Duchenne muscular dystrophy and caregiver burden: a systematic review.

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. INTERPRETATION: Providing informal care to a patient with DMD can be associated with a substantial burden. Yet, more research is needed to better understand the clinical implications of caregiving in DMD and the relationship between caregiver burden and the progression of the disease. Our data synthesis should be helpful in informing clinical and social support programmes directed to families caring for a patient with DMD. WHAT THIS PAPER ADDS: A substantial body of evidence describes caregiver burden in Duchenne muscular dystrophy. Little is known of the family burden beyond caregivers' self-assessments.

Guidelines for the Perianesthesia Care of the Duchenne Muscular Dystrophy/Becker Muscular Dystrophy Patient.

More patients suffering with Duchenne muscular dystrophy (DMD) and Becker muscular dystrophy (BMD) are presenting to perianesthesia settings for emergent and nonemergent treatment and care. A group of collaborative health care providers at Rush University Medical Center in Chicago developed a multidisciplinary DMD/BMD Task Force to study this disorder and create a set of guidelines to aid those engaging in the planning, execution of care, and recovery of this unique population in the perianesthesia setting. Attention to detail, well-executed preplanning, meticulous awareness of the patient, and prearranged implementation and intervention has proven to offset potential problems and complications and is the key to a successful perianesthesia period.

Sleep quality of mother-caregivers of Duchenne muscular dystrophy patients.

BACKGROUND: Sleep disturbance is a common problem for caregivers. In general, patients with Duchenne muscular dystrophy (DMD) use noninvasive ventilation to maintain quality of life and improve survival. OBJECTIVE: The aim of this study was to evaluate the sleep quality of caregiver-mothers of sons with DMD and factors that are associated with their sleep quality. METHODS: We evaluated 32 caregiver-mothers of sons with DMD and 32 mothers of sons without any neuromuscular or chronic disease (control-CTRL group). The evaluation of quality of sleep was made using the Pittsburgh Sleep Quality Index (PSQI). RESULTS: Caregiver-mothers had poor sleep quality, specifically longer sleep latency and reduced sleep efficiency. The impaired sleep quality of the caregiver-mothers was associated with the length of time of noninvasive ventilation used by their sons. CONCLUSIONS: Our results suggest that caregiver-mothers of sons with DMD have poor quality of sleep, and the length of use of noninvasive ventilation of their sons is associated with better sleep of caregiver-mothers.

Health-related quality of life in patients with Duchenne muscular dystrophy: a multinational, cross-sectional study.

AIM: To estimate health-related quality of life (HRQOL) in patients with Duchenne muscular dystrophy (DMD). METHOD: HRQOL was assessed using the Health Utilities Index Questionnaire (HUI) and the Pediatric Quality of Life Inventory (PedsQL) neuromuscular module version 3.0 online. Results were stratified by disease stage (early/late ambulatory/non-ambulatory) and caregivers' perceptions of patients' health and mental status. RESULTS: A total of 770 patient-caregiver pairs (173 German, 122 Italian, 191 UK, and 284 USA) participated. Most caregivers (>84%) perceived their patients as happy/somewhat happy and in excellent/very good/good health, irrespective of current ambulatory class. In contrast, mean patient utility (reflecting public preferences: 0, dead; 1, perfect health) deteriorated with disease course, from 0.75 in early ambulatory males to 0.15 in the most severely affected patients. Mean patient PedsQL scores (0-100, higher score indicating better HRQOL) decreased from 80 to 57 across ambulatory classes. INTERPRETATION: HRQOL in DMD, measured through public preferences, is substantially impaired in relation to the general population and significantly associated with disease progression. Still, most patients are perceived as happy and in good health by their caregivers, indicating that influential domains of HRQOL remain intact through the disease progression. Our findings emphasize the challenges in measuring HRQOL in a rare, progressive childhood condition such as DMD.

Becoming a back-up carer: parenting sons with Duchenne muscular dystrophy transitioning into adulthood.

