Patient Stories- CSS Around the World

In Our Own Words, a forum for folks affected by CSS to share their experiences, is one of the most popular features of the CSSA website. We are very pleased that three patients, from around the world, have shared their stories for this May, 2007 update.

Light at the End of the Tunnel By Graham Shore

Thirty-Five Years with Churg-Strauss (and counting.....) By David Moreland

Light at the End of the Tunnel

By Graham Shore
May, 2007

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.

Thirty-Five Years with Churg-Strauss (and counting.....)

By David Moreland, USA
May 2007

I recently found this site and was impressed by CSSA’s mission. I was diagnosed with Churg-Strauss Syndrome in 1979, and hope that my story will in some way help others to confront and manage their condition. I’m counting my 35 years from when I first started to show some of the many symptoms of CSS, and I’ve described the various sequelae that have expanded its impact.

The usual childhood illnesses hit me very hard, and as a child I had many colds and ear infections. By high school in the late 1960’s, however, I had put in many, many miles of vigorous hiking and bicycling in the hills of Connecticut, and during my last three years I was a halfback (midfielder) on the new soccer team. As a junior in high school I was in extremely good health with excellent stamina, with my only physical problems being nearsightedness and a partial hearing loss in one ear from a childhood cholesteatoma (a benign growth in the middle ear due to inflammation) and subsequent mastoidectomy.

BEGININGS?
In retrospect, the likely first signs of CSS came early in my high school senior year, 1970, when it became very difficult for me to get into and stay in shape for the fall soccer season. I had developed a persistent postnasal drip, which turned out to be the first appearance of what became chronic rhinitis and sinusitis. I spent the season enduring the taunts of my teammates and the wrath of my coach for not staying in condition. In early 1971 I was diagnosed with fall allergies and started treatment with allergy shots, which were ineffective, and OTC medications, which offered some short-term relief. I have had severe chronic sinusitis ever since.

ASTHMA
On to college, where I was still in very good shape in spite of the annoying sinus problem, and where I stayed physically active with backpacking, snowshoeing, and whitewater canoeing and kayaking in the White Mountains of New Hampshire. The allergy shots were continued, to no apparent benefit, and I used OTC nose sprays and decongestants to try to manage the sinus symptoms.

I gradually developed a tightening in the back of my throat that would appear episodically and would go away if I used an OTC epinephrine inhaler. In the fall of 1972 I had my first actual asthma attack, which was triggered by goose down while I was assembling a sleeping bag kit. The attack was severe but was treated successfully with corticosteroids. During the next year I developed steadily worsening chronic asthma. Initially the symptoms were worse from mid-autumn through mid-spring, but gradually became year-round.

The asthma symptoms were controlled poorly. From late September through April or early May I would be awakened by significant – often severe – asthma episodes every morning between 3:00 and 4:00 AM. In the days before inhaled steroids, the only effective treatment for me was systemic corticosteroids, and the conservative medical establishment at the time was extremely reluctant to prescribe long-term steroid treatments. Instead I was given various preparations of theophyllin and alternately encouraged to use or not use an OTC epinephrine inhaler. I received corticosteroids only through emergency room visits and hospitalizations. Initially I was given Tedral or Elixophyllin, which is a rancid solution of theophyllin in ethanol that burned as it was swallowed. It had an imitation tutti-frutti flavor that utterly failed to cover theophyllin’s bitterness. I would sit in near-strangulation with the dose measured out in front of me for long periods before finally forcing myself to swallow it. After a year or so I was switched to two grams per day of Slo-phyllin, a time-release theophyllin preparation. It took quite some for me to become acclimated to theophyllin and stop experiencing its side effects of nausea and vomiting. The nightly asthma episodes continued, however, and since theophyllin, ephedrine, and epinephrine are all stimulants I rarely got a good night’s sleep. Sometimes the episodes could be controlled within a few minutes, but usually it took at least an hour and more often two hours to get back to sleep. I would use an IPPB (Intermittent Positive Pressure Breathing) machine to help force medication and saline into my lungs and to help clear out some of the thick, sticky sputum. It was not unusual for me to not get back to sleep at all, and I went through my last two years in college in a fog of exhaustion. My friends made jokes about heavy breathing, asserted that it was all psychosomatic, and gave advice that it could all be cured with herbs or vitamins. A group of them would break out into loud coughing whenever I entered the room. I would not know from one day to the next what sort of condition I would be in – would I be an athlete or an invalid? I became cautious about making social commitments because I couldn’t know more than a day in advance whether I would be physically able to keep them.

