Factors affecting the health-related quality of life of caregivers of patients with muscular dystrophy.

Muscular dystrophy (MD) is a group of progressive muscle weakness diseases. The caregiver burden, increasing as the disease progresses, can be associated with impaired health-related quality of life (HRQOL). The aims of this study were to investigate the HRQOL in caregivers of patients with MD and identify the factors associated with HRQOL. A cross-sectional assessment of caregiver HRQOL was performed with the Short Form-36 and compared with norms. The factors affecting HRQOL were investigated by patient and caregiver characteristics. The Muscular Dystrophy Functional Rating Scale was used to assess the functional status (mobility, basic activities of daily living, arm function, and impairment) of patients. The demographic data and social interaction activities of caregivers were assessed. Caregivers (n = 62) had poor HRQOL. Caregiver HRQOL was associated with the patient's functional status, especially in the domains of Vitality and Mental Health. Numerous visits by neighbors and close friends of the caregiver family indicated better HRQOL (in the body pain, general health, vitality, role emotion and mental health domains). Caregiver HRQOL was associated with caregiver education level, while patient age, caregiver age, length of caregiving, and family income were not. These findings demonstrate that caregivers have poor HRQOL, and the mental domain of quality of life is associated with the patient's functional status, social interaction, and caregiver education level. We suggest that rehabilitation programs focus on caregiver HRQOL, promote the patient's functional status with assistive technology, enhance professional caring techniques, and encourage participation in social groups to improve caregiver HRQOL.

Family context in muscular dystrophies: psychosocial aspects and social integration.

Muscular dystrophies (MDs) are degenerative diseases which may led to marked functional impairment and reduced life expectancy. Being caregivers of a loved one with MD may be both a rewarding and a demanding experience that may have relevant impact on the quality of life of the whole family. In this short review we summarize the main findings of the first survey on family context in MD in Italy. The study was carried out on 502 key-relatives of patients suffering from Duchenne, Becker, or Limb-Girdle MD, aged between 4 and 25 years, and attending one of 8 participating Centers, all over 2012. The results revealed that practical difficulties were mainly related to relatives' involvement in helping the patient in moving and in relative's constraints of leisure activities. Furthermore, feelings of loss and perception of patient's condition as having negative effects on the family life were the psychological consequences more frequently complained. However, despite the difficulties, 88% of the key-relatives acknowledged the caregiving as a positive experience. In fact 94% of the respondents stated they could rely on friends in case of own physical illness, and 88% in case of psychological stress. Burden was found higher among relatives of patients with lower functional autonomy and longer duration of illness, and among relatives with lower professional and social support. Conversely, the positive aspects of the caregiving were more frequently acknowledged by those who received higher level of professional help and psychological social support. These results reveal that the caregiving experience has a positive impact on key-relatives quality of life despite the practical demands, and that the support of professionals is essential to help families in identifying the benefits of this experience without denying its difficulties.

"I have got something positive out of this situation": psychological benefits of caregiving in relatives of young people with muscular dystrophy.

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 patients with MD. Professionals should help relatives to identify the benefits of caregiving without denying its difficulties. Clinicians themselves should develop positive attitudes towards family involvement in the care of patients with long-term diseases.

Caring for young people with chronic illness: a case study.

This article is a case study of a 16-year-old female with congenital muscular dystrophy. It aims to raise awareness of some of the issues associated with complex illness during adolescence, particularly the transition from child to adult services. To improve care it is argued that the needs and values of the young person should be respected and interventions planned locally with the young person and their family where appropriate.

Factors relating to carer burden for families of persons with muscular dystrophy.

OBJECTIVE: To assess the burden on family carers of persons with muscular dystrophy living in their homes and to determine factors contributing to carer burden. METHODS: 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-). RESULTS: 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. CONCLUSION: This approach should lead to better decision-making by medical teams, patients and their carers.

Being the next of kin of an adult person with muscular dystrophy.

