From Children’s Hospital Medical Center - August 1997: ‘Thanks to excellence and team effort, M beats the odds’.
M.C. marked her 16th birthday at Children’s Hospital Medical Center - and the occasion was truly a cause for celebration. Just six months earlier, M was facing certain death. But on this August 10th day, she could celebrate the fact that she had beaten the odds. A week after her birthday, she was discharged and returned to her home in Naples, Italy. Even the physicians who co-ordinated her care were awed by what they had done.
A rare and worsening condition, M was born with Melnick-Needles Syndrome, a rare disease that affects the development of the bones. Hers was an unusually severe case. As the disease progressed, it caused her spine to curve nearly 90 degrees, which led to severe narrowing of her windpipe and dramatic loss of lung function. If her spine curvature continued to progress, she had no chance of survival.
L. and N.C, had spent 10 years seeking help for their daughter, but no hospital in Europe offered them hope. Because she suffered from dangerous sleep apnea - she would stop breathing within a few minutes of falling asleep - her doctors would not attempt surgery. They believed she could not survive anesthesia.
By the time she arrived at Children’s in February, M had only 10 percent lung capacity and was too weak to walk. M was referred to Children’s by a doctor in France who knew of Dr. Robin Cotton’s work in airway management. Dr. Cotton, director, otolaryngology, consulted with pulmonary medicine, anesthesia, orthopaedic surgery and critical care medicine. Together, these services developed a plan to first improve M’s breathing and later correct her spine.
The plan was bold and risky. M would need to survive anesthesia, survive several complex operations and come through the post-op period. As pulmonologist Raouf Amin, M.D., points out, “There is no case in the literature where a patient who had lost 90 percent of lung capacity was able to withstand three surgeries and survive.”
For the family, the choice was between risky surgery or certain death. They chose surgery. With M awake under a local anesthetic, Paul Samuels, M.D., used a fiberoptic bronchoscope to guide him as he inserted a tube that stabilized her airway. M was very co-operative throughout, he says, and the procedure was successful. Next, M underwent an operation to place a trach tube in her airway, an operation made more complicated by the unusual shape of her airway.
When she had recovered from these procedures, M went back to Italy for months of physical and respiratory therapy. By the time she returned to Cincinnati in July, her lung function had improved by 20 percent. Still, she was at grave risk in undergoing the complex operation that would straighten her spine.
Alvin Crawford, M.D., director, orthopaedic surgery, and Richard Brilli, M.D., clinical director, intensive care unit, calculated that there was a 55 percent chance she would not survive. There was the additional risk of damage to her spinal cord that might leave her paralyzed.
Dr. Crawford performed two operations - first placing M in traction, and then placing rods and hooks in her vertebral column to align her spine. After her spine was straightened, M’s lung function improved another 30 percent, says Dr. Amin.
Physical and respiratory therapy began while M was in the hospital. Before she left, she was able to sit up and was begining to walk. Therapy will continue in Italy. “I really want to thank everyone who helped me,” says M. “It has really changed my life, and everyone has been very kind.” The successful outcome has wide importance, M’s father believes. “Doctors now know they can do surgery on a child with only 10 percent lung capacity. We showed them it’s not impossible”.