I finished undergraduate studies and went to graduate school in California, and hoped that the change in climate would improve my health. It didn’t, and I had a couple of visits to the emergency room, but I eventually did find an allergist who was comfortable prescribing prednisone. He had me take 10-day tapering courses as needed in addition to continuing the Slo-phyllin. I would run about a five-week cycle: after a course of steroids my symptoms would be very good for a couple of weeks, then gradually worsen and become pretty severe before I’d start the next course. I struggled through the first couple of years of graduate school in this mode.

In 1977 or so Vanceril, the first inhaled corticosteroid, became available. I was started on it immediately and it stabilized my symptoms greatly. I still needed the periodic systemic steroid courses, but my symptoms were slower to deteriorate and did not fluctuate as quickly or as widely. Improved beta-blockers also started appearing around that time, and provided better short-term symptom relief than the OTC epinephrine inhalers. Symptoms were still worse during the winter months, but overall my asthma was much more stable and I started to be able to sleep through the night again. The sinusitis persisted, and I had nasal polyps removed in 1978.

Life seemed to be getting better, and during the summers I started playing in pick-up games of soccer and riding my bicycle up into the Berkeley and Oakland hills. My goal was to complete my thesis work, start the job search, and get on with life.

VASCULITIS
With the 1979 fall job recruiting season approaching, I was putting in a lot of hours to produce results on my thesis project. In mid-June I was still keeping up my regular soccer matches, and during one game suffered a split lip in a freak collision. A trip to the infirmary and a tetanus shot patched me up, but that night I developed severe abdominal cramping. The cramping had improved greatly by morning, so I didn’t act on it. During the next few days I had some diarrhea and nausea, and the cramping didn’t go away completely. I passed it off as a summer flu, since there was a little bit of something going around. Besides, I had to get those thesis results…

The cramping never really went away, and during July it began to worsen – in retrospect, probably in sync with my steroid courses. By the time I saw a doctor in mid-August, the pain was very significant and I had lost my appetite completely. Blood work showed elevated eosinophils, and the initial, tentative diagnosis was parasites. The internist I was visiting said the tests were very ambiguous and said they really weren’t sure what was going on. After a couple more office visits and more blood tests, I was admitted to the hospital on Sept 9 – nine days after my 26th birthday – for observation and more tests.

In the hospital my asthma flared up because it was near the end of my usual steroid-course cycle, and it took a few days to control it. When the asthma stabilized I was given a battery of additional tests, including barium scans which indicated intestinal blockage. I was unable to eat, and hyperalimentation (intravenous feeding) was begun to treat what had become malabsorption and malnourishment. In the meantime there were more tests, while the abdominal pain remained very significant and was poorly controlled with Vicodin.

The pain was generalized throughout the central and upper abdomen and, somewhat, in the lower left quadrant. When I finally reported that some pain had developed in my lower right quadrant, I was taken immediately into surgery for an appendectomy – a relatively minor procedure that, hopefully, would provide some information about what was going on. The following day the pathology results were available, showing vasculitis, and a rheumatologist was added to the medical team. From him I first heard about systemic necrotizing vasculitis and Churg-Strauss Syndrome. He diagnosed polyarteritis nodosa or, because of my history of asthma, Churg-Strauss. He described a paper by Anthony Fauci and co-workers that had just appeared in the Aug. 2 issue of the New England Journal of Medicine reporting the use of cyclophosphamide and high-dose prednisone for the treatment of systemic necrotizing vasculitis. Treatment was begun that day, Sept 18. I could not have been more fortunate that the paper was published when it was, and even more so that the rheumatologist was up on his reading.

