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Restore - Stabilizing a Spine—and a Patient’s Quality of Life

Restore Winter 2015

Stabilizing a Spine—and a Patient’s Quality of Life

Date: January 8, 2015

JoAnn Boyd and Samuel Mayer celebrate her progress.
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JoAnn Boyd and Samuel Mayer celebrate her progress.

Within minutes of speaking to JoAnn Boyd, Jay Shapiro knew she’d need surgery. As director of the Osteogenesis Imperfecta (OI) Clinic at the Kennedy Krieger Institute and a member of Johns Hopkins’ Physical Medicine and Rehabilitation faculty, Shapiro sees about 200 patients a year with the rare congenital bone disorder that impairs the body’s ability to make collagen, leading to fractures. These patients, including Boyd, require dozens of operations to stabilize bones and become very attuned to how OI is affecting them. “So when it gets to the point that the patient is complaining,” he says, “we both expect that major-league surgery will follow.”  

For Boyd, who hails from Cincinnati, Ohio, OI has necessitated almost 40 operations since her birth in 1964. Most of her limb rodding procedures occurred before she turned 15. Despite the surgeries, Boyd is fiercely independent: She drives, owns a home and works full time. But last summer, when she started feeling severe pain in her upper back and neck and numbness in her limbs, she suspected trouble.

Shapiro’s decades-long experience with OI, coupled with Johns Hopkins Medicine’s neurosurgical and rehabilitation expertise, inspired Boyd to travel to Baltimore for an evaluation. During that visit, Shapiro confirmed that her spine was pushing into the soft base of her skull and referred Boyd to Johns Hopkins neurosurgeon Jean-Paul Wolinsky and physiatrist Samuel Mayer.

Not long ago, to correct basilar invagination, surgeons would resect the ondontoid process through a transoral-transphryngeal approach. But sometimes the jaw had to be split to reach the back of the throat, causing complications if there’s a cerebrospinal fluid leak. Bacteria from the mouth can mix with the fluid and cause postoperative meningitis. To reduce that risk, in 2005, Wolinsky developed a surgical approach through the neck (see illustration).

The operation first involves removing the C2 bone and the ondontoid to decompress the spinal cord and brainstem. Then Wolinsky turns the patient over and takes the bone off the back of the skull and cervical spine to relieve pressure. This is followed by fusing the head to the cervical spine. Throughout the 14-hour operation, Wolinsky and his colleagues work with custom-made long instruments, including high-speed drills and irrigation devices to remove the bone dust.

“Whereas patients used to stay in the hospital for two months,” says Wolinsky, “now it’s less than two weeks. The patient wears a halo vest for two months, then a cervical collar for another month.”

For patients with OI, there’s a heightened need for careful monitoring and targeted rehab. “Keeping everyone in the loop before and after surgery is important,” says Wolinsky, “because these patients are so fragile.”

To that end, Wolinsky and Shapiro consult regularly with Mayer and his colleagues, who craft a regimen that emphasizes safe handling and protective positioning. In Boyd’s case, Mayer and physical therapists coached her on isometric exercises to strengthen her muscles without putting stress on bones affected by the operation. The regimen included leg lifts, knee bends, and rotating the ankle and feet.

Boyd says she felt relief immediately following the procedure, and she continues to make progress restoring her mobility while the halo is in place—transferring in and out of the wheelchair and performing basic self-care. “I intend to be well,” she says, “by my upcoming 50th birthday.”

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