Just three slices of cake

(© Nicklas Öbom)

William's heart has just three slices of cake; that is how William's big sister Fanny describes it to her preschool companions. And her other little brother, Cristofer-Robin, has a heart with doors that don't close properly.

This is supposed to be the story of William's TCPC operation, but it is so much more. It may actually be a story of percentages.

“When William was born and turned out to have HLHS, the doctors said it affected only a small percentage of children,” says William's mother, Veronica. “When it was confirmed that his little brother Cristofer-Robin also had a heart defect they said the risk of having a sibling with a congenital heart defect was minimal, just a few per cent.”

“Because we had gone in for a targeted ultrasound, and felt relatively reassured, it was a shock when it was discovered after a few days at the hospital that Cristofer-Robin had a heart defect as well. You shouldn’t promise children anything in advance, but I had told Fanny that this time her little brother would be healthy and not have all those tubes when she saw him. So when the doctors told us that he had a heart defect I rang my sister, who was looking after Fanny, and said, ‘You need to bring Fanny at once because Cristofer-Robin has to have an operation.’ I wanted her to get to see her little brother, pristine and beautiful as he was, before the surgery merry-go-round started on its way... again!”

Mechanical valve

At home with William, now two-and-a-half, we find parents Veronica and Nicklas, sister Fanny, 5, and one-year-old baby Cristofer-Robin, who was born with a hole between his atria and a constricted and malformed aortic valve. Cristofer-Robin underwent surgery when he was two months old, and awaits the installation of a mechanical valve at some point in the future. The family lives in a yellow row house in Svartbäcken, outside Stockholm. William’s neighbour and best friend is Ludwig, who was also born with HLHS.

“William's TCPC operation was to be his reward, and afterwards he was to be an almost totally normal little boy,” says Veronica. But things did not turn out quite that way.

William finally underwent surgery on September 8, 2008 in Lund. Despite having seen more than their fair share of paediatric heart surgeries by then, his parents found the experience as frightening as ever. Doctors told them that it was just a routine operation for William, but of course there was always a risk that something could go wrong.

Intact aortic arch

(© Nicklas Öbom)

After the operation, the surgeon said it had gone extremely well and that they had even finished an hour-and-a-half earlier than expected. William met all the criteria for a favourable outcome, with minimal leakage and an intact aortic arch. Still, that small risk eventuated – something did go wrong.

After a few days in intensive care William was transferred to the general ward. Understandably, he had a dreadful fear of bandages, doctors and most things having to do with healthcare. When the nurses approached him he would panic, turning his head this way and that, and kicking his feet. But his arms did not move. “At first we thought maybe he didn't want to move them because he had things inserted into his hands,” says Veronica. But they soon realised there had to be some other reason.

“I said to one of the nurses that it was as if he was paralysed. It wasn’t until a few nights later, when it was Nicklas's turn to sleep by William and he noted that something's not right here that William was sent in for an X-ray.” The X-ray revealed that William had a number of small blood clots in his brain. “Getting that news gave us the same feeling we had when the cardiologist told us that William had a heart defect,” says Veronica.

Oxygen deficiency in the brain

An MRI was performed the next day, revealing an oxygen deficiency in the brain.
“They told us that our son had a CP injury. The news was too much for me,” says Veronica, “and I started to cry. I locked myself in the bathroom and turned on the tap to drown out the noise. Nicklas was left alone listening to the doctors.”

The oxygen deficiency had occurred on the right and left sides at the front of the brain, in the part that controls the motor functions. The deficiency was more pronounced on the right side, the side that controls the left side of the body. Oxygen deficiency had also been detected at the rear of the brain, which controls visual perception. “I didn't believe it was true. ‘They have ruined William,’ I thought. We came down here with a healthy little boy, and he came out from the operation as a vegetable,” says Veronica.

William was unable to sit up and had no stability in his neck or legs. He didn’t move his arms; all he could do was talk and kick his feet. Veronica remembers regretting that she had let William undergo the operation. “But we didn't have a choice,” she says. “If William hadn't had the surgery, we would have caused him a very hard life, which gradually would have led to his death. Well, clearly everyone wants the best for their child. You want to give them quality of life and that's what the TCPC was supposed to give us, a livelier and healthier William. Now he will live as the stubborn little boy he is, full of vitality, but obviously at his own pace and within his limitations, until the day when a transplant becomes relevant. But we're trying not to think too much about that right now, and we hope that it lies far in the future, although the prospect is always with us.”

Physical therapy yielded results

(© Nicklas Öbom)

William’s physical therapist Charlotte was tremendous when things looked most bleak. She refused to give up, and worked with William every day, fighting to get him to be able to move again. William was cross and despondent. He had been able to walk before the operation, and now his legs just gave way beneath him. When William sat in a wheelchair his head had to be supported so that it would not loll to one side. He was frustrated and afraid the entire time; he didn't recognise his own body. Sometimes he would be peaceful as he lay in bed watching Bob the Builder with his mother and father on either side, and his stuffed tiger and teddy bear close enough for him to feel them. He also wanted a soft security blanket below his nose, to comfort him. He would be calm then, but because he could not move his head to see whether his mum and dad were really there, he asked after them at regular intervals. And as soon as he spotted anyone wearing a white or blue hospital gown he would cry out “Oh, oh, oh, it hurts!”

But Charlotte's dark red gown was OK. Charlotte also gave Nicklas and Veronica tips on what they could do, such as massaging the parts of his body that William was unable to move. The psychologist explained that William was severely stressed, that he had passed through the burned-out stage and entered something even worse. It was decided at a meeting between the doctors, psychologist, social welfare officer and physical therapist that William should encounter doctors and nurses only three times a day, and preferably the same people. Because he was so afraid, permission was also granted to sedate him any time a new insertion or intervention was required. “He was sedated a total of 11 times,” says Nicklas.

Miracle boy

Veronica, Nicklas and Charlotte soldiered on and gradually they saw results. Today William can walk again, his neck is stable and he uses both arms, although he is reluctant to use his left hand. “They call him the miracle boy down in Lund,” laughs Nicklas.

Since returning home William has regularly seen a neurologist, who believes his motor function can be restored to 97 per cent. However, the doctors are not yet sure how extensively William’s visual perception has been affected or whether he has learning problems.

“Despite everything that has happened to William in his two-and-a-half years, he is a very social little boy who loves life,” says Veronica, “and it seems that he carries true miracles within himself – he never ceases to amaze us.”

All the family wants is to lead a normal life again, after a year of nothing but hospitals. Nicklas and Veronica also hope that William will start eating again. Since the operation in September he has eaten almost nothing except small bits of cheese. He receives his nourishment from gruel and nutrient drinks. Meals usually take several hours a day.

“We have been battling for two years, and it has left its marks. Fanny has had to deny herself a great deal, both in terms of activities and spending time with friends, and at home. She has spent a lot of time by herself because we’ve been taking care of William,” says Veronica. “Fanny is a helpful and understanding girl, but we have seen times when her eyes have filled with tears and she has gone into her room alone and pottered about while the chaos continued outside her door.”

What went wrong?

Veronica and Nicklas have asked the surgeon what went wrong during the operation. The presumption is that there was a period when the oxygenation level was only 40 per cent. This occurred when a shunt that was to be inserted proved too big for the hole, and a new one had to be ordered. This took four or five minutes – perhaps too long for William. “Having just 40 per cent oxygen for that long doesn't work for all children, and I believe that they have raised that percentage now,” says Veronica as she lifts up Cristofer-Robin, a little boy who still has at least two heart operations to undergo.

Author(s): Ulrika Hallin
Last updated: 2010-03-17