Gill , Essex, UK, aged 45
I was a very active child, teenager and adult who exercised regularly each week until about 26 years old. I wasn’t sick from school much, apart from contracting measles around 9 years old and then from around 16 years old I had repeated attacks of tonsillitis and they were taken out when I was 20. When I look back, it’s difficult to say when it all went wrong, but in hindsight there may have been some early warnings of trouble brewing. At 15 years old, I went to A&E with chest pain and difficulty breathing and at this time this was dismissed, apparently if I was well enough to put make up on then I can’t have been that ill. As a teen, I also suffered from terrible mouth ulcers. I think I had more days with ulcers in my mouth than without, that’s what my memory tells me it felt like.
I always worked hard after leaving school, always striving for that better position. In my early twenties on trips to both Turkey and the Caribbean, I contracted awful stomach bugs or food poisoning. At around 26 years of age, not long after returning from the Caribbean, I contracted a chest infection which left me asthmatic and I ended up on inhalers for some years. I was now diagnosed with asthma and on the occasions I tried to stop the inhalers, it just came back.
Apart from repeated chest infections and asthma, life was still okay until around 2002 (33 years). I started to get regular problems with my sinuses. At this time, I had not long been in a brand new, state of the art (for the time), building. Various things were going on in my life – stress at work and I was getting married in 2003. In 2004, I accidently took the lift to the ground floor one day where they were re-tiling the floor and as the lift doors opened it was as if someone has punched me in the face with the smell of glue that hit me and it felt as if I was being stabbed in the nose. Why bother with this, you may ask. I have two friends from the same building, one started to get ill when they were working there and has now been diagnosed with mycosis fungoides, and another who a couple of years later after leaving got diagnosed with sarcoidosis. Maybe nothing but they are only two people I know of, who happen to be friends.
Between August 2003 (just after returning from the Seychelles) and April 2004 my sinuses got worse and worse. When I look back, and knowing what I now know, it never occurred to me that the repeated antibiotics I was being prescribed were just not working and it should have been investigated further. I started to get a cough.
In March 2004 I was coughing so badly, that on a commute to London, people were trying to move away from me on the train. I had to carry water with me everywhere. I was the only way I felt comfortable to try and alleviate my cough, which of course it didn’t. As I reached London, I realized I could not carry on and lead a whole day meeting and phoned my boss to confirm I was getting back on the next train out to go home. Stress from my job was at an all time high and in April 2004, I left my job with no other to go to. For the first time in 16 years I found myself without a job. From this time my ability to breath through my nose was gradually worsening.
I managed to drag myself to interviews in London and even secured myself a contract, even though I was coughing all the way through the interview which I found quite amusing. My ability to walk at speed was diminishing. Luckily, I was offered a contract closer to home and I started there in May 2004. By this time, I was trying anything to be able to breathe through my nose – Vicks inhaler, decongestants, and steam. I had to leave my friends walking to and from work because I could not keep up, in fact I could barely walk, not because of my legs but my lungs. When I got to both the office and car, I was going through copious amounts of tissues to cough up all the clear sputum that was being mass produced that I just could not get rid of. It was quite disgusting. My husband and I went on a beautiful walk to Pinetum in Kent sometime in June and when I was unable to walk up a slight hill it dawned on me that I was in real trouble and felt the end was close. I saw my GP and she suggested a private referral to a respiratory physician and this happened within a matter of days. That was the best referral I could ever have had. He also specialises in allergy. That consultant has been my rock along the way and boy have I given him some grief.
At his initial consultation from the chest x-ray, after about 20 minutes, he told me the mass on my lungs looked like possibly tuberculosis or lung cancer, the latter of which could be ruled out by taking steroids. Bloods were taken. I started on prednisolone, went back and the patches on my lungs had started to clear very quickly. The bloods showed raised Eosinophils, and he confirmed I had Eosinophillic Pneumonia (EP). Wow, what a difference that made but what I chose not to hear and take in and that time was I remember him telling me that this condition could lead on to another disease. Wish I had my ears pricked up a bit harder. I was just relieved to have a diagnosis that was not cancer and started to feel more human again. The treatment was steroids and since that date I have been taking a seretide inhaler. I had a second case of EP some months later. Sinuses were still causing problems.
In 2005 I became pregnant, now off medication, and had my first child in 2006 and my second in 2007. My bloods were referred to my consultant during pregnancy because they were concerned I might have had AIDS, because my CD4 count was abnormal. Luckily he was able to rule that out. My consultant had predicted that during pregnancy I might feel very well and of course I was. I had not felt so well in ages. Pregnancy and birth were wonderful to me but to stay in that permanent state I would have a huge family and probably be bankrupt by now.
When my second child was 11 months old, I started to feel very fatigued again. Sinuses an every day burden but I think I just got used to the pain. We discovered some mold in our house, which I believe made my sinuses worse and in late 2009, I was rapidly going down hill. The ability to do things was diminishing and so frustrating then I started to get pins and needles in my arms, legs and feet. I was fortunate to be able to ring my consultant’s secretary and on this visit he confirmed that he felt I had now moved on to Churg Strauss Syndrome. At this point I met 5 of the 6 criteria for diagnosis. The only one I have not met is biopsy but most of my problems are lung related and he felt it too invasive.
