living with vasculitis

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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. 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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Living with #raredisease EGPA: Eosinophilic Granulomatosis with Polyangiitis

Disclosure:   I received a small sum from GSK for shooting this video, which I donated to charity.  For more details and a full list of disclosures, please see the Disclaimer page. This 2 min video was recorded at GSK’s offices at the Navy Yard in Philadelphia last month as part of their educational efforts about EGPA/Churg-Strauss vasculitis.  It was filmed for a social media campaign to raise awareness about living with rare diseases like EGPA. Thanks to GSK for their outreach and educational programs on EGPA and other rare diseases, and many many thanks for their work and dedication in developing new treatments for eosinophilic disorders, including EGPA. From GSK’s website: Meet Jennifer: A science researcher whose quest became her own health As a biomedical researcher at a major medical institution, Jennifer’s happiest days are spent reading, learning, and working on discovering something that will help improve people’s health. While her research includes rare diseases, she never imagined that she’d one day put her skills to use on herself, to unlock the strange mystery going on inside her body. Aside from having allergies, Jennifer had a healthy childhood. As her allergies became progressively worse in her 30s and 40s, she also developed asthma. Then things started to get strange. She developed a host of respiratory symptoms, skin rashes, headaches and something called eosinophilia, which is a type of white blood cell in allergy and asthma, the levels of which were unusually high in Jennifer. It had taken Jennifer six months of testing, visiting multiple specialists including Respiratory and Immunology specialists when a Rheumatologist finally diagnosed her with a rare disease, Eosinophilic Granulomatosis with Polyangiitis (EGPA), formerly known as Churg-Strauss syndrome. EGPA affects about 5,000 Americans. It is characterized by asthma, an increase in eosinophils, and inflammation of blood vessels (vasculitis). “When I was diagnosed, I felt very relieved, but then quickly realized I was in this very serious situation. I had this rare disease, there was no cure, and I was going to have to face this for the rest of my life.”  -Jennifer Today, Jennifer plays a proactive role in managing her disease with her healthcare team. “My role in the team is that I am the quarterback,” said Jennifer. “My Rheumatologist is the coach and the other specialists are special teams. I am the one that has to get in there and make the plays, take the hits, and I have to get back up and get back in for the next play.” To help her prepare, Jennifer makes clear notes of what’s going on in her life from what’s working well to what’s not and she brings a summary to her doctor’s appointments. She makes sure that if she feels there’s something wrong, she raises the issue so that it can be addressed. Having a support system around her also helps her stay healthy. If Jennifer could offer advice to other patients, it’s the following: “I think it is really important to take the time to reach out to the people who are supporting you,” said Jennifer. “We know when we’re not feeling well, we know when we suspect a flare, and we know when a doctor is listening to us.  Build the people and team around you that will help you to move forward, because you can get there. Pay attention to your gut and if you are not feeling well, keep pursuing the answers.” You can view the post and video on GSK’s website at the following link:

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Tapers, treatments, and flares: My treatment course for EGPA/Churg-Strauss Vasculitis (Oct 2014 – Apr 2018)

