphasize that what follows is my own experience. Treatments have likely changed over the years, so your experiences might be quite different. In addition, I've been told that younger people can get more serious cases that progress rather quickly compared to most people who get the disease in their 40s or 50s. My case was very difficult and took a long time to control. Thankfully (knock on wood), things have been going well for me for the past 7 years.
Before I begin, I want to make a couple of important recommendation to anyone with PV. The first is to EXERCISE, EXERCISE, EXERCISE. I can't stress this enough and wish someone had told me this when I was first diagnosed. Prednisone, which many people are treated with, really takes its toll on the body over time. Exercise is one thing you can do to help control the bone loss and muscle atrophy that might occur. Prednisone also makes you hungry, retain fluids and can slow down the digestive track. My second recommendation is to eat a well-balanced, low salt diet and drink plenty of water. No Snacking!
Now to the first part of my story…
It was 1992. I had recently graduated from college and was working for a big six accounting firm. A few months into the job, I developed a sore throat that would not go away. It didn't bother me much, but it was always there. After a couple of months, I finally consulted a doctor, and thus began my long journey. I first went to my internist, then his colleagues, then an ENT, then his colleagues, and so on. I saw a total of 15 doctors in a period of 5 months. In the meantime, I was taking small doses of Prednisone (6mg) to treat the sore throat and the blisters that had appeared in my mouth. I was feeling somewhat better, but still, no one knew what I had. Finally, a biopsy was taken. On a Friday afternoon, I pressured a nurse into giving me the results. She told me I had PV. "What?" I said. After she repeated and spelled the diagnosis, I thanked her for the information and promptly looked up "pemphigus" in Webster's Dictionary. I remember quite clearly reading "a near fatal disease". What a long weekend that was! My family and I were understandably very scared. The Internet wasn't a resource back then, so we began to contact friends in the medical field. We eventually identified a doctor in NYC who treated the disease.
In June 1992, I was put on a daily dose of 90 mg of Prednisone and I got better in a matter of weeks. By the fall, I was slowly being lowered off the Prednisone. However, while on a business trip, I noticed that my undershirts were bloody. By December, I had developed blisters on both my back and face. I was trying hard not to let the disease affect my life, but it was difficult just to get ready for work. Even showering was an obstacle. Also, AIDS was very much in the news at the time, especially with Magic Johnson going public with his disease. What did people think I had? I got out of the shower one day and decided to call in sick. I would be out of work until 1995.
Because I was not satisfied with the results of my treatment, I switched doctors and was put on gold therapy. My condition improved, but a blood test revealed that I was having liver enzyme problems. A top liver doctor advised that I stop the gold therapy. During this time, I was also taking Carafate to combat bone loss. This backed up my digestive system and landed me in the hospital for my first of many stays.
My second time in the hospital was 3 months later to treat a kidney stone. This visit actually saved my life. An X-ray revealed something on my lung. I was treated with antibiotics, but it did not go away. The doctors decided to perform a biopsy of the lung, which was a very unpleasant procedure. They discovered that I had PCP Pneumonia that, fortunately, could be easily treated with Bactrium DS. I learned later that doctors were being taught to prescribe Bactrium DS to persons taking high doses of Prednisone. This was not the protocol at the time of my treatment, but I would certainly recommend that people talk to their doctors about it.
Back at home, it was very hard just to sleep and move around. Bathing was very uncomfortable. I kept Vaseline on my body constantly to stay moist. I slept on a board to keep me on an incline for comfort. However, whenever I woke up, my shirt was dry and stuck to my skin. Over time, I learned to get creative with bandages and ointments to cope with the pain.
In March 1993, I went to Yale University to learn about a new procedure called Photoperithesis. Once there, it was determined that the procedure was not appropriate for me. However, the doctors admitted me to the hospital because my skin was very infected and needed to be cleaned. This was the first of my three visits to Yale.
It would be several more months of new doctors and various treatments and drugs before I finally found what would eventually get me better. The next newsletter will include the second installment of my story. If you would like to talk with me before that, please feel free to call or e-mail me. My contact information is in the heart to heart section.
