Parent Perspectives on the Child Experience of Pediatric Home Ventilation via Tracheostomy.

BACKGROUND AND OBJECTIVE: Parents facing the decision of whether to initiate pediatric mechanical ventilation via tracheostomy ("home ventilation") report wanting information about what to expect for life at home for their child. The study objective is to explore parent descriptions of the child experience of home ventilation to increase awareness for clinicians in the inpatient setting caring for these children. METHODS: Semistructured interviews were conducted using purposive sampling of parents with children who initiated home ventilation within the previous 5 years from 3 geographically diverse academic medical centers. RESULTS: We interviewed 21 families from 3 geographic regions in the United States. About 75% of children had respiratory failure in the first year of life, 80% had medical complexity, and half had severe neurologic impairment. Five domains emerged regarding parent perceptions of their child's experience of home ventilation: (1) health and well-being; (2) development; (3) adaptation; (4) mobility and travel; and (5) relationships. Within each domain, several themes were identified. For each theme, there was a positive and negative subtheme to illustrate how the child's experience was modified by home ventilation. Parent descriptions were generally positive, however, in all domains and to varying degrees, parents expressed negative aspects of home ventilation. CONCLUSIONS: By providing information about the realistic experiences of children using home ventilation, clinicians can support families in the inpatient setting as they face this complex decision. Balanced information about home ventilation is critical to enhancing clinician counseling so that families may benefit from the perspectives of experienced parents.

Family-Reflections.com: Creating a parent-to-parent web-based tool regarding pediatric home ventilation.

BACKGROUND: The decision to initiate pediatric mechanical ventilation via tracheostomy ("home ventilation") is complex and parents often desire information from other parents who have faced this decision. However, parent-to-parent communication is challenging as it is difficult to connect new families to experienced families in ways that optimize informed, balanced decision-making. OBJECTIVE: Create a parent-to-parent web-based tool to support decision-making about pediatric home ventilation. PATIENT INVOLVEMENT: The tool was created based on interviews and feedback from parents. METHODS: We interviewed parents who previously chose for, or against, home ventilation for their child. Interview themes and family comments guided website development. Viewer feedback was solicited via an embedded survey in the tool. RESULTS: We created 6 composite character families to communicate 6 themes about home ventilation: 1) Considering treatment options, 2) Talking with medical team, 3) Impact on life at home, 4) Impact on relationships, 5) Experience for the child, and 6) If the child's life is short. Nine families who reviewed the draft tool felt it would have helped with their decision about home ventilation. Specifically, it supported families in thinking through what was "most important about their child's breathing problems" (7 of 9 parents) and feeling "more at peace with the decision" (8 of 9 parents). Between 6/1/20-12/31/22, nearly 5500 viewers have accessed the tool and 56 viewers completed the survey (including 13 families and 39 clinicians). Feedback from experienced families and clinicians reported the tool taught them something new. DISCUSSION: This novel parent-to-parent tool shows promise for expanding access to balanced, family-centered information about pediatric home ventilation. PRACTICAL VALUE: The diverse stories and decisions let parents access multiple family perspectives. The tool's focus is on family-centric information that parents reported was usually missing from clinician counseling. FUNDING: This work was supported by the National Palliative Care Research Center.

Home values and experiences navigation track (HomeVENT): Supporting decisions about pediatric home ventilation.

Objective: To pilot feasibility and acceptability of HomeVENT, a systematic approach to family-clinician decision-making about pediatric home ventilation. Methods: Parents and clinicians of children facing home ventilation decisions were enrolled at 3 centers using a pre/post cohort design. Family interventions included: 1) a website describing the experiences of families who previously chose for and against home ventilation 2) a Question Prompt List (QPL); 3) in-depth interviews exploring home life and values. Clinician HomeVENT intervention included a structured team meeting reviewing treatment options in light of the family's home life and values. All participants were interviewed one month after the decision. Results: We enrolled 30 families and 34 clinicians. Most Usual Care (14/15) but fewer Intervention (10/15) families elected for home ventilation. Families reported the website helped them consider different treatment options, the QPL promoted discussion within the family and with the team, and the interview helped them realize how home ventilation might change their daily life. Clinicians reported the team meeting helped clarify prognosis and prioritize treatment options. Conclusions: The HomeVENT pilot was feasible and acceptable. Innovation: This systematic approach to pediatric home ventilation decisions prioritizes family values and is a novel method to increase the rigor of shared decision-making in a rushed clinical environment.

"What Would Give Her the Best Life?": Understanding Why Families Decline Pediatric Home Ventilation.