NOTE:  This is not a typical treatment course for patients with EGPA/Churg-Strauss (not that there is such a thing as a “typical” EGPA patient!).  It has taken several years and much trial and error to find a combination of medications to try and keep my immune system under control and it’s a work in progress.  One of the most critical factors is to work with clinicians who are experts in treating patients with your form of vasculitis, especially for rare forms of vasculitis like EGPA/Churg-Strauss.  If you are newly diagnosed or having setbacks in your treatment, please do not be discouraged by all of my ups and downs.  Instead, I hope what you will see from my treatment course is that: 1) there are always more options for treatment; 2) progress is possible if you work with experienced clinicians and develop a treatment plan you can commit to; and 3) have some patience, it may take a while. Disclosure:  I was a patient in a GSK-sponsored clinical trial from March 2015 to May 2016 testing mepolizumab in EGPA/Churg-Strauss patients. For more details and a full list of disclosures, please see the Disclaimer page. Flare #7.  I am in my seventh flare since I started treatment for EGPA/Churg Strauss three and a half years ago, so I thought the Zen proverb above suits the occasion.  Below is a summary of my prednisone tapers and vasculitis flares, and the steps taken to find a combination of immunosuppressive treatments to help control the disease activity.  I’ve summarized all of this information in the graph to give a better sense of the literal ups and downs that I’ve been through during my treatment so far.  Even though, as my rheumatologist says, “past performance is no indicator of future results”, I’m pretty sure that this is not going to be my last flare. And that’s okay. Figure legend.  Treatment course showing prednisone tapers, clinical symptoms, disease flares, and treatments received from October 2014 – April 2018.  The graph shows my prednisone dose (mg/day) in red.  I scored my clinical symptoms using the Birmingham Vasculitis Activity Score (BVAS) and BVAS scores are shown on the graph in black.  The dotted line across the graph shows the level of 20 mg prednisone per day – my flares always occurred when I reached this point during my first two years of treatment.  Flares are noted by red arrows at the top of the graph.  The addition of methotrexate, mepolizumab, rituximab, and IVIG treatments are shown at the top of the graph.  Note that the first three mepolizumab injections were received while I was enrolled in a double blind clinical trial in which I was randomized to the placebo control group so these injections were saline only, but subsequent monthly mepolizumab injections are the actual drug. Diagnosis.  I was diagnosed with EGPA/Churg-Strauss Syndrome, a rare and potentially life threatening form of vasculitis, in the fall of 2014.  At the time, I thought that getting the diagnosis was going to be the hard part.  Boy, was I wrong.  I was diagnosed by a rheumatologist who is one of the world experts in EGPA/Churg-Strauss and just happens to be located in Philadelphia where I live. My symptoms included chronic rhinosinusitis, adult-onset asthma, pulmonary congestion, bronchiectasis, eosinophilia, mononeuritis (vocal cord paresis), peripheral neuropathy, purpura, hip joint pain, fatigue. Except for eosinophilia, my bloodwork was otherwise normal for inflammatory markers or antibodies, i.e., a normal ESR, normal CRP, and a negative ANCA titer. Initial treatment approach – prednisone, azathioprine, methotrexate.  I was extremely fortunate to get an early diagnosis so I had only suffered some mild nerve and lung damage, but the potential for further damage to my lungs, nerves, and possibly other organs, was high.  For this reason, my rheumatologist felt it was important take an aggressive approach to control the inflammation.  I would begin a treatment course familiar to many patients with vasculitis – starting with high doses of prednisone, a very powerful and very fast acting anti-inflammatory corticosteroid, followed by the addition of a second drug for maintenance immunosuppression, such as azathioprine or methotrexate. The prednisone would then be tapered gradually once the second drug was on board to control further inflammation. I began at 60 mg prednisone (a target of 1 mg/kg is the typical induction dose and I was 52 kg).  I also started taking Bactrim to help prevent any possible respiratory infection due to having a suppressed immune system.  Azathioprine was then added, but had to be discontinued within a few weeks due to severe GI intolerance.  We switched to methotrexate and chose to use weekly subcutaneous injections to minimize possible GI side effects. After reaching maximum therapeutic doses of methotrexate for a month, we began tapering the prednisone.  When I got down to 20 mg per day of prednisone, I had a flare, meaning that symptoms of active disease returned which included peripheral neuropathy, hip joint pain, fatigue, and purpura on my lower legs.  Due to the flare, my prednisone was increased to 50 mg per day for one month. Mepolizumab clinical trial.  At this point, it seemed the methotrexate was not enough to control the vasculitis, so my next option would be to add a stronger immunosuppressant such as rituximab, which depletes B cells.  Another alternative was an experimental drug, mepolizumab, which was being tested in a Phase III clinical trial to specifically target the eosinophils (the “E” in EGPA).  My rheumatologist was participating in the trial and offered me a chance to enroll. This was the first trial of its kind for EGPA.  It was a randomized double blind study, meaning that I would be randomly selected to receive mepolizumab or placebo control (saline) but neither my doctor nor I would know whether I was receiving the drug or the placebo control. After discussion with my rheumatologist, I decided to enroll in the trial for three reasons:  1) because the previous clinical trials testing mepolizumab in asthma patients showed a good safety profile

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