A pulmonary monitoring and treatment plan for children with Duchenne-type muscular dystrophies.

The Pulmonary Medicine Section of the Department of Pediatrics of the University of Arkansas for Medical Sciences has recently developed an association with the Muscular Dystrophy Association Clinic held at Arkansas Children's Hospital. The slowly progressive, insidious onset of pulmonary problems associated with Duchenne-type muscular dystrophies and other degenerative muscle disorders indicated a need for a aggressive monitoring and treatment plan for these children and their caregivers. We have developed a Respiratory Care Handbook for families with information on the pulmonary consequences of these diseases including pathophysiology, pulmonary function tests, respiratory treatments including mechanical ventilatory support, and anticipation and prevention of pulmonary crises. In addition, we have introduced for the physician a formal monitoring and treatment regimen driven by changes in the vital capacity lung volume. The substance of this plan is presented in this manuscript.