At about this time my hands and feet first became numb, then painful over the course of a day or two, with my left hand and arm eventually becoming very painful while the other hand and my feet improved. The thumb, index finger, and middle finger – serviced by the median nerve – of my left hand became paralyzed and I lost all feeling in them except for neuropathic pain. I developed significant neuropathic pain in my left forearm and shooting pain in the whole arm.

My condition was stable for eight days following the appendectomy, but there was no improvement. On the ninth day the daily blood tests showed that my liver function had become severely impaired and late in the afternoon I was taken into surgery for a general abdominal exploration and an attempt to determine what the problem was with the liver.

When I awoke in the recovery room late that night the surgeon described their findings in great detail. They had removed 18 inches of small intestine and my gall bladder, both of which were necrosed and gangrenous. My hepatic artery was thrombosed and my liver appeared to be completely necrosed. They were unable to restore circulation to the liver, and the damage to it was so severe that, after consulting with all the available surgeons in the operating complex who were scrubbed up, they terminated the surgery without attempting to address either the liver damage or the remaining necrosed portions of my intestines. In the recovery room the surgeon told me that my injuries were fatal – and should have been fatal long before they ever had a chance to see them – that they hadn’t expected me to regain consciousness, and that they were not ordering my evening medications because “it would be pointless”. I was moved to Intensive Care, where I was isolated from the rest of the ward and the nursing staff checked in every 15 minutes or so to record my blood pressure and pulse, both of which were extremely high.

I spent the night performing relaxation exercises and building a rapport with the nursing staff. In the dark morning hours I first became chilled, then went hypothermic, and the nurses took the initiative to contact the key medical team members at home to get permission to treat it the hypothermia. My medications were resumed in the morning, and from that point on I received very attentive care.

During 72 days in the hospital I weathered a partial failure of the surgery, a systemic Candida infection that was treated with amphotericin B, a gastrojejunostomy to bypass a section of intestine that apparently had been too severely damaged to recover, and several other complications before I was discharged. My liver function returned spontaneously. I had reached a low of 129 lbs, down from my normal 175, which I carried on a 6’ frame. I went home from the hospital on the day before Thanksgiving in 1979.

After being discharged I continued oral cyclophosphamide for four months to complete the six-month course, then was put on chlorambucil (Leukeran) for another six months. The fingernails on the paralyzed fingers of my left hand fell out over the course of a month or so after my discharge, with new nails growing in beneath them.

I continued Vanceril and Slo-phyllin for asthma, but after the high-dose corticosteroid was tapered down, started alternate-day corticosteroids instead of the periodic courses I had used previously. Initially my asthma symptoms were improved enormously compared to before the chemotherapy and I was able to taper off the systemic steroids completely. After two years, however, the asthma symptoms worsened and I had to resume alternate-day steroids, arriving at the basic regimen of medications that I used for the next 17 years.

I returned to graduate school determined to complete my degree after a three-month hiatus. The three fingers in my left hand remained paralyzed and it was very painful for me to touch anything with them, so I changed the focus of my studies from laboratory work, which required careful manual manipulations, to computational work, for which I could at least hunt and peck with seven fingers on the keyboard. As it became less painful to touch things I sewed together a parka, which I got as a kit from Frostline to help exercise my hand. I used a sewing machine in their local store and for the duration of the project had to grasp and pull the fabric, and guide it carefully through the machine. It turned out to be a good level of exercise. Feeling and function in the left hand improved gradually over several years, and after many years became near-normal, even though there had been no detectable transmission in the left median nerve at the time of my discharge. My left hand remained weak, however, and cramped up badly with prolonged use.

