Various Press-cuttings

(Krantenknipsels / Press-cuttings)
(Weer succes voor Bredase kinderneuroloog)


Source: Weekly paper “De Bredanaar”, March 13th 1996, by Jacqueline Leenders

‘I’ve always known Sebastiaan wasn’t the only one’

“I’ve always known that Sebastiaan wasn’t the only one”, says his mother Agnes Dekkers in her home in the Belcrum quarter in Breda. Therefor she wasn’t surprised when Dr. De Rijk called her in February, telling her that she’d found two new cases of the disease.
A three year old boy and a two year old girl had been admitted to St. Ignatius Hospital and had Dopamine administered, just like Sebastiaan. Agnes: “I had an appointment with Dr. De Rijk the next day, but I couldn’t wait any longer. I had to get to that hospital!! In a short period of time we built an enormous bond with the other parents. Their children have become my children too”.
Sebastiaan was two years old when Dr. De Rijk could tell the parents that he was suffering from a rare metabolic disease.
An end came to the search for Agnes and Philip Dekkers, because none of the doctors who’d examined Sebastiaan, could give a diagnose. “But you still want to know what’s wrong with your child. Even if I had to search for twenty years, I would have continued doing so”, Agnes says, while Sebastiaan stands in front of the TV and roars with laughter because of the jokes Mr. Bean, the British comedian makes. It seems almost impossible, this energetic toddler being severely handicapped only one year ago. A normal buggy, bike seat or kitchen chair were of no use to him. “He could only sit in the laundry basket”, Agnes Dekkers tells me.

Sebastiaan was born on November 28th 1992 at Baronie Hospital in Breda. The birth went remarkably fast and the child seemed healthy. He did look a bit violet at that time and had trouble keeping temperature, but the next day mother and child could go home. About half a year later the problems started. Sebastiaan was trembling heavily and couldn’t keep his food in after dinner. Of course Philip and Agnes went straight to the doctor, but the family doctor that was on call that day saw nothing strange. The family went on vacation and there the situation escalated. “We gave Sebastiaan a rattle in his hands to cover up the shaking and trembling that was getting worse”, Agnes remembers. 
Because of the often throwing up, the baby lost weight and showed dessicating symptoms. When the family doctor saw Sebastiaan after their vacation, he was immediately referred to the hospital. They did all kinds of tests on him for ten days. The results hit Philip and Agnes like a bomb: their boy would be spastic.
New tests at Dijkzigt Hospital gave the same result. Philip, Agnes and his sister Sabrina had to prepare for a life with a severelly handicapped Sebastiaan.

To make further examination possible, an MRI-scan was necessary. Dijkzigt Hospital had a waiting period of one year, but at Ignatius Hospital it was possible to have Sebastiaan admitted within three months time.
Agnes and Philip chose the fast solution, which was also the nearest-by, and although they didn’t know it at the time, they chose at that point a new life for their son. Sebastiaan came under treatment with the pediatric neurologist Dr. J.F. De Rijk-van Andel.
She’d soon found out that Sebastiaan wasn’t spastic, but it would take a lot of searching for her to give the right diagnose. Sebastiaan wasn’t doing well at that time. He had a severe attack of dyspnoea and was urgently admitted to hospital; it might be epilepsy. The MRI-scan they’d been waiting for for so long didn’t give the result they had hoped for. The cause for Sebastiaan’s disease wasn’t found.

In the meantime it had become the spring of 1994. Sebastiaan was one and a half. Agnes Dekkers: “He was like a living doll. He hung in his adjusted buggy and looked very weary. People always thought that it was his bed-time. He couldn’t hold on for more than one hour”.
In November Dr. De Rijk told the parents that only a spinal punction was possible.
She asked the parents for their permission. Agnes, agitated: “Of course we gave permission. It couldn’t get any worse than this, could it?!”
They had given up hope and were totally astonished when Dr. De Rijk told them on December 14th 1994 that she’d discovered what was wrong with Sebastiaan. Concluding from the spinal fluid, Sebastiaan lacked Dopamine, which is very rare. Not only the parents heard what was wrong with their child, but they were also told that there was medication for Sebastiaan.
Agnes: “That night it finally hit me, I couldn’t conceive everything that had happened. They’ve been searching for years to cure a terrible disease like Aids, without any luck so far. Now my son has a rare disease and they immediately have medication for it. That’s just a miracle”.