Well I cried for 3 weeks after reading about it on the internet, assuming I would be dead within 5 years. I put my consultant through hell. I researched every other possible disease linked to eosinophillia and pulmonary infiltrates that I could find and asked him to confirm to me that none of these matched me. I was even paranoid that I had only fallen ill after being abroad and had I contracted some awful parasitic disease. I even requested a second opinion at the Royal Free, who confirmed the same diagnosis. In the end I thanked him for being patient with me to which he responded it was a pleasure. I guess I was in the denial phase for a long time. All my allergy testing was negative and I am also ANCA negative. In 2010 I was again on steroids but no immune suppressants at this time.
When I tapered off the steroids, I was soon chronically fatigued again. In 2012 my cough was getting worse, although this time a slightly different cough. I didn’t really have any pain, except I was getting more occular migraines and my eye socket on one eye was itchy and swollen. Lying down on my back was slightly uncomfortable. Sinuses as usual were a problem. I went back to see my consultant and the chest x-ray again showed pulmonary infiltrates but also he and I noticed the shape of my heart. I was immediately referred to the heart ward there and then and was given an ultrasound. This time it was a pericardial effusion. So from then on I was seen by a Respiratory Physician, a Rheumtatologist and a Cardiac Consultant. Apart from the cough, this condition did not give me any symptoms at all. So back on the dreaded prednisolone again and I was also started on MMF.
As of today, I am now down to 1mg of steroids and 2000mg of MMF (Mycophenolate mofetil) . I am, according to the doctors, officially in drug induced remission. After 3 years, assuming I don’t go in the wrong direction, they will try and reduce the MMF very slowly.
I know I have been saved any nerve damage so far, even though I am at stage 3, by being referred to the correct consultant for my symptoms at the time. This, I believe is fundamental to everyone’s right direction on their journey forward in receiving the appropriate treatment. I was not coping mentally until I reached acceptance, again I think this takes each individual a different amount of time to come to terms with but makes the journey easier once you have reached this milestone. I have learnt to accept my life will never be the same again. I am no longer regularly under a cardiac consultant but see an ENT instead. I don’t have any polyps, have only a slightly deviated septum and the nasal lining is currently is much less inflamed than it was in 2010. I have also persevered with nasal steroid drops and sprays, which has helped. Because of my deviated septum I have not been able to master the art of nasal rinsing.
Although my consultants are not vasculitis experts, they do have seminars with the specialist doctors who are and at least they know what vasculitis is. Every time I see my consultants, I take brief notes and try and impress them with my knowledge, well let’s face it you don’t get diagnosed with a rare disease and sit back and just do nothing. I know my relationship with them and my GP have helped me greatly.
Obviously, being on MMF has not been without problems. My hair is much thinner and dull and dry. I keep catching infections and after a trip to Greece last year, I ended up in A&E with a non specified infection that I had to fight for antibiotics for. I remember thinking in my head, when they were scratching their heads after ruling out a deep vein thrombosis and not even knowing what MMF was – “just give me the damn tablets, I know I am not showing a fever, but I am on medication that might be masking it”. In the end I ended up getting some tablets and came away with a 2850mg a day dose of two antibiotics for 7 days. Within half a day I was back to normal – well almost apart from the effect of the antibiotics. That’s what you get for asking for it.
I feel very lucky that I am not as badly affected as some of other people with this awful disease. I am not religious, but I believe only those strong enough to deal with it have been given this awful fate. The only thing I get sad about is not having had enough energy for my children but my husband is good with them. I worry of course about passing something onto them as the maternal side of my family are riddled with auto immune conditions.
I am learning to listen to my body when it is telling me something is wrong and I have finally learnt when to sit down and rest. I do part time work two days a week and have set up my own little giant bauble business because I know I can never do the long hours that I used to. I don’t bother trying to explain my disease to people who aren’t really listening because they usually only offer advice on how to boost my immune system which is really what I don’t want to do. Only my few good friends understand. Some people think I am lazy but my “in the mind” response to that is they want to look at themselves and I just ignore their cruel comments. I’m still alive and I am fighting. I still have a sense of humour thank goodness.
Kevin Selig was diagnosed in January 2014 with Churg-Strauss Syndrome and fought a courageous battle against the disease. Kevin passed away on Saturday, June 21, 2014, at the Mayo Clinic Hospital in Jacksonville, Florida.
We in the CSS community were saddened by the loss of such a vibrant human being and reminded how our illness often goes undetected until too late. Glenys , a very brave CSS warrior, set us into motion to bring awareness to our disease with a challenge to raise awareness of Churg Strauss Syndrome by posting a story on the the 1st of every month in an effort to let the world know we exist and we want to help others obtain earlier diagnoses.
Posted in CSS by karen in wonderland with 1 comment.