After completing my degree in 1984 I entered the corporate world in Ann Arbor, Michigan. I was referred to a top-notch pulmonary specialist at a local private hospital, and he recommended a rheumatologist to keep an eye on the vasculitis. I remained active with volleyball, bicycling, and cross-country skiing.

In the late 80s I suffered two seizures and was started on anticonvulsants, which I tolerated poorly, experiencing numerous side effects and depression from several successive therapies. Eventually the seizures were linked to functional reactive hypoglycemia, caused by gastric dumping as a result of the gastrojejunostomy. Ultimately, all I need to do to avoid seizures is to not have sweets on an empty stomach shortly before getting vigorous exercise under stressful conditions. By managing my diet and exercise I no longer need to be on anticonvulsants.

RELAPSE
In the early- to mid 1990’s I began to have some vague urinary tract symptoms – soreness in my kidney area, periods of difficulty urinating, painful urinations and ejaculations, and occasional blood in my urine and semen. My blood tests were unremarkable, and the internist, rheumatologist, and urologists were not terribly concerned about the symptoms. In the mid-90s I started treatment for mild depression brought on by building frustrations with my work situation and with my health.

During these years I periodically developed itchy, leathery nodules on my elbows. These would come and go with no apparent pattern and were unrecognized as potentially related to vasculitis.

When Accolate became available for asthma in 1996 it was added to my therapy and I was able to stop the alternate-day systemic steroids, but had to retain the inhaled steroid (by then it was Flovent 220). Upon stopping the systemic steroids I had a brief episode of soreness in my kidney area, which resolved itself over a day or two. I began going to a nephrologist, who determined that my kidney function was mildly impaired, but also that I had hepatitis C – probably contracted from blood transfusions during the 1979 surgeries. He suggested that my urological symptoms might be due to the virus.

In 1997 I developed inflammation in both of my shoulders, for no obvious reason. Any attempt to move my arms at the shoulders became very painful and my shoulders quickly froze up so I couldn’t raise my arms from my sides. It took over six months of painful physical therapy and exercise to return them to full function. This doesn’t seem to be something that typically happens with CSS, but I note it because it is another manifestation of inflammation.

I have experienced ear infections fairly frequently in my adult life, and during the mid-90s they would occur several times a year, especially in my left ear. They resulted in ruptured eardrums many times, so were treated aggressively with various antibiotics. In May 1998 I was put on a two-week course of ciprofloxacin, a fluoroquinolone antibiotic, to treat an ear infection. I had been on Cipro once before and had experienced abdominal discomfort that resolved quickly once the course was over. As this second course progressed I began to feel fatigue and discomfort, muscle pain, then joint pain, and I stopped the course on my own a few days early. Shortly thereafter I noticed a “floppy foot” condition in my left foot, which also started to go numb. I saw the rheumatologist within a day of this, but he was not concerned by the symptoms. Two days later both hands and both feet started going numb much as they had in 1979. I drove myself to the emergency room, where they diagnosed polyneuritis multiplex and sent me home, in spite of my protestations about having a history of peripheral neuropathy from systemic necrotizing vasculitis. The next day my hands and feet became very painful and I returned to the ER, where, after triage and a brief exam, I was left unattended overnight. I was admitted to the hospital in the morning and after two or three days of the rheumatologist, nephrologists, and the neurologists debating what to do, a kidney biopsy was performed and they decided that it was consistent with the presence of vasculitis. During this time the symptoms worsened, affecting mainly the ulnar nerve in my left arm and the sciatic nerves in both legs, the left being worse. The outside of my left foot became paralyzed.

