Parent perspectives on education to support hospital discharge for children with invasive mechanical ventilation.

BACKGROUND: Children with invasive mechanical ventilation (IMV) often live at home, but for safety, parents must be prepared to assume primary responsibility for all aspects of their child's medically complex care. Prior studies have described discharge education programs, however often without perspectives of parents with lived experience transitioning home. PURPOSE: To describe parent perspectives on hospital-based education for discharging home a child with IMV. DESIGN AND METHODS: A secondary qualitative analysis of 23 parent interviews between February 2019 to January 2022 on topics related to caring for a child with IMV. Each interview was coded independently and discussed to consensus. Data from codes related to parent education and training were analyzed to identify themes and sub-themes. RESULTS: Parents of 23 children with IMV participated in the primary interviews a month after hospital discharge. Four main themes in the secondary dataset were identified: (1) Training context: The hospital can be a stressful and difficult learning environment; (2) Training characteristics: Parents receive thorough training from interdisciplinary providers; (3) Learner characteristics: Parents are motivated learners who independently seek out knowledge; (4) Post-discharge education: Parents gain confidence in their expertise after navigating an emergency. CONCLUSIONS: Parents who have transitioned from hospital-to-home describe sufficiently detailed hospital-based education; many felt trained as capably as nurses. However, parents experienced in-hospital training as inflexible and stressful. PRACTICE IMPLICATIONS: Parents of children with IMV are eager learners but parent education is not always family-centered. Reforming the hospital learning environment to match parent needs will improve family experiences and training.

Paying Family Medical Caregivers for Children's Home Healthcare in Colorado: A Working Medicaid Model.

OBJECTIVE: To compare the characteristics and healthcare use of children with medical complexity who receive paid certified nursing assistant (CNA) care by a family member (family CNA) and by a traditional nonfamily member (nonfamily CNA). STUDY DESIGN: This was retrospective cohort study of children who received CNA care through Colorado's Medicaid paid family caregiving program between 2017 and 2019 by a home healthcare agency. We compared patient characteristics between the family CNA and nonfamily CNA groups. A multivariable Poisson regression model was used to compare hospitalization rates (days in the hospital per year), adjusting for patient age patient sex, nursing care, and complex chronic condition. RESULTS: Of 861 patients, 79% (n = 680) received family CNA care and 21% (n = 181) received nonfamily CNA care. Patient demographics and hospitalization did not differ between the groups, although patients who had family CNAs were less likely to receive additional nursing-level care (42% vs 60%, P < .01). Family and nonfamily CNA caregivers had similar characteristics, except that family CNA caregivers had substantially better 3-year retention (82% vs 9%, P < .01) despite lower average hourly pay ($14.60 vs $17.60 per hour, P < .01). Hospitalizations were rare (<10% of patients). In the adjusted model, patients who received family CNA care experienced 1 more hospitalized day per year, compared with patients who received nonfamily CNA care (P < .001). CONCLUSIONS: Paid family caregivers provided CAN-level care to children with medical complexity with a greater employee retention compared with nonfamily CNA caregivers, with marginally different hospitalization rates using a family-centered approach. This model may help address workforce shortages while also providing income to family caregivers.

Home health nurses for children with invasive mechanical ventilation (IMV): Perspectives on gaps and opportunities for recruitment.

BACKGROUND: Children with medical complexity (CMC) dependent on invasive mechanical ventilation (IMV) often require private duty home nursing; however, there are ubiquitous shortages. Home health is an especially vulnerable nursing sector because of less competitive wages and less prominence during nursing education. We sought to understand nurses' perspectives on gaps and opportunities for recruiting home care nurses for children with IMV. METHODS: Home health nurses experienced with children with IMV were recruited for semi-structured interviews. The interview guide served as the initial codebook which was iteratively modified as themes emerged. This study presents an analysis of quotes related to field entry and home health experiences. FINDINGS: Twenty interviews were completed with primarily female (95%) participants. The majority worked full-time (60%) and had an average of 11 years of experience. During nursing education, participants described a lack of exposure to private duty home health nursing. Many entered the field serendipitously, because of a passion for care of CMC or to continue care for a hospitalized patient. Challenges to employment included lack of competitive wages and benefits. Nurses remained in the field because of the rewarding work with patients and families, schedule flexibility, slower pace, and one-on-one care. DISCUSSION: Home health nurses for IMV describe lack of employment benefits. However, the opportunity to work longitudinally and individually with patients was rewarding. APPLICATION TO PRACTICE: Creative solutions must be explored to recruit and sustain this essential workforce, including exposure during nursing education, improved training and benefits, and targeted recruiting.

