Bram is a drummer

Bram is an enthusiastic drummer. In his bedroom, he is pounding his drums with life-crashing force. He takes after his father, Dree, who caused a stir with the Dutch percussion band Slagerij van Kampen. As he bangs away on his electronic drum kit, it’s obvious that Bram inherited his musical talent from his dad. Dree and Margot look at their son with pride. ‘He’s having more and more trouble climbing the stairs to his room, though,’ Dree says. ‘We have no idea if we’ll be going on holiday with our folding trailer next year.’ The uncertain future of a child with Duchenne Muscular Dystrophy (DMD) has an effect on everyone around him.

"Surely something could be done to remedy the problem, right?"

Eight-year-old Bram is Dree’s and Margot’s only child. ‘We have known for quite some time that Bram has DMD. Ever since he was two years old. Bram started walking three months before he turned two. The doctor at the child health clinic noticed he walked awkwardly and suggested a pelvic examination.’ Dree, who teaches art and culture at a secondary school in Eindhoven, and Margot were referred to a children’s physiotherapist. ‘She happened to work at a rehabilitation center and instantly recognised the symptoms of DMD. Of course, she wasn’t in a position to make the diagnosis. So she advised us to immediately see a paediatrician, hinting there could be something wrong with his muscles. Initially, we were somewhat matter of fact about it. Surely something could be done to remedy the problem, right?’

‘I remember it very vividly,’ Margot recalls. ‘We had an appointment to see the paediatrician on 5 December [when Sinterklaas is celebrated in the Netherlands] at the Catharina Hospital in Eindhoven. After the assistant ran a number of tests, she came back with Dr Brackel. As soon as they started talking, we knew it was bad. From the doctor’s serious tone, it was clear that this wasn’t a joking matter. He put it to us straight, in no uncertain terms. In retrospect, we are thankful for this. He called us every day of the following week. Eventually, a preliminary diagnosis was made by a neurologist. And then our whole world collapsed.’

In the week following 6 December numerous test were taken. On 18 December, they were told that there was an 80 to 90 percent chance of Bram having DMD. Ironically, a week earlier, a neighbour had said: ‘If only it’s not DMD.’ The doctor warned them not to surf the internet because the posted information could be confusing and confrontational. Dree nevertheless logged on. Margot waited until after the diagnosis.

Confusing times

‘For us, December of 2003 was a strange and hectic month. We went to see friends in Spain, which was fun for Bram. He was still small and such a happy little fellow. We spent that holiday taking long walks on the beach and talking a lot. Despite our sadness, we were able to enjoy ourselves. But the next few years, we couldn’t celebrate Sinterklaas or Christmas without remembering those weeks.’ Dree continues, ‘I don’t think about that time much anymore, simply because I’m busy with other things. Bram’s progress and what the disease has in store for us is always on my mind.’

"Bram’s progress and what the disease has in store for us is always on my mind."

‘At first, we were just plain angry,’ says Margot. ‘You sincerely wonder how you can ever be happy again. You even find yourself resenting other children. Of course, you don’t really resent them. But when I see children running around at Bram’s school, I do occasionally think:…. Ihre Sorgen möchte ich haben [I should have your problems].’

"When I see children running around at Bram’s school, I do occasionally think… damn it."

Hero

Bram is the school hero. Everyone sticks up for him. Margot feels fortunate that in his class Bram is surrounded by lots of special attention. But we’ve heard that this is also true for other children with DMD. Anyone who messes with Bram has to answer to his friends, who include boys, girls and the teachers. Last year, he participated in swimming lessons at school. One of the mothers present told me that Bram was one of the last children to finish a lap, and to her astonishment, everyone started cheering. She couldn’t believe her eyes. His classmates, however, thought it was absolutely normal. For him to be able to do this was brilliant, wasn’t it? It brought tears to that mother’s eyes.’

"For him to be able to do this was brilliant, wasn’t it? It brought tears to that mother’s eyes.’"

Duchenne Parent Project does help

Margot: ‘Not knowing where to turn to makes coping difficult at first. The only thing they tell you is that, in time, you should make an appointment with a rehabilitation center. You can already visit one, if you want. But at that point, that’s the last thing you feel like doing. We did start attending the annual Dutch National DMD Day fairly soon.

‘The Duchenne Parent Project answered all of our questions. For instance, one of the things you want to know is whether you can continue living in your home. What does it mean to have a child with such a serious disease? Naturally, we dreaded going there the first time because you know that you will be confronted with other children. This means you will see what your son will look like later. But on the other hand, we heard about exon-skipping for the first time, which was a real eye-opener.

‘Elizabeth Vroom’s story in Dutch women’s magazine Viva inspired me,’ says Margot. ‘I started with organising a charity walk. Over sixty people ended up taking part. Even some of my cousins who lived miles away participated and brought their friends along. All in all, it was a fantastic and fun experience, also because it allowed us to deal with the disease from a more positive angle. We managed to hang on to this positive vibe for a long time. We eventually founded the Bram Foundation, for which the notary provided his services free of charge. From there on, the ball kept on rolling. A year later we organized a bicycle tour and a charity concert with famous bands from Eindhoven like The Watchman , Betty Blue and Slagerij van Kampen. This was also a great success and again we raised a great amount of money.

The Duchenne Parent Project strongly encourages these types of activities, especially during the annual Dutch National DMD Day. The positive energy flowing around there is absolutely amazing. For us, it helped us get our life more or less back to “normal”. As it turned out, leading a life with a DMD child was possible after all.’

