image: Dr. Scott Wolfe and patient Kale Hyder view more
Credit: Hospital for Special Surgery
An innovative and complex surgery involving nerve transfers is restoring hope and transforming lives torn apart by a mysterious and devastating illness. Acute flaccid myelitis, also known as AFM, strikes without warning, shows no mercy and frequently results in paralysis. Most affected patients are children, and nearly all have partial or complete loss of muscle function in their arms or legs.
Dr. Scott Wolfe, an orthopedic surgeon specializing in nerve injuries at Hospital for Special Surgery (HSS), has restored arm movement and function in a number of young AFM patients previously told their paralysis would be permanent.
The journal Pediatric Neurology published two AFM case studies by Dr. Wolfe and colleagues. The article was made available online in the summer of 2018, in advance of final print publication in November 2018. The report documents patients, ages 12 and 14, who had suffered partial paralysis and regained movement in their arms after nerve transfer surgery at HSS.
"We published the case studies to raise awareness in the medical community," said Dr. Wolfe. "Since the procedure is so highly specialized and performed by very few surgeons, most people, even doctors, are unaware that nerve transfer could potentially help AFM patients. But there is a window of opportunity, and the surgery should ideally be performed within six to nine months of disease onset."
Cases of acute flaccid myelitis, which is considered a subtype of transverse myelitis, have been on the rise in the United States since 2014, according to the Centers for Disease Control and Prevention. The illness, which is most common in children and teenagers, appears to occur after a viral infection. Within a day or two, inflammation within the spinal cord leads to muscle weakness and rapid, progressive paralysis of the arms and/or legs. In 2014, and again in 2018, a disturbing spike in cases was reported in certain regions of the United States.
Patients suffer different degrees of paralysis, and while some regain function, many suffer some degree of permanent paralysis. No nonsurgical treatment has been shown to be effective.
At age 15, Kale Hyder of Davenport, Iowa was not only the picture of health, but an accomplished athlete. At 6-foot-two, the active teenager played on his school's basketball team and on an elite traveling team in the summer. One day in June 2015, he woke up with a stiff neck and asked his mother for a new pillow. By the next day, he could not move his arms and could barely stand up. His mother rushed him to the emergency room. Soon after, he was paralyzed from the chest down.
Kale was diagnosed with transverse myelitis. After days and months had passed, Kale and his family were shocked to receive a terrible prognosis. "We went to an excellent neurologist in the Chicago area, and he said, 'I'm so sorry, you're not going to regain function of your hands.' It was devastating, I'll never forget receiving that news," recalls Marcy Hyder, Kale's mother. "Here's my basketball player, sitting next to me in a wheel chair. Everything changed in the blink of an eye. When we got out of there, we all just sobbed."
At that time, Kale was receiving occupational therapy at Shriners Hospitals for Children in Chicago, and the therapist told them about another young girl in her care who had been helped by Dr. Wolfe at HSS. In March 2016, the Hyders made the trip to New York.
Although several doctors had indicated that Kale would require assistive devices and a caregiver to help with activities of daily living, Dr. Wolfe gave them another option. And he gave them hope.
Dr. Wolfe performed nerve transfers to each arm, followed by tendon transfer surgery a year later to restore function in Kale's hands and enable him to lift his arm over his head. Nerve transfer surgery entails taking all or part of a working nerve with a less important or redundant function and transferring it to restore function to one or more muscles that have been paralyzed. The delicate and painstaking surgery requires hours of muscle and nerve testing and meticulous planning by the surgeon and his team beforehand. It's not uncommon for Dr. Wolfe to work much of the day and into the night to plan the surgery. Performed under a microscope, each set of nerve transfer surgeries can take five to seven hours.
"We have been performing nerve transfers for patients with brachial plexus injuries, so it made sense to try it for AFM patients," Dr. Wolfe said. "But it's more challenging. Since the disease causes almost random patterns of muscle paralysis, there's no roadmap to follow and we have to come up with a creative solution for each patient. We take a full inventory of what's working and what's not working in each limb by checking each muscle. We look for donor nerves, so if two muscles move an elbow, for example, we can take one of the nerves controlling that function and use it to restore function to a hand or shoulder."
Marcy says she is extremely grateful to the Shriners occupational therapist and a physician there who told her about HSS and Dr. Wolfe. "We would never have known. We would have missed that window of opportunity for surgery. Without it, Kale would not be functioning as he is today," she says. "Think of everything you do with your hands; he couldn't do it. But he can now. What Dr. Wolfe did for him is absolutely amazing. He gave him his independence."
Now Marcy is on a mission to raise awareness that nerve transfer surgery could be an option for transverse myelitis and AFM patients, but they need to be evaluated sooner rather than later.
Kale, now a freshman at Johns Hopkins University, credits Dr. Wolfe with enabling him to follow his dreams. A pre-med major, he plans to be a neurologist and a researcher so he can help others. He attends classes, engages in volunteer work on campus, types on a keyboard, shops for food and does his own laundry.
"Dr. Wolfe has been a role model and I admire him for the way he works with patients. They have such serious conditions, yet he finds hope in every case and gives his patients hope," Kale says. "And he's a very great problem solver, the way he knows anatomy and physiology. Those problem-solving skills are something that I hope I can have at least half of, one day. It's crazy what he can do."
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Anyone who would like more information on nerve transfer and reconstruction is invited to visit https://www.hss.edu/brachialplexuscenter-treatments.asp
About Hospital for Special Surgery
HSS is the world's leading academic medical center focused on musculoskeletal health. At its core is Hospital for Special Surgery, nationally ranked No. 1 in orthopedics (for the ninth consecutive year) and No. 3 in rheumatology by U.S.News & World Report (2018-2019). Founded in 1863, the Hospital has one of the lowest infection rates in the country and was the first in New York State to receive Magnet Recognition for Excellence in Nursing Service from the American Nurses Credentialing Center four consecutive times. The global standard total knee replacement was developed at HSS in 1969. An affiliate of Weill Cornell Medical College, HSS has a main campus in New York City and facilities in New Jersey, Connecticut and in the Long Island and Westchester County regions of New York State. In addition, HSS will be opening a new facility in Florida in late 2019. In 2017 HSS provided care to 135,000 patients and performed more than 32,000 surgical procedures, and people from all 50 U.S. states and 80 countries travelled to receive care at HSS. In addition to patient care, HSS leads the field in research, innovation and education. The HSS Research Institute comprises 20 laboratories and 300 staff members focused on leading the advancement of musculoskeletal health through prevention of degeneration, tissue repair and tissue regeneration. The HSS Global Innovation Institute was formed in 2016 to realize the potential of new drugs, therapeutics and devices. The HSS Education Institute is the world's leading provider of education on the topic on musculoskeletal health, with its online learning platform offering more than 600 courses to more than 21,000 medical professional members worldwide. Through HSS Global Ventures, the institution is collaborating with medical centers and other organizations to advance the quality and value of musculoskeletal care and to make world-class HSS care more widely accessible nationally and internationally.
Journal
Pediatric Neurology