The tainted blood scandal was hugely tragic but very instructive

I was born in 1952 and diagnosed with severe haemophilia B aged 11 months. My mother’s uncles in England had died of haemophilia when they were young. In Montreal in 1953, a small group of haemophiliacs, their families (including my parents and grandparents) and physicians founded the Canadian Hemophilia Society.

In the 1950s and 1960s treatment for haemophilia B was fresh frozen plasma (FFP) administered intravenously which meant spending on average three to five days in hospital once a month. Each bag of FFP contained only small amounts of the missing clotting factor, so large volumes were needed to stop joint bleeding. FFP was not effective in serious surgery – a simple appendectomy, for example, was likely to be fatal.

It’s a funny disease because one week out of a month you are incapacitated while the rest of the time you are almost normal. I missed a third of my school days, but it meant I had time to read. In the hospital I learned how to learn by myself, which is a tremendous life skill. In those days, kids were regularly staying in the hospital together and naturally you made friends. Today, because of home treatment children will only go into hospital once or twice a year for check-ups, so they don’t foster those hospital friendships with other children with the same condition. These days, we manufacture those valuable connections through summer camps.

In the early 1970s factor concentrates that could be infused at home became available. I learned quickly how to self-infuse and this changed everything. In 1972 I toured Europe by bicycle from England to Athens, which would never have been possible had I not had my concentrates with me. Today we have kids who, because of the advances in treatment, are unable to identify bleeding when it occurs and don’t know the consequences of bleeding. We need to educate these children about the causes of bleeding, how to identify bleeds and how to prevent them. Preventative treatment for children really makes a difference in the long run to the health of their joints. We want to raise kids so their joints are in good shape and they don’t have to be repaired in later life.

My goal now is to pass on my knowledge to the younger generation. My message to young people is be knowledgeable and engaged in your own healthcare care. This is your life.

Older people with haemophilia suffer with every step, because literally every step hurts. The damage was done in their youth. In addition to the pain, there is emotional suffering. Some feel socially isolated and unable to play a full role in society. Some might not be educated because they could not go to school. Some feel like a burden on their families. Young kids in Canada today will avoid almost all that pain and suffering. Yes, there are still frequent needles, but compared with the past, it is almost like night and day. Saying that, inhibitors are a parent’s worst nightmare because when a child develops an inhibitor it’s like travelling back in time 50 years.

By the early 1980s, the Canadian Hemophilia Society helped to build a network of haemophilia treatment centres (HTCs) across Canada to provide comprehensive medical care throughout the lifetime of patients. There are 25 HTCs in Canada. The patient is at the centre of a circle of care which includes a trained haematologist, a nurse coordinator, a physiotherapist for prevention and rehabilitation, a social worker, and a psychologist to support learning to live with a chronic disease.

The challenge for parents is finding the balance of not being over-protective, but equally not being under-protective and denying the disease. Some parents say: “My child is normal, he can do anything.” That isn’t quite true. I encourage people to learn about their child’s condition and to be as open as possible with other people in terms of disclosure.

This has become a very treatable disease in the developed world, with life expectancy close to normal. In many countries, however, it is just like it was 50 years ago, with no access to factor VIII (FVIII) and factor IX (FIX) products, and lack of haemophilia expertise in the hospitals. Globally, 75% of people with haemophilia have inadequate or no access to treatment. Many people die before they reach 20, and if they do survive they are crippled. For the last 20 years the Canadian Hemophilia Society has run 10 successful twinning partnerships with developing countries to help them build their haemophilia organisations and support them to educate their members and advocate for care.

The infection of thousands of Canadians with HIV and hepatitis C was Canada’s worst preventable public health disaster. It was a terrible time for everybody: individuals, families and healthcare providers. There are people still living with the consequences and some have been unable to rebuild trust in the healthcare system. The crisis was hugely tragic but very instructive. The Krever Commission (1993–1997) was set up to investigate allegations that the system of government, private and non-governmental organisations responsible for supplying blood and blood products to the healthcare system had allowed contaminated blood to be used.

We are a militant and demanding community. In general people with haemophilia tend to be obstinate. Once we get an idea we don’t give it up easily.

Many of our members testified, including me. It was extremely hard to hear what had happened, and to learn that some of it could have been avoided. The Krever Commission led to many changes in blood systems worldwide and I am proud of what we achieved.

In Canada at the moment paid plasma donation is a hot issue. Our organisation takes the view that products from paid donors are as safe as those from unpaid donors. We need more plasma and the only way to get more plasma is through paid donors. This is heresy for some people because of the problems in the 1970s and 1980s. However people need to challenge their own preconceptions and look at the science and the facts. Things have really changed since the tainted blood scandal, and with all the scientific advances, lessons have been learned. The key issue is having a safe supply of product for patients.

We are a militant and demanding community. In general people with haemophilia tend to be obstinate. Once we get an idea we don’t give it up easily, which comes from facing challenges as children and often dealing with medical people who didn’t know what they were talking about. We knew which veins to use when we were four, and we were put in the hands of interns, so even as young children we had to assert ourselves to get the care we needed.

Fortunately, the younger people today don’t have to go through what we went through in the 1980s. The Canadian Hemophilia Society is involved in lots of blood safety committees and medical conferences. We try to get the younger generation involved and allow them to take their proper place in decision making. My goal now is to pass on my knowledge to the younger generation. My message to young people is be knowledgeable and engaged in your own healthcare. This is your life.