Understanding carer resilience in Duchenne muscular dystrophy: A systematic narrative review

Children, 2021

The purpose of this integrative review was to explore psycho-social impacts and therapeutic interventions for parent caregivers of young people living with Duchenne’s Muscular Dystrophy (DMD). Electronic databases were searched for research publications between 2010 and 2020. This included Medline, CINAHL, PsycINFO, ERIC, ERC, and AMED. Four central themes emerged: Living with DMD; Knowing and telling; Transitioning; and Building resilience. The impact on parents caring for a child with DMD affected all aspects of their lives, changed over time, and had identifiable peak stress points. Unmet parental information and support needs left parents struggling in their role. Transition required changes to parenting behaviors and required adaptation and resilience. It is proposed that future investment should focus on anticipating family need, targeting intervention cognizant of predictable stress points and building resilience through social community. Parents may then be better positioned...

This paper focuses on the psychological benefits of caregiving in key relatives of patients with muscular dystrophies (MD), a group of rare diseases characterized by progressive weakness and restriction of the patient's functional abilities. We describe whether relatives perceived caregiving to be a positive experience and test whether relatives' perceptions vary in relation to their view of the patient as a valued person, the degree of involvement in care, and the level of support provided by social network and professionals. The study sample included 502 key relatives of patients aged 4–25 years, suffering from Duchenne, Becker, or limb-girdle MD, in treatment for at least 6 months to one of the eight participating centers, living with at least one relative aged 18–80 years. Of key relatives, 88 % stated that they had gotten something positive out of the situation, 96 % considered their patients to be sensitive, and 94 % viewed their patients as talented. Positive aspects of caregiving were more recognized by key relatives who were more convinced that the patient was sensitive and who perceived that they received higher level of professional help and psychological social support. These results suggest that most key relatives consider that their caregiving experience has had a positive impact on their lives, despite the practical difficulties of caring for

Journal of Neurology, 2020

Background Living with a progressive disease as muscular dystrophy (MD) can be challenging for the patient and the entire family from both emotional and practical point of view. We aimed to extend our previously published data about mental health in patients with MDs, also investigating coping profiles of both themselves and their parents. Furthermore, we wanted to verify whether psychological adaptation of patients can be predicted by coping strategies, taking also into account physical impairment, cognitive level and socioeconomic status. Methods 112 patients with MDs, aged 2-32 were included. Their emotional and behavioural features were assessed through parent-and self-report Achenbach System for Empirically Based Assessment questionnaires and Strength and Difficulties Questionnaires. Development and Well-Being Assessment or Autism Diagnostic Observation Schedule were administered to confirm suspected diagnoses. Coping profile of both parents and patients was assessed through the self-administered New Italian Version of the Coping Orientation to the Problems Experienced questionnaire and its relationship with emotional/ behavioural outcome was examined in linear regression analyses. Results High prevalence of intellectual disability and autism spectrum disorders was confirmed in Duchenne MD. Despite the high rate of internalizing symptomatology, we did not report higher rate of psychopathological disorders compared to general population. Parents tend to rely more on positive reinterpretation and less on disengagement coping. Avoidance coping, whether used by parents or patients, and ID, predicted increased emotional/behavioural problems. Conclusions Psychosocial interventions should address problems of anxiety and depression that people with MDs frequently experience, even through fostering parents' and childrens' engagement coping over disengagement coping.

Developmental Medicine & Child Neurology

ABBREVIATIONS DMD Duchenne muscular dystrophy EQ-5D EuroQol-5D HRQoL Health-related quality of life ZBI Zarit Caregiver Burden Interview AIM To conduct a systematic literature review of caregiver burden in Duchenne muscular dystrophy (DMD). METHOD We searched Embase, Web of Science, and PubMed for full-text articles reporting results from studies of caregiver burden in DMD. RESULTS We identified 483 unique publications. Of these, 450 were excluded after title and abstract screening, and 12 after full-text review. A total of 21 articles were included for data synthesis. Results encompassing more than 15 aspects of caregiver burden, investigated through surveys and/or interviews across 15 countries, were identified in the literature. Caregiving in DMD was frequently associated with impaired health-related quality of life, poor sleep quality, reduced family function, depression, pain, stress, sexual dysfunction, and/ or lower self-esteem, as well as a considerable impact on work life and productivity.

