Family-Centered Advance Care Planning: What Matters Most for Parents of Children with Rare Diseases.

Few studies have described the goals and wishes of parents caring for their children with rare diseases, specifically when children are unable to communicate their preferences directly. The purpose of this study was to describe the parent's understanding of their child's illness, goals of care, and what mattered most to their child from the parent's perspective. Six families completed a feasibility study of the FAmily CEntered (FACE)-Rare pACP intervention. Qualitative content analysis was performed on transcripts of videotaped responses to the Respecting Choices Next Steps pACP Conversation facilitated conversation guide about the goals of care. Codes were grouped into themes, with direct participant quotations representing the themes. Five themes emerged: getting out and moving freely; feeling included and engaged; managing symptoms and disease burden; coordinating care among many care team members; and managing today and planning for the future. In the context of pACP, families reported that what mattered most to their children included the freedom of movement and human connection and engagement, while parents strived to be effective caregivers and advocates for their child with a rare and severely disabling disease.

Family caregivers of children and adolescents with rare diseases: a novel palliative care intervention.

OBJECTIVE: To develop and pilot test a palliative care intervention for family caregivers of children with rare diseases (FAmily-CEntered pediatric Advance Care Planning-Rare (FACE-Rare)). METHODS: FACE-Rare development involved an iterative, family-guided process including review by a Patient and Family Advisory Council, semistructured family interviews and adaptation of two evidence-based person-centred approaches and pilot testing their integration. Eligible families were enrolled in FACE-Rare (the Carer Support Needs Assessment Tool (CSNAT) Approach Paediatric sessions 1 and 2; plus Respecting Choices Next Steps pACP intervention sessions 3 and 4). Satisfaction, quality of communication and caregiver appraisal were assessed. RESULTS: Parents were mean age 40 years, and children 7 years. Children's diseases were rare enough that description would identify patients. All children were technology dependent. Telemedicine, used with four of seven families, was an effective engagement strategy and decreased subject burden. Families found FACE-Rare valuable following a strategy that first elicited palliative care needs and a support plan. Eight families were approached for pilot testing. Of the seven mothers who agreed to participate, six began session 1, and of those, 100% completed: all four FACE-Rare sessions, baseline and 2-week postintervention assessments, and a written pACP which described their preferences for medical decision-making to share with their providers. 100% reported FACE-Rare was helpful. The top three CSNAT concerns were: knowing what to expect in the future, having enough time for yourself and financial issues. Benchmarks were achieved and questionnaires were acceptable to parents and thus feasible to use in a larger trial. CONCLUSIONS: FACE-Rare provides an innovative, structured approach for clinicians to deliver person-centred care.

"Can you tell me why you made that choice?": A qualitative study of the influences on treatment decisions in advance care planning among adolescents and young adults undergoing bone marrow transplant.

BACKGROUND: Adolescent and young adult advance care planning is beneficial in improving communication between patients, surrogates, and clinicians. The influences on treatment decisions among adolescents and young adults are underexplored in the literature. AIM: The aim of this study was to explore and better understand the influences on decision-making for adolescent and young adult bone marrow transplant patients about future medical care. DESIGN: Clinical case studies and qualitative inductive content analysis of treatment decisions made during the Respecting Choices® Next Steps Pediatric Advance Care Planning conversation as a component of the Family-Centered Advance Care Planning Intervention. SETTINGS/PARTICIPANTS: A total of 10 adolescent and young adult patients (aged 14-27 years) undergoing bone marrow transplant at an academic Midwest children's hospital were involved in the study. RESULTS: Influences on participants' decisions were consideration for family, quality of life, and awareness of self. Desire to avoid suffering and maintain an acceptable quality of life was often in competition with participant's concern over the perceived negative impact of discontinuing treatment on their families. CONCLUSION: This study highlights that adolescent and young adult bone marrow transplant patients are capable of meaningful deliberation about future treatment decisions. Influences on decision-making should be incorporated into advance care planning conversations to facilitate communication between patients and their surrogates. Longitudinal research is needed to explore these influences throughout the trajectory of illness.

