Original articleParental Stress and Quality of Life in Children With Neuromuscular Disease
Introduction
Children with neuromuscular disease are at risk for developing respiratory insufficiency because of associated muscle weakness, obstructive sleep apnea, or scoliosis. Increasingly, noninvasive positive pressure ventilation has been used for the management of chronic respiratory insufficiency in these patients, as it has been demonstrated to relieve signs of hypoventilation, prolong survival, and enhance quality of life in adult neuromuscular patients [1], [2], [3], [4]. Few studies have examined the impact of noninvasive positive pressure ventilation and other assisted ventilation devices in children with neuromuscular disease [5], [6], [7], [8]. Further, to the best of our knowledge, no studies have investigated the effects of home mechanical ventilation on parental stress and pediatric health-related quality of life across childhood and adolescence.
This study examined pediatric quality of life and parental stress in families of children with neuromuscular disease, including those children requiring home mechanical ventilation. We hypothesized that parents of pediatric neuromuscular patients would report higher levels of stress than those reported in the normative sample, and that the use of home mechanical ventilation would be associated with greater parental stress. In addition, the use of home mechanical ventilation was hypothesized to be associated with lower pediatric health-related quality of life.
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Study Participants
Parents with children followed at the pediatric neuromuscular clinic in Calgary or Edmonton (Alberta, Canada) were invited to participate in this study. The inclusion criteria were: (1) the child with neuromuscular disease was between ages 2-18 years; and (2) the child was followed at the clinic in Edmonton or Calgary for at least 6 months or longer. Families with limited English-language comprehension or those who were unable to give informed consent were excluded. Written, informed consent
Results
A total of 109 (44%) out of 246 eligible families participated in the study (Table 1). Sixty (55%) families were from Calgary, and 49 were from Edmonton. The most common pediatric neuromuscular diagnoses were Duchenne and Becker muscular dystrophies (22%), Charcot-Marie-Tooth disease (22%), and spinal muscular atrophy (16%). The children's mean age was 10.5 years (S.D., 5.2 years), and 75 (69%) were male. The median duration of signs was 4 years (interquartile range, 2-8 years). The
Discussion
Children with complex chronic illnesses have significant impairment in their everyday functioning, and require ongoing medical care and intervention. Relative to healthy children and other chronically ill children, the pediatric neuromuscular patients in this study displayed poorer physical and psychosocial health-related quality of life [9]. The physical weakness experienced by these patients may limit their ability to participate in a variety of physical and social activities, resulting in
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