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In What’s It Like To… New Zealanders from all walks of life share stories of health & wellbeing. Here, Deon York tells Paulette Crowley about living with the hereditary bleeding disorder haemophilia, which is estimated to affect around 430 New Zealanders. Today, Thursday, April 17, marks World Haemophilia Day.
My parents were told I had severe haemophilia when I was 18 months old after an injury in my mouth wouldn’t stop bleeding.
Haemophilia is a hereditary bleeding disorder where the blood doesn’t clot properly, which means bleeding will not stop unless treated. It can be fatal. Back when I was a kid, every time I had a bleed, I was pulled out of school to be treated in hospital. There was a lot of waiting around and the treatment was with this gluggy product that took hours to draw up and administer. I had a bleed every week or so, predominantly in my elbows and ankles. It was a very painful experience and sometimes I wasn’t able to walk.
Living with haemophilia meant I couldn’t participate in sport at school. I spent a lot of time on the sidelines, watching others. Sometimes haemophilia is not so obvious and children would often ask me, “Why are you special? Why do you get to sit this one out? What’s going on?”
That curiosity actually led to a very supportive group of friends who backed me up. Having haemophilia also pushed me to focus more on education and how I was going to do things differently in my life.
Back in the 80s, treatment for haemophilia relied heavily on blood transfusions. I don’t know if you remember, but back then, there was a blood scandal with HIV in the blood supplies. Anyone who had a blood transfusion around this time was potentially exposed to HIV and the haemophilia community was affected badly. As a child, I faced stigma because of it. There were schools that would not take me because they thought I would infect other children.
Luckily, I dodged the HIV bullet but there was also hepatitis C in the blood supplies in the 80s and 90s. I was about 10 when I was exposed to hep C and contracted it. The treatment for it in those days was brutal but I was very lucky that I had my therapy when I was young, which meant a higher chance of it working.
I’m virus-free today but more than 180 people from our community were affected by the disease. A lot of them died from liver cancer and cirrhosis of the liver. Many people with haemophilia still live with the effects of hep C today.
For the first 18 years of my life, I was treated only if I had a bleed, which caused a lot of lasting problems. When you have blood going into joints, it can cause permanent damage, a little bit like osteoarthritis. There’s now preventive treatment that minimises the damage for kids growing up with haemophilia, although it doesn’t completely remove it.
The treatment for bleeds has improved, too – a huge leap forward from the 1980s. These days, a lot of treatments are synthetic therapies, which eliminate risk from blood contamination. Most treatments can be self-administered at home rather than having intravenous infusions or subcutaneous injections in hospital. Many of us now inject preventive treatments only once every week, once every fortnight or once a month, as opposed to three intravenous injections a week.
Kids also now learn how to self-infuse and self-treat, which is another big step forward and a huge time saver for families.
The last time I had a bleed would have been about a month ago – in my left ankle. I’m not sure what caused it. People imagine that if a someone with haemophilia is cut blood spurts everywhere and you bleed out. But that’s a misconception. Quite often, people can have spontaneous internal bleeds with no particular cause, like my last bleed. All I know is that the characteristic swelling happened, I was limping more than usual and I knew that I needed to treat it. As well as having treatment for the bleed, you need to rest and have physiotherapy.
These days, I probably have fewer than five bleeds a year. But that’s not the reality for everybody and some people with haemophilia continue to bleed often, despite the advances that we’ve seen. We still have people in our community who have intracranial bleeds, which is one of the most serious things that can happen. Once you have a head bleed, it’s akin to having a stroke and affecting your mobility. It can change your personality. So we really need to remind people, despite having a “wonder drug”, haemophilia is still a serious condition if not treated.
Aside from a bleed every now and then, my life is pretty normal. I can go for quite a long period of feeling almost invincible, but a bleed will remind me that I have a serious condition and I need to take care of myself.
The treatment has been amazing because it’s been able to transform people from not being able to hold down a job or get an education. Most of the time, people with haemophilia can have a job, a family and a life without any problems. Some people now work in trades and do things that were not open to me growing up. I always knew I was likely to do a desk job but a lot of people now can pursue dreams that they couldn’t pursue before.
Haemophilia New Zealand works to improve health, wellbeing, and long-term outcomes for all people affected by bleeding disorders across the country. It relies almost entirely on the generosity of the public. To support Haemophilia New Zealand visit https://haemophilia.donatenow.co.nz/
