Annual Report 2016

EVEN WHEN I'M IN PAIN, I FOCUS ON ALL THE POSITIVE THINGS.

ERIC, 18 UNIVERSITY STUDENT, STUDYING HISTORY, WINNIPEG, CANADA

I am grateful for everything I have in life. Living with severe haemophilia A has made me humble and has taught me a lot about how to cope with challenges. I don’t think there is a lot of value in feeling down about yourself. Yes, the diagnosis sucks, but at some point you realise: this is my life, and feeling sad doesn’t help me or anybody else. You always have something to be grateful for. Even when I am in pain, I focus on all the positive things; for example, at least I can still move my arms.

Even though things can get really bad, it does get better. For me, I have suffered a lot with chronic nerve pain and my inhibitor. I don’t want to cheapen living with haemophilia, but haemophilia with an inhibitor is almost a completely different condition. An inhibitor is brutal. When I was 16 months old, I developed an inhibitor which means that my immune system identified my factor product as being non-self and developed antibodies to fight it. My body eliminated the factor immediately, so clots wouldn’t form. As a small child I was put on immune tolerance induction (ITI). It’s a tough regimen which involves daily treatment with large volumes. The treatment didn't work so I went on factor VIIa prophylactic treatment and also used it on demand.

Even though things can get really bad, it does get better. For me, I have suffered a lot with chronic nerve pain and my inhibitor. I don’t want to cheapen living with haemophilia, but haemophilia with an inhibitor is almost a completely different condition. An inhibitor is brutal. When I was 16 months old, I developed an inhibitor which means that my immune system identified my factor product as being non-self and developed antibodies to fight it. My body eliminated the factor immediately, so clots wouldn’t form. As a small child I was put on immune tolerance induction (ITI). It’s a tough regimen which involves daily treatment with large volumes. The treatment didn't work so I went on factor VIIa prophylactic treatment and also used it on demand.

In eighth grade I had what I thought were a series of bleeds in my knee, back and hip. I had a month and a half of bed rest where I could only lie on my side which was very frustrating. Eventually, after an MRI scan, they discovered there had been no bleeding and there was no physical damage. My body was simulating pain and inflammation. I was diagnosed with chronic nerve pain. We had been chasing phantom bleeds – I had gone through all that pain for nothing. I felt disillusioned. This had been draining on me, the family’s resources and on the healthcare system. I eventually came out of that difficult situation through a combination of the correct medication and physiotherapy.

When I was 14 my haematologist suggested we give ITI another try. I was a teenager at this point, so the decision was mine to take. I committed to an 18-month trial and was tolerised in only three months; I was 15 years old and that was a huge turning point for me. I moved to treatment every other day with 10,000 units of wilate®. Now I can live my life the way I want to within my own limits. wilate® is how I control my quality of life by protecting me from bleeds.

“For me, when I feel I am experiencing the beginning of a bleed, it is not a question of “if” I treat but “when”; I always have my factor available. Many people around the world don’t have that luxury.”

Haemophilia has taught me how to deal with disappointment. Growing up, I got used to planned family holidays having to be cancelled because I got a bleed. It’s disappointing for all the family, and although it’s my fault I can’t allow myself to feel super bad about it.

I’ve been talking about how complicated my life has been, but there are so many people around the world who do not have access to the treatment they need. Attending the 2016 World Federation of Hemophilia (WFH) World Congress in Orlando was a really humbling experience which blew me away. The major theme is “Treatment for All”. Haemophilia is a manageable condition when you have access to factor concentrates, but 75% of people don’t have access. For me, when I feel I am experiencing the beginning of a bleed, it is not a question of “if” I treat but “when”; I always have my factor available. Many people around the world don’t have that luxury.

Haemophilia has shaped my interests and friends in many ways. As a kid I couldn’t play sports. I played video games and board games. Having haemophilia means a lot of my interests are non-physical. I partake in what I call “mental athletics”. In November 2016 I competed in the world championships of Netrunner, which is a two player card game set in a dystopian future. When I am playing competitive card games at the highest level I still feel the pain and the stiffness in my joints, but when I am truly involved in a game everything else falls away and I can just let go.

