Adaptive Care for Perioperative Patients With Developmental Disabilities: An Exploration of Interventions and Family Experience.

INTRODUCTION: Patients with developmental disabilities commonly experience psychological distress during health care visits. There is limited research describing which individualized interventions are being implemented to promote optimal care in the perioperative area. METHOD: In this prospective observational study of 60 patients with developmental diagnoses, aged 3-21 years, we recorded adaptive care plan (ACP) interventions and assessed family experience. RESULTS: Patients receiving ACPs had diverse diagnoses, including autism spectrum and 10 other unique syndromes. Most patients received previsit planning (90%), adaptations to standard protocols (60%), child life specialist preparation (67%), procedural support (90%), and were given fast-acting anxiolytics before anesthesia induction (68%). Families reported that ACPs were important for managing a child's stress (94%) and promoting safety (92%). DISCUSSION: ACPs promote safe, productive health care encounters for patients with various diagnoses, ages, and coping abilities. Families find ACPs important for stress management and promoting safety.

Optimizing Pediatric Induction Experiences Using Human-centered Design.

PURPOSE: Anesthesia inhalation induction (falling asleep for a surgery using a medical mask) is often stressful for children. When children become anxious about induction, they may resist wearing the anesthesia mask. High anxiety during induction is associated with poorer outcomes after surgery, such as increased emergence delirium, increased pain and negative behavioral changes after discharge. The purpose of this project was to design an optimal anesthesia induction experience for children, with a focus on decreasing patient anxiety during inhalation induction. DESIGN: Designing for the perioperative environment requires in-depth knowledge of existing processes, needs of key stakeholders, strengths/shortcomings of existing approaches, and iterative testing of design concepts. We used a human centered design model (design research) to approach this challenge. METHODS: Hospital staff partnered with a design team at the Live Well Collaborative. Families and staff were consulted during all phases of the design process. The iterative design research process (research, ideation, refinement) was used to develop product and process concepts for optimizing the induction experience. Requirements for an optimal induction experience were defined during the research phase through reviewing the scientific literature, process mapping and benchmarking with other products. Product and process concepts were developed during the ideation phase. Concepts were tested and refined during the refinement phase. FINDINGS: The research phase provided insights about the current anesthesia induction process and what interventions help engage children in medical care. Key insights included normalizing the anesthesia mask through medical play, providing patients with an increased sense of control preoperatively and during induction, and engaging multiple senses during the process. The ideation phase led to the development of several design concepts, including an app that is activated by breathing into the anesthesia mask. During the refinement phase, concepts were shared and refined with input from families and clinical staff. This phase led to the development of a novel zoo-themed gaming app. CONCLUSIONS: The design research process facilitated the creation of a new anxiety reduction tool for the perioperative environment. The breath-controlled induction app simultaneously employs multiple evidence-based anxiety reduction techniques and is designed to seamlessly integrate into the fast-paced perioperative workflow during key stress points. Testing in the clinical setting is needed to determine the effectiveness of the app for preoperative anxiety reduction.

Validation of a Simple Tool for Electronic Documentation of Behavioral Responses to Anesthesia Induction.

BACKGROUND: Anxiety and distress behaviors during anesthesia induction are associated with negative postoperative outcomes for pediatric patients. Documenting behavioral responses to induction is useful to evaluate induction quality at hospitals and to optimize future anesthetics for returning patients, but we lack a simple tool for clinical documentation. The Induction Compliance Checklist is a tool for grading induction behaviors that is well validated for research purposes, but it is not practical for routine documentation in busy clinical practice settings. The Child Induction Behavioral Assessment tool was developed to provide a simple and easy to use electronic tool for clinical documentation of induction behaviors. The aim of this study was to test the Child Induction Behavioral Assessment tool's concurrent validity with the Induction Compliance Checklist and the interrater reliability. METHODS: This prospective, observational study included 384 pediatric patients undergoing anesthesia inhalation induction. Concurrent validity with the Induction Compliance Checklist and interrater reliability of the Child Induction Behavioral Assessment were evaluated. Two researchers alternated scoring the Induction Compliance Checklist. The 2 researchers independently scored the Child Induction Behavioral Assessment. The anesthesia clinician caring for the patient also independently scored the Child Induction Behavioral Assessment by completing their routine documentation in the patient's medical record. Two age groups were evaluated (ages 1-3 and 4-12 years old). RESULTS: Clinicians' and researchers' Child Induction Behavioral Assessment scores demonstrated a strong correlation with the Induction Compliance Checklist (P < .0001). There was an excellent agreement between the 2 researchers' Child Induction Behavioral Assessment scores for the younger and older age groups, respectively (Kappa [95% CI] = 0.97 (0.94-0.99); K = 0.94 (0.89-0.99)]. The agreement between the researchers and the 117 clinicians who documented Child Induction Behavioral Assessment assessments in the medical record was good overall (intraclass correlation coefficient = 0.70), with fair agreement with the 1- to 3-year-old patients (intraclass correlation coefficient = 0.56) and good agreement for the 4- to 12-year-old patients (intraclass correlation coefficient = 0.74). CONCLUSIONS: The Child Induction Behavioral Assessment scale is a simple and practical electronic tool used to document pediatric behavioral responses to anesthesia inductions. This study provides evidence of the tool's validity and reliability for inhalation inductions. Future research is needed at other hospitals to confirm validity.

