VF in the News

Bill’s Story

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?”

“Just when the caterpillar thought the world was over, it became a butterfly.”

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.

“The greatest pleasure in life is doing what people say you cannot do.” Walter Bagehot

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.

“Life is what you make it, always has been, always will be.” Grandma Moses

We have learned many lessons together:

·        According to the National Institutes of Health, 25 million Americans suffer from rare illnesses, many of which go undiagnosed for years. A Mayo Clinic study found that the true cause of illness had either been missed or misdiagnosed in 20% of patient fatalities in the ICU.
·        Therefore, do whatever is necessary to obtain an accurate diagnosis from a medical professional who knows and understands vasculitis, and is fully aware of the spectrum of possible symptoms.
·        Some doctors have difficulty admitting “I do not know what you have” or “My first diagnosis was wrong.” Since Wegener’s and other vasculitides can present themselves in so many different ways, do not hesitate to seek a second opinion, or a third.
·        Take control of your own health care as a top priority in your life. Nobody cares as much about your health as you and your loved ones.
·        Listen to your body. If you think something is wrong, there probably is something wrong. You are not a hypochondriac – the reality is you have a very serious medical condition that needs immediate and ongoing attention.
·        If you have the resources, complement the medical care you receive from your local team of doctors by visiting one of the national centers who specialize in the diagnosis and proper treatment of this family of diseases: Cleveland Clinic, Mayo Clinic, and Johns Hopkins to name three world class medical facilities in the United States.
·        A strong support network of family, friends, and clergy members is very helpful as you navigate the journey. These same people, who cheered you on during the difficult early times will also be there to share in the success when you receive an accurate diagnosis, start on an appropriate treatment plan and ultimately, when you achieve remission.
·        If available in your area, consider joining a local vasculitis support group, or even an internet chat room. Knowledge is powerful.
·        The Vasculitis Foundation is a valuable resource to assist in this process, as is their network of Area Contacts – people around the world who are living every day with one of these “orphan” diseases, just like you and me.

·        While not every day will be sunny….everything is easier with a positive attitude!

“And in the end, it’s not the years in a life; it’s the life in the years.” Abraham Lincoln

(Bill was diagnosed with Wegener’s Granulomatosis in 2006. He and his wife Maureen live in Laguna Vista, TX. Bill also serves as a Texas Area Contact for the Vasculitis Foundation.)

Published: May 2011

To read more stories, please visit the Vasculitis Stories landing page.