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!

Being encouraged to exercise by a sweet little freind

 

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.

Micrograph of eosinophilic vasculitis

 

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.

 

Recovering after surgery, April 2012

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.

The effects of steroid use.

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.

CSS AWARENESS CAMPAIGN MAIN PAGE

 

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