Steve's story
I have always been an active person, working full time, enjoyed running long distance, sailing, biking and volunteer work in aviation and search and rescue. Coming to a grinding halt with a debilitating rare disease was not on the radar.I ran my last half marathon on 15 March 2020. I developed a light cough during the race and was a little more tired than usual. I completed the race in 2 hours 14, which was around 10 minutes longer than I expected. Looking back I believe I was already suffering the effects of the illness that two months later would see me admitted to hospital with multiple organ failure.I should have been diagnosed earlier, but with Covid having been recently released into the community it was difficult to get in front of a doctor. GP’s especially didn’t want to see people with coughs. I finally got someone to organise a blood test, and the day I had that test I was admitted acutely to hospital.The team at the hospital hammered my system with high doses of Prednisone which reduced the massive inflammation my body was experiencing and slowed the rate of damage to my body in order to give them time to work out the diagnosis. Both medical teams treating me worked on it and after three days in hospital came up with the likely diagnosis. Even then, how I presented wasn’t typical of the disease, it seems the variation of symptoms between people is infinite.
The diagnosis was confirmed by checking antibodies, and a biopsy of my kidneys. At this stage my kidney function was down to 17 percent (normal >90) and my liver was also pretty shot. By then I had permanent scarring in my larynx too which means I still cough, not a good thing during Covid, and something that still alarms my family when they hear it.My illness was determined to be ANCA Positive Vasculitis, previously called ‘Wegeners’. It involves the immune system attacking and destroying small blood vessels throughout the body. It is classed as a rare disorder, affecting around 3 people in 100 000 which is estimated to be around 150 people in New Zealand.The only treatment option was with chemotherapy. I had more high dose prednisone and once discharged from hospital was given six infusions as an outpatient of a medication called Cyclophosphamide which stopped my immune system working. Once in remission I was put on immune suppressant medication which I was expected to be on for at least five years. I had a bad reaction to the initial suppressant which put me back into the hospital. This was due to the lack of some enzyme meaning my system was only able to tolerate 50 percent of the recommended dosage. I continued with that medication but remained unwell so it was changed to another which I’ve been on since.My medical condition has improved steadily over the last three years. I have to be careful about infection, catching a cold or Covid or getting an infected wound would be serious. I’ve also been left battling chronic fatigue which is bad enough at times that on bad days I would have little strength in my right leg and have to use a walking stick and severely limit day to day activities. The nausea of the early days has gone, but there remain odd symptoms that I wonder…is it the medication, is it the disease, or is it something else.Currently my blood test results show that my kidney function is almost in the normal range for my age. I am now on minimum dosage of the immune suppressant, and expect to come off it completely in October 2025, unless the illness becomes active again. I understand I have a 50% chance of getting to the 5 year mark still in remission, and am always on the alert because if a return of the disease isn’t picked up early enough it could lead to more harm to already damaged blood vessels and organs.For me, an important part of staying well with ANCA vasculitis is staying fit, within my ability to do so. This requires constant effort. It’s made harder because I don’t appear ‘sick’ which means other people may struggle to understand my limitations, or why I might be very restricted one day, and able to do things the next. I’ve now been medically retired and currently I walk my dog up to around 2 hours per day and at least once a month I am up to sailing my small boat, or can go kayaking. The time, when I’m not resting, is spent doing small amounts of housework, gardening and cooking as I can.Even though the medication I have been prescribed is working for me, there are other patients in NZ that are not so lucky and struggle to have access to medications readily available in Australia, so to help those people, I've decided to start running again at events to raise awareness of rare disorders.I will start with 10 km races, and hopefully if my condition keeps improving I will be able to do at least one more half marathon. I would also like to train for a triathlon, which was a goal I had before I became ill.