The population of adults with Duchenne muscular dystrophy is increasing rapidly. However, information for individuals with DMD and their parents about the transition to adulthood is lacking; young adult sons and their parents may struggle to maintain smooth family functioning and well-being during this period. This study examined the process of change in parental behaviors during their son's transition. The participants were 18 parents with sons aged 15-30 years. Data were obtained from semi-structured interviews and analyzed using a grounded theory approach. Eleven categories of behaviors were identified across three domains: emotional, physical, and determination. The changes made by parents were directed toward becoming a back-up carer: letting go of some control but still being active participants in their sons' lives. We identified several issues important for well-being in the transition period: psychological support, the aging of the parents (the primary caregivers) and the concomitant emergency and specialized care needs, and parents' intervention in the self-determination of adult sons with DMD. The findings of this study may provide a rationale to advocate for policies to improve support for parents of sons with DMD transitioning to adulthood and provide information to help parents in their role as primary care providers.

Psychosocial challenges in family caregiving with children suffering from Duchenne muscular dystrophy.

Families of children with Duchenne muscular dystrophy (DMD) go through significant challenges in dealing with the condition. Few studies have looked into the situation, especially in the sociocultural scenario that is unique to India. The authors' aim was to identify the psychosocial challenges for caregivers of children with DMD. A cross-sectional descriptive study was perfomed among the caregivers of 60 children with DMD who were attending the neuromuscular disorders clinic of a national tertiary referral center for neurological disorders. The knowledge and attitude, psychosocial needs, burden, and coping patterns were assessed in an interview. The findings showed that parents of children with DMD tended to have inadequate understanding of the disease but had a positive attitude, had a moderate family burden, and tended to rely more on religion, focus on and venting of emotions, and instrumental and emotional social support for coping. Caregivers of children with DMD would benefit from psychosocial intervention to address their understanding of and attitude toward the disease, as well as burden of dealing with it, and to help them develop their coping skills and meet their children's and their own needs.

Breathe easy.

Staff at a children's hospice and the respiratory nurse team at an acute hospital are working together to help young people with Duchenne muscular dystrophy lead more active lives. The young people receive a non-invasive form of ventilation that can be used at home.

Dependence on care experienced by people living with Duchenne muscular dystrophy and spinal cord injury.

Being dependent on care in a hospital or in a traditional homecare setting may generate an experience of inferiority in patients. In a private home, dependence is easier to bear if the dependent person has the possibility to influence the planning of care. Little is known about the experience of being dependent on care in a private home, where the dependent person employs his or her own helpers. The aim of this study was to describe the meaning of dependence on care in a private home setting among people living with help requirements for all aspects of daily life. The article draws on two interview studies of people with high cervical spinal cord injury and men with Duschenne muscular dystrophy. Transcriptions of the interviews were analyzed according to a phenomenological hermeneutic approach influenced by Paul Ricoeur's philosophy of interpretation. The meaning of all the interview texts is presented as four short stories. Four themes were identified: the helper as liberating, the paramount verbalization of own needs, the creative engagement in life, and accessibility as an issue in everyday life. Dependence on care was identified to be a movement between freedom and restriction, where the helpers played a crucial role, because it was key that they were sensitive to the signals they got and were able to transform words into meticulous actions.

Subjective caregiver burden of parents of adults with Duchenne muscular dystrophy.

PURPOSE: To describe subjective caregiver burden of parents of adults with Duchenne muscular dystrophy (DMD) and to identify factors associated with the level of subjective burden. METHODS: In a cross-sectional study in 80 parents of 57 adult, severely disabled DMD patients' level of subjective caregiver burden was measured with the Caregiver Strain Index (CSI) and the Self Rated Burden Scale. A visual analogue scale was used to measure happiness. The expanded CSI version, the CarerQoL and open questions were used to analyse caregiving in more depth. In uni and multivariate analyses, associations of objective care characteristics, patient characteristics and caregiver characteristics with burden were explored. RESULTS: Parents indicated substantial burden, but valued giving care as being important and rewarding. Subjective burden was associated with received support, tracheotomy, active coping by the patient and anxiety in patient and parents, together explaining 34%-36% of variance. Living situation was not associated with experienced burden. CONCLUSIONS: Caring for an adult son with DMD is burdensome, but rewarding. Subjective caregiver burden of parents may be alleviated by adequate support and respite care, by avoiding tracheotomy and by intervention programs targeting anxiety and promoting active coping by the patient from childhood.

