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

STUDY PURPOSE: The purpose of this qualitative study was to understand, from the parent perspective, the experience of the family whose child has Type 1 spinal muscular atrophy (Type 1 SMA), in the emergency center, hospital, and clinical care settings to identify opportunities for improved family-centered care (FCC). DESIGN AND METHODS: This study used a qualitative descriptive design with individual or small group interviews guided by a semi-structured questionnaire. Reviewers used framework analysis to identify gaps in the provision of FCC and opportunities for improvement with respect to services health professionals may provide families of children with Type 1 SMA. RESULTS: Nineteen families with 22 children with Type 1 SMA participated. Results are organized according to eight basic tenets of FCC. Family-to-family interactions strongly impacted participants' decision-making and perceived level of support. Participants valued strong family/provider partnerships, feeling heard and respected by their providers, and receiving complete education regarding disease trajectory. CONCLUSIONS: Our analyses revealed both successful application of FCC and gaps in care where FCC could have been used to benefit families who have children with Type 1 SMA. As a pediatric chronic illness affects the whole family, FCC is important in maintaining the providers' focus on the family during the child's care. PRACTICE IMPLICATIONS: There are opportunities for nursing, social work, care managers and others to engage as care coordinators to explain the family's goals and values to the medical team. Care coordinators help ensure understanding between families and providers, empowering the family to articulate their hopes and concerns.

Provider Perceptions for Withdrawing Life Sustaining Therapies at a Large Pediatric Hospital.

CONTEXT: More than 74% of pediatric deaths occur in an intensive care unit (ICU), with 40% occurring after withdrawal of life-sustaining therapies (WOLST). No needs assessment has described provider needs or suggestions for improving the WOLST process in pediatrics. OBJECTIVES: This study aims to describe interdisciplinary provider self-reported confidence, needs, and suggestions for improving the WOLST process. METHODS: A convergent parallel mixed-methods design was used. An online survey was distributed to providers involved in WOLSTs in a quaternary children's hospital between January and December 2018. The survey assessed providers' self-reported confidence in their role, in providing guidance to families about the WOLST, experiences with the WOLST process, areas for improvement, and symptom management. Kruskal-Wallis testing was used for quantitative data analysis with P values <0.05 considered significant. Analysis was performed with SPSS v27. Qualitative data were thematically analyzed using Atlas.ti.8 and NVivo. RESULTS: A total of 297 surveys were received (48% survey completion) that consisted of multiple choice, Likert-type, and yes/no questions with options for open-ended responses. Mean provider self-rated confidence was high and varied significantly between disciplines. Qualitative analysis identified four areas for refining communication: 1) between the primary team and family, 2) within the primary team, 3) between the primary team and consulting providers, and 4) logistical challenges. CONCLUSIONS: While participants' self-rated confidence was high, it varied between disciplines. Participants identified opportunities for improved communication and planning before a WOLST. Future work includes development and implementation of a best practice guideline to address gaps and standardize care delivery.

The Experience of Families With Children With Spinal Muscular Atrophy Type I Across Health Care Systems.

Spinal muscular atrophy type I is a genetic disease characterized by degeneration of spinal cord motor neurons resulting in weakness, technology dependence and early demise. While the newly approved treatment nusinersen may alter the morbidity/mortality of this disease there continues to be complex treatment challenges to consider. The aim of this qualitative study was to understand from the parent's perspective, experiences of the family and child in the emergency center, hospital, and clinical care settings to identify gaps in care. Nineteen families interviewed had 22 children with spinal muscular atrophy I (11 deceased, 11 living). Three overarching themes emerged from parent interviews describing a range of experiences surrounding diagnosis, informed medical decision making and acute care practice. Identified quality improvements include development of a diagnostic screening tool, a medical decision tool, and emergency center informational template individualized to the child and providing an overview of spinal muscular atrophy I.