Introduction: Previous studies have shown that long-term ventilation (LTV) 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.
Aim: 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. Another aim was to explore the contentment of the patients about the provision of information on LTV.
Methods: Postal questionnaires requesting information on the current practice of respiratory care were sent to physicians, including pulmonologists and neurologists known or suspected to be involved in the care of children and adults with DMD. 26 of 30 (87%) physicians returned the questionnaire. A second questionnaire was sent to 90 DMD patients of whom 43 (48%) returned the questionnaire.
Results: 88% of the physicians indicated to discuss the option of LTV with their patients and families, but 50% of the surveyed physicians start such discussions only after the patient has already developed respiratory failure. Regular pulmonary function assessments are performed by the majority physicians in DMD patients, but only 50% of the physicians perform regular sleep studies to detect sleep disordered breathing.
Conclusion: The majority of Swiss physicians involved in the care of DMD patients disclose information on LTV to DMD patients, but commonly late in the disease course after respiratory problems have developed. The current routine of respiratory follow-up of DMD patients seems insufficient, at least with respect to early detection of nocturnal hypoventilation.