Interview: A blood transfusion every four weeks

David (37) and Yessica (37) have an adopted son, Vaglary (8), who has sickle cell disease. He has HBSS.

How long has Vaglary been a part of your family?

For three years now.

Where does he come from?

He's from Haiti, Port-au-Prince.

Did you know he had sickle cell disease when he came to the Netherlands?

Yes, we did. Before he arrived, we had looked on the Internet and we found out that the AMC has a special team. We became members of the Oscar Association and the Wereldkinderen Association invited us to visit a family who also had a son with sickle cell anemia.

What kind of impression did the disease make on you?

It was hard. During the adoption process, we were given a choice of disorders and we thought we could handle this. We didn’t have any expectations, really. The boy from the family we visited suffered a lot of crises, that really made an impression. But sickle cell anemia can vary a lot, it's very unpredictable.

Did you worry about it beforehand?

No, we thought we’d take things as they come. We did prepare ourselves to the extent that we had everything ready at home. We also had to wait a very long time for him to come to the Netherlands, almost three years. We went through all the reports we received from Haiti.

How did he do when he first arrived?

He was five when he joined us. After three and six weeks, he had quite a crisis, probably caused by all the stress after his arrival. Initially, Ede hospital wouldn’t treat him because they didn't have a protocol for treating sickle cell disease. Since then, we carry a digital version of the protocol wherever we go. He was the first person in Ede with sickle cell disease.

What were his complaints?

He had terrible abdominal pains that wouldn’t subside. Eventually, they started hyperhydration, that means they administered a lot of water. That was enough to fight the crisis. Along with Paracetamol and Diclophenac.

How’s he doing now?

In terms of crises, he’s okay. We’ve learned to give him Paracetamol in time and we give him plenty to drink. The only thing is that he now suffers from another severe complication, a vascular constriction in the brain. The sharp ends of the sickle cells damage the vessels, causing scar tissue and that constricts the vessels. This means a higher risk of blockages. For two years, he needs a blood transfusion every four weeks and after that, we’ll have another look to see if it had any effect.

How did they discover the vascular constriction in his brain?

They found out during the check-up. They made an echo because they knew this can happen. And after an MRI, we knew for sure. We couldn’t see any small infarctions at the time.

How’s the treatment going?

He’s now also being treated in Ede, he has a blood transfusion every four weeks. And once every four months, we go to the AMC hospital for a big check-up, we combine it with a blood transfusion. He hardly has any crises now, although he had one two weeks ago. That was a bit of a tense weekend.

Is stress a weak link?

For him, stress is the biggest factor. He has only been in the Netherlands for three years and a lot of things are still new and exciting. He’s very afraid of injections and that’s not nice when you need them every four weeks. Sometimes, they can’t find a vein and they have to inject him two or three times.

How are things at school?

He's doing fine. He understands things quickly. He does have run-ins with the teacher now and then. It's important for him to drink a lot and take a rest in time. The drinking thing is not easy. When we talk about it with his teacher, she says he bears personal responsibility too. We don’t agree with that. He has two bags, one for his sickle cell disease and one for the adoption. He has a developmental delay, and he needs help with that. Otherwise, he’s doing really well.

School staff want to encourage his self-reliance; you still want to protect him because of his adoption. How will you resolve this?

We first spoke to his teacher but we felt we were seen as over-concerned parents. The educational department of the AMC hospital then contacted the school and it emerged that other professionals had the same opinion as we, the parents. So that's when things started to fall into place. And we’re going to do the same thing again, but this time about drinks.

How does he hold up in class, does he talk about it?

In general, he talks about it as little as possible. That's a conscious decision. He doesn’t like being different than others. He has coloured skin, he's adopted and he’s aware of that. In a new class, they ask about his skin colour, because we are white. If we notice he’s being asked too many questions, we tell the class about his adoption and sometimes, also a bit about sickle cell disease. He won’t say anything until he gets to know a person better, one-on-one. He won’t stand in front of the classroom. He won’t hold a presentation, we do that for him, so to speak. He doesn’t have the courage yet to do it himself.

Do you think the disease affects his life?

If you don’t know, you don't really notice, to be honest with you. If he stops doing something, he just gives them another reason. He doesn’t say “I’m tired, I need to take a rest.”

Does he find it hard to keep up with others?

When he plays outside, he’ll come inside when he's been going on for too long, because he has abdominal pains. Sometimes his abdomen, other times his bones.

Does he look after himself when he needs to?

It varies. Eight times out of ten. Sometimes, whatever he’s doing is too much fun and he’ll carry on for too long, but don’t we all?

