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Vasculitis Webinar

Managing vasculitis: A patient’s perspective

In March 2017, I recorded this webinar for the Vasculitis Foundation’s Road Map to Wellness webinar series.  In the webinar, I talk about my diagnosis with EGPA/Churg-Strauss vasculitis and the first few years of my treatment course. I also talk about some of my approaches for tracking symptoms, working with doctors, and strategies for managing vasculitis. https://youtu.be/iYQWI1Wtruo For more about my disease course.  For a detailed summary of my diagnosis with EGPA/Churg-Strauss Syndrome, see here.  For a  summary of my treatment course through April 2018, see here.

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EGPA diagnosis

Step one: My Diagnosis with EGPA/Churg-Strauss Vasculitis

Below is a summary of the events leading to my diagnosis with EGPA/Churg-Strauss Syndrome, a rare form of vasculitis, in 2014.  Looking back, I can see worsening symptoms developing for about a year prior to my diagnosis.  Getting a definitive diagnosis can be a long and difficult road for many vasculitis patients.  I know how lucky I am to have gotten a diagnosis, and in a relatively short period of time. Before EGPA/Churg-Strauss Syndrome. Life was good before EGPA/Churg-Strauss.  I was active, worked long hours, and was always on the go.  I never got sick, never missed a day of work.  I was able to do anything and everything I wanted to do.  Then a series of strange things started to happen… Adult-onset asthma.  As a kid I was pretty healthy. I had the occasional stomach bug or respiratory infection, and some seasonal allergies, but nothing serious and no major illnesses or hospitalizations.  In my thirties, I developed asthma and a persistent annoying cough, and my allergies evolved into year round stuffiness and congestion.  My asthma doctor gave me every form of inhaler, nasal spray, antihistamine, and decongestant on the market but none of them seemed to help much. I just dealt with it. Laryngitis and vocal cord damage.  One day, I got an upper respiratory infection and an acute case of laryngitis.  I remember exactly when this happened because I was at a research conference in New York and had to give a presentation in a near whisper from the podium.  I made it through the talk, but my voice did not recover completely afterwards.  I struggled with my voice while teaching during the next semester, then I sought out an ENT who was a vocal cord specialist over the summer of 2013 to see what was going on.  He visualized my vocal cords using a camera (by laryngoscopy – not fun!) and he told me he could see exactly what the problem was – my right vocal cord was partially paralyzed!  He said this was due to a problem with my vagus nerve which controls the vocal cord nerves. My right vocal cord was too weak and would not line up with the left which created a big gap between the two cords that was making it difficult for me to speak.  I had MRIs of my brain and neck to rule out any signs of compression of the nerves controlling my right vocal cord such as a tumor or other anatomical problem, but he could not identify what was the underlying reason for the paresis. The MRI did, however, show signs of chronic rhinosinusitis, which I shrugged off.  He performed surgery (medialization thyroplasty) to insert an implant in my right vocal cord in order to reposition the cord to improve my voice.  The surgery definitely improved my voice, but I continue to have problems, especially in low humidity and in the wintertime, sometimes when I’m having a flare, and when I need to project my voice in noisy environments or large classrooms. Lung problems and skin rashes.  A few months after my vocal cord surgery, in the fall of 2013, I started to develop lung congestion.  I kept thinking it could be a respiratory infection, or seasonal allergies, but the congestion persisted and increased to the point where I was coughing up quite a lot of sputum all day long.  At the same time, strange rashes appeared on my lower legs, which I now know were purpura. My asthma doctor gave me a course of antibiotics but the congestion did not improve.  When she gave me a short course of prednisone (my first time ever taking prednisone), I felt relief within hours and the lung congestion cleared up immediately.  The congestion quickly began to return a few weeks after I finished the course of prednisone. Bronchiectasis.  After taking a second antibiotic which did nothing for the congestion, my asthma doctor ordered a chest CT.  The chest CT showed I had bronchiectasis, an unusual lung condition, which is a form of COPD.  When I read about bronchiectasis I found that it is caused by chronic inflammation and can result in permanent damage to the airways and loss of lung capacity.  Since I am in a big city with several university hospitals, I was able to find experts in bronchiectasis nearby.  Coincidentally, the pulmonologist who specialized in bronchiectasis was at the same university as my ENT/vocal cord specialist. Time to get serious.  At this point, in March of 2014, a lightbulb went off in my head.  I had spent the last four months with persistent lung congestion and shortness of breath, as well as just feeling plain lousy all the time.  Antibiotics did not help but the prednisone worked wonders, even though the congestion kept coming back as soon as I stopped the prednisone.  I knew there was something really wrong with me and I needed to get to the bottom of it.  I would spend the next six months learning everything I could about my symptoms and going back and forth between different specialists for lots of tests. Eosinophilia.  The lung doctor ran a whole bunch of pulmonary function tests, sputum cultures, imaging, and bloodwork, including screening for cystic fibrosis.  Other than some mildly reduced lung function, everything came back normal except for one thing – my blood count showed very high levels of eosinophils.  Eosinophils (the “E” in EGPA) are a type of white blood cell which are important for controlling allergies and parasitic infections.  This turned out to be a very important clue since eosinophilia is unusual and there are a limited number of conditions that can cause high eosinophils.  The lung doctor continued with additional tests and referred me to an immunologist to help track down the cause of the eosinophilia. Peripheral neuropathy.  A few months later, in August of 2013, I began experiencing pains in my right wrist and hand, as well as numbness and tingling in my hands and fingers.  I started dropping things

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