Outcome of noninvasive ventilation in children with neuromuscular disease

• Published in: Neurology Vol. 68; no. 3; pp. 198 - 201
• Main Authors: YOUNG, H. K, LOWE, A, FITZGERALD, D. A, SETON, C, WATERS, K. A, KENNY, E, HYNAN, L. S, IANNACCONE, S. T, NORTH, K. N, RYAN, M. M
• Format: Journal Article
• Language: English
• Published: Hagerstown, MD Lippincott Williams & Wilkins 16-01-2007
• Subjects: Biological and medical sciences; Child; Child, Preschool; Cohort Studies; Degenerative and inherited degenerative diseases of the nervous system. Leukodystrophies. Prion diseases; Female; Follow-Up Studies; Headache. Facial pains. Syncopes. Epilepsia. Intracranial hypertension. Brain oedema. Cerebral palsy; Humans; Infant; Male; Medical sciences; Nervous system (semeiology, syndromes); Neurology; Neuromuscular Diseases - complications; Neuromuscular Diseases - diagnosis; Neuromuscular Diseases - therapy; Outcome Assessment (Health Care); Quality of Life; Respiration, Artificial - methods; Sleep Wake Disorders - diagnosis; Sleep Wake Disorders - etiology; Sleep Wake Disorders - prevention & control; Treatment Outcome; Human; Neuromuscular diseases; Nervous system diseases; Prognosis; Child
• ISSN: 0028-3878; 1526-632X
• DOI: 10.1212/01.wnl.0000251299.54608.13

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.
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.
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.
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.