by Kirsten R Bellur
It is almost four years ago that, after many failed attempts, I was finally accurately diagnosed with Pemphigus. In the face of that solemn pronouncement, I was told there was good news: it was only Foliaceous, a more benign form, that was easily treatable with Prednisone. And under that treatment it would most likely go away. But this sanguine vision and mitigating explanation of the seriousness of the illness did not obviate the fact that I was unable to regain the integrity of my skin.
As others living with this condition have experienced, after several months of Prednisone, alone, I did not get a favorable result. Therefore, Imuran was added to the drug regimen. After four months, the side effects were too intense, and my steroid-sparing drug therapy was changed to CellCept. For almost two years, I did a “yo-yo” diet of steroids. I was down to 9mg of Prednisone every other day, only to experience a flare, necessitating a difficultly steep, fast-track steroid schedule to combat it, one which my body was ultimately unable to follow. After almost 6 months, I reduced to 20mg of Prednisone a day, and experienced another flare. And I was advised, once again, to go back up to a high dose of Prednisone.
Going up and down repeatedly on Prednisone, I was losing faith that I was making any progress whatsoever, and suffering serious side effects. When dealing with PF, one can never ignore an itch or a blister, as it may well be the harbinger of a flare-up of the disease. This flare-up would have been building precipitously beforehand with no visible signs and has now, with perceptible manifestations, reached a level that is more advanced, requiring higher doses of medications to get it under control. Was there any way to accurately predict a flare-up before the itching started, when the antibody levels were lower, and less of the harsh medications would be required to quell it?
My desire to answer that question became even more urgent as I analyzed my drug therapy over time and came to an alarming possibility. For every subsequent flare of the illness, more Prednisone was required, and for a longer period of time, to suppress it. The “yo-yo” diet seemed, perhaps, somehow to be making the illness less responsive to the medications. The implications of this, given the deleterious side effects of Prednisone, could be dire.
Desperate to make progress toward remission, I sought answers widely, leading me to Dr. Grant J. Anhalt at the Johns Hopkins Medical Center. He met with me and assessed my condition. His first observation was that my dosage of CellCept (2000mg/day changed to 1500mg/day increased again to 2000mg/day), though appropriate for certain organ transplants, would most likely be more effective for PF at the higher constant dose of 3000mg/day. But the most valuable insight was this: the Enzyme-Linked ImmunoSorbent Assay (ELISA) test may be able to predict flare-ups before they manifest themselves as perceptible symptoms, allowing them to be suppressed with a smaller amount of medication. As the ELISA test is better at assessing this than the commonly used Indirect Immunofluorescent test (IIF).
The ELISA test provides a sensitive, specific, and quantitative tool to obtain precise information about autoantibody specificity. Monitoring the disease activity can be extremely useful in planning a tapering schedule for Prednisone and predicting flare-ups and relapses by detecting increases in antibodies before clinical evidence is noticed. Early intervention is the best defense. One could, therefore, then imagine taking the ELISA test monthly, and when a rise of antibodies seemed to be happening, well before the patient could feel it, the medication could be raised slightly to stop a flare. The implications to the patient, who could be spared dangerously high doses of harsh medications, are staggeringly positive.
Such a discovery faced some adversity: getting my health insurance provider to cover the cost. Very typically, a myopic view about the ultimate cost savings of preventative, proactive care most likely led them to this decision. After they first denied coverage, I filed a grievance with their Medical Review Board, and backed up my claim with relevant articles and research. With no background in medicine, this was an intimidating task, but I knew that I was fighting for the very quality of my life. Without going into all the details, my request was granted – they would reimburse the cost of the test.
The blood collection for the ELISA test is very simple, and can be done from anywhere (I live on the other side of the country from Johns Hopkins). I am provided with pre-paid mailers that the local lab uses to express mail the samples to the Johns Hopkins Lab. My primary care physician has filed a standing order for monthly completion of this test, along with Complete Blood Count and Hepatic Function Panel (CBC & LFT’s). All the tests results are also communicated to my dermatologist, an outside provider. Initially the ELISA test should be done monthly and then, with progress, can be done every three months, as the antibody levels neither increase or decrease at a rapid rate.
It has given me great comfort and peace of mind to get the ELISA values as an added assurance, that the occasional itch or blister I experience is not my next flare or relapse, but that I may finally be on the track to a durable remission. It is also important for me when faced with making informed decisions with regard to other medical matters that affects my life – like elective surgery, as I have experienced first hand, medicine is not an integrated art, but the art is to find the right medicine.
I hope sharing my personal experience may help others in finding the right treatment.