A RARE AND BAFFLING ILLNESS – CHURG STRAUSS SYNDROME!
This is the first time I have written about the very rare auto-immune condition I was diagnosed with in February 2012. I had been rushed to hospital a couple of weeks earlier after collapsing with a suspected heart attack. It was all 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 , nor is it inherited. It is extremely hard to diagnose, and variable in both presentation and progression. I like to call it Pandora’s Box. because so many nasty things can emerge! However, there are generally three stages. The first involves adult-onset asthma and allergic rhinitis. I was first afflicted with these horrors in my late 30s. Repeated surgery to remove nasal polyps is common although I got away with one operation. Many of us lose our sense of small and taste, which I have written about HERE.
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 can be life-threatening. For several years pre diagnosis I had been experiencing (and foolishly ignoring) episodes of what felt like vertigo. This was the disease active in my brain.
Several months after my original stay in hospital I was readmitted for surgery to remove fluid from around my heart. A permanent ‘window’ was created in the surrounding sac in case the problem should reoccur. It was quite a setback, but I recovered well.
Spending time in my Blue Mountains garden was very therapeutic. Nothing lifts a battered heart and brain as much as the tinkling chorus of fairy wrens, or the hearty laugh of a kookaburra.
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 mid 2012. Looking at it now, I can see that my face was already filling out due to steroid use. One of the great challenges for patients with CSS is to slowly decrease the amount required without causing a flare.
Against the odds, I was not only well enough to travel to the UK for the launch of The Water Doctor’s Daughters in March 2013, but to return for the launch of my second book in August the same year.
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 have some residual numbness in my feet, but otherwise I feel perfectly well. I am one of very few people who manage to reach this stage. Of course, there is no guarantee that I will not have a ‘flare’. However, my immunologist tells me that if this occurs it is likely to be less severe, and therefore reaching remission should be easier.
This specialist is very cheeky, with a sense of humour that I suspect bemuses my GP. Here is an extract from a review visit when we were building our house; ”I am writing about our friend Pauline, whom I reviewed today. She was unescorted because her husband was besieged by tradesmen and hopefully they did all the right things.’”
I belong to a wonderful CSS support group called Churg Strauss Friends. The group currently has over 500 members world-wide. We have chosen 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 to the miracle of a cure. Legend has it that when the beautiful Pandora so unwisely opened that box she released all the evils of the world. However, for sufferers of Churg Strauss Syndrome and every other incurable condition, there is something worth remembering. When she finally forced the lid shut, Pandora captured something hiding at the very bottom … the spirit of HOPE.
The impetus for our PR blitz was the death of one of our members, who died six months after diagnosis, aged just 44. RIP Kevin.
If you or someone you know has been diagnosed with this condition, please feel free to contact me via this site, or on my email address at [email protected]
UPDATE – JUNE 2019 I have now been free of medications and in full remission for nearly six years.