Tony Smith, Bathurst (Central Tablelands of New South Wales). November, 2006 The diagnosis I got a ‘probable’ diagnosis of CSS (eosinophilic vasculitis) in August 2004. This came about as a result of my admission to Sydney’s Royal Prince Alfred Hospital, a major teaching institution beside the University of Sydney. I do not know how many Australian sufferers there are. I seem to recall being told that there could be 1:50,000 people. That would make about 400 Australians. Lately, I heard a patient at another hospital had been told that the number is under one hundred. I was in the Cardiology Unit because three weeks before my eventual discharge from RPA, I presented at my local Bathurst District Hospital with chest pains. There they did all the right things for a 57 y.o. man presenting with chest pains and reporting arm pain as well. They assumed that my heart was having some trauma. So I got stabilising medications and was flown to Sydney on the air ambulance for a cardiogram. I received very good care in the Cardiology Unit, but the cardiogram reported ‘minus’. Most of my ward mates got a stent or two to open blocked arteries and were sent home. But I had inflammation and a rapid and erratic heartbeat. Over 3 weeks, I was visited by haematologists, respiratory physicians, rheumatologists, and infectious disease experts, and told my story quite a few times. Eventually the immunologists decided to adopt me, and the cardiologists were happy to release me into their care. They agreed some form of vasculitis was likely. I had a collapsed lung, but that healed with antibiotics, so we assumed that was due to an infection acquired after admission. The journey to hospital In describing my medical history it is difficult to know what is relevant. I am an only child born in 1947. My parents were heavy smokers. My father’s wartime experience probably ruined his life, and he tended to be frozen in time by membership of a veterans’ club. This did not make my mother’s life happy, especially as he drank too much but mainly at weekends. My mother died in her early fifties of heart attack. She refused to go to doctors. My father died at 67. He had been told continuously by veterans’ affairs doctors that he would die young if he did not change his lifestyle. I was a pretty fat little kid, but slimmed down dramatically in secondary school when I discovered cricket and puberty. This was at 13. I had the usual vaccinations including polio, and do not recall any major diseases. I had my tonsils and adenoids extracted at 6 and contracted German measles when 17. We lived within a mile of an industrial complex at Clyde. The skyline was dominated by an oil refinery. At 21 I spent two years in the military and had more vaccinations, including smallpox but had no major reactions. I recall two contacts with medicos. One night I had a very high temperature, but the next day it had gone and was not explained. I cut my scalp and received some sutures. I also had some dental work, including insertion of amalgam fillings. Between 23 and about 43 I was very healthy. I married and we moved to the north west of NSW and stayed there for 16 years. I was involved in teaching and farming (milking goats). I had some sinus problems, but these were common in the district. I caught mumps but had no major complications. When I was 41 we moved to Cowra on the south west slopes of NSW. A couple of years later I started to develop asthma symptoms and some rhinitis. I did yoga there, but pulled a calf muscle badly. I began to investigate the possibility that the proliferation of canola crops could have been an asthma trigger. We moved to Bathurst when I was 46. I was hospitalised with a severe asthma attack associated with a persistent lung infection. I remember using chalk on a blackboard on the afternoon of the attack. I began attending the Respiratory Medicine Centre at RPA and participated in some trials. This stabilised my asthma as the doctors there were very objective and relied on lung function tests. From about 1995 to early 2004 I took pulmicort regularly but hardly needed any Ventolin for relief. One of the trials I was on involved sodium monteleukast. I have no idea whether I was had the drug or a placebo, but some symptoms returned because I had to stop using pulmicort. Between 1995 and 2004 – about midway – I started to develop what I thought originally was R.S.I.. Then I was sent to a rheumatologist who was keen to find someone in my ancestry who had psoriasis. She suspected psoriatic arthritis or spondylo-arthropathy. The standard anti-inflammatories did not agree with my stomach and I did not feel great relief, so I stopped them. I took quite a lot of neurofen. The arthritis type pain started in my shoulders and meant that I could not swim overarm or serve overarm in tennis. Then my legs began to ache a good deal. The aches reminded me of aches I had had as a teenager. I also had a strange experience occasionally. When walking my knee would give way without warning. In early 2004, my asthma symptoms began to return. I had a short course of prednisone and within about ten days of tapering off, I started to get excruciating elbow pain. Nothing the GP gave me helped. I just had to sit these unpredictable spells out. A few months later, the process repeated