Uma criança hiperativa com hemofilia é um coquetel perigoso

I was a very hyperactive child and that is hazardous when you also have haemophilia. I have attention deficit hyperactivity disorder (ADHD). When I was young I would constantly run through the house and jump around. I was always up to something. With hindsight I can admit that I was quite annoying. I had so much energy and was extremely active, which resulted in a lot of bleeds. Bringing up a boy like me was quite intense for my parents and it was especially difficult for my mother. To have a hyperactive kid with haemophilia is a dangerous cocktail.

As a kid I found it very hard to accept my haemophilia. I used to wish that I didn’t have bleeds and didn’t have to go to hospital so often. Sometimes I would stay in hospital overnight. It was tough, but occasionally I enjoyed the hospital stays because it meant I could watch videos.

The most challenging time for me and my mother was from 9–11 years old. I found it very difficult when I had to learn to inject myself with FVIII product. It is tough for a young lad to infuse himself. It was very hard for my mother to manage everything that was happening and to cope with having a little kid that doesn’t want to look after himself. I remember being alone in my room and crying. I felt so frustrated. I couldn’t play football with my friends. I always wanted to play but it was just too dangerous, especially since my ankle was a target joint for bleeds. All my sports teachers knew about my condition, and that meant that I couldn’t take part in some sports lessons. I was angry that I wasn’t allowed to be normal.

I grew up believing I had moderate haemophilia, but recently my doctor told me that I have severe haemophilia. It seems that I don’t have as much factor VIII activity as I used to. My older brother has mild haemophilia and he is just like a normal guy. Today he is doing his dream job – he is a train driver. I am happy for him. He didn’t have the problems I had growing up. He was never under prophylactic treatment. He was also a much calmer child. I was the big challenge for my mother.

I would rather see more factor concentrate treatment available for people throughout the world than a pill which was only available in Europe.

My grandfather had haemophilia and he was a great man. I know that he didn’t have the chance to get the therapy he needed. If he was alive today he would be very happy to see the medicine that is available and all the advances that have been made. I think as young people we should appreciate all that we have today. Sometimes we don’t appreciate it enough; I know I don’t. I have experienced a lot of challenges, but I was never in a situation where I had a bleed and no access to therapy. Unfortunately I take my medicine for granted.

I should take my prophylactic factor product twice a week, but sometimes I don’t have the time to do it or I just don’t feel like doing it. Taking this medicine feels like a burden. It’s a stupid attitude, I know. I understand that it is important to take it in order to stay protected, but when you are young you don’t always look after yourself as you should.

Today I lead a nearly normal life, although I have target joint bleeds in my ankle. My foot swells from the inside and turns blue, and if it is really severe I can’t walk. I am very involved with the German Haemophilia Society. I am a Youth Representative on the Youth Council of Germany. This work is very enjoyable and it has emotional worth for me. Spending such a long time in the community forms a deep and heartfelt connection. My involvement started at its summer camp, which I attended almost every year from age nine until my 18th birthday. You get to know a lot of kids with haemophilia and you realise that you are not alone. As I got older I became more involved in the running of the camp and was given more responsibilities. The summer camps were amazing; in fact, these were some of the best times of my life.

I attended the World Federation of Hemophilia (WFH) World Congresses in Orlando and Paris – these events offer a great experience. You get the opportunity to meet people from all over the world. I would love to see the majority of haemophilia patients having access to products. Today, only 25% of people have access. If I had the choice in the future I would rather see more factor concentrate treatment available for people throughout the world, than a pill which was only available in Europe.

I am a musician and have played guitar since I was 11 years old. Reading and writing fiction are also great passions of mine. I recently went hiking in the mountains with a friend whom I met at summer camp. The fact that two young guys with haemophilia can go hiking really shows what modern treatment can allow. I would say to a young guy with haemophilia that he should participate in the community because we need him. I would tell him that he shouldn’t take his treatment for granted. Live the life you want, because the medication today gives you that freedom.

We shouldn't grant this disease too much power over our lives. One can live a happy life despite having haemophilia.

Susanne, Jannik's mother

When my sons were born I knew there was a chance they would have haemophilia; it is normal for us. Haemophilia has been in our family since the 1700s. My father had haemophilia and grew up during a time when no treatment was available and the disease was little understood. The doctors thought that vitamin C and eating lots of peanuts would help. Children were told they could not do sports and after injuries often they had to lay in bed for weeks.

My father wanted to be a chef, but was told he couldn’t because he would have to handle sharp knives. My father learned not to trust doctors. He believed that you were more likely to die in hospital than if you treated yourself at home. Even dentists were too scared to give him treatment. His brother lost a leg when he was young after cutting himself – they couldn’t stop the bleeding. The first time my father was infused with a factor concentrate, in 1980, he contracted hepatitis C. I had convinced him to go to hospital for treatment to stop his nose bleeding. I felt guilty for a long time after that.

The connection between sons and mothers is usually very close and most mothers feel guilty about being a carrier of this disease. Even when you know you cannot change it and it was not your choice, in your heart you feel differently.

I was expecting my younger son Jannik to have mild haemophilia like my older son, but when he was born an inexperienced doctor said: “Don’t let him cry, he will get bleeding in his brain.” I was shocked. Despite knowing this was incorrect, he made me feel very insecure. I replied that it cannot be true because everyone in the family has always had mild haemophilia. Jannik was always considered to have moderate haemophilia. Only recently he has been shown to have severe haemophilia after all.

I didn’t expect Jannik to have so many bleeds throughout his childhood. You could never predict what would happen next because of his hyperactivity. Being at home was sometimes like being in intensive care. It became too much for me – I had a lot on my shoulders. I felt destroyed. My husband could not help me that much, he could not see blood and he stood back. When Jannik was 10 years old I went with my two sons into rehabilitation for four weeks. I felt exhausted and during that time I asked the doctors to perform the injections. In the following year I realised it was finally time for Jannik to do his injections himself. But he said: “You do it or nobody will do it.” When he was 11 years old, with the support of the home care service, Jannik learned how to infuse himself. What a relief!

In 2009 we built up a network for haemophilia families throughout Germany. The network consists of families with haemophilia who offer a helping hand or an open ear to other families. For three years I was a member of the Board for the German Haemophilia Society. I learned a lot during this time. In November I stepped down from the Board and now I am fully focussing my efforts on the network.

I am a Senior Home Care Specialist. We support patients and families with children with haemophilia with their therapies, and train them on home care treatment. Now we are seeing many refugees with haemophilia entering Germany. Some of these people have never had access to treatment before; some have subsequently developed inhibitors after treatment. It is very difficult for these people and there are some language barriers. We try to find a way through the chaos to support them.

We shouldn't grant this disease too much power over our lives. One can live a happy life despite having haemophilia. If one can accept help from others, many things become easier. I am really curious about the developments in the future and I hope that this disease can soon be treated even better.