by Erica Byrne

My journey began two years ago, when I was diagnosed with pemphigus vulgaris. I tried many different medications, none of which gave me sustained improvement or remission. I am only 27 years old and I plan to have children, so I ruled out using daily oral cyclophosphamide (Cytoxan) because of the possible side effect of infertility.

In November 1998 my doctor, Grant Anhalt, told me he was trying to get approval for an experimental bone marrow ablation (chemotherapy) for pemphigus. He assured me that the high dose of Cytoxan used in this procedure is not known to cause infertility. After many months, the procedure was approved by the Internal Review Board at Johns Hopkins Hospital. I opted for this route.

I met Dr. Robert Brodsky, the oncologist in charge of my treatment, who answered my hundreds of questions. I had to have a complete physical examination, as well as extensive blood work, tests on my heart, lungs, and other functions. When I "passed" all of these tests, I was scheduled to have a Hickman Catheter (central line) placed. This enabled me to receive medications and give blood without being stuck repeatedly. For some reason I was most scared about this procedure, but it was not as bad as I anticipated. Although it was uncomfortable, it was painful only for about two days, at the site of the line. I then went to the IPOP (Inpatient/Outpatient Clinic) which is located on the Oncology Unit.

The following day, I was admitted to the Oncology Unit of the hospital. After making myself comfortable in the hospital room, I was "hooked up" to what would be my friend and shadow for many days. All of the medication hangs from a pole with wheels, which escorted me everywhere while I was in the hospital. I soon got used to pulling it along! After about two hours of hydration through the Hickman catheter, I received my first dose of cytoxan over a period of two hours. This occurred every day for a total of four days. I also received other medication to help reduce the nausea, and later I received antibiotics.

On the third day I also received two units of red blood cells, due to a drop in my count. Unfortunately, I also developed a fever. Since it was uncertain as to why I had a fever, many precautions were taken, including a chest x-ray. Luckily, nothing was found. I like to say that I remember and was awake for most of the time that I was in the hospital, but my family tells me otherwise. My husband, Tyson, my parents and friends were with me in the hospital for a large percentage of the time. I cannot begin to describe how wonderful or helpful they were, and still are.

Once I was discharged (on day five) from the inpatient unit, I returned to the IPOP daily for almost three weeks to receive medication and to give blood. Let me try to describe the daily routine. Upon arrival, my assigned nurse would check my weight, temperature and blood pressure. She would then take a blood sample to send to the lab to check my blood counts. Next I would get hooked up to my rolling friend, while receiving antibiotics (for the first 7 days), and on the tenth day of treatment I began to receive Growth Factor, which essentially jump-starts the white blood cells. All of this took at least several hours.

Sometime during my wait, the nurse brought the blood work results to me on a small slip of paper. It contained three numbers: platelet count, white blood count, and hematocrit (red blood cell count). I watched each day, as the numbers dropped lower and lower (as planned). When the platelet count dropped below 10,000 ("normal" is usually 300,000 or more), I received a platelet transfusion through the Hickman line. The white blood cells went as low as 11 ("normal" is 15,000 or higher). This was considered to be zero, which was the goal. At this time I had no immune system, and wore a mask everywhere except in my house. I also limited the people with whom I had contact, due to my susceptibility to infection.

So, for about three weeks, the only places I went were my house and the IPOP clinic at Hopkins. While I was home, I took my temperature several times each day, to be sure I did not have a fever. This is fairly common, due to having almost no immune system, but I was lucky. No fever meant no readmission to the hospital. After several long weeks, you can imagine how happy I was to finally get permission to go "in public!"

Ten days after I started the treatment, my hair started to fall out, in clumps, as promised. At first it did not bother me too much, but it was ALL out by the end of the eleventh day. I could not believe how quickly my formerly thick hair fell out. When I only had a few wisps left, and thought that I looked very sick, Tyson shaved the rest off for me. This was the first time I cried during my treatment process. I could barely look in the mirror, and quickly tied a bandanna around my head. After several days, I got used to my new look, and to wearing hats when I went out of the house. (I also bought two wigs in advance.)

One day at IPOP I looked at the small slip of paper and was amazed to see that the blood counts were rising again. I was right on track. Once they started rising, they rose to the desired level in only a matter of a few days. With a quick yank, the central line was removed, and I was discharged from the IPOP. Believe it or not, it was hard to leave. The nurses and doctors who took care of me were the most wonderful, caring, friendly, helpful, professional group of people I have ever met. After celebrating my discharge with a lunch out, I went home to rest, as was so often the case.

I am pleased to report that my blood work has looked very good so far. And, almost more importantly, most of the lesions have cleared up! I am down to 20/0 of Prednisone, which is the lowest I have been since I was diagnosed. The only other medication I am taking is an antibiotic which I will take for six months. I recently ate pizza for the first time in recent memory. I also returned to work the last week of August. Other than being more easily tired, I feel great. My hair is even starting to grow again. (It is too bad that society places such a high value on hair styles, because I am getting used to having a cooler head, and not having to wash and dry my hair every day. I sure do get ready quickly now!)

Although this process is not over, I know I am well on my way. My husband has been by my side through the whole process, and has accompanied me to most of my medical appointments since the beginning. Somehow he puts up with the Prednisone mood swings, and everything else. Although it probably saved my life, I will only be too happy to get off the Prednisone. If the treatment was as successful as we hope, I will be able to taper off Prednisone completely, and remain PV-free!