Living in Northern California with her husband Steve and dog Dexter, Caz was fully prepared to reap the benefits of retired life. After a career in healthcare, she was more than ready to dedicate serious time to gardening, reading, sipping a good cup of coffee and attending live theater productions. Or so she thought. Days after retiring at the young age of 55, and after being so unusually healthy as to not have used a single sick day in the past 20 years, Caz began experiencing symptoms that eventually led to her being diagnosed with EGPA. She sat down with us to share her story.
Q: What were the early warning signs that something was wrong?
It all started with sudden hearing loss. I awoke one morning with significant hearing loss in one ear. I figured it was congestion-related but still wanted to get it checked out when it didn’t immediately resolve. I saw doctors who recommended over the counter medications and agreed it would likely get better on its own. But after a month without improvement, I decided to have an audiogram and was shocked to learn my hearing loss was sensorineural and not conductive, and that it wasn’t going to get better.
Fast forward a few months and I had a bout of flu-like symptoms, then a sinus infection. Then I had another infection, and then another. It was odd because I’d never had sinus infections before. My doctors prescribed rounds of antibiotics and steroids, did chest X-rays and sinus CTs. I also saw an ear, nose and throat doctor (ENT), an allergist and a sinus specialist. Meanwhile, I started having coughing fits unlike anything I had ever experienced. I couldn’t sleep. I had to walk out of theaters and concerts because I couldn’t stop coughing. I lost my appetite and started losing weight — once seven pounds in a week. I felt so tired. I had ongoing communication with my physicians, but each individual symptom was commonplace, so they didn’t raise any alarms.
Q: What finally led to being diagnosed with EPGA?
The breaking point was several days of crazy back pain that finally got me to the emergency room. I was told that I had asthma, though several doctors up to that point had thought the coughing was simply post-nasal drip from the sinus infections. So, the staff did a bunch of tests. The back pain turned out to be pancreatitis, and I was admitted to the hospital where things began to go downhill quickly. I was put on oxygen because my pulse oxygen level was very low. Vague numbness in my fingers and toes quickly turned into significant immobility. I couldn’t walk, stand or even move myself on the hospital bed. But it still wasn’t clear what was wrong with me.
My complete blood count showed eosinophil levels were 31%. Normal is typically under 5%. My symptoms led me to be screened for autoimmune diseases and cancer. I was visited by neurologists, pulmonologists, rheumatologists and more who I envisioned brainstorming “Dr. House style” with lot of scribbles on a whiteboard. I had CTs and MRIs where they found granulomas in my lungs. Ultimately one of my doctors did a nerve conduction study and it was confirmed that I had vasculitis. Aggressive treatment began immediately.
In a lot of ways, I’m incredibly lucky. I was only in the hospital for three days before I was diagnosed with EGPA. Though it had taken seven months since my first clear symptom — hearing loss — before I had an answer to what was happening, many people with this disease go years without a clear diagnosis. The disease is so rare that many physicians may never see it in their career and many of the symptoms are so common it is difficult to pin things down.
Q: How has your recovery been?
At the beginning of treatment, I spent nine days in the hospital and another 12 in a rehabilitation facility receiving intensive physical and occupational therapy. It was not at all clear how much of my loss of function would be permanent. Thankfully, my response to medication was swift and over time I recovered a fair amount of what I had lost. I received my first chemotherapy infusion in the hospital and continue to take prescription medication three years later to keep the disease under control.
I’m feeling much better and feel fortunate to have achieved remission so quickly. But the lack of active disease doesn’t mean I am symptom-free. Medications have side effects and I still struggle with damage caused by the initial onset of disease. Ongoing difficulty such as hearing loss, cognitive deficit, neuropathy and fatigue makes my life very different than it was before.
The other big thing I will always deal with is the uncertainty of living with an incurable disease. I don’t know when or if my EGPA is going to flare back up. And when it returns, I don’t know how it will present itself — if it will affect the same organs as before or new body parts will be targeted. But I feel lucky to have had such a strong response to treatment and try to stay optimistic and focused on the present.
I’m well connected with support groups. I have met and connected with others who also live with EGPA and other similar illnesses. I have become active in the vasculitis community and continue to learn everything I can about my disease. I have a loving family and I’m back to doing many of the things I had hoped to do in retirement. EGPA has changed my life, but it hasn’t stopped me living it!
Learn more about EGPA at Lung.org/EGPA.
Development of this educational content supported through an educational grant from GSK
Blog last updated: November 2, 2020