Siblings of young people with Duchenne muscular dystrophy--a qualitative study of impact and coping.

BACKGROUND: Duchenne Muscular Dystrophy (DMD) is a progressively debilitating neuromuscular disorder markedly affecting family life. AIMS OF THE STUDY: To obtain descriptive accounts from siblings about impact and coping with DMD and consider implications for psychological function. METHODS: Semi-structured interviews with a purposive sample of healthy siblings of young people with DMD attending a regional centre. Main carers were interviewed for corroborative evidence. Interviews were recorded and transcribed verbatim prior to a thematic analysis. RESULTS: 35 siblings (18 girls, 17 boys from 29 families) and their parents were interviewed. Mean sibling age was 14.3 years (sd 2.4) (range 11-18). Young people with DMD were aged 5-22 years; most were wheelchair users and 3 required nocturnal ventilation. Six primary themes were identified. Knowledge about the nature of DMD had been acquired gradually and varied in breadth. Whilst siblings were involved and generally accepting of caring responsibilities, they were nevertheless able to pursue other activities and interests of their choice. Positive impacts included increased family cohesion, knowledge and maturity in siblings; negative impacts included inequality of parental attention/availability. Coping was aided by the normality of a situation gradually developing since early childhood, and by support from friends, relatives, teachers and mentors. Coping techniques included getting on with life one day at a time, proactively restraining negative emotions, humour, distractions, and physical relocation. CONCLUSIONS: DMD generates situations that have the potential to increase the risk of emotional problems in unaffected siblings, but its chronic course also provides opportunities for positive family psychological adjustment.

Opportunities for people with disabilities in the virtual world of Second Life.

The virtual world of Second Life (SL) offers people with disabilities a chance to explore new worlds without being limited by their disabilities. Many people with disabilities use SL for information, support, and entertainment. SL is a computer-based simulated environment in which participants are represented by a human-like avatar. The avatar can move through the environment, manipulate objects, and participate in day-to-day activities that most people take for granted, such as walking, dancing, and communicating. In this article, the authors focus on the benefits that information, socialization, and community membership can offer people with disabilities and some of the resources that are available for them in SL. SL communities, groups, and activities also help increase self-worth and empower people with disabilities. Participating in a virtual world enriches the overall quality of life of people with disabilities and may enhance their physical, emotional, and social adjustment.

The effect of caregiving on women in families with Duchenne/Becker muscular dystrophy.

Duchenne/Becker muscular dystrophy (DBMD) is a disorder of progressive muscle weakness that causes an increasing need for assistance with activities of daily living. Our objective was to assess the psychosocial health and contributing factors among female caregivers in families with DBMD. We conducted a survey of adult women among families with DBMD in the United States (US) from June 2006 through January 2007, collecting data related to the care recipient, perception of caregiving demands, personal factors, and socio-ecologic factors. Life satisfaction, stress, and distress were assessed as outcomes. Existing validated instruments were used when available. We received responses from 1238 women who were caring for someone with DBMD, 24.2% of whom were caring for two or more people with DBMD. Caregivers were more likely to be married/cohabitating than women in the general US population, and a high level of resiliency was reported by 89.3% of caregivers. However, the rate of serious psychological distress was significantly higher among caregivers than among the general population. Likewise, 46.4% reported a high level of stress, and only 61.7% reported that they were satisfied with their life. A high level of caregiving demands based on the Zarit Burden Interview (ZBI) was reported by 50.4% of caregivers. The post-ambulatory phase of DBMD was associated with decreased social support and increased ZBI scores. In multivariate logistic regression modelling, life satisfaction was dependent on high social support, high resiliency, high income, and form of DBMD. Distress and high stress were predicted by low resiliency, low social support, and low income. Employment outside of the home was also a predictor of high stress. Interventions focused on resiliency and social support are likely to improve the quality of life of DBMD caregivers, and perhaps caregivers of children with other disabilities or special health care needs as well.