Part 1: When your child is incurably ill
‘They told us that the life expectancy of those
children is low. Every day feels like a bonus’
When Maria Feenstra gave birth to her third child three years ago she felt the most lucky woman on earth. With two older sons she finally received the healthy baby girl she had longed for. Maria feels like she is on the top of the world. But within a few days she starts to worry. Marlie fails the hearing test. However, it will take three months before their parents receive the devastating diagnosis. Their daughter Marlie suffers from the Vici Variant Syndrome and therefore has multiple disabilities. “In the hospital they are very straightforward,” says Mary. “They immediately told us that the life expectancy of those children is very low. For us every day feels like a bonus. Another day to enjoy our princess. Even though life with a special needs child is never easy. “
During the pregnancy everything seems perfectly fine. Except for the high blood pressure Maria suffers from, as during her former pregnancies. As precaution the general practitioner does many ultrasound examinations from the start of the pregnancy. To her big surprise Maria discovers her neighbour is pregnant as well. Her due date differs one day from Maria’s. Fantastic, until the neighbour gets the horrible news that the fetus is not viable. It throws a dark shadow on the happiness they shared just before.
At the end of the pregnancy Maria ends up in hospital. Her blood pressure is too high and the position of the baby isskewed. The baby has to get out soon. Maria is fine with that as long as she doesn’t need to deliver on the same day as her neighbour. ‘She wanted to carry her baby as long as possible’, she remembers. . Maria asks for a caesarean section, but first they try to induce the delivery three weeks before the due date. “Eventually, a caesarean was needed anyway,” she says. “But as soon as you hold the baby in your arms, that’s forgotten.”
During the first week after the baby was born, everything seems to go well, until the baby keeps failing the hearing test. Tests at the audiological center in Zwolle show that there is nothing wrong with Marlie’s hearing. ‘Everything was going to be fine.’ But at home it’s not easy. ‘Deep down inside me I knew something wasn’t right. But I didn’t know what.’ Maria fights a lonely fight. ‘Marlie was a very sweet baby. She didn’t cry. After two rowdy boys, I enjoyed the peace and quiet.’ It takes one-and-a-half-hour to feed the baby. ‘All day long I was busy with bottles. Secretly I didn’t mind having her close all day.’
Over time Maria picks up on some worrying signals. ‘She doesn’t look at me,’ she tell others, but nobody responds to that. Her husband Paul tells her not to worry. ‘She does look at me,’ he assures her. Nevertheless Maria starts investigating it. An osteopath notices a small developmental delay. Not surprising, since Marlie was delivered early. ‘Perhaps she just needed more time, I thought’ But a children’s physiotherapist says: ‘If it was my child, I would be worried too.’
Paul, Maria and their three children.
The next step is visiting an optometrist. ‘One of my sons had to get glasses when he was three years old, maybe that was the problem?’ The optometrist suspects it might be glaucoma, and refers Maria to the hospital in Groningen. All seems to be OK. ‘My husband is a truck driver on an international route and he’s away from home for the whole week. I always take my mother with me to the hospital. Together we celebrated the good news with coffee and cake. At one hand I was happy, but at the other hand I wasn’t. Because if the eyes are OK, there must be something wrong with the brain!’
Desperate Maria consults a pediatrician. ‘We have been dealing with this uncertainty for three months now,’ she says. ‘I want to go now!’ Paul thinks she’s imagining things. ‘She does look at me,’ he says. ‘I was fighting on my own,’ tells Maria. Paul doesn’t want to stay home and leaves with the truck. But in the hospital the pediatrician immediately says: ‘This is a special little girl.’ Somehow Maria is tremendously relieved. ‘Finally somebody says so. I have been in doubt so much. I even looked up the symptoms of post-partum depression. Now I know it’s not me.’
That ‘relief’ is short lived. Straight away Maria has to make decisions. ‘Do you want to go home first, or do you want to have her admitted to hospital to get a MRI-scan?’ Maria calls Paul. ‘You have to come home straight away. It’s not good.’ He still doesn’t believe it, but he comes directly. When they get the results of the MRI-scan, they hear that Maria doesn’t have a corpus callosum. ‘You can stop there, I know enough,’ she says shocked. ‘How can you know what that means?’ asks the doctor surprised. ‘We don’t even know ourselves yet.’ But Maria has a cousin and a friend with a child without a corpus callosum. ‘Both children passed away in the past few years.’ Maria and Paul are devastated.
They discover that Marlie suffers from the Vici Variant Syndrome. This syndrome is caused by a genetic mistake on chromosome 18 epg 5. The results are disastrous. ‘Marlie can’t sit, stand or talk. Her eyes might be good, but that’s difficult to test. The left and right part of her brain don’t exchange information, because the connecting part of the brain, the curpus callosum, is missing.’ Unknowingly Maria and Paul both carry this gene.
Now they know that this means for their children. The chance a child will suffer from the syndrome is 25 percent and the chance it will be healthy is the same. The other 50 percent of children is healthy, but carries the gene. ‘Thankfully our two boys are healthy, but we don’t know if they carry the gene or not. When they are eighteen they can have that tested. You understand it’s not easy for them, knowing it’s possible they carry the gene. It’s very difficult.’
Dutch text: Alice ten Napel. Pictures: Maria Feenstra
English translation: Daniëlle van Westen.
Next time in part 2: ‘What kind of treatment do you choose if Marlie has to be admitted to hospital with life threatening circumstances?’