Stuart says certain experiences are common with uncommon diseases, such as difficulty getting a correct diagnosis or how fast a patient can rip through medical benefits and caregivers through their family leave time and cash and the energy to cope with a healing process that takes months and sometimes years. Crow and Puckett know well the challenge. It has become the story of their lives.
The long haul
While the disease mostly strikes older people, it sometimes takes a younger one, like Crow — and by extension and inadvertently, his caregiver Puckett — out of the work force. Stuart's seen it in teens and adults who in some cases can never work again because of flares and subsequent difficulty bringing the pemphigus under control. "If you can get good control of the disease — that's both if and when — on average there's about two years when you're really suffering with the thing and not able to work," she says.
Two years isn't much over the course of a long life. But it's forever when, like Tina Puckett, you're trying to get by on the time the Family Medical Leave Act excuses you from work. She didn't make it and lost her job. And there's another irony to the timing: It takes a couple of years for people who struggle with something like pemphigus to qualify for certain types of disability. For those who are lucky and overcome the disease, about the time you qualify, the condition is controlled and the disability ends before it officially starts. And for those who never get it quite under control, two years with no help is a very long time, Stuart says.
Still, she notes, "the bigger crisis is the period before diagnosis." The longer it builds up, the more damage the disease causes and the harder it is to overcome. But because pemphigus is so rare, many doctors have never sees a single case and they know nothing about it. When the foundation surveyed those who have been diagnosed with pemphigus or pemphigoid, they found many had seen more than 10 doctors before they learned the name of their disease. Finding someone capable of treating it with expertise is a genuine quest.
The foundation estimates there are perhaps 35 people who know how to handle the disease well in the United States. Some pemphigus patients will never find those specialists — or will search for a very long time. Because the disease typically starts with blisters in the mouth, people often go first to a dentist to find out what's going on. And a dentist may not recognize the disease; if he does a scraping it increases the likelihood miserable symptoms will flare and spread. Even with a diagnosis, many providers don't know to watch for bone loss or to keep an eye on the red blood count, says Stuart.
Experts including Zone are teaming up to come up with a protocol to help others do the right thing consistently when it comes to treatment. Stuart says 33 experts gathered at the National Institutes of Health, which incidentally funds much of the research being done, to share what they know and to come up with a plan to teach others.
Still, "it is the smallness and rarenesss and insignificance on the 30,000-foot level that makes it all so tragic," she says.
Zone's dermatology practice includes 15 or 20 people in the region who have pemphigus. Tina Puckett was lucky to find a knowledgeable dermatology clinic just an hour's drive from home.
Lester Crow's case, Zone notes, is one of the most severe he's ever seen. By treating the condition aggressively with cortisone and medicine that suppresses Crow's immune system, Zone is fairly certain the skin will eventually heal and the disease will go into remission. But getting there has been fraught with challenges. Crow's skin must be treated "gingerly," so it doesn't break or peel off. It has had to be kept "absolutely clean" to avoid infections. Dressing changes for a while were done at the University Hospital burn unit, with staff's vast experience that again highlights how similar his wounds were to burns.
Just bumping his skin is potentially serious. Stuart knows people who place shower curtains or plastic sheets on their beds and pour vegetable oil on it so they won't stick to anything when they have pemphigus.
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