CHRISTINE, ERIC’S MOTHER
Haemophilia is in our personal lives and is now my occupation as well. I am Executive Director of the Canadian Hemophilia Society’s Manitoba chapter.

At 14 months Eric bit his tongue; it was a tiny little cut but the bleeding didn’t stop for days. We took him into the hospital and he was diagnosed with severe haemophilia A. Eric’s diagnosis was like an out of body experience. We were processing things three seconds after they were happening. It is devastating to discover that your perfect child has a chronic condition. You experience anger and denial. You think of all the things that won’t be; I remember my husband Shane saying: “He’ll never be a police officer.”

“I am so proud to see Eric’s positive approach to life. When he was little and expressed anger we would talk about all the good things that have come from his condition – all the life experience we have and the people we know. You have to remember to see all the silver linings.”

Once you have been through the grief process, you realise that this little person has all these other fantastic things to offer. You learn to become good managers of the condition – it becomes your normal. There was no way I would have put Eric into day care. I sold my business so I could stay at home with him. I was lucky to be in a position to do that. He is so cute, so it’s worth it.

My advice to newly diagnosed parents is to learn all you need to know, but not from the internet which is full of scary things. Whatever you dreamed up in your head about the reality of this condition, it might not be the reality. Educate yourself and reach out to the community. During that dark time after diagnosis we very quickly made a strong community connection. During our first community event we saw kids running around being normal children. It was a huge relief to see that these were not disabled children, and to meet other parents who had been through what we were going through.

As a small child Eric developed an inhibitor and was put on immune tolerance induction (ITI). We did treatments every day through a port-a-cath. We did that for a very long time, but we couldn’t get his titre level to change. We discontinued ITI then began using another plasma product that we gave prophylactically every other day two or three times a week – and used a bypassing agent when he was bleeding.

When Eric had his inhibitor he experienced profound bleeding episodes. When he expressed that he had pain there was instant panic. The experience of living with the inhibitor was a significant part of his young life. Since he tolerised at 15 years old, I have watched him gradually let go of his anxiety. Now he can experience the body pain everyone experiences. Yes, he pays attention, but there is not that same level of fear and anxiety, and that’s the case for me too. When we got the news that he had tolerised I felt a weight lift off me that I wasn’t even aware I was carrying. The difference between life with the inhibitor and life free from the inhibitor is incredible.

After everything we’ve been through we have learned an important lesson: every challenge is temporary. During the tough times it may not seem temporary, but you have to persevere and dig into your internal resources. You do the best you can, knowing that it won’t always be like this. Having the chronic nerve pain diagnosed was a huge improvement in Eric’s life because he finally knew why he was in pain all the time. Pain is so little understood, and is often undermanaged in bleeding disorders. Our nurses have become a part of our family. The care from the team has been amazing. These people have known Eric all his life. Our haematologist, who is Director of the Manitoba Bleeding Disorders Program, was a Fellow when Eric was diagnosed. I have experienced the healthcare professionals working in the haemophilia realm to be truly dedicated to the people they serve, not only in terms of the care they provide but also their advocacy. We have been very lucky.

Eric has a resiliency and maturity not typical of his age. As a child Eric was socialised to adults early on; he was much more mature than his peers. Some older gentlemen in the community, who had experienced bleeding in their childhood and could remember what it was like not to have product, showed Eric kindness because of what he was going through. They became friends. The social skills he built up being around adults all of the time have served him very well.

Eric was 16 when he joined the gaming community, which is a beautiful community of people who have welcomed him and boosted his self-confidence.

I am so proud to see Eric’s positive approach to life. When he was little and expressed anger we would talk about all the good things that have come from his condition – all the life experience we have and the people we know. You have to remember to see all the silver linings.