Identifying Opportunities to Provide Family-centered Care for Families With Children With Type 1 Spinal Muscular Atrophy

Murrell, Diane V.; Crawford, Claire; Jackson, Chanti T.; Lotze, Timothy; Wiemann, Constance M.

doi:10.1016/j.pedn.2018.09.007

Cited by 19 publications

(9 citation statements)

References 38 publications

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“…Moving forward, there is a need for a multidisciplinary team to provide a holistic care from diagnosis to treatment, rehabilitation, and psychosocial support. Transdisciplinary collaboration and open communication between all the stakeholders of various specialties focusing on patient and family centered goals are paramount [ 29 ]. Collaboration between established NGOs, healthcare providers and community support would be vital to aid with knowledge and capacity building focusing on how to care for PWSMA.…”

Section: Discussionmentioning

confidence: 99%

A mixed method study on the impact of living with spinal muscular atrophy in Malaysia from patients’ and caregivers’ perspectives

Ch’ng

Koh

Ahmad‐Annuar

et al. 2022

Orphanet J Rare Dis

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Background Spinal Muscular Atrophy (SMA) is a rare, recessively inherited neuromuscular disorder that causes progressive muscle weakness. There is a low degree of awareness about SMA amongst the public and healthcare providers, which may impact the perception of the disease and its proper management. To understand how this lack of awareness may have affected diagnosis, care and support for SMA patients and their caregivers, this study aims to investigate the impact of SMA on the lives and daily activities of SMA patients and their caregivers in Malaysia. Methods Nationwide recruitment was carried out via invitations coordinated by a local SMA advocacy organization. A mixed method cross-sectional study consisting of a self-administered questionnaire followed by in-depth interviews (IDIs) and focus group discussions (FGDs) was conducted. The interview sessions were audio-taped, and verbatim transcripts analyzed thematically. Results Participants reported feeling stressed, anxious and depressed. There were issues with delayed diagnosis, lack of information from healthcare professionals about the disease progression, and limited access to supportive services like physiotherapy. Participants expressed their concerns living with self-doubt and turmoil with having to modify their lifestyles, relationships with family and friends, and challenges with educational and career opportunities. Various themes of their hopes for the future touched on having access to treatment, clinical trials, holistic care for symptom management, as well as improving infrastructure for disability access. Conclusion This study, to the best of our knowledge represents the first comprehensive study on SMA in South East Asia, highlights a plethora of issues and challenges experienced by persons with spinal muscular atrophy (PWSMA) and their caregivers in Malaysia, from the point of SMA diagnosis and throughout the management of care, in addition to the deep psychosocial impact of living with SMA. The significant findings of this study may contribute to a better understanding among stakeholders to make improvements in clinical practice, the education system, the work environment as well as holistic care support and society at large.

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Section: Discussionmentioning

confidence: 99%

A mixed method study on the impact of living with spinal muscular atrophy in Malaysia from patients’ and caregivers’ perspectives

Ch’ng

Koh

Ahmad‐Annuar

et al. 2022

Orphanet J Rare Dis

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“…Full texts were retrieved if title and abstract were of interest, or the eligibility was unclear. Studies, which seemed to meet the inclusion criteria at first, were mostly excluded because of missing psychosocial outcomes for parents (e.g., [ 23 ]), because the sample included only few cases of SMA (e.g., [ 24 ]), or because a large fraction of parents was deceased (e.g., [ 25 ]). For details of the study selection process, see Fig.…”

Section: Methodsmentioning

confidence: 99%

Parents as informal caregivers of children and adolescents with spinal muscular atrophy: a systematic review of quantitative and qualitative data on the psychosocial situation, caregiver burden, and family needs

Brandt

Johannsen

Inhestern

et al. 2022

Orphanet J Rare Dis

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Background Spinal muscular atrophy (SMA) is a rare degenerative neuromuscular disease, mostly occurring in infants and children, leading to muscle wasting and weakness, and premature death. Due to new developments of multiple disease-modifying treatments within the last years, the interest of research in patients affected by SMA increased steadily. However, the psychosocial situation of parents as informal caregivers is still rarely addressed. Objectives This review aims to highlight quantitative and qualitative data about the psychosocial situation, caregiver burden, and needs of parents as informal caregivers for children and adolescents with SMA. Methods A systematic literature review was performed including quantitative and qualitative original studies focusing on different psychosocial aspects and outcomes for parents of children and adolescents < 21 years of age with SMA type I–IV (PROSPERO; registration number CRD42020219020). We searched the following databases in November 2020 with a research update in August 2021: MEDLINE, CINAHL, PsycINFO and Web of Science. Results In total, 24 articles from 23 studies were selected for inclusion (15 quantitative studies, 7 articles from 6 qualitative studies, 2 mixed methods studies). The synthesis of included studies shows multiple sources of psychosocial burden for parents of children and adolescents affected by SMA: Most studies found reduced levels of quality of life, moderate to high levels of caregiver burden and distress, as well as physical and mental health symptoms. Further, findings indicate several unmet family needs regarding information, care coordination, treatment decisions, financial support, and adequate supportive care services. Conclusion Parents of children and adolescents with SMA face multiple sources of psychosocial stressors, caregiver burden and various unmet family needs. To unburden families, the needs of parents as caregivers should be included in integrated care paths for SMA to improve their psychosocial situation and thus their ability to care for their children and to treat or prevent physical and mental health problems due to overburdening. Future research should focus not only on quality of life and on caregiving-related burden but should also examine the clinical relevance of reported symptoms to support the implementation of adequate support services for families affected by SMA.

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“…As many professionals are involved in the management of those children, parents as caregivers play a major role in transmitting information, ensuring a continuum of care as no home hospitalization settings enables constant nurse or medical staff presence. This empowerment of parents in their child's care has been claimed in recent studies (24,25,28,29) and seems a key point to ensure the best care for the child in real life. In our study, not only did parents report as "Clinical Research Assistant" their child's symptoms and treatments in the HB, but they also spontaneously evaluated the treatments and recommendations made by care providers, and most of all they made propositions on everyday management of a child with SMA-1 (plays, installation, and feeding), enlightening that in addition to being a caregiver, they take care of their child as every parent does.…”

Section: Discussionmentioning

confidence: 86%

“…The implementation of specific pediatric palliative care in the context of SMA-1 patients needs active collaboration and coordination between the different actors involved to ensure the child's and family's best quality of life. This need for coordination has recently been supported in qualitative studies (25,26), as well as the importance of parents' input about their wishes for their child's treatments and end-of-life conditions (22,24,25,(27)(28)(29).…”

Section: Discussionmentioning

confidence: 99%

Palliative Care in SMA Type 1: A Prospective Multicenter French Study Based on Parents' Reports

Hully

Barnérias²,

Chabalier³

et al. 2020

Front. Pediatr.

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Identifying Opportunities to Provide Family-centered Care for Families With Children With Type 1 Spinal Muscular Atrophy

Cited by 19 publications

References 38 publications

A mixed method study on the impact of living with spinal muscular atrophy in Malaysia from patients’ and caregivers’ perspectives

A mixed method study on the impact of living with spinal muscular atrophy in Malaysia from patients’ and caregivers’ perspectives

Parents as informal caregivers of children and adolescents with spinal muscular atrophy: a systematic review of quantitative and qualitative data on the psychosocial situation, caregiver burden, and family needs

Palliative Care in SMA Type 1: A Prospective Multicenter French Study Based on Parents' Reports

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