[ Riky de Jong and Peter Boos from the Netherlands have four children: Merel (six years old), Stijn (four years old) and the twins David and Viktor (two years old). The boys all have severe haemophilia A. Riky stays home with the children. ]
“I like being home to take care of them, but we really could not do it any other way. Peter works full-time and his jo takes him throughout the country, leaving home early each morning.
My mother was a carrier of haemophilia, but was not aware of it. My brother has haemophilia and I am a carrier, I had this checked when I knew I would like to have children. I considered not having kids because of haemophilia, but due to the fact that good treatment is available, I decided to go ahead anyway.
In 1998, our daughter Merel was born and in 2000 our first son, Stijn. When he was five weeks old, we had him tested for haemophilia. Strangely enough, I was convinced he would not have it. It was a shock to find out he did indeed have haemophilia.
The toughest part of Stijn’s early years were the injections in the hospital. At first, this was only needed with a bleed. He was not easy to inject and it always took several tries. I hated seeing three nurses holding down this angry boy! At these moments, I was very aware that I was the one who gave him haemophilia. This changed when Stijn turned two. He received a “port-a-cath”, so injecting would be less of a burden on him and we could treat him at home. This was a great improvement.
We really wanted another child, just one that is. At the first pregnancy check, it turned out they were twins, identical twins. That was a surprise, but soon we both loved the idea. An ultrasound proved they were both boys. Later on in the pregnancy I elected to have an amniocentesis, so we could prepare well for the delivery. When the doctor called to tell us both babies had haemophilia, we just atarted to laugh, it was such a strange situation. We were not too worried, we saw how well Stijn was doing and we received wonderful support from the Van Creveld clinic.
The boys were carried to term and were delivered by Caesarian as one of them was lying breech. We expected things to go like with Stijn, with a first bleeding happening when they would start to crawl. But when they were seven months old, Viktor was crying in his bed one night.
He was ill and had a fever and when I picked him up I noticed one side of his face had strange twitches. Peter went to the emergency room where they immediately gave him factor VIII. This turned out to be a good thing, as the next morning our fears became reality, the MRI showed Viktor had had a stroke. Viktor was transferred to the Wilhelmina Children’s Hospital where he spent two weeks with a continuous IV of factor VIII. We were very lucky that the bleed did not cause any permanent damage.
At a check-up a short time later, it was discovered Viktor had developed an inhibitor. We have had to give him a high dosage of factor VIII daily since then to suppress the inhibitor. This meant he received a port-a-cath and that his bleeds had to be treated with factor VIIa. In the meantime, Viktor and David learned to crawl and walk and this resulted in the first joint bleeds.
David’s bleeds happened the way Stijn’s had, but Viktor was a different story. Since he could not be treated with factor VIII, his bleeds were different. They were more serious and lasted longer. He was admitted to the hospital several times in order to treat these bleeds appropriately. This gave us a glimpse into how it must have been in the early days of haemophilia treatment. It made me realise haemophilia is a very serious condition. How lucky for us to be living in a country where patients can be treated well. I find that something to be thankful for.
The hospital stays are logistical situations for the entire family, finding babysitters for the others and travelling back and forth to the hospital. This is quite tiring. The grand parents help out as do other mothers from school. I regularly keep one of the kids with me in the hospital for the entire day, especially David since he is still so small.
Viktor resents the injections much more than his brothers, he will start screaming as soon as he realises it is that time again. He even hates it if someone just wants to check on him at the hospital. It has to be because of all the injections and examinations. There was a time when he was very restless and had a fear to be abandoned, was not sleeping well, and could not really sleep without one of us next to him.
Stijn had his port-a-cath for a year and a half. At a given moment the injections did not go easily at all and it turned out there was a hole in the tube. We then tried to inject him intravenously. We had to go to the hospital twice a week to do this. At first it did not go so well. Every time a different doctor tried and often it would not work in one try. I found it hard to be assertive and ask for a good injector, I wanted to stay friendly with them, until the moment that someone who had never injected a child gave it a shot. It was a disaster and even Stijn at some point called out “What in the world are you doing?” That’s when I was fed up and said something.
We then discussed the situation with our haematologist and from that moment on, the same paediatrician injects Stijn every time, and succeeds in the first try each time. Things were getting better.
The port-a-cath was removed and Peter learned to inject. I will learn but am hesitant as I am afraid of needles. I can handle injecting into the port-a-cath. David also has a port-a-cath, and receives prophylactic treatment three times a week, like Stijn
I often worry about Merel. When I found I was young I found it hard to have one brother with haemophilia and she has three of them. She is doing great, however, and feels good about herself. We always explain everything, use the medical terms and are honest.
I sometimes talk to her about my own experiences as a child. At times, she is upset that she does not get as much attention when she falls. And she is upset if we go to the hospital without her. She wants to be there.
From March onward we have had two nurses come to our house to inject Viktor and David. I decided this since at one point I felt more like a nurse than a mother because of all the medical things I had to do. Especially with Viktor, I wanted to hold and comfort him, not put a needle into him. The children do not mind the new arrangement and it helps me.
We are now debating what to do in the years ahead when it comes to injecting. Stijn currently gets injected at night when Peter comes home from work, but now that he goes to school he has had some bleeds and we will have to do it before school. We will likely have the nurses come at 7am to inject the boys while I dress them and get breakfast ready. We are still trying to figure out how to do all that.
This summer, we went camping. We like it and are not planning to let haemophilia get in the way of that. We bought a small fridge for the factor and sealed crates for the injection accessories.
I am also making sure we find campgrounds that are fairly close to hospitals. For now we stay within the country, we are not ready to go abroad. We inject at the table, outside. With Viktor we go into the tent since he yells so hard each time and neighbours would probably wonder what was going on. We are trying to maintain the routine for injecting, which is hard, especially on vacation.
We are amazed time and time again about how much resilience children have. It gives us energy. There are also so many more pleasant than unpleasant moments, we thoroughly enjoy our family. I always look ahead, what has been, has been.
Fortunately, we have been able to accept that this is a large part of our lives. We are the incredibly proud parents of four kids and are looking forward to the future!”
[Taken from The Source Magazine – Spring 2006]