itself – symptoms, steroid, pains. My sense of smell returned and has stayed with me since! While I was seeing rheumatologists and seeking a diagnosis, I had some other symptoms. I had some vision problems – blind spot, flickering fountain lights, glass triangles as in a kaleidoscope. An ophthalmologist put me on aspirin and ordered further tests. I also had some sweats. A couple were at night, a couple in the day. Again, I could not predict these. Interestingly however, when I had these sweats, I had no elbow pains! Eventually, my GP gave me a Vioxx to try. Coincidence or not, a few hours later, the pains spread to my chest and my wife drove me to the casualty department in Bathurst. Among the other medical experiences I recalled were three operations. I had an apesectomy (root canal therapy). An American doctor used a filling he assured me was not available in Australia! I had a sebaceous cyst in my ear lobe (for 50 years) and it exploded at Easter 2002. I had a general anaesthetic for the curetting and took some time to recover. (The ear marsupialised – the wall of the cyst replaced the destroyed tissue). I reckoned I was still feeling the effects ten days later even though I was unconscious for only about 20 minutes. This experience convinced me that I should avoid generals. So in November 2003, when I needed a hernia repaired, I had it done with local anaesthetic. That was a little uncomfortable I have to admit. They had a new theatre light and it reflected very well. It was like watching the op on television! I squirmed a little as they tucked my tripes back in. Ah well. Treatment Since diagnosis, I have been pretty well really. I left hospital with a number of heart meds. I have now stopped all of those except aspirin, but have had a blood pressure med added. The CSS treatment began at 60mg prednisone per day (reducing) plus 100mg Imuran. About three months later with the prednisone at 30mg per day, my liver function tests played up and I was taken off the Imuran. I continued reducing prednisone anyway but at about 10mgs I started to get asthma symptoms. My eosinophils have been pretty low all the way through since leaving hospital. So, my prednisone went back up to 30mg and I was put on methotrexate and have been taking 25mg a week (on Wednesdays) for about a year and a half. This winter I had my ‘flu injection as usual, but I had 3 infections in various parts of the respiratory tract. I missed a week of mtx each time while taking antibiotics, and increased my prednisone to 30mg. Basically, I am now on 7.5 mgs prednisone a day and am due for review in December. I have some arm pain but only at the level of an ache rather than a pain. I have not had to take anything for it, but it has made me hesitate about carrying buckets of water around. I had another vision problem which suggested an ischemic experience. A CT scan showed some hypodensity in deep white brain cells. An echo cardiogram showed that my heart was good. I tried asasantin as a stronger anticoagulant but got headaches. I tried cloydrogel but got stomach problems. So I am back to aspirin. I take a weekly Fosamax for my bones and take a weekly megafol two days after the mtx to ‘give something back’ to my immune system. I take pulmicort – 400 morning and night. The future? I am reasonably optimistic. I have good care and am glad that we still have a sound health system in Australia. I always recommend that anyone diagnosed with an immune system disorder at least have their case reviewed by an immunologist. I have great support at home, and live in rural splendour. Well, the splendour is turning to dust in Australia’s worst drought on record, but it is not all depressing. Like everyone else I suppose, I have mood swings. I have monthly blood tests, and pay attention to what my body tells me. I eat well and drink a little (wine for the heart of course!). I am lucky that I do not have to work nine-to-five. I do not feel that I could be reliable, and I can do without the stress. Nor is my balance 100%, especially after exercise. I work a little from home. I love getting out into the garden and cooking the produce. We have many birds here – over ten varieties of parrots alone - and have visits from possums, kangaroos, and other animals including reptiles. We are within driving distance of Sydney for cultural activities and we drive to the South Coast regularly to swim in the ocean. We are expecting our first grandchild in February. I would dearly love to live long enough to see this disease better understood. Control is one step. A permanent cure would be better. But best of all would be an appreciation of the cause(s). It is possible that we are like the canaries used in coal mines to detect poisonous gases. Perhaps something in the environment or foodstuffs causes this disease, and perhaps there could be an epidemic in the future unless greater resources are devoted to understanding CSS. I am very glad to have the mutual support of others who contribute to this web site. It really helps to hear other experiences, especially when it is time to talk to my doctors. I have been able to ask many, many questions as a result of the postings, so I encourage everyone to keep writing in, even when they think their problems are too small to bother about. You are all V.I.P.s!