I’ll wager that when your automobile’s “check engine” indicator flashes on, you quickly get your car into the repair shop. So why, when my personal “check engine” light flashed, did I ignore it?
When I was diagnosed with Pemphigus Vulgaris at the age of 47, the first information I unearthed regarding the disease was at my local bookstore. It was there, sitting cross-legged on the floor, that I found one medical book that referred to pemphigus as “a blistering disease that is usually fatal”. Ha! Fifteen years later, I’m alive, and usually well, excepting the occasional flare. But that doesn’t mean the road I’ve travelled hasn’t been without its potholes. Thanks to my doctors, my family, and, finally, the IPPF, I’m up and running, ready to coast through the next 15—or 30!—years. One of my greatest sources of hope has been the IPPF Annual Meeting, which was held in Philadelphia this past April. But it took me awhile to get there.
When I first became ill in 1995, I was fortunate to have been diagnosed quickly—only four months from start to finish. I had great doctors who, by the way, had never seen or treated pemphigus. I take that back. My dermatologist had seen one case while in medical school but that patient died from her PV shortly afterwards. To say that my doctors were big ‘fraidy cats would be an understatement! I was placed on 20 mg of prednisone—too little to do any good.
It took some research (remember, 15 years ago the Internet was an infant) but I eventually discovered the perfect PV mechanic in Janet Segall. At that point, Janet was working out of her house to help others with pemphigus — some 3,000 miles west in California. Janet was a fountain of information and, after several discussions, she suggested I make an appointment with either Dr. Jean-Claude Bystryn at NYU Hospital or Dr. Grant Anhalt at Johns Hopkins, since they were both within a manageable five-hour drive from my home.
I chose Dr. Anhalt and was given an immediate appointment. What a relief it was when he reassured me that I was not going to die, and that I was not contagious. I remember thinking: “Really? I can kiss my baby grandson?” Back home, we got down to business. Under Dr. Anhalt’s direction, my family doctor and dermatologist worked together and ordered baseline tests for bone density, a thorough eye exam, blood work, chest X-rays—you name it, I had it. Their close observation resulted in a wonderful 10-year remission. Trust me, I was a perfect patient—until 2005, when I was promoted into my dream job.
I put pemphigus on the back burner, and my job on the front burner. I worked 60 hours a week and travelled constantly. But within two years, my tank was on empty. I was exhausted, developing harsh colds accompanied by fever, coughing spasms, and severe laryngitis that silenced me for weeks. Eventually, I would regain my health, get on with life and wait for the next onslaught.
One morning, I woke to discover a bloodstain the size of a dinner plate on my hotel room pillow. I was startled but took no action. Several months later, it happened again on an international business trip. My expectant daughter, who was with me, was horrified. For her sake, I laughed it off: It’s happened before, it will be okay. She was not so easily pacified; daughters can be very tiresome creatures!
I should have known then that my personal “engine light” was flashing. But I ignored it. Unfortunately, the day after arriving back in the States, I experienced a searing chest pain and with one cough shredded five inches of my esophageal membrane. No siree, pemphigus was not ignoring me. It had found the perfect breeding ground and, now, my chassis was parked in the hospital.
I was released in seven days, not only reeling with invasive PV, but also as a newly minted Type II Diabetic from the massive doses of prednisone. Four days later I suffered a large retinal tear in my right eye and required two laser surgeries. I was terrified of what might happen next; it was a hideous state of affairs.
I was out of work for four months and used that time to seriously re-evaluate my lifestyle and the changes age had brought about. I finally acknowledged my reckless habit of undermining my health in what was a clear case of “disease to please” everyone but myself. I immediately made changes. I was now 58 and could retire with benefits at 60. My management and company medical department provided unconditional support. I gradually resumed my workload to a more-manageable 40 hours a week or less, and I no longer travelled. It was not unusual for my manager to stroll into my office at any time of day and direct me to go home. I loved him!
I retired two years ago this month (May). I set only a few goals. I would correct my six- year-old grandson’s batting stance, start a local Pemphigus support group, and refuse to engage in activities, social gatherings, or anything that did not enhance my well-being. I completely embraced my new freedom and after 28 years of “oh-dark-thirty” alarms, discovered the delight of waking naturally and savoring that first hot cup of coffee.
Along the way, I found my locality does not lend itself to a pemphigus support group; there just are not enough of us. So, I recently became active in the IPPF. I am now a Peer Health Coach so that I can “pay forward” the terrific support I have received from the IPPF. My grandmother, a depression-era medicine woman, would be so proud.
Although I was really too ill from a flare-up to attend the recent IPPF Annual Meeting in Philadelphia, I hauled myself there anyway. I’m so glad I made the effort! Going to the conference served as a huge wake-up call for me. It had been three years since I last attended a meeting, and I was delighted to hear of newer treatments, success rates, studies, wound care, etc. Break-out sessions on several topics allowed me to tailor my learning to fit my situation. Finally, I saw Dr. Anhalt there, and he once again has given me hope, and put me back on the path to controlling my flare-ups.
I’ve learned my lesson: a tune-up is not an option; it’s a requirement. So, just as you’d take your car in for annual service, make a plan to attend the annual conference next year. Consider it your personal, annual tune-up. The conference is a great place to network with physicians and professionals on the cutting edge, to ask questions of experts and receive down-to-earth answers from the people that “get” you. The forums are informal, open settings that allow everyone to absorb the information. The icing on the cake is the exposure and interaction with others who have walked the walk. I guarantee you will connect with someone with whom you will stay in touch.
In writing my story, I hope to encourage you to take advantage of the many wonderful opportunities the IPPF provides. We have at our disposal dedicated doctors, researchers, a physician referral list, clinical trials, and pictures and information available to become knowledgeable, articulate, and on par with (or ahead of) the physicians that treat us. Visit www.pemphigus.org and connect globally in forums and chat rooms with others who understand what you are experiencing. Through the Health Management Program, an email or phone call will put you in personal contact with a Peer Health Coach.
I am excited about the IPPF’s confidential and secure Pemphigus-Pemphigoid Registry! We will have the opportunity to register our personal stats, thereby drawing nearer to the common goal of a cure for the diseases and variants of pemphigus and pemphigoid. It’s heady stuff, on an international scale!
Contrary to the inadequate information I uncovered 15 years ago sitting cross-legged on the floor of a bookstore, the real message about pemphigus is one of hope, camaraderie, and medical advances. Come join me at next year’s conference to fuel up on all three!