Exon-skipping

Bram is eligible for exon skipping. Dree and Margot would jump for joy if Bram were selected for a clinical trial in the future. ‘But I believe that’s all rather complicated,’ says Dree. At the Radboud Hospital in Nijmegen, Bram is currently in a programme for DMD patients called Rust Roest. The aim of this new research is to find out whether he can keep his muscles supple through working out. The hospital will provide him with a home trainer to exercise his legs and arms. The project is being led by rehabilitation specialist Dr Imelda de Groot.’

Bram is doing less well

Bram has now reached a phase in his physical development where Dree and Margot will have to start thinking about making adjustments to their unique home. Due to his short calves, Bram has trouble keeping his back heels down when standing. As a result, he has been walking on his toes for some time now. Getting the right angle is becoming more and more difficult. He also has to wear splints at night to prevent his feet from growing crooked. ‘Bram can still climb the stairs… but only just. The last six months, he has been having more and more trouble doing so. Until recently, he has been relatively stable,’ says Margot. ‘But we have started to notice that he’s getting worse. Getting up from the floor takes him a lot longer now. ‘Sometimes, he cannot cope without supporting himself with something,’ Margot adds. ‘Whether this is early on, we don’t know. Some children are still healthy at the age of ten, while other children’s health deteriorates as early as at the age of seven.'

"Getting up in the morning takes him forever. He used to be able to get out of bed from a sitting posture in ten seconds. This takes him a lot longer now."

Renovating or moving house

Dree and Margot are still in limbo as far as the renovation is concerned. Their home has been designed by the Dutch architect Dudok, which makes it a listed house. ‘We aren’t allowed to extend. So at the moment we are looking into the possibility of having a shaft lift installed, from the living room all the way up to the attic. But this has taken some doing. Initially, we were told that we had to move to a more appropriate dwelling. Renovating would be far too expensive. Then a municipal official reopened our file.

Margot continues, ‘Dree and I are very attached to this house.’ ‘I have lived here for thirty years,’ Dree adds. ‘But it isn’t easy to sell your home in the current market. And that’s not all. You still have to buy an even bigger house. We don’t actually want to move. Bram’s school is excellent for him. Dree and Margot still have their doubts. Dree says, ‘Bram is our number one priority. I may want to stay here, but at the end of the day, we have to consider what’s best for Bram.’

Still enjoying life

Dree and Margot say they still want to enjoy life. ‘Giving in to the sadness doesn’t do Bram any good. So we don’t. Although this may not always be a financially wise thing to do.’ Margot: ‘When Bram was about three, it dawned on me that his condition has made my life a lot simpler. I used to worry about small things. Having one goal in life – making Bram’s life with DMD fun and happy – is actually quite nice. This doesn’t mean you have to ignore your own needs. Quite the contrary. It’s just that you have a clearly-defined life goal. And this also gives a sense of peace.’

"Having one goal in life – making Bram’s life with DMD fun and happy – is actually quite nice."

Sorrow

Dree and Margot just got back from a holiday in Spain. ‘This vacation in particular brought home the harsh reality of DMD. We spent the holiday with friends. They have a daughter who is in Bram’s class. Last year, this worked very well. But this time, the little girl had already found some friends to play with since our friends had arrived prior to us. This meant that Bram more or less tagged along with them, which he didn’t always manage. But you should have seen his perseverance. He wanted to keep up more than anything. Just looking at him tired me out. Now and again, he would feel sad about it. And as a mother you feel his pain. Suddenly, you realise that each year will bring new adjustments.’

"But you should have seen his perseverance. He wanted to keep up more than anything."

Self-confidence

‘You keep asking yourself how you can help him to boost his self-confidence, despite the fact that he knows he’s different from other children. You don’t want him to feel inferior. And you don’t want him to think that other children won’t like him because of it. You have to be able to address these matters. That’s what I find the most difficult,’ says Margot. ‘He is becoming more and more aware of his condition. He says things like: “My muscles are sick.” When we organised the event with Slagerij van Kampen, Bram was five years old. We printed posters with a picture of Bram on his tricycle. At the time, Bram wanted to know why he was on them, so we had to give him an honest answer. What’s more, all of his classmates were already riding bikes without training wheels. Of course, he also wanted to do that. But that just wasn’t possible. He then wanted to know if he would ever ride without training wheels. We had to honestly answer that question, too. Bram cried his heart out. Over those training wheels… At that moment, he couldn’t be bothered with his sick muscles. Later we got a duo bicycle, to which he responded: “See, now I can ride without training wheels.”’

"That’s what I find the most difficult. He is becoming more and more aware of his condition. He says things like: “My muscles are sick.”"

‘Another time, Bram made a striking comment in response to a friend who had said he was really sad about Bram’s disease. “But I get to have a special bicycle and a cool wheelchair,” he said. As if it was some kind of present. Bram now has one of those motorised bicycles.’

"But I get to have a special bicycle and a cool wheelchair."

‘As far as the future goes, we take it one day at a time,’ Dree continues. ‘You can’t plan things. For years, we have being going on holidays with our folding trailer. But that’s becoming increasingly difficult. It’s simply impossible to plan ahead for next year’s holiday. We also don’t know how long he can attend a regular school, even though his school is willing to make all the necessary adjustments, which is fantastic. All we want is to make Bram’s life as happy as possible, but we haven’t got a clear picture of how it will be.’

"All we want is to make Bram’s life as happy as possible, but we haven’t got a clear picture of how it will be."