title: UK physicians' attitudes and practices in long-term non-invasive ventilation of Duchenne Muscular Dystrophy.
abstract: Previous studies have shown that long-term non-invasive ventilation (NIV) is not always routinely offered by all physicians in Duchenne Muscular Dystrophy (DMD), despite evidence that this treatment improves quality of life and survival. This study examined UK physicians' practices related to respiratory follow-up and DMD ventilation. A mailed questionnaire was used. Thirty-eight of the 59 (64%) UK physicians identified via the Muscular Dystrophy Campaign (MDC) responded. Eighty-one per cent of respondents felt ethically obliged to discuss NIV with families while 13% believed that NIV results in poor quality of life. Forty-seven per cent of physicians discuss in-depth the use of NIV when the patient is in respiratory failure. Eighty-four ventilated DMD patients in the respondents' practice use NIV (via Bi-Pap Nasal mask). Nearly 66% of physicians do not consider the public cost to be an impediment to offering NIV, despite significant problems with resources' allocation in their area. While the majority of UK physicians have comparable attitudes and practices regarding NIV, the questionnaire highlighted that not all specialists were aware of the existence of consensus guidelines regarding respiratory monitoring. In addition, different practices of disclosure of life-prolonging ventilation options were used by different physicians. Seventy-one per cent of physicians wished for national consensus guidelines for different DMD age groups.
authors.affiliation: Dubowitz Neuromuscular Centre, Department of Paediatrics, Imperial College, London, UK.
authors.forename: M
authors.initials: M
authors.lastname: Kinali
authors.forename: A Y
authors.initials: AY
authors.lastname: Manzur
authors.forename: E
authors.initials: E
authors.lastname: Mercuri
authors.forename: B E
authors.initials: BE
authors.lastname: Gibson
authors.forename: L
authors.initials: L
authors.lastname: Hartley
authors.forename: A K
authors.initials: AK
authors.lastname: Simonds
authors.forename: F
authors.initials: F
authors.lastname: Muntoni
mesh_terms: D000328:Adult; D004992:Ethics, Medical; D005260:Female; D007722:Health Knowledge, Attitudes, Practice; D006801:Humans; D008297:Male; D008875:Middle Aged; D020388:Muscular Dystrophy, Duchenne / Q000534:rehabilitation*; D010818:Practice Patterns, Physicians' / Q000706:statistics & numerical data*; D011788:Quality of Life; D012121:Respiration, Artificial / Q000941:ethics; D011795:Surveys and Questionnaires; D006113:United Kingdom