Benjamin Harper with his brother Jackson
Baby Benjamin Harper lies in his cot with a slim feeding tube trailing from his nose, his little body working gently, eyes wide and his mouth relaxing into a smile in response to a bit of face pulling. Delivered seven weeks premature and clocking up three operations in the nearly five months since, he has been slow to put on weight.
But considering the amazing operation that he had before he was even born, he is doing really well.
In fact, Benjamin is a pioneer, the first Kiwi baby to have surgery for spina bifida while still in the womb. The operation was carried out at 24 weeks gestation and has significantly improved his chances of walking. Despite ongoing issues, his parents, Catherine and Paul, are very happy with the results and grateful to all who made it possible.
When Catherine went for her 20-week scan early last December the couple learned that their new baby had the most severe form of spina bifida – myelomeningocele – as well as a Chiari malformation of the cerebellum, the part of the brain that controls balance.
The baby did not have the characteristic “bulge” of tissue and fluid, but “an open railway track” on the spine, indicating a high risk that he would not be able to walk and could have trouble controlling his bladder or bowel. The leaking of cerebrospinal fluid from the spinal lesion was pulling the brain backwards, the ventricles were enlarged and fluid was accumulating there, meaning there was a high likelihood of Benjamin needing a shunt.
It was devastating news for Harpers. Since the birth of their first child, Catherine had suffered four miscarriages. Now the first baby to have “stuck” in the three years since Jackson was born would most likely have severe disabilities.
“We went home and cried a lot,” says Catherine. It did not help that her ob-gyn specialist delivered the diagnosis with advice to terminate the pregnancy. “He didn’t want us to suffer, but for us it was a matter of, ‘Well, he has this; is it going to change how much we love him? No.’”
Instead, Catherine got busy on the internet finding out all she could about spina bifida. In terms of support and information leads, Facebook was out in front: “Social media is fantastic because you hear from people who have coped with this challenge, and the achievements of people born with it, and it’s so encouraging.”
The big find, however, was that surgeons in the United States were operating in utero on babies like Benjamin. Following research that began in the 1980s, doctors at three specialist centres in the US began a study called MOMS (Management of Myelomeningocele Study). The benefits were so obvious that the trial was ended early and the surgery has become standard at some fetal medicine centres.
It involves making a vertical incision in the mother’s abdomen to expose the unborn baby so doctors can stitch up the opening on its back. At best it means the child will be able to walk and not need a shunt – a tube to drain fluid from the brain to the tummy – or the ongoing need for replacements.
However, what the specialists call “prenatal fetal repair” currently requires a large and highly skilled medical team, comprehensive care for both mother and baby, and comes at considerable cost. For these reasons, presumably, it is not yet available in a small country like New Zealand (although fetal heart surgery was pioneered here by William Liley) and is offered at only one hospital in Australia.
When Catherine discovered that Dr Glenn Gardener and his team were doing the surgery at the Mater Hospital in Brisbane her hopes rose. She sent him an email and within 24 hours she had heard back with an invitation to send her case file immediately. The window of opportunity was small: she was already nearly 22 weeks pregnant and the surgery is not done after 25 weeks.
There followed a period of intense activity: getting the information to Dr Gardener; an application by Catherine’s specialist at Auckland Hospital to see if the New Zealand government would meet the cost of the procedure (it did); a crowd funding campaign launched by family and friends to meet transport and other costs; a trip to Brisbane for preliminary tests, and an assessment of the data by specialists in the United States that would determine whether the Brisbane team would go ahead.
Back in Auckland the Harpers had a call from Dr Gardener (“He’s an angel!”) Christmas Eve saying they could go ahead, and booking the surgery for the 29th.
“It was Christmas and nearly all his staff were on leave, but they all came back for it. They were fantastic, doing all this for a Kiwi,” says Catherine. Her specialist from Auckland Hospital also came to observe.
Catherine and her baby were only the third joint candidates in Australasia for a complex procedure involving high risks for both mother and baby and intensive care for weeks afterwards. It was, she admits, “super scary” when the day arrived. But a month later she was back in Auckland and looking forward to the day when her baby could be safely delivered by caesarean section – ideally, not earlier than 37 weeks. She would be on bed rest for the duration.
Her first scan in Auckland amazed the doctors as it showed that the Chiari formation had reversed. Closing the opening on the baby’s backbone had also stopped ongoing damage to the spinal nerves, and the doctors were impressed with neatness of the repair, given that it was done inside the womb. But the Harpers had been warned not to expect too much. “We were told to expect no movement, no feeling below his knees.”
Ultimately Catherine did not make it to 37 weeks; amniotic fluid began leaking from her uterus and Benjamin had to be delivered at 33 weeks. But, to the great joy of his parents he came out crying lustily and kicking his feet, and continued breathing on his own, not needing oxygen. Steroid shots prior to birth had helped with that.
That was on March 1st. The path since then has not all been smooth. After three weeks a scan showed that a syrinx – a fluid-filled cavity — had formed at the junction of the brain stem and spinal cord and a shunt had to be inserted to drain it. This has been replaced twice. Further surgery may be needed to deal with the syrinx. It has been hard for his parents to see their little boy suffer these surgeries and know there is more to come.
And yet, there he is, smiling in his cot, and his mum says he is beginning to chuckle. His cognitive function is high and his bowels and bladder normal.
“We are just so blessed to have him. Big brother Jackson loves him and is always kissing him. He tells Benjamin, ‘I am going to teach you how walk, and how to run!’ His little brother has taught him empathy,” says Catherine. “We’re so happy that he chose us.”
The pre-natal procedure and c-section have left her own body weaker but it could still be possible to have another baby.
Would she recommend this possibility to others?
“It definitely is a good opportunity to help your baby, although it does require a lot of care afterwards. Good support is essential and you need to be mentally strong to handle it. People have said to me that they couldn’t handle the challenges, the suffering, but I would say that you can’t tell until the situation is right in front of you. Don’t underestimate yourself.”
Catherine is happy that there is now interest in the procedure here, but, surgery or not, she wants to help others faced with a pre-natal diagnosis of spina bifida. There’s a well-known video online called “Project Eli”, which is beautiful but out of date, she says.
“I want to make a new video, one that focuses on the child, not the condition. The incidence of spina bifida in New Zealand is 1 in 1000 births, so it is reasonably high, and yet 80 percent of these babies are aborted. I can understand why: the professional attitude is that you are vulnerable, and it costs a lot. And yet, thanks to social media, we know there are many good news stories out there from people affected by it.”
Besides, she says, technology is advancing all the time, and already some fetal surgery is done laparoscopically, reducing trauma and recovery time.
And the more parents who embrace a baby with spina bifida, the stronger the incentive to take the technology forward.
Carolyn Moynihan is deputy editor of MercatorNet.