Parent education programs for children assisted by invasive mechanical ventilation: A scoping review.

PROBLEM: The population of children assisted by invasive mechanical ventilation (IMV) and living at home is growing. Although parent education is essential for safe transitions from hospital-to-home, little is known about how this education is delivered. The aim of this review is to identify existing literature about parent education programs, synthesize the evidence, and identify gaps in the literature for future inquiry. ELIGIBILITY CRITERIA: This scoping review was conducted in accordance with the Joanna Briggs Institute methodology. Full-text publications in the English language focused on describing parent education programs for children assisted by IMV, indexed in CINAHL, PubMed, OVID, and PsycINFO and published from 2010 to 2021 were included. Reference lists of relevant articles were reviewed, and a hand search was completed to locate any additional literature outside the original search. SAMPLE: A total of 2472 citations were identified. After screening titles and abstracts, 37 full-text articles were retrieved and assessed for eligibility. Two independent reviewers completed the screening process. A hand search located one additional article. A final sample of 18 articles were included in the review. RESULTS: The parent education programs described in the final sample included standardized discharge education programs, simulation training, resourcefulness training, patient-specific action plans, disaster preparedness, and symptom and technology management. CONCLUSION: Although most parent education programs identified in this review focused on teaching caregiver skills, program characteristics and outcome measures varied widely. IMPLICATIONS: This review recommends directions for future research to optimize parent education for children assisted by IMV.

Attributable Delay of Discharge for Children with Long-Term Mechanical Ventilation.

OBJECTIVE: To assess the reasons for discharge delays for children with long-term mechanical ventilation. STUDY DESIGN: Charts of children (0-18 years of age) with a new tracheostomy in the Pulmonary Habilitation Program at the Ann and Robert H. Lurie Children's Hospital of Chicago were retrospectively reviewed for demographic information, medical diagnoses, medical stability, discharge to home, reasons for discharge delay, and hours of staffed home nursing. All patients were discharged on mechanical ventilation. Discharge delay was defined as >10 days after medical stability. Hospital charges were analyzed and excess charges quantified beginning with the date of delay. Descriptive statistics and Pearson χ2 tests were used to compare nursing hours and demographics. RESULTS: Of 72 patients, 55% were male with mean age 1.8 years (SD 3.8) at tracheostomy placement. The most common long-term mechanical ventilation indication was chronic lung disease (n = 47, 65%); 54% had discharge delays, the majority were primarily due to lack of home nursing (62%), followed by delay of caregiver training (18%), caregiver health and social issues (8%), and delay in a transitional care facility bed (8%). Of the 39 delayed patients, 10% ($13 217 889) of hospital charges occurred during excess days with a median of $186 061 (IQR $117 661-$386 905) per patient. CONCLUSIONS: Over one-half of children discharged to the community from a large inpatient pediatric long-term mechanical ventilation program had a nonmedical delay of discharge home, most commonly because of home nurse staffing. This case series provides further evidence that limited availability of home nursing impedes efficient discharge and prolongs hospitalizations.

Maternal body mass index (BMI) is independently associated with the control of diabetes mellitus in young patients.

OBJECTIVE: Investigate the cross-sectional association of glycemic control of ethnically diverse youth with diabetes mellitus with family characteristics. DESIGN: Family study of 91 youth (probands) with diabetes mellitus and 142 parents. RESULTS: Children's age and HbA1c averaged 11.9 years and 8.9%, respectively; 69% were minorities. After adjustment, poor glycemic control was associated with minority race/ethnicity, more television viewing, and higher maternal body mass index (BMI). Average HbA1c was 1.2 and 1.9% units higher for children of overweight and obese mothers, respectively (p = 0.004). CONCLUSIONS: The positive association between maternal body composition and child HbA1c likely represents the unique behavioral influence of mothers.

Home Health Nursing Agencies' Services for Children With Medical Complexity: Parent and Nurse Perspectives.

