“I was alone with her at that moment and it’s really something you can’t prepare for. It was so surreal, I immediately felt a primal feeling. I calmed down, acted and immediately thought: the next phase has arrived. It was a moment of ‘damn, she already has this’. It immediately goes to your throat. Will she have more outbursts soon? That’s what you’re really afraid of.”

To tell or not to tell

Pleun is on medication for her epilepsy, so she has not had an attack for a year and a half. Evelien explains that Pleun herself does not know that she is ill or what awaits her. “We don’t want that either. Of course we had our struggles about this in the beginning: should you tell us or not? Should you keep such a secret from her?”

“But how are you going to tell your child that she will forget everything she learns, that she will end up in a wheelchair and will soon no longer be able to talk? That is already too much for us to comprehend, so what is that like for her? Pleun gets answers when she comes up with questions, but we provide the information in doses.”

Pleun has been blind since she was eight. Photo: Sushilla Kouwen

Raw edge

Evelien and her husband are completely on the same page when it comes to caring for Pleun – and also their other daughter. “We are very balanced. If one person is high in his or her emotions, the other can take over for a while. It’s just a lot to go through and I can’t always indicate what I need. People talk about ‘living loss’, but I find that complicated. Should I accept that loss? No, I’d rather be in life. Our lives have a raw edge because of Pleun’s disease. I don’t want to be an ostrich, but prefer to celebrate the beautiful moments and successes, no matter how small they are.”

Although Evelien and her husband indicate that they are handling the situation well, that does not mean that it is easy. Every day they wake up wondering when Pleun will enter the next phase. “I’m always paying attention to see if anything has changed. Does she remember everything? Doesn’t she forget anything? I’m constantly on and I’m everywhere. Batten disease is always there. No one knows the course of life, but we actually know too much about what is coming.”

“At the moment things are actually going well and Pleun is never sad, angry or frustrated – not even about the fact that she has become blind. Sometimes I think: if this is it, then it’s fine, because we can deal with this. It’s just that you know what’s coming next. When I look at her now and think that she will soon no longer be able to walk, that we will no longer be able to communicate, that she will no longer be able to eat… That is simply not possible,” she says emotionally.

Pleun is a happy child and does not know that she is ill. Photo: Sushilla Kouwen

Daughter Billie

A second big blow came when Pleun’s diagnosis in 2019 revealed that Evelien and Koos both have the same error in their DNA, which causes Pleun’s problems. This means that their children have a 25 percent chance of the disease. But what does this news mean for their youngest daughter Billie? Would she have it too? And do they want to have her tested for Batten disease?

“At first we thought: we don’t want to know, we’ll leave it be, we’ll wait and see. But it didn’t work that way in my head. I was constantly checking whether her vision was deteriorating, for example by secretly holding my fingers above the table to check whether she could still see everything. At a certain point – we were just setting up our catering tent for the summer season – I felt fed up and knew that something had to be done. I had to know, we just had to test.”

Worst day

And so in 2020 they decided to do a test in the hospital in Utrecht. “I think that was the worst day of my entire life. After the test we had to wait for the results at home. My best friend sat heavily pregnant next to me on the couch awaiting new life and I sat there awaiting a second death sentence. I was convinced that Billie also had Batten’s disease.

When I heard the news I wanted to throw up and I hung up the phone. I was completely taken aback when they said, ‘It’s okay, she doesn’t have it.’ It wasn’t until hours later that I was able to call back to ask if they were really sure. At first I thought: what is the benefit of knowing? But I now know that it really brought peace.”

Evelien’s daughter has progressive Batten’s disease: ‘How do you explain this to a child?’ Read also

Medical trial

Making memories together for a long time to come is of course the family’s biggest dream. They are therefore doing everything they can to see if Pleun can participate in a medical trial, if necessary in America. Rather, they hope to receive the treatment in Europe or even the Netherlands, preferably from their own pediatrician. A process that, if possible, will cost a lot of money.

“They are doing a trial in America for children aged 17 and the first results are cautiously positive. The disease appears to be slowing down and this gives time. For us, time is everything. That is why we want to join in on this. When we were diagnosed five years ago, there was nothing at all to help Pleun. Now I keep hope because the medical world is really working on it.”

rainbow ice cream

In recent years, Evelien and Koos have raised a huge amount of money for scientific research. In 2022 they also started the Foundation for Pleun and rapper and singer Snelle has stepped up as an ambassador. Pleun herself is also involved in raising money, although she does not know this herself. When she could still see, she was allowed to design an ice cream for an ice cream maker friend. The result was Pleun’s Rainbow Ice Cream.

Pleun and rapper Snelle at the promotion of the Regenboogijsje. Photo: Rick de Visser

Father Koos shared the impressive ranking of the responsible, healthy ice cream on LinkedIn. To date, 75,000 ice creams have been sold, of which 50 cents per ice cream sold goes to Pleun’s charity. “We especially want to raise awareness for this disease in a positive way, and it is nice that there will soon be money if necessary. We really hope that the trial will come to the Netherlands and that we will then be ready to join in, which is why from this year on the proceeds will go to our own foundation #voorpleun.”

Rapper Snelle gives his car rainbow colors to support terminally ill Pleun (7). Read also
2024-03-03 07:35:41
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