Parent Experiences with the Process of Sharing Inpatient Safety Concerns for Children with Medical Complexity: A Qualitative Analysis.

OBJECTIVE: To describe the process of identifying and reporting inpatient safety concerns from the perspective of parents of children with medical complexity (CMC). METHODS: We conducted a secondary analysis of qualitative data from semi-structured interviews with 31 English and Spanish-speaking parents of CMC at two tertiary children's hospitals. Interviews lasted 45-60 minutes and were audio-recorded, translated, and transcribed. Three researchers inductively and deductively coded transcripts using an iteratively refined codebook with validation by a fourth researcher. Thematic analysis was used to develop a conceptual model of the process of inpatient parent safety reporting. RESULTS: We identified four steps illustrating the process of inpatient parent safety concern reporting 1) parent recognizing concern, 2) parent reporting concern, 3) staff/hospital response continuum, and 4) parent feelings of validation/invalidation. Many parents endorsed that they were the first to catch a safety concern and were identified as unique reporters of safety information. Parents typically described reporting their concerns verbally and in real-time to the person they felt could quickly remedy the situation. There was a spectrum of validation. Some parents reported their concerns were not acknowledged and addressed, which led them to feel overlooked, disregarded, or judged. Others reported their concerns were acknowledged and addressed, resulting in parents feeling heard and seen and often leading to changes in clinical care. CONCLUSIONS: Parents described a multi-step process of reporting safety concerns during hospitalization and a spectrum of staff response and validation. These findings can inform family-centered interventions that support safety concern reporting in the inpatient setting.

Family Safety Reporting in Medically Complex Children: Parent, Staff, and Leader Perspectives.

BACKGROUND AND OBJECTIVES: Despite compelling evidence that patients and families report valid and unique safety information, particularly for children with medical complexity (CMC), hospitals typically do not proactively solicit patient or family concerns about patient safety. We sought to understand parent, staff, and hospital leader perspectives about family safety reporting in CMC to inform future interventions. METHODS: This qualitative study was conducted at 2 tertiary care children's hospitals with dedicated inpatient complex care services. A research team conducted approximately 60-minute semistructured, individual interviews with English and Spanish-speaking parents of CMC, physicians, nurses, and hospital leaders. Audio-recorded interviews were translated, transcribed, and verified. Two researchers coded data inductively and deductively developed and iteratively refined the codebook with validation by a third researcher. Thematic analysis allowed for identification of emerging themes. RESULTS: We interviewed 80 participants (34 parents, 19 nurses and allied health professionals, 11 physicians, and 16 hospital leaders). Four themes related to family safety reporting were identified: (1) unclear, nontransparent, and variable existing processes, (2) a continuum of staff and leadership buy-in, (3) a family decision-making calculus about whether to report, and (4) misaligned staff and parent priorities and expectations. We also identified potential strategies for engaging families and staff in family reporting. CONCLUSIONS: Although parents were deemed experts about their children, buy-in about the value of family safety reporting among staff and leaders varied, staff and parent priorities and expectations were misaligned, and family decision-making around reporting was complex. Strategies to address these areas can inform design of family safety reporting interventions attuned to all stakeholder groups.

Three-generation family with novel contiguous gene deletion on chromosome 2p22 associated with thoracic aortic aneurysm syndrome.

Latent transforming growth factor binding proteins (LTBP) are a family of extracellular matrix glycoproteins that play an important role in the regulation of transforming growth factor beta (TGF-ß) activation. Dysregulation of the TGF-ß pathway has been implicated in the pathogenesis of inherited disorders predisposing to thoracic aortic aneurysms syndromes (TAAS) including Marfan syndrome (MFS; FBN1) and Loeys-Dietz syndrome (LDS; TGFBR1, TGFBR2, TGFB2, TGFB3, SMAD2, SMAD3). While these syndromes have distinct clinical criteria, they share clinical features including aortic root dilation and musculoskeletal findings. LTBP1 is a component of the TGF-ß pathway that binds to fibrillin-1 in the extracellular matrix rendering TGF-ß inactive. We describe a three-generation family case series with a heterozygous ∼5.1 Mb novel contiguous gene deletion of chromosome 2p22.3-p22.2 involving 11 genes, including LTBP1. The deletion has been identified in the proband, father and grandfather, who all have a phenotype consistent with a TAAS. Findings include thoracic aortic dilation, ptosis, malar hypoplasia, high arched palate, retrognathia, pes planus, hindfoot deformity, obstructive sleep apnea, and low truncal tone during childhood with joint laxity that progressed to reduced joint mobility over time. While the three affected individuals did not meet criteria for either MFS or LDS, they shared features of both. Although the deletion includes 11 genes, given the relationship between LTBP1, TGF-ß, and fibrillin-1, LTBP1 stands out as one of the possible candidate genes for the clinical syndrome observed in this family. More studies are necessary to evaluate the potential role of LTBP1 in the pathophysiology of TAAS.