Background: Families who must decide about pediatric home ventilation rely on the clinicians who counsel them for guidance. Most studies about pediatric home ventilation decisions focus on families who opt for this intervention, leaving much unknown about the families who decline. Objective: To describe the rationales of families who decline home ventilation. Design: Semi-structured interview study. Setting/Subjects: We interviewed 16 families in hospitals across 3 U.S. states, identified by their clinicians as previously deciding to not pursue home ventilation via tracheostomy within the past five years. Measurements: Targeted content and narrative analyses were used to understand family intentions and reasons for declining. Results: The clinical and social context varied among the 16 families in this study. Families' intentions in saying "no" fell into two categories: (1) definitive "No": Families who stood firm on in their decision and (2) contingent "No": Families who may consider this in the future. Families described four reasons why their child did not receive home ventilation: (1) concern about medical impacts, (2) concern about physical and/or communication restrictions, (3) concern that there would be no clear health benefit, and (4) concern about no clear meaningful life. Most families mentioned all four reasons, but concern about no clear meaningful life predominated. Conclusions: Though these families did not see home ventilation as an appropriate option, each reported a complex interplay of intentions behind and reasons for declining. Clinicians who counsel families about home ventilation could share the reasons that families commonly decline this intervention to facilitate a balanced discussion.

Home mechanical ventilation for children with severe neurological impairment: Parents' perspectives on clinician counselling.

AIM: To retrospectively explore the perspectives of parents of children with severe neurological impairment (SNI), such as those with severe cerebral palsy, epilepsy syndromes, and structural brain differences, on clinician counseling regarding home mechanical ventilation (HMV). METHOD: Inductive thematic analysis was performed on data from telephone interviews with parents who chose for and against HMV for their child with SNI at three academic children's hospitals across the USA. RESULTS: Twenty-six parents/legal guardians of 24 children were interviewed. Fourteen children had static encephalopathy, 11 received HMV, and 20 were alive at the time of parent interviews. Themes included how HMV related to the child's prognosis, risk of death, and integration with goals of care. Although clinicians voiced uncertainty about how HMV would impact their child, parents felt this was coupled with prescriptive/intimidating examples about the child's end of life and judgments about the child's quality of life. INTERPRETATION: While prognositc uncertainty exists, this study suggests that parents of children with SNI seek clinician counseling about HMV that considers their goals of care and views on their child's quality of life.

Parent perspectives on facilitating decision-making around pediatric home ventilation.

RATIONALE: Deciding about pediatric home ventilation is exceptionally challenging for parents. Understanding the decision-making needs of parents who made different choices for their children could inform clinician counseling that better supports parents' diverse values and goals. OBJECTIVES: To determine how clinicians can meet the decisional needs of parents considering home ventilation using a balanced sample of families who chose for or against intervention. METHODS: We conducted semi-structured interviews of parents who chose for or against home ventilation for their child within the previous 5 years. Parents were recruited from three academic centers across the United States. Interviews focused on parent-clinician communication during decision-making and how clinicians made the process easier or more difficult. Qualitative analysis was used to generate themes and identify key results. RESULTS: Thirty-eight parents were interviewed; 20 chose for and 18 chose against home ventilation. Five themes described their perspectives on how clinicians can facilitate high-quality decision-making: demonstrating dedication to families, effectively managing the medical team, introducing the concept of home ventilation with intention, facilitating meaningful conversation about the treatment options, and supporting and respecting the family's decision. CONCLUSIONS: High-quality decision-making around home ventilation depends on individual clinician actions and the complex operations of large academic settings. Strong working relationships with parents, collaborative alliances with colleagues, and appropriate delivery of key content can help meet the needs of parents considering invasive breathing supports for their children.

Life with pediatric home ventilation: Expectations versus experience.

OBJECTIVES: To explore the family experience of home ventilation through a comparison of anticipated home life changes with subsequent experiences. STUDY DESIGN: Guided interviews with parents across three states who chose home ventilation for their child within the last 5 years. PATIENT-SUBJECT SELECTION: Purposive sampling of parents who chose home ventilation for their child within the last 5 years. METHODS: Interviews were transcribed for qualitative analysis and analyzed for thematic saturation and prevalence of codes. RESULTS: Twenty families were interviewed. Families generally reported not considering potential home life changes when facing the decision about home ventilation; instead, they worried most about medical management. These concerns reversed in importance later. Families learned medical management quickly but felt largely unprepared for the extensive changes to their home life, including isolation, altered relationships with extended family and community, effects on siblings, financial strain, and need for physical changes to their house. Families had not anticipated how much they would be affected by home healthcare as a new part of their life. CONCLUSIONS: The priorities that families consider during decisions about pediatric home ventilation may not be aligned with the actual home experience of this technology. Given that the success of home ventilation largely rests with the family's care, family expectations for home life adaptations must be augmented, as should postdischarge supports for families with complex home care experiences.