On January 5th 1995 Sebastiaan had his first DOPAMINE administered. On January 6th suddenly he corrected his posture by sitting up straight all by himself. A couple of days later he said ‘mama’ and from that moment on it changes came rapidly.
He learned to roll over and started playing with his toys. In the summer his parents bought a normal bike seat and buggy, which he could sit in straight up immediately. In August he’s suddenly standing up in his bed, and in October he climbs the settee without help. Sebastiaan learns to talk, to walk and grows into an active, naughty toddler that recently even locked his father out of the house by accident.
In his neighbourhood it starts to show that Sebastiaan has become a totally different child. For example: the family sudenly got a gift-voucher worth 25 guilders from a man, with the words: “I’ve always wanted to do something for that boy. You might buy him some toys?”
There’s yet no telling what Sebastiaan’s future is gonna look like. He has development arrears, but Dr. De Rijk thinks he’ll be able to lead a normal life. Agnes can still hardly believe it: “I’m still scared that he’ll have a relapse”. Her husband Philip: “I don’t have blinkers on: I see what he can and cannot do. You have to be realistic, but the way it looks right now, he might very well be able to attend a normal primary school”.

Sebastiaan’s wonderful story made the newspaper, his parents and Dr. De Rijk appeared on Ivo Niehe’s ‘TV Show’ and on ‘Hart van Nederland’ (Heart of the Netherlands) on the channel SBS 6. Philip and Agnes Dekkers purposely contact the media, because they think that there are more children with the same disease Sebastiaan has. They opened a post office box and receive ninety messages from The Netherlands, but also from Spain and Israel. Everyone that writes to them, is send an extensive story about Sebastiaan’s life.
Dr. De Rijk is now treating yet two more patients thanks to all the publicity. Meanwhile the Dekkers family are busy founding a parents association.
Agnes Dekkers: “Even if we can help just a few children, our goals will be achieved”.

Kaylee wasn’t spastic, handicapped or epileptic, like the doctors initially thought. Last year a pediatric neurologist in Breda found out by accident that in Sebastiaan’s (3) case it was a rare metabolic disease. It makes that signals from the brain don’t reach the muscles. The pediatric neurologist knew how to treat Sebastiaan’s disease with dopamine, which is also administered to Parkinson’s Disease patients.
Kaylee’s parents heard in a news item and a TV-show about the success pediatric neurologist Dr. J. De Rijk-van Andel had in treating Sebastiaan. Kaylee’s symptoms were exactly the same. She was born healthy. When she was about five months old a true nightmare started for her parents. “She didn’t talk, she didn’t eat well, could hardly breathe, perspired a lot when she exerted herself and clenched her fists spastically. She couldn’t stay awake longer than one hour because of weariness. Toys lay purposely in front of her. She could only look at it”. The results of the dopamine-treatment are startling. “The first day after the medication had been administered, she started playing with a little bell. The next day she jabbered a bit”. The Timmermans family fell from one surprise into the other. They kept a diary of the Kaylee’s rapid progress. “The physician at Wilhelmina’s Children Hospital in Utrecht looked like he saw water burning. First Kaylee was lifeless, now she pulls the stethoscope and pencils fly all over the desk”.
Kaylee still has to make up her arrears. Timmerman hopes that more publicity can help other children. The disease was practically unknown up till now, doctors couldn’t do anything. “I don’t want to give other parents false hope however. Not all children that are motionless, have this disease”.

Nobody seemed able to help the severely handicapped Sebastiaan Dekkers. The boy couldn’t sit, walk or talk. He slept or just stared a bit. The specialists that examined him, couldn’t really diagnose him. Until Dr. De Rijk-van Andel saw the child and searched until she found the cause for all the misery. It appeared that the boy had a lack of dopamine. After administering this substance, Sebastisaan really recuperated. From a passive ‘lying-baby’ he changed into a naughty toddler. “Before long he starts at mytylschool ‘De Schalm’ (a school for handicapped children)”, says his mother Agnes Dekkers proudly.
When it was obvious that Sebastiaan was benefited by the treatment, Agnes and Philip Dekkers searched the media to make other parents and physicians aware of this diasease. They appeared on Ivo Niehe’s TV Show and also ‘Het Hart van Nederland’ by the channel SBS 6 paid attention to the subject. Meanwhile Agnes and Philip started the foundation “The Missing Link’. The foundation aims at parents of children with development arrears because of a lack of dopamine. “Every child who has this disease and isn’t treated, is one too many”, is how Agnes Dekkers argues their action.
Parents that suspect their child to have the same disease Sebastiaan has, can ask the foundation for documentation. There’s also a folder that will festively be handed to Dr. De Rijk next Wednesday. The folder is made by the foundation, with approval of Ignatius Hospital.