Does he play any sports?

He does breakdancing. We agreed that if he gets tired, he can stop earlier. He can complete the workout - push-ups, abdominal muscles and running - at his own pace. They do encourage him but they accept his limits and that’s really nice. He keeps up with them well because he's able to determine how far to go. He does come home all sweaty and stuff.

What does he think of that?

He doesn’t like that but he does like being with the group. He has to go to hospital every four weeks and at times, he gets angry about his sickle cell disease. He’ll tell you and he can. Sometimes, he hits himself on the head, to get rid of those things, that's how he sees it. He gets angry simply because of what has happened to him, also the adoption. We bought him those foam noodles, he can use them to hit things to vent his anger. He’s allowed to hit me with them, just to get rid of all those feelings. At times, he shouts as well. His breakdancing is also a way to vent. So every now and then, we move the table, put some music on with the volume up and away we dance.

Does he wet his bed?

Yes, at home, he only sleeps with a nappy. He does have sleepovers but only with children who are close to him. He gets hot very quickly but he still wants to wear pyjamas over his nappy. He feels others shouldn’t know about it. If he has friends over to play, he asks if his bed is clean as he thinks others shouldn’t have to see it.

Does he take any medicine?

He takes folic acid and vitamin D. Broxil doesn’t agree with him, it gives him such terrible diarrhoea that he loses weight. As he's been with us a bit longer now, we could try again. Although, he has blood transfusions now, so he wouldn't need it as much. He's also used to the different food now, he suffers from less stress and things. We do have it at home, in case of an emergency.

Do you go abroad for holidays?

Not yet. He has a safe place here. Holidays are something different altogether. We’ve been to the province of Zeeland, out camping and he was extremely excited. Initially, he didn’t want to sleep because of the excitement.

How’s the contact with his biological parents?

Very special and he wants that contact. The adoption organisation didn’t want to cooperate, not until he was 18. But we thought that would be too long so we started looking ourselves. His parents live in the slums. We found them by word of mouth because they all know each other. A month ago, we had contact with them via Skype. Vaglary was extremely nervous. Things were a bit awkward at first, also because of the language barrier. At one point, he started pulling funny faces which made them laugh and that broke the ice.
They also made sure the entire family was there, his mother, father and three sisters. We were able to see all of them. That was very special and emotional. You can just tell they miss him and you know why they put him up for adoption, because of his sickle cell disease They didn't have the money for it and because he’d been in hospital, his dad immediately lost his job. Plenty of others to take your place, right? Two sisters were also tested but they didn’t have it. The youngest, who’s one, hasn’t been tested yet. If they put up another child for adoption because of sickle cell disease, we’re open to it. We’ve told them so. He would love to go to Haiti once. But we’ll have to find out what to take with us.

Does Vaglary have a lot of friends?

Yes, he’s very social, not shy or anything.

Is he in contact with other children with sickle cell anemia?

We went to a sickle cell day at the AMC hospital. It was quite a revelation for him to find out there were other people who suffer from sickle cell disease too, even grown-ups! That was extremely good for him. And recently, we went to a Cliniclowns day and some of the children there had sickle cell disease too. That was nice for him because at least he didn’t have to keep explaining why he took a rest every now and then. There was also another adopted boy, so that was good for him too.

Did you pick up any tips that day?

Yes, the microwave bear. You put it in the microwave and it gets nice and warm. It helps him when he gets abdominal pains. Even if it’s only for comfort.

What's your opinion on bone marrow transplants?

We’ve thought about it but it's not a light decision to take. His opinion is also important. We’ll wait until he's a bit older, when he understands it better. If it turns out that it improves and that he needs to continue with the transfusions, we’ll start researching it. So that we have the information for him when he's ready. It's his body, so he's entitled to an opinion.

How does Vaglary deal with all the care providers?

He has little control in his life. First, the adoption and now, hospitals and injections every four weeks. That's why we now allow him to remove the drip himself, so he's regained a bit of control. When he's being examined or when the doctors do something he doesn’t like or doesn’t understand, he's allowed to say something about it or ask questions. I think that’s very important. He can say what he wants to do with his body. We give him control whenever we can.

What’s your vision on his future?

As it is, he can lead a normal life as long as he takes his rest and keeps an eye on his food and fluid intake. He has a blood transfusion every four weeks now. Perhaps, once that constriction is gone, he no longer needs them. If not, he will need transfusions for the rest of his life. That will affect his life. You try and study or find a job when you have to have a day off every four weeks ...

What does he want to be when he's grown up?

He wants to be a doctor because he wants to invent injections that don’t hurt.