Family Experiences Deciding For and Against Pediatric Home Ventilation.

OBJECTIVE: To understand what considerations drive family decisions for, and against, pediatric home ventilation. STUDY DESIGN: Qualitative interviews with parents of children who faced a decision about home ventilation in the previous 5 years at 3 geographically dispersed institutions. RESULTS: In total, 38 families (42 parents) were interviewed; 20 families opted for pediatric home ventilation, and 18 families opted against. Approximately one-quarter of children had isolated chronic lung disease; the remainder had medical complexity that was expected to remain static or decline. Parent perspectives about home ventilation generally reflected whether the child was early, or later, in their disease trajectory. Early on, parents often interpreted prognostic uncertainty as hope and saw home ventilation as a tool permitting time for improvement. For families of children later in their disease course, often already with home technology and home nursing, home ventilation held less possibility for meaningful improvement. Nearly all families experienced the decision as very emotionally distressing. Reflecting back, most families described feeling satisfied with whatever decision they made. CONCLUSIONS: The 2 principal groups of families in our cohort-those with children whose respiratory insufficiency might improve, and those with children facing chronic decline-warrant targeted counseling approaches about initiating home ventilation. The distressing nature of this decision should be anticipated and family supports reinforced.

Material Hardship and Indoor Allergen Exposure among Low-Income, Urban, Minority Children with Persistent Asthma.

Traditional measures of socioeconomic status (SES) are associated with asthma morbidity, but their specific contributions are unclear. Increased exposure to indoor allergens among low SES children is an important consideration. Material hardship, a concept describing poor access to basic goods and services, may explain the relationship between low SES and indoor allergen exposure, and thereby, the increased risk of asthma morbidity. We sought to (i) describe the specific hardships experienced by low-Income, urban, minority children with asthma and indoor allergen sensitization and (ii) determine if material hardship is associated with indoor allergen exposure in this population. We conducted a cross-sectional analysis of children undergoing the baseline assessment for a clinical trial of home environmental modification. Participants were scored in five domains of material hardship. Domain scores were assigned based on caregiver responses to a questionnaire and were summed to generate a total material hardship score. Linear regression was used to examine the relationship between material hardship scores and bedroom floor concentrations of five common indoor allergens. Participants experienced high levels of material hardship in each of the five domains, with 33% not having access to a car, 35% not being able to pay utility bills, and 28% not being able to pay rent in the past year. Each one-point increase in material hardship was associated with an increase in cockroach allergen of 16.2% (95% CI 9.4%, 24.6%) and an increase in mouse allergen of 9.4% (95% CI 1.0%, 18.5%). After adjusting for traditional measures of SES, including household income, health insurance type, caregiver education, and caregiver employment status, the association between material hardship and cockroach allergen, but not mouse allergen, remained. These data suggest that a significant proportion of families of low-income, minority children with asthma may experience material hardship, and that they may be at greater risk of cockroach allergen exposure than their peers with similar income, but without material hardship.

Gaucher disease: chemotactic factors and immunological cell invasion in a mouse model.

Gaucher disease results from mutations in GBA1 that cause functional disruption of the encoded lysosomal enzyme, acid ß-glucosidase. The consequent excess accumulation of glucosylceramide and glucosylsphingosine in lysosomes is central to the disease pathogenesis with classical involvement of macrophage (Mфs) lineage cells of visceral organs, bone, or brain. Several studies have implicated the increased secretion of chemokines and infiltration of a variety of immunological cells into tissues of Gaucher disease patients. Trafficking of immunological cells to the sites of inflammation requires the presence of chemokines. Although increases of different immunological cells and several chemokines are present in Gaucher disease, the specific chemoattractants that cause the increased influx of immunological cells are not fully defined. Here, increased levels of I-309, MCP-5, CXCL-2, CXCL-9, CXCL-10, CXCL-11, CXCL-13, and their corresponding leukocytes, i.e., MOs (monocytes), Mфs, dendritic cells (DCs), polymorphonuclear neutrophils (PMNs), and T, and B cells were identified in the circulation of mice with Gba1 mutations (D409V/null). Sera from D409V/null mice contained chemoattractants for a variety of immunological cells as shown by ex vivo chemotaxis studies and by flow cytometry. Enhanced chemotaxis towards 9V/null sera was found for 9V/null lung-, spleen-, liver-, and bone marrow-derived Mфs (CD11b(+) F480(+)), PMNs (Gr1(high) CD11b(+)), DCs (CD11c(+) CD11b(+)), T lymphocytes (CD3(+) TCRB(+)), and B lymphocytes (B220(+) CD19(+)). These data support these chemotactic factors as causative to increased tissue infiltration of leukocytes in Gaucher disease.