OBJECTIVE: Many children with medical complexity (CMC) require the services of home health nurses (HHNs). Home health agencies (HHAs) hire, train, and manage nurses. For children to flourish, families, nurses, and HHAs must establish successful working relationships. Our objective was to understand the perspectives of parents and nurses about HHAs. METHODS: In Illinois (IL) from 2019 to 2022, HHNs for and parents of children with invasive mechanical ventilation were interviewed. In North Carolina (NC) from 2012 to 2013, parents of CMC were interviewed, and from 2013 to 2014 HHNs participated in focus groups. Each dataset was initially analyzed separately for main themes relating to HHAs. Using collaborative thematic analysis, we determined themes common across datasets. RESULTS: In IL, 23 mothers, 12 fathers, and 20 nurses were interviewed. In NC, 19 mothers, 6 fathers, and 1 grandmother were interviewed; and 4 focus groups of 18 nurses were conducted. Four common themes were identified. 1) HHAs do not have a uniform process for hiring and assigning nurses to cases. 2) HHAs have marked variability in training offered to nurses. 3) Shift scheduling, notifications, and communications with the HHAs frustrate parents and nurses. 4) Nurses and parents have little allegiance to specific HHAs; they frequently change agencies or work with several simultaneously. CONCLUSIONS: Parents and nurses perceive practices for hiring, training, and staffing as inconsistent, and experience communication challenges. HHA-level problems may contribute to issues with HHN retention and complicate the lives of the families of CMC. Further research about this critical health care sector is needed.

Autism and neurodevelopmental disability risks in children with tracheostomies and ventilators.

BACKGROUND/OBJECTIVE: Infants who survive prematurity and other critical illnesses and require continued invasive mechanical ventilation (IMV) postdischarge (at home) are at high risk of developmental delays and disabilities. Studies of extremely preterm cohorts (<28-week gestation) demonstrate rates of 25% for intellectual disability (ID) and 7% for autism spectrum disorder (ASD). Rates of ASD and ID in children with IMV are unknown. This study aimed to determine neurodevelopmental disability risk in a cohort of children with postdischarge IMV. DESIGN/METHODS: A consecutive series of children with IMV were assessed 1 month, 6 months, and 1 year after discharge. Cognitive, social, and communicative domains were assessed by a Developmental and Behavioral Pediatrician using (1) clinical adaptive test/clinical linguistic and auditory milestone scale (CAT/CLAMS) of the capute scales; (2) pediatric evaluation of disability inventory computer adaptive test (PEDI-CAT); and (3) modified checklist for autism in toddlers, revised (MCHAT-R). Red flag signs and symptoms of ASD using DSM-V criteria were noted. Longitudinal testing was reviewed. Expert consensus impressions of evolving ASD and/or ID were determined. RESULTS: Eighteen children were followed for 1 year; at 1 year, the median age (range) was 23 (17-42) months. Children were 44% male, 33% non-Hispanic White, 39% non-Hispanic Black, and 28% Hispanic. Fifteen (83%) children were prematurity survivors. Median (range) developmental quotients (DQs): full-scale DQ 59 (11-86), CAT DQ 66.5 (8-96), and CLAMS DQ 49.5 (13-100). Twelve (67%) children were highly suspicious for ASD and/or evolving ID. CONCLUSIONS/SIGNIFICANCE: This cohort of children with at-home IMV demonstrates a higher risk of ASD and ID than prior premature cohorts. Larger investigations with longer follow-up are needed.

Newborn Screening for Neurodevelopmental Disorders May Exacerbate Health Disparities.

Newborn screening (NBS) began in the early 1960s with screening for phenylketonuria on blood collected on filter paper. The number of conditions included in NBS programs expanded significantly with the adoption of tandem mass spectrometry. The recommended uniform screening panel provides national guidance and has reduced state variability. Universality and uniformity have been supported to promote equity. Recently, a number of researchers have suggested expanding NBS to include genomic sequencing to identify all genetic disorders in newborns. This has been specifically suggested for genes that increase the risk for neurodevelopmental disorders (NDDs), with the presumption that early identification in the newborn period would reduce disabilities. We offer arguments to show that genomic sequencing of newborns for NDDs risks exacerbating disparities. First, the diagnosis of NDD requires clinical expertise, and both genetic and neurodevelopmental expertise are in short supply, leading to disparities in access to timely follow-up. Second, therapies for children with NDDs are insufficient to meet their needs. Increasing early identification for those at risk who may never manifest developmental delays could shift limited resources to those children whose parents are more poised to advocate, worsening disparities in access to services. Rather, we suggest an alternative: genomic sequencing of all children with diagnosed NDDs. This focused strategy would have the potential to target genomic sequencing at children who manifest NDDs across diverse populations which could better improve our understanding of contributory genes to NDDs.

