OBJECTIVE: To assess the effect of institution of noninvasive ventilation (NIV) on clinical outcome and quality of life (QOL) in a cohort of children with severe neuromuscular disorders. METHODS: We reviewed records and obtained clinical data from the year prior to commencing NIV and annually thereafter. Data obtained included diagnosis, patient symptoms, mortality, NIV adverse effects, pulmonary function tests, polysomnographic data, length of hospitalizations, and health care costs. Patients and parents completed questionnaires assessing QOL with NIV and recalling QOL before NIV. RESULTS: Fourteen of 17 (82%) suitable patients were enrolled. Follow-up ranged from 6 to 84 months (median 30). Symptoms of daytime sleepiness (p = 0.003) and headache (p = 0.046) improved after initiation of NIV. Sleep quality assessed by polysomnography also improved. Hospitalization rates (p = 0.002) and health care costs (p = 0.003) decreased. QOL remained stable after NIV, despite disease progression. CONCLUSION: Treatment of respiratory failure, in children with neuromuscular disease, with noninvasive ventilation results in a reduction in symptoms, hospitalizations, and health care costs without adverse effects on quality of life.
OBJECTIVE: To assess the effect of institution of noninvasive ventilation (NIV) on clinical outcome and quality of life (QOL) in a cohort of children with severe neuromuscular disorders. METHODS: We reviewed records and obtained clinical data from the year prior to commencing NIV and annually thereafter. Data obtained included diagnosis, patient symptoms, mortality, NIV adverse effects, pulmonary function tests, polysomnographic data, length of hospitalizations, and health care costs. Patients and parents completed questionnaires assessing QOL with NIV and recalling QOL before NIV. RESULTS: Fourteen of 17 (82%) suitable patients were enrolled. Follow-up ranged from 6 to 84 months (median 30). Symptoms of daytime sleepiness (p = 0.003) and headache (p = 0.046) improved after initiation of NIV. Sleep quality assessed by polysomnography also improved. Hospitalization rates (p = 0.002) and health care costs (p = 0.003) decreased. QOL remained stable after NIV, despite disease progression. CONCLUSION: Treatment of respiratory failure, in children with neuromuscular disease, with noninvasive ventilation results in a reduction in symptoms, hospitalizations, and health care costs without adverse effects on quality of life.
Authors: Susan T Iannaccone; Linda S Hynan; Anne Morton; Renee Buchanan; Christine A Limbers; James W Varni Journal: Neuromuscul Disord Date: 2009-10-28 Impact factor: 4.296
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Authors: Craig M McDonald; Dawn A McDonald; Anita Bagley; Susan Sienko Thomas; Cathleen E Buckon; Eric Henricson; Alina Nicorici; Michael D Sussman Journal: J Child Neurol Date: 2010-06-17 Impact factor: 1.987
Authors: Ching H Wang; Carsten G Bonnemann; Anne Rutkowski; Thomas Sejersen; Jonathan Bellini; Vanessa Battista; Julaine M Florence; Ulrike Schara; Pamela M Schuler; Karim Wahbi; Annie Aloysius; Robert O Bash; Christophe Béroud; Enrico Bertini; Kate Bushby; Ronald D Cohn; Anne M Connolly; Nicolas Deconinck; Isabelle Desguerre; Michelle Eagle; Brigitte Estournet-Mathiaud; Ana Ferreiro; Albert Fujak; Nathalie Goemans; Susan T Iannaccone; Patricia Jouinot; Marion Main; Paola Melacini; Wolfgang Mueller-Felber; Francesco Muntoni; Leslie L Nelson; Jes Rahbek; Susana Quijano-Roy; Caroline Sewry; Kari Storhaug; Anita Simonds; Brian Tseng; Jiri Vajsar; Andrea Vianello; Reinhard Zeller Journal: J Child Neurol Date: 2010-11-15 Impact factor: 1.987