“From everything we know about pemphigus vulgaris, you’re in remission.”
I just stared at the doctor in disbelief. I think I actually said, “You’re kidding me, right?” Probably not the best response. But he smiled, as did the other two doctors in the room, and said, “No, I’m not kidding.”
It took me till I left the appointment, got in my car and was stuck bumper-to-bumper in Boston traffic to realize that I had done it. I had licked this insidious disease – well, as licked as an autoimmune disease can get. Anyone who has battled one knows that there is always the chance that it will rear its ugly head again – “not curable, just treatable,” is the refrain we all hear way too often.
But for me, the word “remission” was just as good. Medically, according to my dermatologist Dr. Arturo Saavedra-Lauzon, who practices at Brigham and Women’s Hospital, remission in pemphigus vulgaris means three things: There is no clinical evidence of active disease or true acantholysis, a breakdown of the cell layer of the epidermis; my B cells, which had been taken to zero through a 12-treatment course of Rituxan®, had recovered to normal in the absence of the disease; and no IGG antibody is noted on my indirect immunoflorescence tests.
Ironically, it had been six years almost to the date since I had first noticed something going horribly wrong with my immune system. In early 2003, after what seemed like the longest five months of my life, my mother passed away. At the time, I was an editor for a computer magazine, zig-zagging across the country developing and leading one-day tech seminars. Some weeks, I’d fly to California and make my way back East, stopping to do classes in three different cities in three days. It was a grueling schedule, made even more hectic as I tried to spend as much time with my mother in a Pennsylvania hospital as possible.
I continued that pace through the summer. Then in the fall, I decided to take a break and go to London with some friends. However, it ended up being my mental and physical breaking point. On the flight home, I noticed red rimming around my eyes. I called my internist when I got home and he told me I must have gotten “pink eye” or some other virus during my trip. Within days, my eyes were no longer just rimmed with red, they were downright bloody-looking and, what I now know were blisters and lesions, had started to appear in the mucosa of my nose, gums, lips and throat.
I was peppered with blisters to the point I couldn’t eat. They were quickly marching their way toward my voice box, which meant I couldn’t talk, so I was placed on short-term disability. Also, we still didn’t know what I had and had to assume it could be contagious.
Finally we thought we were on to something, we just didn’t know what, because the doctor prescribed a short course of high doses of Prednisone. Of course, once I started on the Prednisone, the lesions started to clear, so I returned to work and we all thought I had been cured. However, as I tapered off the Prednisone, the lesions reappeared and this time, they were worse, blanketing not only my internal mucosa, but also my stomach and leg. The infectious diseases doctor said he couldn’t help me and sent me on my way. Again, I was sidelined from work and out of luck. Upon my sister’s urging, I returned – as a last-ditch effort before the ER – to my internist.
He concluded that because it was now visible on my skin, maybe a dermatologist would be able to help and sent me to Dr. Jay Cohen at Newton-Wellesley Hospital near Boston. Luckily, Dr. Cohen had just recently seen another patient with pemphigus vulgaris – his first in years, he said. He immediately took a biopsy of my abdomen lesions and confirmed the diagnosis with Dr. Razzaque Ahmed, an expert in this field. He called Dr. Ahmed personally and asked him to see me as soon as possible. This was around Christmas of 2003. By New Year’s, I was a patient with Dr. Ahmed. I will be forever grateful to Dr. Cohen for taking such a personal interest in my case and changing the trajectory of this disease’s effect on me.
I tried resuming my work travel schedule but within a few months found it too demanding and I quit to become a free-lance writer. As an aside, that was probably the best thing that ever happened to me. It was a lifelong dream fulfilled. As my wise nephew said, “See, there have been some good things about battling this disease.”
The last straw for me was when the entire lining of my throat sloughed off in a single piece of tissue. The pain was so excruciating that I felt defeated. I had been on IVIG for years at that point and I seemed to be in a stalled pattern that would not let me off the Prednisone. Finally, we decided that I would undergo the Rituxan® protocol that others were seeing success with. I tolerated it well in tandem with IVIG and was able to taper off the Prednisone completely. Although I was doing comparatively better, I still had blistering here and there and my gums were bright red and pulpy.
I didn’t care, I felt good. I had even been working out with a personal trainer and thanks to tapering off the Prednisone and eating better, had lost the bloating caused by Prednisone. My energy level had also rebounded so I poured myself into my niece’s wedding, took another niece and nephew on a cruise to Alaska; and ran a 5K – all within one summer.
I don’t have to tell you what happened next. I suffered a pretty massive relapse. One that crushed me physically and mentally. Where I had almost been finished with IVIG – one more round – I suddenly was back to every four weeks after having made it to 14-week intervals and I was back on Prednisone. And the worst part, I had done it to myself. So I decided from that point on – no extra stress and only low-impact exercise. When my health insurance forced me to switch dermatologists, I took the opportunity to start doing IVIG closer to home — a 10-minute drive to a nearby hospital infusion clinic vs. a two-hour drive into Boston. That alone relieved a tremendous amount of stress.
And amazingly, over the past few months, I’ve had no lesions and my gums are the beautiful pink/salmon color that I had admired in others.
So that brings me to today and that wonderful word “remission.” As you can see, it’s been a long journey and I have every right to be skeptical of how long this will all last. To say it’s out of my head now would be a lie. I still avoid most foods that I’ve been told could cause lesions such as acidic or sharp, pointy foods. And I do a site-check of my mouth every day to see how my gums and tongue are faring. I’m cautious not to over-exert myself and worry any time I’m around someone with a virus because I know that is a possible way to start all this back up. And to be honest, I still wake up nervous that this is going to be the day I relapse. My family is excited – but wary – about my remission. They are thrilled to see me “back to normal” but know that one bad cold or too much stress could dash it all.
I’m sure the further into remission I get, the more that panic will subside for all of us. For now, I’ve learned to be thankful for every day that I get that’s blister-free, but I’ll take what I can get.
by Sandra Gittlen
PV Patient and Freelance Journalist
Sandra Gittlen is a free-lance business, technology and lifestyle writer in the greater Boston area.
She can be e-mailed at firstname.lastname@example.org.