Early childhood developmental skills of children with tracheostomies and ventilators recently discharged home.

BACKGROUND: Infants who survive prematurity and critical illnesses but require ongoing invasive mechanical ventilation (IMV) are at high risk for developmental disabilities. However, their detailed developmental profiles are largely unknown. OBJECTIVE: To understand the developmental profiles of a cohort of young children with IMV after hospital-to-home transition. DESIGN/METHODS: Developmental testing was completed 1 month after hospital discharge to determine functioning within motor, cognitive, and communicative domains using (1) the Clinical Adaptive Test/Clinical Linguistic and Auditory Milestone Scale (CAT/CLAMS); (2) the Pediatric Evaluation of Disability Inventory Computer Adaptive Test (PEDI-CAT); and (3) The Warner Initial Developmental Evaluation of Adaptive and Functional Skills (WIDEA-FS). To complement validated testing, semi-structured interviews were conducted with parents to understand perceived influences on neurodevelopment. RESULTS: Twenty children were evaluated at median (range) age 11 (5-27) months. Nearly half (45%) were male, 40% non-Hispanic Black, and 25% Hispanic. Sixteen (80%) children were survivors of prematurity. After gestational age correction, median (range) full-scale developmental quotients (DQ) were 69.5 (8-119): CAT DQ 62.5 (3-113) and CLAMS DQ 71.5 (12-125). Parents described that prolonged restriction within the hospital crib away from caregivers, despite hospital therapists, impedes development. Home environments improve development primarily through increased time with parents, but also play outside of the crib. One month after hospital discharge only 10% were receiving early intervention therapies. CONCLUSIONS/SIGNIFICANCE: Children with IMV display wide ranges of neurodevelopmental skills with a majority experiencing substantial delays in motor, communicative, and adaptive functioning. The degree and cross-domain challenges highlight the need for targeted and timely therapeutic strategies.

Diagnosis of autism in a rare case of tyrosine hydroxylase deficiency: a case report.

BACKGROUND: Tyrosine hydroxylase deficiency (THD) is a rare movement disorder with broad phenotypic expression caused by bi-allelic mutations in the TH gene, which encode for tyrosine hydroxylase (TH) protein. Some patients with THD have improvement in dystonia with carbidopa-levodopa, a synthetic form of dopamine typically used in Parkinson's disease, and are considered to have dopa-responsive THD. THD has been found in 0.5-1 per million persons, although due to overlapping symptoms with other disorders and the need for genetic testing, prevalence is likely underestimated. Existing literature describes some patients with THD having intellectual disability, but comorbid autism spectrum disorder (ASD) has not been reported. CASE PRESENTATION: A nearly 3-year-old boy was referred to pediatric neurology due to hypotonia, delayed motor milestones, and expressive speech delay. Whole exome sequencing confirmed tyrosine hydroxylase deficiency, detecting a novel variant p.S307C first reported here. The child was treated with carbidopa-levodopa with an excellent response, resulting in improved balance, fewer falls, and improved ability to jump, run and climb stairs. He was determined to have dopa-responsive THD. Due to his delays in expressive speech, the boy also had an assessment with a developmental and behavioral pediatrician, who identified a pattern of social pragmatic speech delay, sensory sensitivities, and restricted interests, and determined that he met criteria for a diagnosis of ASD. CONCLUSIONS: While ASD can stand alone as a clinical diagnosis, it is also a cardinal feature of other genetically-based neurological disorders. To our knowledge, this is the first case that describes a patient with both disorders. Perhaps THD may be among the genetic disorders linked with ASD.

Prevalence of and Spending on Ear, Nose, Throat, and Respiratory Infections Among Children With Chronic Complex Conditions.

