Respiratory muscle weakness is a common feature of many neuromuscular disorders contributing to the development of daytime respiratory failure. However sleep-related breathing abnormalities and sleep disturbance can occur months or years before the emergence of daytime hypercapnia. Although obstructive events are commonly seen in the early stages of many disorders with age and disease progression central events and nocturnal hypoventilation occur. Patients may complain of unrefreshing sleep daytime sleepiness and impaired concentration although these symptoms can develop insidiously and be overlooked or mistaken as features of primary disease process. As management of neuromuscular disorders is primarily supportive not curative therapeutic intervention must be directed towards managing symptoms and maintaining quality of life. Non-invasive ventilation (NIV) has been convincingly shown to improve physiologic parameters and extend survival in patients with a wide variety of neuromuscular disorders. Although an improvement in symptoms once therapy is commenced is frequently reported the impact on sleep quality and health-related quality of life (HRQOL) has received far less attention. Assessing quality of life is essential to evaluate the financial and human costs and benefits of therapy. Health professionals frequently underestimate the quality of life in patients with neuromuscular disorders and significant disability and this can affect the type of therapeutic options that are offered to patients. As sleep quality has been shown to be a major determinant of global quality of life in patients with chronic respiratory failure evaluating sleepiness specific sleep-related quality of life issues and neuropsychological function should be undertaken routinely before the institution of home ventilation and during follow up. © 2008 Humana Press, Totowa, NJ.
CITATION STYLE
Piper, A. J. (2008). Sleep and quality of life in neuromuscular disease. In Sleep and Quality of Life in Clinical Medicine (pp. 209–220). Humana Press. https://doi.org/10.1007/978-1-60327-343-5_23
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