A six-month course of cyclophosphamide was initiated, this time given intravenously once a month, and a high-dose course of prednisone was begun. The combination of Vicodin, oxycontin, amitryptillin, and Neurontin did not fully alleviate the nerve pain, and I developed significant edema in my lower legs. I was sent home after a week, initially with home nursing to administer IV medications. I was unable to walk without crutches because of the pain in my left foot, and needed a home care worker to shop and to do some light food preparation. I could put in only short days at work before the pain and swelling in my foot would become too severe for me to concentrate. Fortunately I was able to do quite a bit of work at home.

By the end of the six-month cyclophosphamide course I was able to walk with considerable discomfort without crutches but was able to function more or less normally. I had stopped all of the painkillers except Neurontin, which I continued for a year or so. I had also gained 20 lbs from inactivity and during the next couple of years worked back down to my original weight of 170. The pain in my foot and thighs gradually diminished over several years to near-normal, but the paralysis in my left foot remained. The ulnar side of my left hand improved to the level of the median side, with nearly full function and sensation.

TODAY
My condition is now being managed by a rheumatologist at the University of Michigan Hospital who specializes in autoimmune conditions and vasculitis. Following the 1998 cyclophosphamide course he initiated methotrexate at 20 mg/wk with alternate-day prednisone. I have been unable to taper the prednisone dose below 10 mg, with pulmonary function being significantly better at 12.5 mg alternate-day. My asthma deteriorates with any reduction in either the inhaled or the systemic steroid. I continue with Flovent and albuterol for asthma and with Nasonex, ipatropium bromide, fexofenidine, gentomycin flushes, and warm saline flushes with a Hydropulse Irrigation System for the sinusitis.

Sinusitis is still a significant problem for me. I’ve had tinnitus as long as I can remember, especially in my left ear, and it fluctuates in severity. I have had two surgeries to remove sinus tissue and polyps, and for the past several years have had a tube in my left eardrum. Ear infections are now rare, though I have constant sinus drainage.

My pulmonary function is quite good. In spite of the asthma my lung volume is large for my age and size. The efficiency of gas exchange in my lungs, however, is only 60% of what it should be. CT scans show some irregularities that appear to be transient and that biopsies show are not infectious, vasculitic, or cancerous.

I have residual effects from the vasculitic damage. I have some difficulty fastening and unfastening shirt buttons and tend to use my middle and ring fingers of my left hand with my thumb rather than my index finger. My left hand still cramps up occasionally, making it difficult to perform tasks around the house that require fine manipulations for extended periods. The thumb gets stuck against the palm and I have to massage it and pull it open again. The edema in my lower legs comes and goes, and the left side of my left foot is still paralyzed.

My liver and kidney functions are stable and biopsies have shown the liver to be in very good shape, in spite of what it went through in 1979 and in spite of the hepatitis C and the methotrexate. We have not attempted to treat the HVC for fear of exacerbating the vasculitis.

The condition of my stomach is now being monitored carefully. The 1979 gastrojejunostomy altered its functioning enough that the stomach lining becomes irritated by bile and endoscopies have shown dysplasia – abnormal cells that can lead to cancer – and polyps. While the dysplasia was resolved with a more careful diet, the stomach lining remains irritated and polyps appear occasionally and must be removed.

I have long had stretch marks on my torso from the two times I was bedridden and taking high-dose corticosteroids. Since the mid-90s other effects of taking corticosteroids for 30 or so years have become more apparent. My blood pressure has become slightly elevated but is treated very effectively with lisinopril. My skin – especially on my shins – is fragile, breaks easily, and heals slowly, so I wear my old soccer shin guards for activities, like clearing brush, that present a risk. I always have a collection of small bruises from bumping into things or from carrying something heavy against my legs. Several years ago my bone density was found to have decreased significantly, but it largely has been restored by taking calcium and Fosamax. My face is perhaps slightly more rounded than it might be otherwise, but that is one place where the steroids have had little effect.