A chronic disorder affects all members of the family in various ways. The aim of this study is to elucidate the next of kin's (N= 36) experiences when an adult family member has muscular dystrophy. The relationships were partner (36%, n= 14), parent (18%, n= 7), child (21%, n= 8), sibling (15%, n= 6), and other relative (3%, n= 1). Latent content analysis is employed and involves an interpretation of the interviewtext. The results showthe meaning of being close to a person with muscular dystrophy through the themes that emerged: exposure of the family; the span between obligation and love; being vigilant, protective, and supportive; and striving for an ordinary life. This study reveals a need for healthcare staff to understand the next of kin's narrated meaning of changes when a family member has a progressive disease.

Transportation of a client with muscular dystrophy on commercial air: a unique setting for a rehabilitation nurse.

This experience was very well suited to rehabilitation nursing, demanding specialized knowledge and care. Our talents can be used in unique situations. We can make a difference in the lives of persons who have altered functional abilities to promote optional function--no matter what the location.

Caring for a child with a progressive illness during the complex chronic phase: parents' experience of facing adversity.

The qualitative study explored the day-to-day experiences of parents caring at home for a child with a progressive life-threatening illness at a certain point in their illness trajectory. This point in the trajectory is when the child lives with a complex chronic condition, and is in need of specialized and time-consuming care, but is not yet in a terminal phase. The naturalistic research design of phenomenology was chosen for the study's methodology. Parents' experiences of caring for their child were conceptualized as an ongoing process of 'facing adversity', as parents had continuously to redefine and then manage those changes resulting from the progressive nature of their child's condition. Concepts of normalization and chronic sorrow are considered in the conceptualization, as are the challenges of caregiving (particularly of mothers) who faced many hardships in their role, including the myriad of changes related to the increased burden of care. Implications for nursing practice are identified.

Nursing practice management: muscular dystrophy.

The Nursing Practice Management section displays a health care plan for a student with Duchenne Muscular Dystrophy. Following a brief overview of the pathophysiology of the disorder, a case study is presented from which a nursing process-based plan of care is derived.

Touch and visualisation to facilitate a therapeutic relationship in an intensive care unit--a personal experience.

This paper describes how the nature of touch was explored and utilised in nursing practice to promote a therapeutic relationship between nurse and client in an intensive care setting. It explains how this, and other complementary therapies such as visualisation and relaxation techniques, were used to facilitate a counselling situation, and also to effect the resolution of acute anxiety as an alternative to the traditional allopathic approach.

Traveling with technology.

This article reports on a 2-week trip in a motor home through the southeastern United States with an oxygen- and ventilator-dependent young woman with muscular dystrophy. The article describes the planning, implementation, and evaluation of traveling with technology. Discussed are the amount and types of equipment and supplies to take on such a trip, modification of equipment and the motor home, the roles and responsibilities of the nurses, interactions with the client and family, and legal issues. The impact of the trip on the client's behavior--which was to transform her from a passive recipient of care to an actively involved participant--is reported.

The family's experience of living with a child with Duchenne muscular dystrophy.

An ethnographic inquiry was conducted to better understand the experience of families living with a child with Duchenne muscular dystrophy. Participant-observation and in-depth interviews were used to collect data. Six themes emerged as primary descriptors of the experience: (a) Disillusionment: The Erosion of the Hope for Normalcy; (b) Society Confirms the Impossibility of Normalcy; (c) Dynamics of the Family: Who's Disabled Anyway?; (d) A Smaller World; (e) Letting Go or Hanging On; and (f) Things Must Change. Implications of these themes are discussed in terms of need for counseling and support services for families, in-service education for health professionals, and activities for children diagnosed with Duchenne muscular dystrophy.

The impact of Duchenne muscular dystrophy on families.

To understand the experiences of families having a child with Duchenne muscular dystrophy, the investigator studied three families for 10 weeks with a follow-up at 1 year. Six themes recurred in the common experiences in the families: the erosion of hope for normalcy; society's confirmation of the impossibility of normalcy; the dynamics of the family; a smaller world; letting go or hanging on; and things must change. This article illustrates these six themes, compares them to Roy's Adaptation Model, and discusses nursing interventions.