My story with pemphigus vulgaris began just as I was turning 65 and beginning my retirement after 20 years working as an RN in an acute care hospital in Central Florida. The accumulated stress of preparing for Christmas 2013, my December retirement, and the onslaught of oak pollen season in Florida in February caused my body to revolt.
I have memories of sitting at the computer and feeling a small fluid-filled blister under my tongue. It wasn’t painful and did not burst. Eventually, though, my mouth became a haven for inflammation with large white areas. Due to my oak pollen allergy, I also suffered head congestion and drainage followed by eye irritation with redness and itching. I was taking my generic Claritin® with minimal relief. In early March 2014 I went to the walk-in clinic associated with my medical facility and was given nystatin, thinking oral thrush was upon me. With no improvement in a week, an ENT doctor prescribed a course of mouth rinses, oral antibiotics, and, thankfully, prednisone for my inflammation. A biopsy of a lesion on the inside of my lower lip came back only as negative for squamous cell cancer. Regrettably, I was yet to be tested for pemphigus. The only things I could put in my mouth were liquids. Protein smoothies were my friend. I lost almost 20 pounds — which I could afford to lose, thank goodness! As I weaned off of prednisone (50mg), the oral irritations and erosions returned.
By this point, I was also seeing a rheumatologist, who suggested that I might have Behcet’s syndrome after ruling out several other options with lab tests. We were getting close. At the end of July 2014, when I was up to 80mg of prednisone with oral improvement (able to eat roasted chicken!) my ENT doctor attempted to wean me off of the prednisone with only oral rinses for support. Well, my mouth took revenge and my extremities took notice with blisters on my back, arms, legs, groin, and chest.
I returned to the walk-in clinic. The doctor took one look at my mouth and body blisters, referred to her book, and said, “This looks like pemphigus vulgaris. I’m sending you to a dermatologist for a skin biopsy.” On August 6, 2014, the result came back positive for pemphigus. The clinic dermatologist, in the meantime, treated my skin blisters with triamcinolone cream, oral tetracycline, and oral prednisone (40mg). Since she was not familiar with PV treatment, she referred me to a dermatologist at the University of South Florida Medical School in Tampa with a scheduled appointment one month away. Unfortunately, the new doctor had to cancel the appointment because of a move to California! The best I could be offered was an appointment two weeks after the original one and with a third year resident. But by that time I was becoming anxious, and my husband and I decided to do some work on our own. My husband found the IPPF.
I placed a call to the IPPF, and the Patient Services Coordinator, Noelle, sent me a list of Central Florida doctors. Within two days of emailing one of them (Sand Lake Dermatology Center in Orlando), I had an appointment that confirmed the diagnosis of PV and was told by Dr. Allison Arthur to stay on prednisone (40mg). I was further prescribed CellCept (1500mg) to begin immediately.
Now it is the end of July 2015. I am taking CellCept 3000mg and prednisone 10mg daily. The skin blisters on my extremeties are long gone. My oral cavity remains irritated with inner cheek erosions, small blisters under my tongue, a slightly swollen and tender tongue, and with sticky saliva covering everything. I am able to eat soft, solid foods, taking small bites and chewing slowly. Of course, eating from a menu at restaurants can be tricky. I can’t bear fresh fruit, tomatoes, raw onion, or garlic. I can’t manage coffee or most common condiments.
Dr. Arthur suggested Rituxan infusion therapy as my next option in treatment. With the collaboration of my Watson Clinic dermatologist, Dr. Sharon Fairbee, I saw a clinic oncologist late in June, and my Rituxan therapy has begun after I was approved for financial assistance via the drug maker. My second infusion in late July went well. I have noticed slight improvement, noticeably on my tongue. Chewing seems much easier. The sticky saliva seems reduced. I am maintaining an upbeat attitude to keep my immune system happy! Hopefully my road back to health will continue without any traffic stops, and remission is in my very near future!
Toni Addy is a retired RN and PV patient living in Lakeland, Florida, with her spouse of 44 years. She became an RN after having a “mid-life crisis” and switching careers. She was previously a school teacher. Living with PV and sharing the need for better understanding of PV diagnois and treatment is her priority.
Toni Addy, BS, MA, AS, living in Lakeland, Florida is a PV patient and retired RN. She wrote her story for herself and other PV patients and families.