Quasi-Experimental Evaluation of LifeCourse on Utilization and Patient and Caregiver Quality of Life and Experience.

Whole-person care is a new paradigm for serious illness, but few programs have been robustly studied. We sought to test the effect of LifeCourse (LC), a person-centered program for patients living with serious illness, on health-care utilization, care experience, and quality of life, employing a quasi-experimental design with a Usual Care (UC) comparison group. The study was conducted 2012 to 2017 at an upper-Midwest not-for-profit health-care system with outcomes measured every 3 months until the end of life. Enrolled patients (N = 903) were estimated to be within 3 years of end of life and diagnosed with 1+ serious illness. Exclusion criteria included hospice enrollment at time of screening or active dying. Community health workers (CHWs) delivered standardized monthly 1-hour home visits based on palliative care guidelines and motivational interviewing to promote patients' physical, psychosocial, and financial well-being. Primary outcomes included health-care utilization and patient- and caregiver-experience and quality of life. Patients were elderly (LC 74, UC 78 years) and primarily non-Hispanic, white, living at home with cardiovascular disease as the primary diagnosis (LC 69%, UC 57%). A higher proportion of LC patients completed advance directives (N = 173, 38%) than UC patients (N = 66, 15%; P < .001). LifeCourse patients who died spent more days in hospice (88 ± 191 days) compared to UC patients (44 ± 71 days; P = .018). LifeCourse patients reported greater improvements than UC in communication as part of the care experience ( P = .016). Implementation of person-centered programs delivered by CHWs is feasible; inexpensive upstream expansion of palliative care models can yield benefits for patients and caregivers. Trial Registration: Trial NCT01746446 was registered on November 27, 2012 at ClinicalTrials.gov .

Expanding the Palliative Care Domains to Meet the Needs of a Community-Based Supportive Care Model.

BACKGROUND: Whole person care is appropriate for seriously ill persons. The current framework of palliative care domains in the National Consensus Project (NCP) Guidelines for Quality Palliative Care offers an opportunity to reassess the domains of care delivered at home, earlier in the course of illness. OBJECTIVE: This qualitative study was used to test the applicability of a proposed, expanded set of domains. The results were used to inform a home-based, upstream model of supportive care for serious illness. METHODS: Quotes relating to the experience of late-life serious illness were derived from transcripts of 12 semi-structured group interviews conducted with patients, family, and professionals. Quotes originally coded to the NCP domains of palliative care were then coded to the proposed domain set, which included new categories of family/caregiver, legal/financial, and legacy/bereavement domains. RESULTS: A total of 489 quotes were assigned to the proposed expanded set of domains. One hundred one (19%) coded to the family/caregiver domain, 28 (5%) to the legacy/bereavement domain, and 27 (5%) to the legal/financial domain. Ninety-seven (87%) of the 111 quotes coded to family/caregiver had been initially coded to the NCP social aspects of care. Family/caregiver themes included challenges, rewards, insights, and family growth. CONCLUSION: The preponderance of family-related issues suggests that including the family domain may promote recognition and support of family caregivers and the services they provide. Although this study provides some support for including the legacy/bereavement and legal/financial domains, additional research is needed to determine whether there is a basis for including them in the domain structure.

Combining Best Practices and Patient, Caregiver, and Healthcare Provider Perspectives for Late-Life Supportive Care: LifeCourse.

Healthcare systems seek effective ways to support and treat the growing number of individuals living with serious illness. The nature of these care episodes challenges delivery systems to attain proficiency in dealing with the multiplicity of chronic conditions in individuals and populations through understanding and attending to patients' medical and nonmedical aspects of health. This article describes LifeCourse, a healthcare approach that provides palliative care practices to patients with serious illness years prior to death.