OBJECTIVE: Ear, nose, throat, and respiratory infections (ENTRI) may affect children with complex chronic conditions (CCC) differently than their peers. We compared ENTRI prevalence and spending in children with and without CCCs. METHODS: Retrospective analysis of 3,880,456 children ages 0-to-18 years enrolled in 9 US state Medicaid programs in 2018 contained in the IBM Watson Marketscan Database. Type and number of CCCs were distinguished with Feudtner's system. ENTRI prevalence, defined as ≥1 healthcare encounters for ENTRI, and Medicaid spending on ENTRI were compared by CCC using chi-square tests and logistic regression. RESULTS: ENTRIs were greater in children with vs. without a CCC (57.7% vs 43.5% [P < .001]). Children with a CCC (5.5%, n = 213,425) accounted for nearly one-fourth ($145.8 million [US]) of total spending on ENTRI. Aside from throat and sinus infection, ENTRI prevalence increased with number of CCCs (P < .001). For example, as number of CCCs increased from zero to ≥3, lower-airway infection increased from 12.5% to 37.5%, P < .001 (OR 4.10; 95% CI 3.95-4.26). ENTRI spending attributable to inpatient care increased from 9.7% to 92.8% (P < .001) as the number of CCCs increased from zero to ≥3. CONCLUSION: Most children with a CCC pursued care for ENTRI in 2018 and these children accounted for a disproportionate share of ENTRI spending. Children with multiple CCCs had a high prevalence of lower-airway infection; most of their ENTRI spending was for inpatient care. Providers can use these findings to counsel patients and families and to inform future investigations on how best to manage ENTRI in children with CCCs.

Understanding the Barriers to Receiving Autism Diagnoses for Hispanic and Latinx Families.

Significant disparities exist in early identification of autism spectrum disorder (ASD) for Hispanic and Latinx children. ASD prevalence estimates are approximately identical for White and Black children but lower for Hispanic and Latinx children. Reasons for these racial and ethnic variations are likely multifactorial. This review sought to understand previously described barriers and limitations to accessing ASD diagnostic services in the Latinx and Hispanic communities. Three main categories of existing barriers were identified: (1) parent/family, (2) community, and (3) systemic. These barriers are complex and multifactorial in nature, including circumstantial limitations such as limited English proficiency, noncitizenship, and low-income status. These can limit health care access, and can lead to family and community cultural barriers, poor knowledge about ASD, and social stigma related to disabilities. Understanding and mitigating barriers is essential to reduce disparities to ASD diagnosis in the Hispanic and Latinx community. [Pediatr Ann. 2022;51(4):e167-e171.].

The Role of Care Coordinators for Children with Respiratory Technologies and Home Nursing.

Background: Children with respiratory technologies, particularly those with mechanical ventilation, represent a growing population that require complex home nursing, medical equipment, outpatient medical and habilitative supports to live and thrive in their community. Care coordination is essential to support these children and their families to navigate and integrate key community-based health and educational services, however, care is often fragmented and care coordination needs unmet. Therefore, to fully support children with respiratory technologies, it is critical to understand the role of care coordinators (CCs) and how to sustain this workforce. The aim of this article is to describe CCs' perspective on (1) their role in supporting families in a home care program for children with respiratory technologies and home nursing, and (2) the core components of recruiting into and sustaining the CC workforce. Methods: Semistructured interviews were conducted with 15 CC from the Division of Specialized Care for Children (DSCC) Home Care program for children with technology dependence and home nursing in Illinois. Two independent coders utilized a modified template approach and discussed to agreement to analyze transcripts. Results: CC averaged 6.6 years of CC experience; the majority had social work or nursing backgrounds. CCs' job satisfaction was derived from their role supporting hospital discharge, seeing children improve over time, and navigating challenges with families. CCs enjoyed working in a collaborative environment where they could draw from their colleagues' experience to solve problems. Job dissatisfaction and job turnover stemmed from difficult family interactions, high caseloads, and redundant and time-intensive administrative tasks, which interfered with family engagement. Conclusions: CCs for children with respiratory technologies require diverse skills, but interdisciplinary teams enable collaborative support of families. Seeing children thrive can sustain the workforce, however, CCs report challenges due to high caseloads and administrative tasks, which impede direct family involvement.

PICU Survivorship: Factors Affecting Feasibility and Cohort Retention in a Long-Term Outcomes Study.