International Journal of Academic Research in Psychology, 2019

Muscular dystrophies are a heterogeneous group of mainly hereditary diseases that affect the functioning of the muscle system. Few studies have focused on the psychological adjustment of parents of children with Duchenne muscular dystrophy. This study's objective was to investigate the parents' experience and the process of adjustment to the complex nature of their child's disorder. The study used a qualitative research design. Qualitative thematic analysis was used to analyze the data collected from parents (n=9) of children with Duchenne muscular dystrophy (n=10) using the technique of semi-structured interviews. The parents' experience is characterized by painful and recurrent losses, which intensify their feelings of isolation and parental responsibility. On the other hand, parents develop internal coping mechanisms and identify sources of empowerment, which they evaluate as positive aspects of their experience. Recurrent losses alternate with periods of stability, hope and efforts toward normalcy in family life. Results of this study implicate the importance of adequate psychosocial interventions that will allow parents to cope with complex challenges, elaborate on their experiences and identify internal and external resources.

Journal of Rehabilitation Medicine, 2006

To assess the burden on family carers of persons with muscular dystrophy living in their homes and to determine factors contributing to carer burden. The study included 56 dyads of people with muscular dystrophy and their family carers. The variables for carer burden were compared by logistic regression in 2 carer groups (burden + /burden-). The mean age of the patients with muscular dystrophy was 32.7 years (median 26.7, range 15-65 years) and that of the carers 51 years (median 48, range 30-80 years). The carers reported the care burden using the Zarit Burden Inventory (median score 23, range 0-57/88). Multivariate analysis produced 3 adjusted explicative factors: carer characteristics related to risk of perceived burden are self-report of poor social functioning on the SF-36 (OR = 26.6 (2.6-278); p=0.006), self report of anxiety on the Hospital Anxiety Scale (OR = 7.1 (1.4-36); p=0.02) and being a carer under 48 years of age (OR = 7.8 (1.7-34.5); p=0.007). However, it was difficult to dissociate the different health variables of the carers from each other. This approach should lead to better decision-making by medical teams, patients and their carers.

Muscular Dystrophy refers to a group of disorders of progressive muscular degeneration and weakness. It leads to loss of strength of the skeletal muscles that control movement. The impact of muscular dystrophy is not only limited to physical disability, but it also impairs cognitive functions. Aim: The current study aims to understand the impact of children with muscular dystrophy on family. The specific areas explored in the study include struggles in providing physical care, financial burden, caregiver's health, career adjustments, psychological burden and limitations in social activities. Method: The study focused on the family of two boys with muscular dystrophy. A semi-structured interview schedule was used to gather data about how a family coped with muscular dystrophy in two children. Results: The findings of the study showed that physical disability and the consequent cognitive impairment has a significant negative impact on family. Conclusion: The impact of disability on family due to muscular dystrophy is significant. The primary concerns in terms of impact is found to be on caregiver's health, financial and psychological burden.

Journal of Autism and Developmental Disorders, 2020

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Journal of Developmental & Behavioral Pediatrics, 2011

Objective: To examine what contributes to resiliency in children living with Duchenne muscular dystrophy (DMD), a chronic, progressive neuromuscular disorder that also influences cognitive ability. The authors hypothesized that family and social support will moderate the effects of individual symptoms of illness severity and influence positive adjustment in boys with DMD. Method: One hundred forty-six boys with DMD were included. Child adjustment, as determined by parent ratings of their son's behavior using the Total Behavior score from the Child Behavior Checklist (CBCL), was examined as an outcome measure. The contributions of individual variables (including age [which serves also as a proxy for degree of physical disability], wheelchair use, and estimated verbal IQ), family variables (the Parental Distress score from the Parent Stress Index), and social environment variables (the Social Competence score from the CBCL) on child adjustment were examined in a linear regression analysis. Results: Both family and social environment variables significantly contributed to the variance in the CBCL Total Behavior score. In contrast, individual factors that are related to illness severity (age, degree of physical involvement, and estimated verbal IQ) were not associated with child adjustment. Conclusion: Increased children's social networks and decreased parents' stress levels positively contributed to good child adjustment, whereas degree of individual clinical severity did not. Thus, emphasis on providing opportunities for friendships and social support and on parents' adjustment will aid in children's resilience, ensuring they can live well, even while living with the significant burdens associated with DMD.

Journal of Pediatric Nursing, 2002

This university hospital-based case control study included 31 parents with a Duchenne muscular dystrophy (DMD) child and 30 parents (control group) of a child with a fever. The study was conducted in southern Taiwan. The study compared the differences of stress and coping strategies between the two groups using closed-ended questionnaires. The control group (fever group) showed higher "stress," "conflict," and "help needs." The DMD subjects had a tendency to use wish-fulfilling fantasy to cope. "Impact" was influenced by income and religion, and income and mother's age influenced coping strategies. Professionals need to manage the parent's conflict, to provide information and resources, and to support the parent's emotional reactions to caring for a child with acute and chronic illness.