The Child Induction Behavioral Assessment Tool: A Tool to Facilitate the Electronic Documentation of Behavioral Responses to Anesthesia Inductions.

PURPOSE: The purpose of this study was to develop and implement an electronic tool for documenting pediatric patients' behavioral responses to anesthesia induction. DESIGN: Quality improvement methodology was used in the tool development and implementation. METHODS: The Child Induction Behavioral Assessment (CIBA) tool was developed based upon existing validated tools and through discussions with content experts and key stakeholders. Staff usage of the tool was monitored and the clinical utility of the tool was assessed. FINDINGS: The CIBA tool facilitated frequent documentation of behavioral responses to inductions. The majority of clinicians reported that they found the prior CIBA ratings useful when developing induction plans for returning patients. CONCLUSIONS: Electronic documentation using the CIBA tool may provide useful information for optimizing induction plans for returning patients. Future research is needed to directly test the CIBA tool's validity.

Genomewide association analysis of respiratory syncytial virus infection in mice.

Respiratory syncytial virus (RSV) is the major cause of lower respiratory tract infection in infants, with about half being infected in their first year of life. Yet only 2 to 3% of infants are hospitalized for RSV infection, suggesting that individual susceptibility contributes to disease severity. Previously, we determined that AKR/J (susceptible) mice developed high lung RSV titers and showed delayed weight recovery, whereas C57BL/6J (resistant) mice demonstrated low lung RSV titers and rapid weight recovery. In addition, we have reported that gene-targeted mice lacking the cystic fibrosis transmembrane conductance regulator (Cftr; ATP-binding cassette subfamily C, member 7) are susceptible to RSV infection. For this report, recombinant backcross and F2 progeny derived from C57BL/6J and AKR/J mice were infected with RSV, their lung titers were measured, and quantitative trait locus (QTL) analysis was performed. A major QTL, designated Rsvs1, was identified on proximal mouse chromosome 6 in both recombinant populations. Microarray analysis comparing lung transcripts of the parental strains during infection identified several candidate genes that mapped to the Rsvs1 interval, including Cftr. These findings add to our understanding of individual RSV susceptibility and strongly support a modifier role for CFTR in RSV infection, a significant cause of respiratory morbidity in infants with cystic fibrosis.

Reciprocal backcross mice confirm major loci linked to hyperoxic acute lung injury survival time.

Morbidity and mortality associated with acute lung injury (ALI) and acute respiratory distress syndrome remain substantial. Although many candidate genes have been tested, a clear understanding of the pathogenesis is lacking, as is our ability to predict individual outcome. Because ALI is a complex disease, single gene approaches cannot easily identify effectors that must be treated concurrently. We employed a strategy to help identify critical genes and gene combinations involved in ALI mortality. Using hyperoxia to induce ALI, a mouse model for genetic analyses of ALI survival time was identified: C57BL/6J (B) mice are sensitive (i.e., die early), whereas 129X1/SvJ (S) mice are significantly more resistant, but with low penetrance. Segregation analysis of reciprocal F(2) mice generated from B and S strains revealed significant sex, cross, and parent of origin effects. Quantitative trait locus (QTL) analysis identified five chromosomal regions significantly linked to hyperoxic ALI survival time (named Shali1-Shali5). Further analyses demonstrated that both parental strains contribute resistance alleles to their offspring and that the phenotype demonstrated parent of origin effects. To validate earlier findings, we generated and tested mice from all eight possible B-S-derived backcrosses. Results from segregation and QTL analyses of 935 backcrosses, alone and combined with the previous 840 B-S-derived F(2) population, further supported the highly significant QTLs on chromosomes 1 (Shali1) and 4 (Shali2) and confirmed that the sex, cross, and parent of origin all contribute to survival time with hyperoxic ALI.

Reciprocal congenic lines of mice capture the aliq1 effect on acute lung injury survival time.

