When NYU recruited Dr. Shay Bess, they wanted him to tackle the most complex spine cases. The expert orthopedic surgeon, who is also a leader in spinal deformity research, has now joined forces with the Dysautonomia Center to take on the spinal problems of our patients with FD.

Ninety-percent of patients with FD develop Scoliosis. As the spine grows and the vertebra elongate, they start to bend and twist sharply, which curves the spine and creates scoliosis. These abnormal spinal curves become more pronounced around the time of puberty, when the spine grows most rapidly. It’s not difficult to imagine how a crooked back could impact self-esteem. But in severe cases, the spine can also compress the lung beneath the curve.

Although scoliosis is a major problem, and one that occurs at an alarming rate, we still know very little about what makes the spine curve abnormally. Dr. Bess is hoping to change this. He believes that the abnormal spinal curves in patients with FD bear a striking resemblance to those of other neuromuscular diseases. Dr. Kaufmann agrees and he has long thought that the scoliosis of patients with FD is related to the underlying problems with the sensory nerve signaling.

90% of patients with FD will develop scoliosis, 25% will also develop kyphosis

There are 140 overlapping muscles that must act together to support the spine and create the range of motion. These muscles work synergistically to keep us upright, but allow us to bend and twist. Their coordination is dependent on muscle spindles, tiny sensors imbedded deep in the muscle and joints that detect muscle tension. It is these muscle spindles that allow us to constantly make adjustments to fine-tune body position (read more: here). Mutant mice lacking this incoming sensory information from the muscles also develop scoliosis.

Fortunately, Dr. Bess is bringing pioneering new ways to treat scoliosis in patients with FD. For some, the spine curvature can start earlier, before their 7^th birthday. Scoliosis treatment in these younger FD children requires an entirely different approach as the spine is still growing. Instead of fusing the vertebrae together, adjustable metal rods are anchored to the spine and lengthened at regular intervals as the patient grows. But this traditional approach is risky as it requires multiple surgeries under general anesthesia every 6-months or so.

But this may be changing. Dr. Bess recently has started using magnetic growing rods in children with FD who have early onset scoliosis. These magnetic rods can be adjusted in the clinic with a remote control. By bringing these new techniques to the Center, the hope for children with FD and early-onset scoliosis is less time spent in the hospital, less exposure to general anesthesia and fewer scars.

Dr. Bess works closely with the Dysautonomia Center Team and sees children and adults with FD at the Center.