Anyone that has asked the foundation for information in the past, or is going to do so, will receive the folder. Agnes Dekkers: “People can also get he folder in hospitals, from neurologists and pediatricians. Further more we’ve contacted the local Red Cross Services. Almost every child will have a consult at a children’s health service, where they can find out the disease in an early stage”. 
To be able to make this folder, the foundation was sponsored by a well-known bank. “The managing director came to our house and saw the videotapes of the TV-broadcasts. Tears were in his eyes”, Agnes Dekkers says. For the festive handing over of the folder the news program ‘Hart van Nederland’ by channel SBS 6 is invited again. Agnes Dekkers also expects the Belgian media to come. “Parents of twenty Belgian children have already asked us for information”.

According to spokesman Luc Demarbaix of Ignatius Hospital, it’s very special that parents exert themselves like this. “I frequently meet people who, on their vacation in Roumania or Russia, are so startled by the situation in hospitals there, that they organize a big money collection in Holland. But I admire the perseverance of these people. You often see parents being very impressed at first, but who’ve already gotten used to the situation after a month.
These people are still enthusiastic, I respect them for what they’re doing”, Luc Demarbaix says. While Sebastiaan’s parents make this disease publicly known, Dr. De Rijk fills in her colleagues in other cities about the disease. When a physician makes a discovery this size, it’s customary that the findings are published in a well-known medical journal. This hasn’t happened yet, because the ‘Dr. De Rijk Syndrome’ has to be well argued scientifically. “One success just isn’t enough”, Demarbaix says. Moreover more patients with the same symptoms have applied for a consult with Dr. De Rijk, and a couple of children already benefit from the treatment with dopamine.

Because of the attention Dr. De Rijk is getting, the number of patients that consults Pediatric Neurology at Ignatius Hospital has grown considerably. Parents praise the neurologist’s enormous effort and concern. 
“We’re also very proud of her”, Demarbaix acknowledges. For now the lack of dopamine will be called the ‘Dr. De Rijk Syndrome’, but according to Demarbaix it’s not really sure whether the disease will keep that name or not. “The name is actually a popular called one. A child would be brought in with the same symptoms Sebastiaan had, and the nurses would say to each other: Another one with the Dr. De Rijk Syndrome”.
Anyone who wants more information from the foundation ‘The Missing Link’, can write a note.

Some mothers are absolutely courageous mothers

When you’re pregnant, of course you hope for a healthy baby. But sometimes it appears there’s something wrong with your baby and as parents you’ll have to deal with the enormous impact of it. Agnes Dekkers has a diseased child. Now she’s giving parents in the same situation a helping hand.

When Sebastiaan was six months old, Agnes Dekkers was told that her son was spastic. An enormous shock and a lot of worry. Further tests however showed that Sebastiaan wasn’t spastic, but was suffering from a shortage of dopamine. And that there was medication for it. Now Agnes helps in tracing other children with this deficiency.

“Sebastiaan is born twice to us”, Agnes Dekkers says.
“The first time six years ago, the second time four years ago, after he’d been given medication for the first time. That was incredible. We were told that he was spastic. And would therefor end up severely handicapped in an institution. But in just one hour’s time he started moving, more than he did in the entire year before that. Very unreal”.

“Sebastiaan was born after just three throes. He didn’t cry, his temperature was below average and didn’t breath well. Something told me it wasn’t right. Also because he was throwing up and screaming a lot. And he felt so stiff. Call it maternal instinct. But then again, everything seemed to go well the first six months. So you wipe away your worries. Sebastiaan was growing fine. Until he had an attack. Suddenly he started trembling heavily. He was four months old. We were terribly worried, but we got the brush-off from our family doctor. We were just making a mountain out of a mole hill. According to him it was just a cold. We were about to go on vacation. That shouldn’t be a problem according to the doctor. So we went, because you trust a doctor’s opinion. However, we had better stayed home that week. Sebastiaan was shaking, his eyes looked weird… It was hell, we were so concerned about him. When we got home, he was admitted to hospital. All tests they did showed he was a healthy child. But he didn’t do anything. Just sprawling his legs. He clinched his fists, his feet pointed. What do you mean, a ‘healthy child’? Then we were told Sebastiaan was spastic. Whick shook us up awfully. It was quite a fight, accepting that diagnosis. Children Sebastiaans age started walking, me however walked anxiously into his room everyday. Would he still be there?”

Source: Belgian ‘Libelle’, by Diane Broeckhoven