Acute lung injury (ALI) is a devastating condition resulting from diverse causes. Genetic studies of human populations indicate that ALI is a complex disease with substantial phenotypic variance, incomplete penetrance, and gene-environment interactions. To identify genes controlling ALI mortality, we previously investigated mean survival time (MST) differences between sensitive A/J (A) and resistant C57BL/6J (B) mice in ozone using quantitative trait locus (QTL) analysis. MST was significantly linked to QTLs (Aliq1-3) on chromosomes 11, 13, and 17, respectively. Additional QTL analyses of separate and combined backcross and F(2) populations supported linkage to Aliq1 and Aliq2, and established significance for previously suggestive QTLs on chromosomes 7 and 12 (named Aliq5 and Aliq6, respectively). Decreased MSTs of corresponding chromosome substitution strains (CSSs) verified the contribution of most QTL-containing chromosomes to ALI survival. Multilocus models demonstrated that three QTLs could explain the MST difference between progenitor strains, agreeing with calculated estimates for number of genes involved. Based on results of QTL genotype analysis, a double CSS (B.A-6,11) was generated that contained Aliq1 and Aliq4 chromosomes. Surprisingly, MST and pulmonary edema after exposure of B.A-6,11 mice were comparable to B mice, revealing an unpredicted loss of sensitivity compared with separate CSSs. Reciprocal congenic lines for Aliq1 captured the corresponding phenotype in both background strains and further refined the QTL interval. Together, these findings support most of the previously identified QTLs linked to ALI survival and established lines of mice to further resolve Aliq1.

A genetic mouse model to investigate hyperoxic acute lung injury survival.

Acute lung injury (ALI) is a devastating disease that maintains a high mortality rate, despite decades of research. Hyperoxia, a universal treatment for ALI and other critically ill patients, can itself cause pulmonary damage, which drastically restricts its therapeutic potential. We stipulate that having the ability to use higher levels of supplemental O2 for longer periods would improve recovery rates. Toward this goal, a mouse model was sought to identify genes contributing to hyperoxic ALI (HALI) mortality. Eighteen inbred mouse strains were screened in continuous >95% O2. A significant survival difference was identified between sensitive C57BL/6J and resistant 129X1/SvJ strains. Although resistant, only one-fourth of 129X1/SvJ mice survived longer than any C57BL/6J mouse, demonstrating decreased penetrance of resistance. A survival time difference between reciprocal F1 mice implicated a parent-of-origin (imprinting) effect. To further evaluate imprinting and begin to delineate the genetic components of HALI survival, we generated and phenotyped offspring from all four possible intercrosses. Segregation analysis supported maternal inheritance of one or more genes but paternal inheritance of one or more contributor genes. A significant sex effect was demonstrated, with males more resistant than females for all F2 crosses. Survival time ranges and sensitive-to-resistant ratios of the different F2 crosses also supported imprinting and predicted that increased survival is due to dominant resistance alleles contributed by both the resistant and sensitive parental strains. HALI survival is multigenic with a complex mode of inheritance, which should be amenable to genetic dissection with this mouse model.

Genetic analysis of hyperoxic acute lung injury survival in reciprocal intercross mice.

Acute lung injury (ALI) and its most severe presentation, acute respiratory distress syndrome, represent a full spectrum of a complex and devastating illness, with associated mortality that still hovers around 30-40%. Even supplemental O2, a routine and necessary therapy for such patients, paradoxically causes lung injury. The detrimental effects of O2 have established hyperoxic ALI (HALI) as a conventional model to study neonatal and adult forms of respiratory distress syndromes in experimental animals. To confront the high ALI mortality problem quite differently, we recently identified a mouse model (sensitive C57BL/6J and resistant 129X1/SvJ mice) to assess the genetic complexity of HALI and to identify genes affecting strain survival differences. Segregation analysis of 840 F2 mice generated from all four possible intercrosses between C57BL/6J and 129X1/SvJ mice demonstrated that survival time is a quantitative trait with decreased penetrance, and significant sex, cross, and parent-of-origin effects. Quantitative trait locus (QTL) analyses of the total F2 population identified three highly significant (named Shali1, Shali2 and Shali3, for Survival to hyperoxic acute lung injury) and one significant (Shali4) linkage. Analysis of F2 subpopulations further identified a male-specific QTL (Shali5). QTL allelic comparisons supported cross and sex effects and were consistent with imprinting. Genome-wide pairwise analysis predicted additive gene-gene interactions between the QTLs and also revealed a significant epistatic interaction with an otherwise unlinked region. QTL results confirmed that both parental strains contribute dominant resistance alleles to their offspring to determine individual HALI susceptibility.