Disclaimer: I did not undergo in-utero surgery for my son’s spina bifida. I have studied this report on the MOMS trial extensively and used it as an example in my biostatistics and public health policy courses during my MPH studies.

Parents who receive a prenatal diagnosis for spina bifida are in a unique situation. They have the option of a relatively new procedure for performing in-utero surgery to correct the defect. But since the surgery can only be performed within a narrow window of time, they have to make a decision about a major medical procedure in the course of a few weeks. Bioethics textbooks have thrown this scenario around a lot because of the competing interests in research and the vulnerable emotional state of parents make it very difficult make an informed decision.

People don’t usually think of the diagnosis of a special needs child as a grieving process, but it is. You expected a healthy baby like any other. When you find out your baby has spina bifida (or another birth defect), you begin the process of mourning the child you thought you were going to have. Your grief is OK though. By grieving, you will be able to open up yourself to accepting the beautiful child you have.

The third stage of grief is bargaining. This is when we attempt to regain control over the situation when we feel so helpless. Emotionally, getting a new procedure that promises the possibility of better function appeals to this. Further complicating the situation are news media reports that hail in-utero surgery as a cure for spina bifida.

I have seen some centers that offer the surgery focus their initial information on a few success stories or just listing off the possible positive benefits, glossing over the possible risks and the unknowns. It’s in their interest to have parents choose in-utero surgery, which gives these centers a conflict of interest in advising parents. (Others are forthright with parents warning that the surgery has potential benefits, but is not a guarantee and does entail risks.)

And so, I’m writing this post with the hope that it will provide parents of a newly diagnosed spina bifida baby with some information that will make the process a little easier.

What is the actual impact of spina bifida when treated with conventional (post-birth) surgery on the baby’s back?

To really understand this, we need to back up a bit. Spina bifida has been identified as a birth defect for hundreds of years, but without any treatment, babies died. Before 1960 when surgery to close the lesion was well-established, the survival rate for all forms of spina bifida was 10% to 12%. Surgeons typically postponed treatment until age 2, believing that only the strongest babies would survive that long. Before the introduction of antibiotics in the 1940s, most infants with myelomeningocele died from meningitis. Hydrocephalus was another cause of death until the development of shunts in the late 1950s revolutionized the treatment of hydrocephalus.

Those who survived meningitis and hydrocephalus faced kidney complications until the 1960s when clean intermittent catheterization was developed to preserve kidney function and allow children to gain social continence. In the 1950’s, Dr. Robert Zachary pioneered the practice of performing surgery on newborns rather than waiting until the age of two. His trial showed no improvements by postponing surgery and he was the first to recommend that babies with spina bifida receive surgery right after birth.

However, many doctors felt that even with treatment available, children with myelomeningocele were too burdensome to care for. Dr. John Lorber said: ” the majority of children with myelomeningocele have very few or no friends; most are left without jobs, they have no chance of love or marriage, and when their exhausted parents can no longer cope, they will end their days in an institution.”

Lorber’s colleague, Dr. Robert Zachary took the opposite view saying that our discomfort with children who have spina bifida has more to do with our own anxieties about quality of life and less to do with their condition. He believed that children with spina bifida could lead happy and productive lives with good treatment and a good attitude.

Unfortunately, his view was largely rejected until about 30-40 years ago. Until the 1980’s, doctors usually advised parents that even with treatment, children with spina bifida would have no quality of life and that withholding treatment and allowing death to happen sooner was best-despite the fact that medical technology and care existed to save the lives of infants with spina bifida and give them a better life. In fact, the team that performed a large study on the outcomes of a multidisciplinary approach for treating children with spina bifida was roundly criticized at first and accused of ethical violations.

So it’s in this context that many doctors and specialists look at spina bifida. There is still a bias against babies with spina bifida and the expectation that they will be “vegetables” or “too burdensome”. However, with conventional treatment of myelomeningocele, a baby can be expected to live well into adulthood and do many things like work and play sports with some adaptations. I have known adults with spina bifida who have gone on to get married and have children of their own.

The exact “combination” and severity of issues will vary from child to child. My son walks with orthotics and a walker and we have rarely used a wheelchair with him. He does not require catheterization , but has issues with bowel and bladder control. He has a VP shunt, but has had no revisions in his seven years of life. My childhood next door neighbor has a daughter with myelomengocele and her daughter walks well without assistance, does not use catheterization, but has had multiple shunt revisions.

This degree of variability is what makes it difficult to say whether in-utero surgery will be beneficial for an individual child.

Will the surgery improve my child’s function?

Maybe, maybe not. Since it’s hard to say exactly what function your individual child would have if he grew up without the surgery, no one can say definitively whether the surgery will improve your individual child’s function. This is why the 2013 study on prenatal surgery for spina bifida compared averages.

On average, children who had the prenatal surgery had a reduced need for VP shunts at 12 months of age, higher scores on the Bayley Mental Development Index at 30 months of age, and a greater likelihood of walking without orthotics at 30 months of age.

Also, this study reported results from between 12-30 months of age. We don’t know what the majority of kids who undergo prenatal surgery will experience as they get older. Many kids with spina bifida lose the ability to walk as they get older. Children who undergo the prenatal surgery may be less likely to lose the ability to walk, but we just don’t know. We also don’t know the likelihood of receiving a shunt later in their childhood years or as adults.

What are the risks?

Most of the babies who were in the study who received the prenatal surgery were born prematurely. Care for premature babies is pretty good here in the US, but there’s still the risk of disability. According to the March of Dimes, complications associated with premature birth include neurological disorders like cerebral palsy, autism spectrum disorder, asthma, bronchopulmonary dysplasia, hearing impairment, vision impairment and learning disabilities.

Many babies who are born prematurely grow up to be healthy, but premature babies are at a greater risk for issues like the ones above. As follow-up continues, we’ll know more about how prematurity might affect these babies long-term and if the risks of prematurity outweigh the benefits of the prenatal surgery- or vice versa.

In the prenatal surgery group, two babies were stillborn on day five after the surgery. In the postnatal surgery group, two babies died from severe hydrocephalus after receiving shunts.

Also, all babies the mother has after the prenatal surgery will have to be c-sections because the scarring on the uterus will be even more extensive than that of a regular c-section scar.

A few final thoughts…

MSDWIHE EC002

Sonora and Al after Sonora’s  first blind dive in the movie Wild Hearts Can’t Be Broken.

Have you ever seen the movie Wild Hearts Can’t Be Broken? It was a 1991 movie based on the life of a woman named Sonora Webster Carver. She was in a horse diving act during the 1930’s. She later became blind after an accident and learned to ride and dive afterwards continuing the show with her husband. The audience never knew she was blind.

It didn’t happen in real life, but there’s a scene in the movie where Sonora argues with her fiance Al that her sight will return and she’ll wait for that to happen. Al sits with her and says that the blindness is permanent,  that she can continue to deny it, but it is permanent. Once the character of Sonora accepts that her sight won’t return (the acceptance stage of grief), she is able to move on and train herself to ride and dive without her sight.

I can say from my experience that my son is not a burden. He has a few challenges that are different from other kids, but everyone has challenges. He has lots of friends (he’s a social butterfly who charms everyone) and he helps around the house, exercises and participates in a ton of fun things.

Spina bifida is permanent. It won’t go away. The surgery may improve your child’s function or it might not. But whatever the outcome, if you’re prepared to embrace having a child with spina bifida, you can choose how to frame spina bifida for you and your kid.

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