July CSS Awareness Post
Pauline Conolly’s CSS post can also be found on her web page, Pauline Conolly, ‘writer, historian, lover of books and birds’
A RARE AND BAFFLING ILLNESS – CHURG STRAUSS SYNDROME!
This is the first time I have written a blog about the very rare auto-immune condition I was diagnosed with after collapsing in January, 2012. It was a huge shock, especially as I had just signed the contract for my first book.
Churg Strauss Syndrome (CSS) is a form of vasculitis (inflammation of the blood vessels) . It was first described by Jacob Churg and Lotte Strauss at New York’s Mount Sinai Hospital, in 1951. Coincidently, this was the year I was born. Recently the more medically descriptive (but impossible to pronounce or remember) name of eosinophillic granulomatosis with polyangitis (EGPA) has been preferred, though certainly not by me! The disease in not contagious and nor is it inherited. It is extremely hard to diagnose and variable in both presentation and progression. However, there are generally three stages. The first involves adult-onset asthma and allergic rhinitis. Repeated surgery to remove nasal polyps is common.
In the second stage, white blood cells called eosinophils become elevated and can cause insidious damage to organs such as the lungs and digestive system. Despite having smoked for many years in my foolish youth, my lungs somehow escaped unscathed.
The third and most serious stage leads to neuropathy (nerve damage) and full-on vasculitis. If the heart and brain are affected, as in my case, the condition is life-threatening. Several months after my original stay in hospital I was readmitted for surgery to remove fluid from around my heart. It was a setback, but I recovered well.
At this point, there is no cure for CSS. Worse still, the medications used to control it and to achieve remission include steroids and powerful immunosuppressants. Both drugs have severe side-effects. Prolonged steroid use leads to puffiness of the face , weight gain and loss of bone density . Immunosuppressants leave the body vulnerable to infections and cause hair loss. Changes to body image are difficult to cope with , especially when insensitive remarks are made. Naturally the great hope among medical experts and patients is that a cure will be found, or that less toxic drugs will be developed. Happily, there ARE new treatments on the horizon.
The following picture was taken as a publicity shot for my publishers. Looking at it now, I can see that the shape of my face was changing.
I consider myself extremely fortunate. Like many patients, I was already in stage three at diagnosis, but most of the damage that had occurred was reversed. After 18 months of treatment I achieved full remission and am now off all CSS medication. I am still more easily fatigued and have some residual numbness in my feet, but otherwise I feel perfectly well. I am one of the few people who manage to reach this stage. Of course, there is no guarantee that I will not have a ‘flare’. However, my specialist tells me that if this occurs it is likely to be less severe, and therefore reaching remission would be easier.
I belong to a wonderful CSS support group, which currently has over 450 members world-wide. We have chosen July 1 to speak out about our condition in an effort to raise awareness of what is a debilitating and still sometimes fatal disease. Increased awareness will lead to greater research and perhaps the miracle of a cure. The impetus for our PR blitz was the recent death of one of our members, who died six months after diagnosis, aged just 44. RIP Kevin.
I am currently collaborating with several other people on a book about Churg Strauss Syndrome, to be edited by US group member, Amy Launiere.
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