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Outcome of noninvasive ventilation in children with neuromuscular disease

From the Institute for Neuromuscular Research (H.K.Y., K.N.N.), Respiratory Support Service and Sleep Unit (A.L., C.S., K.A.W., E.K.), The Children's Hospital at Westmead, Westmead, Australia; Discipline of Paediatrics and Child Health (D.A.F., K.A.W., K.N.N., M.M.R.), Department of Respiratory Medicine (D.A.F.), The University of Sydney, Sydney, Australia; Departments of Clinical Sciences and Psychiatry (L.S.H.) and Pediatric Neurology and Pediatrics (S.T.I.), University of Texas Southwestern Medical Center at Dallas; Children's Medical Center (S.T.I.) and Department of Neuromuscular Disease and Neurorehabilitation (S.T.I.), Texas Scottish Rite Hospital for Children, Dallas, TX.

Address correspondence and reprint requests to Dr. Monique M. Ryan, Neurosciences Department, Royal Children's Hospital, Flemington Rd, Parkville Victoria 3052, Australia; e-mail: monique.ryan{at}rch.org.au

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.

Additional material related to this article can be found on the Neurology Web site. Go to www.neurology.org and scroll down the Table of Contents for the January 16 issue to find the link for this article.

Disclosure: The authors report no conflicts of interest.

Received May 5, 2006. Accepted in final form October 9, 2006.

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