bridging the care gap
Specialists at Barrow, Children's Health Center and Children's
Rehabilitative Services provide lifelong care for people with birth
defects

Until recently, those two facts would not have coexisted, because few babies born with the neurological disease, in which the fetal spine fails to completely close, would have survived past childhood. Now, thanks to dramatic medical advances, spina bifida babies are able to have normal
lifespans.

But this outcome is possible only if, when they reach adulthood, they receive the same expert care they received as children, and this is far from assured. Most physicians who specialize in treating children with spina bifida and other neurological disorders are based at children’s hospitals. Relatively few non-pediatric specialists have experience treating those same patients, who, as adults with congenital diseases, have different needs than people who develop neurological problems later in life, says Dr. Harold Rekate, chief of Pediatric Neurosciences at Barrow.

Bridging this care gap has become Dr. Rekate’s passion.

“It’s something I think about all the time,” he says. “Now we have a huge and increasing population of people whose birth defects are still affecting them, even though they’re no longer children. Finding specialists with interest and expertise in treating these conditions is a significant challenge.”

Children’s Rehabilitative Services, Barrow and St. Joseph’s are rare because they are structured to care for patients from infancy through
adulthood.

Children’s Rehabilitative Services

The state’s Children’s Rehabilitative Services (CRS) provides care for child residents who have a disability or chronic illness. Administered by a branch of the Arizona Department of Health Services, CRS makes sure kids have the specialty medical care they need so they can grow into thriving adults. In central Arizona, the program is administered at St. Joseph’s Children’s Health Center.

“I’ve always said I was unlucky to be born with spina bifida, but lucky enough to be born in Arizona,” Larry says. “CRS is a wonderful program. It’s kept me alive, just like the strong hands of Dr. Rekate.”

Young CRS patients grow up seeing a variety of spe- cialists on a regular basis.

“Up to 21, we get care and build up a relationship with physicians and surgeons. When we graduate from the program and go out to the real world, we see them on the outside,” says Larry, who is living proof of how spina-bifida patients can thrive in adulthood. He is self-sufficient, working until recently as a desk clerk and translator for CRS. He’s also active in politics, such as campaigning for Congressman Ed Pastor.

Transitioning to adulthood

Care beyond childhood is crucial because adult patients face different issues than they did as children. For example, like many spina-bifida patients, Larry has a related condition called hydrocephalus (“water on the brain”), in which spinal fluid normally siphoned off by the body builds up inside the skull. If not released, it can cause severe headaches, blindness and, ultimately, brain death.

Hydrocephalus is treated with a shunt implanted in the patient’s skull. The invention of the shunt is a key reason spina-bifida patients are now able to reach adulthood. Larry, who was born in Phoenix, received his first shunt when he was two weeks old.

But shunts wear out or need adjustment. In one out of five child patients, a shunt’s failure is not reflected on a brain scan, a fact that pediatric specialists are aware of. The statistic still applies when the patients reach adulthood, but specialists in adult neurology don’t necessarily have the same awareness that a shunt’s failure may not show up on a CT or MRI scan. That’s one reason nonpediatric specialists are reluctant to take on such patients.

“Very few general neurosurgeons are comfortable with management of shunts put in during childhood,” Dr. Rekate says.

Learning to manage life with spina bifida

Adult patients face other issues they didn’t as children. The spine, for instance, tends to wear outover time, Dr. Rekate says. It’s up to the physician to determine when surgery will help stave off further damage or repair the spine and when it won’t.

Spina-bifida patients also have urological, orthopedic and other issues to contend with. Most spina-bifida patients lack bowel and bladder control and must learn to catheterize themselves every few hours to prevent urine from backing up into their kidneys. They also have varying degrees of mobility, with most using wheelchairs or leg braces. Larry walks with the help of a cane but has very little feeling in his feet, which means he must be vigilant about protecting them.

“When it’s hot, I could walk down the street on concrete in the middle of July in bare feet and not feel a thing,” says Larry, who has friends who have lost limbs to gangrene.

Larry credits CRS’s weekly spina-bifida clinics for teaching him how to manage life with the disease. The clinics bring together many specialists in one place—neurosurgeon, pediatrician, social worker, nutritionist, psychologist, urologist, orthopedist, phys- ical therapist, occupational therapist and so on. Patients generally visit the clinic at least once a year.

By the time they reach adulthood, they’ve formed relationships with their specialists that continue, which Dr. Rekate says is key to patients’ continuing health.

“They’ve been nurtured in this environment where they’ve been taken care of by this group of experts who still practice at St. Joseph’s,” he says. “When they graduate, they can still be taken care of in the same nurturing environment.”