Dataset Information

Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study.

ABSTRACT:

Background

Spinal muscular atrophy (SMA) is an autosomal-recessive motor neuron disease leading to dysfunction of multiple organs. SMA can impair the quality of life (QoL) of patients and family. We aimed to evaluate the QoL of children with SMA and their caregivers and to identify the factors associated with QoL in a cross-sectional study conducted in China.

Methods

We recruited 101 children aged 0-17 years with SMA and their caregivers from a children's hospital in China. Twenty-six children had type I SMA, 56 type II and 19 type III. Each child's QoL was measured by the Pediatric Quality of Life Inventory 3.0 Neuromuscular Module (PedsQL NMM), which was completed by the child's caregivers. The caregiver's QoL was measured by the Pediatric Quality of Life Inventory Family Impact Module (PedsQL FIM). Information on sociodemographic characteristics, disease-specific characteristics, and treatments were collected using the proxy-reported questionnaire. Two-sample t tests and one-way ANOVA were used to compare differences in average scores of QoL across subgroups.

Results

Children with type III SMA had a higher average Total score of PedsQL NMM and higher average scores in domains Neuromuscular disease and Family resources than children with type I or type II SMA (p?ConclusionOur study has demonstrated factors that may impair or improve QoL of children patients with SMA and their parents. Particularly, QoL was relatively poor in children with type I and type II SMA as well as in their caregivers compared to those with type III SMA. We strongly recommend that standard of care in a multidisciplinary team be strengthened to improve the QoL of SMA patients. Our study called for increased attention from clinical physicians on measuring QoL in their clinical practices in order to enhance the understanding of impacts of SMA and to make better decisions regarding treatment.

SUBMITTER: Yao M

PROVIDER: S-EPMC7789582 | biostudies-literature | 2021 Jan

REPOSITORIES: biostudies-literature

ACCESS DATA

Publications

Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study.

Yao Mei M Ma Ying Y Qian Ruiying R Xia Yu Y Yuan Changzheng C Bai Guannan G Mao Shanshan S

Orphanet journal of rare diseases 20210106 1

<h4>Background</h4>Spinal muscular atrophy (SMA) is an autosomal-recessive motor neuron disease leading to dysfunction of multiple organs. SMA can impair the quality of life (QoL) of patients and family. We aimed to evaluate the QoL of children with SMA and their caregivers and to identify the factors associated with QoL in a cross-sectional study conducted in China.<h4>Methods</h4>We recruited 101 children aged 0-17 years with SMA and their caregivers from a children's hospital in China. Twenty ...[more]

PMID: 33407670

Similar Datasets

Project description:To assess the quality of life (QoL) of caregivers of children with sickle cell disease (SCD) and to determine the risk factors associated with poor QoL.A cross sectional study was conducted between 01 and 30 June 2015, in a tertiary care center in western Saudi Arabia to assess various dimensions of QoL by using TNO-AZL Questionnaire for Adult's Health-related Quality of Life (TAAQOL). A total 164 adult caregivers (aged 16 years or more) of children with SCD, who were regularly visiting the department were enrolled (refusal rate?=?61.6%). The questionnaire scores were transformed into 0-100 scale; with higher scores indicating less difficulty and better QoL Demographic, socioeconomic data and a satisfaction questionnaire regarding participants' lifestyle were collected and analyzed as risk factors for impaired QoL, by comparing different QoL dimensions' scores using independent t-test, Oneway ANOVA, or linear regression, as appropriate.Sixty-three caregivers were included; 79.4% were mothers, age range 21-71 years, 64.5% were from low social class receiving insufficient support and financial needs were unmet for considerable number of families. Analysis of QoL using TAAQOL showed that emotions (median [75th centile]?=?44.44 [66.67] for negative and 61.11 [72.22] for positive emotions), sleep quality (66.67 [91.67]) and sexual life (50.00 [83.33]) were the most affected dimensions. Professional achievement (91.67 [100]), cognitive skills (83.33 [100]), and social contact (100 [100]) were relatively preserved. Negative emotions were more marked in mothers and mostly predicted by satisfaction with social relations notably with partner (B?=?3.14, p?=?0.016), friends (B?=?2.51, p?=?0.015) and relatives (B?=?2.69, p?=?0.016). Positive emotions were predicted by the levels of satisfaction of the caregiver with his/her health (B?=?2.56, p?=?0.001), job achievement (B?=?4.54, p?=?0.001), living conditions (B?=?2.60, p?=?0.034) and the condition of the diseased child (B?=?2.55, p?=?0.011). A strong correlation was found between sleep quality and cognitive skills.There are notable financial and emotional burdens on the caregivers of children with SCD affecting various aspects of their QoL, which are likely to be impacted by the individual levels of social and professional achievement. Physicians and health authorities should give particular attention to the QoL of caregivers and families of children with SCD, to help them cope up with the disease and overcome its related psychological and financial impacts.

Dataset Information

Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study.

Background

Methods

Results

Publications

Quality of life of children with spinal muscular atrophy and their caregivers from the perspective of caregivers: a Chinese cross-sectional study.

Similar Datasets

OmicsDI is part of the ELIXIR infrastructure

Tweets