Spinal Surgery, Assisted Ventilation Better Together in DMD

A British study has found that adding spinal surgery to nighttime assisted ventilation in Duchenne muscular dystrophy (DMD) improves median survival from 22.2 years to 30 years. (“Median” survival was calculated as the time at which half the study subjects remained alive.)

Michelle Eagle at the University of Newcastle and Newcastle Upon Tyne Hospitals Trust and colleagues conducted the study by analyzing the records of boys with DMD born between 1970 and 1990 and followed at the Newcastle Muscle Centre.

They reviewed the records of 27 boys who had surgery to correct a spinal curvature and used noninvasive (nontracheostomy) ventilation during the night; 13 who used ventilation but didn’t have spinal surgery; and 35 who received neither treatment.
 
The median survival time for the first group (surgery and ventilation) was 30 years; for the second group (ventilation only), it was 22.2 years; and for the third group (neither intervention), it was 17.1 years.

“Our current results suggest that the combination of spinal correction and the provision of home nocturnal ventilation have an additive effect on survival for boys with DMD with a progressive scoliosis [spinal curvature] and respiratory failure but without severe progressive cardiomyopathy [cardiac muscle disease],” the investigators say in their report, published in the June issue of Neuromuscular Disorders.

They say the two interventions together enhance longevity to a greater extent than either one alone.

The researchers also note that longer survival in DMD since the 1960s is the result of many factors. Among them are better management of chest infections with antibiotics beginning in the 1970s and 1980s; and, starting in the 1990s, better monitoring of respiratory function and the introduction of home nocturnal ventilation, wider use of surgery to correct spinal curvatures, and a lower threshold for both detection and treatment of cardiac muscle disease.

They note that the recent introduction of corticosteroids (the prednisone family of medications) to the standard treatment of boys with DMD improves strength and prolongs walking, but they say it’s too early to tell whether steroids will also affect survival.