Continued from previous news letter:
In the Winter 2002 newsletter, I recounted my experience with PV from my first symptoms in February 1992 to my initial treatments over the course of the next 9 months. In March of 1993, I was admitted to Yale-New Haven Hospital because my condition had worsened and all my previous treatments were not effective. Upon admission to Yale, a resident greeted me who had never seen someone with such a severe a case of PV. Months later, when I happened to bump into this resident, he admitted that when he first met me, he didn't think I was going to make it. This really put the severity of my case into perspective. At Yale, I was treated with Clorox baths to clean my skin. I was also treated with topical steroids, Bactriban, Vigilon, Xeroform and Vaseline and was wrapped in gauze and stockingnet. The entire process took about two hours each day. Eventually, it was determined that the treatments weren't helping, and the doctors decided to try Plasmapheresis. Because the veins in my arms were not in good shape due to all the blood tests, I had a catheter inserted in my groin area in order to perform the procedure. It was very strange to watch all of my blood flow through a machine and back into my body. I had two treatments over 3 days. I was prescribed Imuran, Cyclosporine and a higher dosage of Predisone as a back up. My kidneys had to be flushed with fluids and monitored frequently because of the potential side effects of Cyclosporine. I started getting better, and was released from the hospital after a few weeks. However, I continued to travel 3 hours roundtrip to Yale a few times a week to take the whirlpool baths.
At home, my muscles atrophied and I gained a lot of weight because I was not able to exercise. My mother, and occasionally a visiting nurse, helped me through a stringent routine at home. Everyday, I woke up early, ate breakfast and took my morning medications. At 10 a.m., the bandages from the previous day were removed and I took a Clorox bath. This was the part of my day I looked forward to because the weightlessness and warm water was very soothing. After bathing, ointments were reapplied and I was rewrapped in gauze and stockingnet. I ate lunch, took my afternoon medications and watched TV. Dinner was at 6 p.m. I was on a low salt diet to treat the high blood pressure that resulted from my medications. After dinner, I took more medications and watched more TV. I treated the constant pain with Percocet. At 10 p.m. I ate a snack and took my last medications before bed. The summer heat made the ointment wrap very uncomfortable.
In July 1993, I was readmitted to Yale for a week because I developed Strep on my skin. Once back home, we continued the same daily routine and regular trips to New Haven. We came up with creative ways to use stockingnet to make me more comfortable. On two occasions, my back went out on me because my muscles were in terrible shape. We ended up renting a hospital bed to make it easier for me to lie down and get up.
My journey turned a significant corner during one of my visits to Yale when we asked the attending doctor who we should consult for another opinion. The attending suggested a few people, but the name that stuck out was Dr. Grant Anhalt whom I had seen listed in a medical book. My mother was able to reach Dr. Anhalt immediately at his office at Johns Hopkins. She instantly liked him. He was extremely helpful and, after hearing the specifics of my case, insisted that I be put on a different protocol. I was admitted to the hospital and the Yale doctors began to follow Dr. Anhalt's protocol that called for three treatments of Plasmapheresis one week and two the following week in conjunction with 150 mg of Cytoxen. Because of Cytoxen's potential side effect of sterility, I first had to bank my sperm.
I was in pain, could barely move, and had to lie flat on my back for days. My skin was falling off and I looked terrible. I was on a morphine drip and needed a hepron shot twice a day to prevent blood clots. But the results of the protocol were miraculous. My skin started to heal before my eyes. However, I still had to address the side effects of Prednisone. I was confined to bed for a few weeks and was unable to do much of anything. A physical therapist taught me how to sit up, stand, take a few steps, walk and go up and down stairs. On October 16, after 7 weeks in the hospital, I was sent home.
My condition gradually improved — my cushionoid look slowly dissipated. I lost some weight and learned how to walk completely on my own. It was a slow process, but I was coming back to life. After about eight months, I suffered yet another side effect of the Prednisone. The redistribution of fat in my body wrapped around my appendix and I needed an appendectomy. Afterwards, I continued to improve. I finally made it.
I went back to work in January 1995. I met my wife in the fall of 1997 and we got married in May 2000. I have been living a happy, active, "normal" life for the past 7 years. However, I do remember. Everyday I am reminded by the stretch marks on my body and my ritual of drinking orange juice to see if there is any pain in my mouth from the acidity. And when life gets me stressed or down, I remember everything I went through, and it puts things into perspective. I was very lucky to have a great support system of family and friends through my ordeal. My mother took a year and a half off from work to take care of me. I know a lot of people were thinking about me and praying that I would get better. Knowing people cared really got me through the tough days.
If anyone would like to contact me please look for my information in the heart to heart section of this newsletter. Thank you.