Parental Stress and Quality of Life in Children With Neuromuscular Disease

doi:10.1016/j.pediatrneurol.2008.04.011

Pediatric Neurology

Volume 39, Issue 2, August 2008, Pages 102-107

https://doi.org/10.1016/j.pediatrneurol.2008.04.011 Get rights and content

This study examined health-related quality of life and parental stress among pediatric neuromuscular patients with or without home mechanical ventilation. Parents completed the Parenting Stress Index or Stress Index for Parents of Adolescents, depending on their child's age. The Pediatric Quality of Life Inventory measured quality of life in children with neuromuscular disease. One hundred and nine families participated; 19 (17%) families had a child with neuromuscular disease requiring home mechanical ventilation. Overall, children on home mechanical ventilation had significantly lower mean total Pediatric Quality of Life Inventory scores than nonventilated children (47.9 versus 61.5, respectively; P = 0.013). No significant difference in mean total stress scores was found between parents of pediatric neuromuscular patients with or without home mechanical ventilation. Despite their child's lower health-related quality of life, parents of pediatric neuromuscular patients requiring home mechanical ventilation did not report significantly higher parental stress than parents of nonventilated children or parents in the normative sample. We postulated that for parents living with the constant demands of caring for their child with neuromuscular disease requiring home mechanical ventilation, these caretaking demands, over time, had become part of “normal” life and were not identified as creating additional stress.

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.

Section snippets

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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With regard to schooling, we asked about the amount of time children spent unaccompanied by their parents during class time as respite care due to medical treatment by school nurses. HRQOL in CMC associated with age, medical dependency, motor functioning, and intellectual development (Dickinson et al., 2007; Mah et al., 2008; Maher et al., 2008; Moore et al., 2009; Sabaz et al., 2001; Varni et al., 2007). Sociodemographic data related to parents and their children were gathered using an information questionnaire.
This study examined the impact of respite care received by children with medical complexity (CMC) on their health-related quality of life (HRQOL). We hypothesized that out-of-home respite care would increase both opportunities to engage in activities and participation with non-family members and help with acquiring autonomy and social skills.
This cross-sectional study of CMC aged between 8 and 18 years living at home used a web-based questionnaire survey that parents living with the target CMC answered for proxy evaluation of CMC's HRQOL (KIDSCREEN-27). We asked 3142 parents to participate in the study through 237 special-needs schools throughout Japan. Path analysis was used to estimate the variation in each aspect of HRQOL with respite care time of in-home care services, day care services, short-stay services, and school time.
We analyzed the responses from 618 parents of CMC. The results showed that respite care by day care services and special-needs schools increased “physical well-being,” “psychological well-being,” and “peers and social support,” which are components of the HRQOL. Furthermore, respite care at schools had an impact on “school environment.”
Respite care provided by special-needs schools and day care services has implications not only in terms of relief for caregivers but also in improving the HRQOL of CMC.
Nurses can provide respite care that does not require parental accompaniment at school or day care facilities, which can lead to CMC's involvement in fostering autonomy and social skills. (249/250 words).
Health-related quality of life in Thai children with spinal muscular atrophy
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Spinal muscular atrophy (SMA) is a chronic genetic disease that causes varying degrees of disability. There is currently no data specific to the health-related quality of life (HRQoL) of Thai children and adolescents with SMA. Accordingly, this study aimed to evaluate the HRQoL of Thai pediatric SMA, and to identify factors significantly associated with HRQoL and other aspects of quality of life. The findings of this study will help to improve overall care in this vulnerable pediatric population.
This prospective cross-sectional descriptive study was conducted at the Division of Neurology, Department of Pediatrics, Faculty of Medicine Siriraj Hospital, Mahidol University, Bangkok, Thailand during 2015–2018. HRQoL was measured using the Thai language version of the Pediatric Quality of Life Inventory™ 4.0 Generic Core Scale (PedsQL™). The PedsQL™ was administered to SMA children aged 2–18 years and their parents. Disease severity was evaluated by pediatric neurologists, and patients were classified according to their SMA subtype.
Forty-two Thai pediatric SMA (mean age: 9.8 ± 5.0 years, 25 males) and their families were recruited. The mean PedsQL™ total score was 57.3 ± 13.6 by child self-report, and 54.3 ± 14.8 by parent proxy-report. The PedsQL™ scores of Thai SMA children were found to be significantly lower than those reported in healthy Thai children. The mean PedsQL™ total score among healthy Thai children was 78.7 ± 9.3 by child self-report, and 79.0 ± 12.8 by parent proxy-report. Factors independently associated with lower HRQoL were non-ambulation, household income less than 18,500 Thai baht/month, mechanical ventilation, and inability to attend school.
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A critical review of patient and parent caregiver oriented tools to assess health-related quality of life, activity of daily living and caregiver burden in spinal muscular atrophy
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The most recent ones also explore how the patients feel about preserving or improving individual functional capacities in relation to possible trials. A number of tools focus on caregiver burden in SMA, considering the socio-economic burden more than the psychological impact of the disease [34,45,56,59]. New scales, such as the SMAIS, have been specifically developed for SMA and have recently been proposed for clinical trials and appear to have a number of items that are relevant for SMA but data on their validation are not yet available.
The positive outcome of different therapeutic approaches for spinal muscular atrophy (SMA) in clinical trials and in clinical practice have highlighted the need to establish if functional changes are associated with possible changes of patient health-related quality of life or have an effect on activities of daily living and caregiver burden. The aim of this paper is to provide a critical review of the tools previously or currently used to measure quality of life, activity of daily living, and caregiver burden in SMA. We identified 36 measures. Only 6 tools were specifically developed for SMA while the others had been used and at least partially validated in wider groups of neuromuscular disorders including SMA. Twelve of the 36 focused on health-related quality of life, 5 on activities of daily living and 9 on caregiver burden. Ten included a combination of items.
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Original articleParental Stress and Quality of Life in Children With Neuromuscular Disease

Introduction

Section snippets

Study Participants

Results

Discussion

Ambul Pediatr

Outcome of domiciliary nasal intermittent positive pressure ventilation in restrictive and obstructive disorders

Thorax

Long-term effects of nasal intermittent positive-pressure ventilation on pulmonary function and sleep architecture in patients with neuromuscular diseases

Chest

Sleep and neuromuscular disease: Bilevel positive airway pressure by nasal mask as a treatment for sleep-disordered breathing in patients with neuromuscular disease

J Neurol

Long term noninvasive ventilation in the community for patients with musculoskeletal disorders: 46 year experience and review

Thorax

Spinal muscular atrophy type 1: Management and outcomes

Pediatr Pulmonol

Outcome of noninvasive positive pressure ventilation in pediatric neuromuscular disease

Arch Dis Child

Outcome of noninvasive ventilation in children with neuromuscular disease

Neurology

Respiratory support for the severely handicapped child with neuromuscular disease: Ethics and practicality

Semin Respir Crit Care Med

The PedsQL™ 4.0: Reliability and validity of the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales in healthy and patient populations

Med Care

Parenting stress index

Child abuse potential and parenting stress: Relationships in maltreating parentsPsychological assessment

J Consult Clin Psychol

Child-related and parenting stress: Similarities and differences between mothers and fathers of children with disabilities

Am J Ment Retard

Original article
Parental Stress and Quality of Life in Children With Neuromuscular Disease

The PedsQL^™ 4.0: Reliability and validity of the Pediatric Quality of Life Inventory Version 4.0 Generic Core Scales in healthy and patient populations