VF in the News

My name is Bill. Soon, the traditional antibiotic treatments no longer worked. My bouts with sinusitis became more severe and more frequent. From 2000-2006, I had three sinus surgeries – ethmoid, sphenoid and reconstructive nose surgery to correct a saddle nose deformity. The results were unremarkable and the infections continued. I was also treated for allergies for three years without success.

In September of 2006, I had an upper respiratory infection that was so severe; I had difficulty breathing, accompanied by regular bouts of drenching ‘night sweats’. My ENT admitted me to the hospital, where it was discovered I also had methicillin-resistant staphylococcus aureus (MRSA). The infectious disease doctors were called in. With no improvement over four days, my ENT did a surgical biopsy of my sinuses, along with numerous blood tests including a cytoplasmic-ANCA (c-ANCA).

It was only then, after at least six years of repeated upper respiratory problems and three apparently unnecessary surgeries, that I was finally diagnosed with Wegener’s Granulomatosis.   Bewilderment and fear took over.

What was this thing called “Wegener’s” all about? While in the hospital, I immediately “googled” everything I could find about this newly-diagnosed disease. When the rheumatologist visited me the next day, I remember saying, “I think I’m going to die. How much time do I have?”

While serious and potentially fatal (emphasis on potentially), he explained that recent advances in treatment made the prognosis good with a full life expectancy possible, providing the disease was managed and kept under control.   I was immediately placed on cyclophosphamide with prednisone, and vancomycin (via a PICC line) to treat the staph infection.

The recovery took longer than expected. I was out of work for six months, during which time and with great fortune, I was referred to Dr. Gary Hoffman at the Cleveland Clinic. After the first appointment in Cleveland, I felt confident that I was properly diagnosed and the treatment regimen was appropriate for my condition.   Imuran (azathioprine) replaced cyclophosphamide and I returned to work.

However, despite my best intentions, after four months I was unable to continue in my job with a large, multi-national company due to lack of energy, daily malaise and exhaustion after only minimal effort. I couldn’t handle the pace or daily stress levels. Air travel was particularly difficult. My life was forever changed.

My priorities changed too. At age 53, my career of 30+ years was over. I had a new goal – to successfully manage this illusive and often misunderstood disease, while at the same time attempting to maintain a “normal” lifestyle to the extent possible. Meeting with customers and reaching sales objectives were replaced by learning to understand and interpret sedimentation rate and C-reactive protein results. International travel to exotic locations was replaced by volunteering and community service, something I always wanted to do but never seemed to have the time.

Fast forward five years and I am pleased to report good, but not uninterrupted progress. Living with Wegener’s is an evolutionary process, not a singular event.   I have experienced three flares, each requiring an increased dosing of prednisone.   The introduction and recent FDA approval of Rituxan (rituximab) for the treatment of WG is a God send. I have had three prior Rituxan infusions with another scheduled for later this year. I am hopeful that the effectiveness of this product will make it possible to eliminate the daily dosing of Imuran, along with some of the undesirable side effects of immunosuppressant therapy.

I see my local rheumatologist (Dr. Paul Pickrell, Austin, TX) on a monthly or bi-monthly basis, in order to closely manage my clinical condition, and to look for any signs of inflammation. Blood work is done every 2-4 weeks. I continue to visit the Cleveland Clinic at least twice a year, and am very fortunate to receive outstanding medical care and guidance from Dr. Carol Langford, Director of Vasculitis Care and Research, and one of the most knowledgeable people in the world regarding the diagnosis and treatment of Wegener’s.

There have been setbacks, including the three flares, numerous chest CT scans to identify the presence of pulmonary nodules, and hip replacement surgery in 2010 – due in part of the regular use of steroids over a multi-year period.   Getting plenty of rest and sleep is essential.

Since being diagnosed, the last five years have been an unexpected, but stimulating learning experience for me, and for my wife Maureen. Her unending encouragement and support are a blessing every day.

We have learned many lessons together: