By Graham Shore
My name is Graham Shore. I was born in Sheffield, UK, and this is an account of my experience of Churg-Strauss Syndrome (CSS). Note that not all incidences of CSS are anywhere near as severe as my particular experience.
I was born 27th December 1961. My earliest recollections of health problems are the usual run of measles, chickenpox, mumps but also severe anaemia. Apart from this, I was remarkably healthy and never even suffered with hay fever, let alone asthma.
Around 1989/90 I started to have sinus problems; I put this down to moving in with a girlfriend who had a dog and I guessed it was some sort of allergic reaction. Anyhow, back in my own house and things got worse; after walking home one evening in very cold air I had my first real breathing problems, but these were gone in the morning and I thought no more about them.
The breathing problems got much worse over the next few weeks and I ended up not only with asthma but I also lost a lot of weight (down from 82.5 Kg (13st) to about 63.5 Kg (10st) and was totally without energy. It was 1992, and the doctors were perplexed.
Fortunately, I have lived in two great areas for UK hospitals, Sheffield and latterly Redhill then Leatherhead, near London. At this time in Sheffield I was the talk of the medical practice because nobody had the slightest idea what the problem was!
I had a chest x-ray, and immediately ended up in out-patients ward of the
Royal Hallamshire Hospital for an examination. The radiographer thought the film was fogged and had to take the image again; the consultant said it looked as though a bomb had gone off in my chest. I was jaundiced too - bright yellow and my boss thought I had cancer.
I was admitted for a couple of nights, so that an exploratory bronchoscopy could be carried out. They needed to know what was actually in the lungs; they did this by removing a tiny bit of the inside of the lung.
Now, the diagnosis for all this was a rare syndrome known as Loffler's syndrome, described on the net as 'mild'!! High doses of prednisone and some rest saw me make a full recovery. However the sinus problems came back again and in a couple of years I had to be admitted for an operation to remove nasal polyps.
So, operation over I thought that was the last of it for a good many years. Wrong. The asthma attacks were severe, sometimes requiring time off work, and of course very uncomfortable.
One evening while lying in bed after a very bad day of asthma, my heart stopped, or at least it felt that way. Both my arms felt totally dead, just like two lead pipes attached to my body. I juddered and came to, but it was very frightening. The next day my partner Sue took me to hospital and they admitted me straight away when I recounted the story. The staff at the hospital thought I had developed the initial stages of ischemic heart disease, and were sorry to ‘tell me the bad news’. But my ankles were not swollen, and I didn’t exhibit any other symptoms of heart failure. Odd. So I left the following day and started to eat more healthily and even to run a little, which did help until I sprained my ankle and that had to stop.
Another year down the line and its 2006. I started a diet around April time. I had had two weeks off work with a bad chest infection and asthma, and by now was getting royally p****d off with the situation (as were my employers). I took drastic action to loose weight; I went on a carbohydrate free diet (not Atkins) and got totally immersed in feeling better about myself by losing weight. That’s right, a 44 year old male with anorexic tendencies. I lost about 19Kg (3 st), but felt good, hardly any asthma at all. Then the asthma vanished completely. Pleased with myself, thinking I had conquered my condition single-handedly, I could have stopped dieting, but I didn't. Not only that, but I watched my weight begin to really plummet. This was getting silly - about 1Kg (2-3 pounds) lost each day. I was getting weaker too - I noticed I couldn't carry the shopping bags back to the office, and had to start using the lift again (breaking my new years resolution!). It was time for some more sick leave, and a visit to my GP. He didn't think that much was wrong, and was even patronising in my opinion. However I went back a few days later and demanded something be done. He could tell something was wrong this time, and got on the phone the local hospital (East Surrey in Redhill) and I went along to the A&E - they were primed as to what to expect. Fortunately, at the time I lived literally next to the hospital. I had a long wait sat on a hard plastic chair, then a chest x-ray and some blood was taken from an artery in my hand; the result came back that I had had a 'Silent MI' - a heart attack. I was admitted to the Coronary Care Unit (CCU) that evening.
Not feeling too bad by now, but mildly shocked by my predicament, I spent a day in hospital more or less recovering then saw several visitors the following day. My readings were good - sats were up, blood pressure ok too; so I was alright if a bit weak.
It was a Sunday afternoon; my friend Lisa had been to visit for about an hour, then went off to see John’s flat, a mutual friend who lived in the same block of apartments as me. I can’t remember if there was any time in between, but Linda, the receptionist from work, came to visit me. We chatted and everything seemed fine; I was doing well as far as the readings on my monitors were concerned, and pretty soon the nurse came round (Keith I think) and declared that I was doing well and shortly after another nurse came round to say that I could actually go from the CCU ward onto the less dependant Holmwood ward, with patients who were mainly in for stent’s and so forth.
I couldn’t walk very well; I was shaky when I stood up and Linda had to hold my arm and escort me into the other room (ward). She was disgusted by the fact that my belongings were summarily dumped on my bed and there was no help to move to the next ward. Anyway, in the next ward I soon settled down and chatted to Linda until Mac and Ralf came to visit. This was very pleasant and we chatted for some time about lots of things; I remember Ralf recounting what his arrhythmia experience was like and I hoped I didn’t suffer a similar fate. One of the things he said, it might not have been at this particular time, was that it was like the long fall on a big dipper, but without the slowing down and stopping at the end! He said you just kept on going, and it was terrifying. After they left I had to wait a long time for some dinner (I think they had forgotten me) and then I asked one of the nurses if I could have a bath. This was the first time I had met Barry (Baz), a real character but underneath it all a genuinely nice guy. He hummed and arred for a while but eventually agreed and led me to the little bathroom. The bath was some large hospital type bath, with very easy access. Although I was weak and clearly not very well at all, it was comforting to get clean and relax off the ward for a while. When I was finished I felt better for the bath, but not better in general; in fact I felt quite a bit worse. I didn’t sleep at all well that night, and I had to get up in the middle of the night and go for a walk to get some tea. I can remember not feeling well at all; I could hardly walk and I had to lean heavily on the nurse’s desk while I was asking for the tea. I went back to bed and slept fitfully for a while; I was woken about 7:30 by the general bustle and chatter in the ward. The guy across from me was talking to someone at the opposite end of the ward so he had to shout; I nearly complained but in the end decided to let it go.
However, a few minutes later I took a turn for the worse, and I can remember sitting up on the side of the bed, crouched over. I said to the noisy but kindly chap next to me that I wasn’t feeling too well; he said “Shall I get the nurse?”, to which I replied “I think so” and he put on his dressing gown and went off to find one. The next thing I can remember is that quite a few nursing staff were busily preparing to move me, and one nurse asked if I would like some morphine to ‘take the edge off things’. I said yes; I think it was 250ml. Barry, the male nurse, said “I knew we shouldn’t have let you have that bath”.
Then I was carted off to a side room and filled with some drugs, another nurse asked if my next of kin could be contacted; this was to become a familiar question in subsequent weeks. I always replied yes, and didn’t think any more of it; after all I didn’t have a clue what was happening. However I did say to one of the nurses “I’m poorly aren’t I?” and she replied, “Yes Mr Shore, you are very poorly indeed”.
I held onto the steel guard adjacent to the bed, and thought to myself “well, I am at peace, and I am not in any (apparent) pain. If this is it, then it’s not a bad way to go.” At that point, I believe I was given the choice of life or death; I can’t remember this as a conscious thought, but that’s how I felt. There was no terror, no trauma; it was a concentrated feeling of living in the moment, a nexus of life experiences; it was almost beautiful.
Having survived this I found myself in intensive care and everything had moved up a gear.
I was sent to the Intensive Therapy Unit (ITU) and given Cytoxin (cyclophosphamide) and put on a machine called a CPAP, which helps to relieve strain on the heart. This carried on for a while, perhaps a week. I was very weak, could hardly move, and the descriptions of life in Ben Watts book “Patient” fit me perfectly.
I was doing well-ish and ended up on the CCU again, but worse was to come, much worse. I arrested in my sleep and woke up to find several nurses and doctors buzzing around me. A central line was fitted into my neck. They didn't really know what to think, and were in constant consultation with Royal Brompton Hospital where the first suggestions that it might be CSS were mooted. The eosinophil count was sky high, and my temperature elevated. They were disappointed; they said I was "doing so well" and talk was of putting me on a ventilator. A few more days in ITU and I was put back into CCU, same bed, same spot near the window, quite nice really....
One day I felt very poorly. It was a Wednesday and some friends came to visit me. They were shocked. One of them said "Graham, I have had a full blood transfusion, if I can survive that you will get through this". I thanked him for his good-natured comments. Later, some more visitors; one chap held my hand but I was drifting off to sleep; the last thing I remember was he squeezed my hand and left. There were nurses and various medical staff lined up along the corridor; I was moving past them, slowly at first and they acknowledged me but no more than that; the pace crept up and I was moving faster and faster past them, until I was in a spin and the whole scene was out of control. Oh! I shouted and jumped upright from my bed. I had been shocked back to life by two pads stuck to my chest; the chest hair was burnt and I was breathing rapidly; several nurses and the doctor were at the foot of my bed. Back on the corridor, the staff lined up. I moved past them and again was acknowledged. I've been here before, and it started to get faster and faster and the room span and Ahrrh! I was jolted back to life again, same thing, doctors, nurses staring intently at the readings on the machines behind my bed. Back on the corridor, same story, medics lined up and moving past them ever faster till Arrrh - jolted back to life. This time I remained conscious.
A WONDERFUL nurse called Shoona, every inch the consummate professional, had taken hold of my hand and was gently rubbing it; it was so comforting I can't begin to express my gratitude.
The staff were watching my machines intently, looking for signs of stabilisation. "Ok Mr Shore, you've had an arrhythmia storm, but you seem to be stable now” the doctor said. A few seconds went by. Initial crises over; the heart had been so weakened by the CSS it had failed; it was "fat and floppy" as one cardiologist put it. “We are going to put a temporary pacing wire into your chest, so we will need to take you to the procedure room”. Great, I thought. Then came a long explanation about the dangers of the procedure, which made me snap back “JUST DO IT!”. I apologised, and the doctor said “Mr Shore it’s perfectly understandable under the circumstances”. Still, it’s never a good idea to upset the one person in the world who is about to save your life.
So, we went (my bed was being pushed) to the procedure room. I wasn’t scared or anything; I knew it was serious but I was looking forward to the pacing wire going in so that I would remain stable. We reached the room and I was prepared; I was placed under a large x-ray machine and some iodine was dabbed on my chest; the operation was carried out under local anaesthetic. The doctor remarked “you are almost the perfect patient – you just lay back and let it all happen”, to which I replied “I haven’t got any option”; not in a nasty way just as a matter of fact. She was brilliant, young and very, very good at her job; she even tutored a student doctor on the procedure while it was happening; he asked all the right questions and she told him it was a good skill to have (Good! Bloody marvelous I would have said!). So, the small operation over and I was wheeled back into the CCU. It must have been about 5 in the morning.
Two friends came to see me, Shaun and Kathleen.
Kathleen had been tremendous throughout my stay in hospital, a professional nurse herself, she had run errands for me, checked the medical side of things with the staff and had generally been an angel. Amazingly, she was moving into new accommodation so was living with a friend just around the corner from the hospital during my illness! The great doctor who operated on me had got to know her well and had phoned her for ‘tea and biscuits’ – a euphemism that I was in imminent danger.
Shaun is a great friend and confidant; a hard man athlete and rugby player, he always seems to know the right thing to say. They walked in, one either side of my bed, both silent for a moment. Shaun knew what to say: “Graham, you look scary”. I half smiled, clearly seeing that he was concerned I said “Don’t worry mate”, but it was too late for that, he was very upset. Kathleen was too, but used to dealing with these situations she took a more pragmatic approach. “GRAHAM! Enough already!” she half shouted at me. Then she proceeded to plead with me to tell my sister to make arrangements for my death, even though “God forbid anything like that should happen.” It was true; I had purposely kept my sister a little in the dark, and not told my elderly parents anything at all, save that I had had a bad asthma attack. After that the conversation was brief, and they left me to get some rest. I thought it was early evening still, never thought to thank them for getting up in the middle of the night.
A nurse appeared and asked if I wanted a sleeping tablet, Zopiclone, which usually did the trick. I admitted I was frightened it might spark off the arrhythmia but she assured me it wouldn’t, and anyway I was in exactly the right place if anything did happen. I drifted off till morning, all things considered not feeling too bad when I woke up the next day. But I couldn’t know; I was motionless in bed and any chance of me getting out of bed was nil.
More phone calls were made; they were desperately trying to get me into either the Royal Brompton or Harefield hospital where I could be treated by specialists. I think it was a couple of days later that I finally got a bed at Harefield (it should really have been the Brompton but there was ‘no room at the inn’). I was taken by ambulance, flashing lights and siren when the traffic got heavy. I remember looking up at the trees from the window (my narrow field of view only allowing anything higher than 20 feet to be in sight). I somehow felt much better just for getting out; it was relief after being cooped up for so long.
I got to Harefield and was taken to the heart transplant ward; this was disconcerting, I hadn’t considered that as a possibility. I was placed in a room in the very high dependency section known as the ‘Square’, where all the nurses were kept busy by their monitors. The food was being prepared just outside my door; I was ravenous, being on large doses of intravenous steroids. Dinner was served and it was delicious – all the food at Harefield was great, come to think it was good at East Surrey too. Two friends dropped by (Kathleen and Olivia) with my favourite fruit juices and soya milk (I still had a reaction to cow’s milk) and a big box of chocolates and some books.
The specialist came in to discuss what was about to happen. I was informed the blood pressure was adequate, so I would not be put on any other machines. The treatment was basically to be a course of medication, to be changed over time until they found just the right combination; Ramipril and Prednisone were high on the list. So far so good. Later that evening, a consultant came in to see me and told me that I would need a pacemaker fitting, a special type known as an Implantable Cardioverter Defibrillator (ICD) “to prevent sudden death”. Hmmm. I was concerned about the operation for this, but over the next few days gradually forgot all about it.
My short term memory around this time was really bad; sometimes I would get a phone call from a friend and be asked what I had for lunch that day; no chance! It just wasn’t there, like the data had been erased. Apparently short-term memory is the first thing to go when you have heart attacks and the blood supply to the brain is restricted.
The staff at Harefield were wonderful; I am still under the care of Dr. Emma Birks and from day one I started my slow but sure recovery. I had lots of visitors, and Sue and my sister and brother in-law came to see me every weekend without fail. That was a round trip of 400 miles.
After a few days the central line was taken out; then another line and the catheter (thank God!) and I could actually lay on my side to sleep, a real luxury. I asked Kathleen on one of her visits if I was out of the woods yet, and she assured me that I was, which was a tremendous relief. I don’t know to this day if she believed it but it helped anyway.
Moved to another room, slightly less dependant and things began to improve rapidly. I was given telemetry so I could move around without the wires trailing back to the monitors, and eventually could walk to the loo and shower room by myself, fantastic! Some days were good, others great, some bad and a set-back usually took a few days to recover from, but all in all the progress was good. The pacemaker was fitted in late October; by now I was strong enough to appear ‘normal’ most of the time. I should mention that I woke up half way through the operation and asked the surgeon “Do you need a hand down there!”
I was released from hospital late October, and started my shaky recovery at home. An over-night stay at the Brompton, prompted by some strange blood results was needed early December 2006, but since then progress has been very rapid, and after Christmas I went back to work full-time. This was a bad mistake as I became very fatigued and now only work 15 hours a week, at least until my strength returns. I believe there are problems with fatigue for anyone with vasculitis.
So, there we have it, and to say that I am grateful and appreciative of all the help and support I’ve been given is a massive understatement, and I am only too willing to extend any help I can give to other sufferers of this rare, serious but ultimately treatable disease.