Our understanding of longitudinal outcomes of Pediatric Intensive Care Unit (PICU) survivors is limited by the heterogeneity of follow-up intervals, populations, and outcomes assessed. We sought to demonstrate (1) the feasibility of longitudinal multidimensional outcome assessment and (2) methods to promote cohort retention. The objective of this presented study was to provide details of follow-up methodology in a PICU survivor cohort and not to present the outcomes at long-term follow-up for this cohort. We enrolled 152 children aged 0 to 17 years admitted to the PICU in a prospective longitudinal cohort study. We examined resource utilization, family impact of critical illness, and neurodevelopment using the PICU Outcomes Portfolio (POP) Survey which included a study-specific survey and validated tools: 1. Functional Status Scale, 2. Pediatric Evaluation of Disability Inventory Computer Adaptive Test, 3. Pediatric Quality of Life Inventory, 4. Strengths and Difficulties Questionnaire, and 5. Vanderbilt Assessment Scales for Attention Deficit-Hyperactivity Disorder. POP Survey completion rates were 89%, 78%, and 84% at 1, 3, and 6 months. Follow-up rates at 1, 2, and 3 years were 80%, 55%, and 43%. Implementing a longitudinal multidimensional outcome portfolio for PICU survivors is feasible within an urban, tertiary-care, academic hospital. Our attrition after one year demonstrates the long-term follow-up challenges in this population. Our findings inform ongoing efforts to implement core outcome sets after pediatric critical illness.

A Literature Review of Feeding Disorders in Children with Tracheostomies and Ventilators.

Feeding disorders and gastrostomy use are highly prevalent in children with invasive mechanical ventilation (IMV) due to both common risk factors (eg, prematurity, neurological disorders) and resultant experiential deprivation (eg, long hospitalizations, delayed feeding experiences). Feeding in children with IMV is complicated by the presence of a tracheostomy, lung vulnerability, and medical complexity. The potential comorbidity of swallowing difficulties (dysphagia) and atypical early feeding experiences can result in complex feeding disorders. In this review of pediatric feeding disorders in children with invasive mechanical ventilation (IMV), we identify gaps in clinical translational research for this patient population and opportunities for improving evidence-based management. To improve long-term feeding outcomes and maximize oral feeding in this vulnerable population, children would benefit from earlier feeding opportunities during critical developmental windows, standardized protocols for advancing oral feeding, and involvement of intensive, comprehensive therapies throughout hospitalizations and early childhood. [Pediatr Ann. 2022;51(7):e291-e296.].

Home Health Care Utilization in Children With Medicaid.

BACKGROUND: Although many children with medical complexity (CMC) use home health care (HHC), little is known about all pediatric HHC utilizers. Our objective was to assess characteristics of pediatric HHC recipients, providers, and payments. METHODS: We conducted a retrospective analysis of 5 209 525 children age 0-to-17 years enrolled Medicaid in the 2016 IBM Watson MarketScan Medicaid Database. HHC utilizers had ≥ 1 HHC claim. Healthcare Common Procedure Coding System (HCPCS) and Current Procedural Terminology (CPT) codes were reviewed to codify provider types when possible: registered nurse (RN), licensed practical nurse (LPN), home health aide (HHA), certified nursing assistant (CNA), or companion/personal attendant. Enrollee clinical characteristics, HHC provider type, and payments were assessed. Chronic conditions were evaluated with Agency for Healthcare Research and Quality's Chronic Condition Indicators and Feudtner's Complex Chronic Conditions. RESULTS: Of the 0.8% of children who used HHC, 43.8% were age <1 year, 25% had no chronic condition, 38.6% had a noncomplex chronic condition, 21.5% had a complex chronic condition without technology assistance, and 15.5% had technology assistance (eg, tracheostomy). HHC for children with technology assistance accounted for 72.6% of all HHC spending. Forty-five percent of HHC utilizers received RN/LPN-level care, 7.9% companion/personal attendant care, 5.9% HHA/CNA-level care, and 36% received care from an unspecified provider. For children with technology assistance, the majority (77.2%) received RN/LPN care, 17.5% companion/personal assistant care, and 13.8% HHA/CNA care. CONCLUSIONS: Children using HHC are a heterogeneous population who receive it from a variety of providers. Future investigations should explore the role of nonnurse caregivers, particularly with CMC.