In all, I am still able to be very active. I participate in a volunteer group in town and serve as a guide for blind cross-country skiers and kayakers at events in the state. During the summer I in-line skate two or three times a week at an aerobic level, kayak once a week or so – also at an aerobic level, and play volleyball occasionally. I work at strengthening the calf muscle in my left leg, but still cannot lift my own weight with it. During the winter I work out at a gym and cross-country ski when I can. I seem to be limited in the level of conditioning and muscle strength I can attain, and it’s not clear whether this is related to the autoimmune condition, the medications, the virus, or just age. My lung’s inability to absorb oxygen normally limits the intensity of my activity. My digestive system does not handle fat very well and I need to be careful of my sugar intake, so I maintain a high-fiber, low-sugar, low-fat diet, which is a good thing. The problem is that I have difficulty maintaining my weight above 160 lbs during the summer, since I seem unable to digest enough food fast enough to keep up with the exercise.

I have found that I am generally the most unusual case that’s walked into a given physician’s office in a long time, and I have encountered physicians who want to take on my case whether they are qualified to or not. It has at times been difficult finding appropriate care. I am fortunate now to be near a large teaching hospital. The primary physician, the rheumatologist, the pulmonary specialist, the nephrologist, and the gastroenterologists with whom I work now have varying levels of experience with autoimmune conditions, but they are all very good in their fields, communicate with each other well, and are frank about what they do and don’t know. Their focus is to prevent more relapses rather than just to react to them.

I often think back to a guy, Don, who was in my junior- and senior high schools. He was an Eagle Scout in the same troop I was in, and was driven to complete all of the challenges he undertook. Don had cerebral palsy. He had to struggle fiercely to get his muscles to do his will, even for the simplest motion. Every movement he made was an excruciating effort, his own body fighting every step and every attempt to communicate. Yet walk and communicate he did. He could carry a backpack just as heavy as mine, and would force himself along the same paths and up the same hills. If he fell he wouldn’t accept help getting up, but would do it on his own. In school he pecked away on the electric typewriter the school provided, holding himself to the same standards as everyone else. The memory of his determined efforts puts things in perspective when I find myself drifting towards self-pity.

During college I spent a summer as a counselor at a camp for diabetic boys. The camp’s theme was that as long as the boys managed their diabetes there was no activity they couldn’t do, and the program centered on their being very active and athletic. Each of the campers had their ups and downs and there were always challenges balancing diet, insulin, and exercise, but they kept at it and found they were no more limited than anyone else, and could enjoy very rich and varied lives. I had just developed asthma, and the camp’s message resonated with my new situation.

I am thankful to have these role models from my past. There’s no question that a major chronic illness is a burden, and takes time and energy – physical, mental, and emotional energy – that would otherwise be applied to other aspects of life. My energy level and mood fluctuate widely, but slowly. I wrestle with the question, to what degree do I use my health as an excuse, and when is it an actual reason for doing or not doing something? Some of the effects are obvious – I can’t do significant exercise on the days I take methotrexate, for instance, because it makes my strength and stamina very low. Other effects are more subtle and I’m sure that I’m only aware of some of them. If I’m feeling listless or fatigued, is it because of medications, because of having been too active over the past week, because I need to rest up from using mental and emotional energy for coping, or is it the mild depression back again? In any case, is it a reason not to go out dancing tonight? It often is. The burden clearly has an influence on my level of motivation, my concentration, and my confidence. It’s not easy to follow the example of Don the Eagle Scout and find a way to forge ahead in spite of one’s burdens. But, it’s either do that or miss out on many of life’s offerings.

It is clear that Churg-Strauss Syndrome, vasculitis, and autoimmunity are below the radar for much of the medical community At the time of my original diagnosis my brother-in-law physician observed that medical professionals are trained to look for a horse when they hear the sound of hoof beats, but it my case it turned out to be a zebra. I hope that the CSSA and this web site will help raise awareness to a level where future patients can obtain prompt and accurate diagnoses and receive the most effective treatment possible in a timely manner.