harriet

Interview: Additional emotional and behavioural problems

Harriët Heijboer is a pediatrician/hematologist and clinic director of the Children’s department (aged 1-10) at Emma AMC children’s hospital. She specialises in, among other things, sickle cell disease in children.

When did you become interested in sickle cell disease?

I learned about sickle cell disease during my training as a children’s hematologist. I thought it was very interesting because it’s a disease that only occurs in foreign cultures. Patients are often not properly informed and there’s also a lot of fear and superstition. In their native countries, children with sickle cell disease often die at a young age. When parents are told that their child has sickle cell disease, their reactions vary from denial to despair that their child is going to die. I was attracted to the challenge to guide these people, who are often not properly informed.

How do you do that?

I always try to find out what knowledge parents (and later on the children themselves) have about sickle cell disease and what their thoughts about the disease and its origins are. For example, I ask them to explain to me what the disease entails. There and then, I can already tell what is correct or what not and explain for example why it’s important that they give antibiotics every day. Once they understand why they have to that, they often become very motivated.

What about therapy compliance in relation to medication?

Since everything is explained over and over again and because a lot of attention is paid to this during every visit, I think therapy compliance is achieved in about 80 to 90% of my patients. I always ask about it and also compliment them if they do well. Most of them are quite open about it. Sometimes, they say: I only give the medication once a day or sometimes I skip a day. I then explain to them why they really should not do this and try to keep motivating them. Sometimes, you’re brutally reminded of the consequences. A mother, for example, was struggling to administer the drugs to her baby. That’s when we called home care to help her twice a day and after a while, it went well. However, when the mother was left to do it on her own again, things went wrong and after a week, the child had to be admitted with a serious infection. It’s a textbook example of what can happen if the medication is not administered or received.

What does a young child with stable sickle cell disease look like?

It often has yellow eyes (sometimes not, or only occasionally) and looks pale (paleness in someone with a dark skin can best be seen by looking at the palms). He has a fast pulse and heart murmur because more blood needs to be pumped through it. He is lean and small and the abdomen shows an enlarged liver and an enlarged spleen (or, in some older children, a reduced spleen or the spleen is altogether absent). In very young children, the spleen is enlarged but from about eight years onwards, the spleen becomes smaller due to infarction.

And what about adolescents?

Children with sickle cell disease generally reach puberty one or two years later than normal. They do continue to grow for longer, but their growth spurt is delayed. They’re often small for their age and only develop secondary sexual characteristics later on. In the case of boys, you can give hormone injections. This won’t make them grow any taller, but they do develop secondary sexual characteristics sooner. Most boys don’t opt ​​for this once they know that nature will eventually work it out for itself.

What do they find most annoying about their illness?

The yellow eyes are something they complain about most. The fact that they can be called names, making references to a yellow banana. They also find it annoying if they can’t keep up with physical education or sports or if they develop pain after swimming. They don’t want to be the odd one out, of course. In fact, they hardly practise sports within a club context. Not so much because it’s not permitted medically, but mostly because the parents can’t afford it. Practising sports is good and healthy for children with sickle cell disease, as long as they can tell others what their limitations are. Bed-wetting as a teenager can be an extremely difficult and embarrassing problem. It stops some children from going on overnight trips or school camp.

Are girls afraid of having a child with sickle cell disease?

I can’t say that I ever hear them talk about that. I do discuss the possibility of having children with them from the age of sixteen, but often they haven’t really thought about it yet themselves. They’re not interested yet and often don’t have a boyfriend yet either. This is probably also due to their late maturing. With that, they don’t go out yet during the weekends. Many parents are very protective. They keep their children with sickle cell disease on a shorter leash compared to others because they’re afraid of a crisis. When having children is discussed, the heredity of sickle cell disease is discussed as well and how important it is to have your future partner tested for carrier status.

Do children suffer from depressive feelings more often?

This is heavily researched at this moment. About one-third of all patients suffer from so-called silent infarcts in the brain. This is a chronic process that causes a decline in cognitive abilities over the course of time. The children also tend to suffer from emotional and behavioural issues more. Providing proper psychological support in the event of cognitive disorders and emotional and behavioural issues is paramount in order to prevent school results from deteriorating and to ensure appropriate help can be offered.

What size is the group of serious patients?

Out of 240 children, about one-third suffer from serious crises on a regular basis. We don’t know why some children suffer from more serious crises more frequently than others. At birth, we can’t predict which children will go on to develop serious symptoms and which ones will not. We submit all children to a transcranial Doppler ultrasound to detect narrowing of the brain vessels. Out of all children with such vascular stenosis, about 40% suffer a cerebral infarction and 60% do not. It would be a very good thing for this group of children if we could tell them apart because now, all children who suffer from vascular stenosis must be treated. We are currently conducting research into such predictive factors.

Which children are eligible for a stem cell transplant?

Only children who develop serious symptoms or vascular stenosis qualify and for whom traditional methods of treatment, therefore, don’t offer improvement. We’ve only had two of such cases in the past few years. It’s possible that in the near future, the criteria for eligibility for a stem cell transplant will be adjusted downwards.

What pre-conceptual information for carriers do you provide?

We always start with the parents. Many young parents still want more children. And we always discuss the risk of conceiving subsequent children with sickle cell disease and refer them to our genetic consultant for hereditary advice. There is the possibility of performing antenatal diagnostics in a subsequent pregnancy, but only a limited number of parents make use of it. If she becomes involved, the genetic consultant always asks about possible brothers and sisters of the parents and offers tests for the entire family.

How can you raise awareness of carrier status?

We should educate more through secondary education and provide more information to young people who want to have children. In other words, how likely is it that someone is a carrier of hereditary anemia (which includes sickle cell disease) and what options are there to have this investigated. Providing public information is difficult. Some time ago, during a Ghanaian-themed day attended by hundreds of people, we were represented through an information stand. Only a handful showed an interest. So that was a waste of time. In 2005, we set up a number of public meetings with the municipal health service and the national hereditary information centre in Amsterdam in the form of a play created by the target group itself, which proved to be reasonably successful but ultimately, you only reach a limited number of people.

Do cultural differences play a role for you?

Lots of people say you have to provide specialised care aimed at the culture, but I don’t agree with that. My aim is to try to reach a consensus on a more personal basis. You see, you’re usually dealing with mothers. So you try to find a common ground. I think personal contact is much more important than the cultural aspect. It doesn’t matter whether someone is from Ghana or Surinam or any other country; the aim to provide the best possible care for the child (each within their own discipline and within their own range of responsibilities) is something that binds and is transcultural.

Interviews