Swiss physicians' practices of long-term mechanical ventilatory support of children and adolescent with Duchenne muscular dystrophy

Introduction: Previous studies have shown that long-term ventilation is still not routinely offered to or discussed with patients with Duchenne muscular dystrophy (DMD). This is in contrast to the increasing evidence that these interventions can successfully improve quality of life and prolong survival of affected individuals. The aim of this survey was to describe the clinical practice and the attitudes of Swiss physicians in the respiratory follow up of DMD individuals and to understand better the current provision of respiratory services in Switzerland.

Methods: Postal questionnaire about clinical information were sent to all physicians, including paediatricians, pneumologists and neurologists known or suspected to be involved in the care of children and adults with DMD. 26 of 30 physicians (87%) returned the questionnaire.

Results: 88% of the respondents discuss assisted ventilation with their patients and families as this is felt an ethical obligation. 68% of the physicians delay discussion until symptoms or signs of respiratory compromise have developed. Non-ambulant DMD patients are offered on average 8.2-monthly pulmonary function testing. 53% of the physicians perform sleep studies on average every 6–9 months in patients older than 10 years.

Conclusion: The majority of Swiss physicians who responded to this survey have comparable attitudes and practices regarding long-term ventilation and promote a positive culture of involving patients in their decision.