Raising a Child With Congenital Muscular Dystrophy: Impact on the Family

PURPOSE: Rare childhood disorders are often chronically debilitating, and affected children may have life-threatening conditions that require special care from family members or caregivers. Early-onset rare disorders are difficult to diagnose and treat due to shortages of skilled health professionals, treatment options, and health care services. Congenital muscular dystrophy (CMD) is a rare disorder with an onset in infancy. Most previous studies have investigated the natural history and clinical features of CMD, while the impact of the children’s health conditions on their parents’ daily lives and parenting occupations has received less research. For families with a child with chronic disability, child rearing, meeting the demands of daily life, and managing the child’s health and well-being are critical parent and family occupations. To increase understanding of the impact of raising a child with CMD, this study compared parental quality of life (QoL), family functioning, and family cohesion between families with and without a child with CMD.

METHOD: A cross-sectional design was used for this study. Parents of children ages 5–19 with CMD (n = 28) and parents of age-matched unaffected children (n = 28) participated in this study. Both groups of parents completed questionnaires measuring parental QoL, family functioning, and family cohesion. Parental QoL and family functioning were examined using the PedsQL™ Family Impact Module, and family cohesion was assessed using the Family Adaptability and Cohesion Evaluation Scale–IV; higher scores on each instrument indicate better functioning. Distribution of the data was tested using the Shapiro–Wilk test. Mann–Whitney U tests were conducted to compare parental QoL, family functioning, and family cohesion between parents of children with CMD and parents of unaffected children.

RESULTS: Shapiro–Wilk tests demonstrated that not all scores were normally distributed (p < .05). The results of the Mann–Whitney U tests showed that all three factors were significantly different between the two groups (p < .05). The parental QoL score of the unaffected group (Mdn = 74.74) was significantly higher than for the affected group (Mdn = 49.79; U = 125.50, z = –4.367, p < .0001). The family functioning score was significantly higher in the unaffected group (Mdn = 62.50) than in the affected group (Mdn = 48.75; U = 205, z = –3.066, p = .002). However, in family cohesion, the score (total ratio) of the affected group (Mdn =3.35) was significantly higher than in the unaffected group (Mdn = 2.66; U = 514, z = 1.999, p = .046).

CONCLUSION: The findings of this study show that parental QoL and family functioning of families of children with CMD were negatively affected by their child’s health. However, cohesion in the families of children with CMD was significantly higher than that in the families of unaffected children, possibly indicating that families of children with CMD are successfully working together to meet the challenges they face. The findings suggest the need for development of effective strategies to support parents’ and families’ occupations and positive family functioning. This study provides empirical evidence important for strengthening evidence-based approaches supporting parents and families.

References

Bertini, E., D’Amico, A., Gualandi, F., & Petrini, S. (2011). Congenital muscular dystrophies: A brief review. Seminars in Pediatric Neurology, 18, 277–288. https://doi.org/10.1016/j.spen.2011.10.010

DeGrace, B. W. (2004). The everyday occupation of families with children with autism. American Journal of Occupational Therapy, 58, 543–550. https://doi.org/10.5014/ajot.58.5.543

DeGrace, B. W., Hoffman, C., Hutson, T. L., & Kolobe, T. H. A. (2014). Families’ experiences and occupations following the diagnosis of autism. Journal of Occupational Science, 21, 309–321. https://doi.org/10.1080/14427591.2014.923366

Grut, L., & Kvam, H. M. (2013). Facing ignorance: People with rare disorders and their experiences with public health and welfare services. Scandinavian Journal of Disability Research, 15, 20–32. https://doi.org/10.1080/15017419.2011.645870