Background -Tiredness and daytime respiratory failure occur frequently in myotonic dystrophy. Sleep hypoxaemia was studied in 12 patients with myotonic dystrophy and correlations were sought with their daytime lung and respiratory muscle function. Methods -All patients underwent overnight sleep studies, clinical assessment, measurement of flow-volume loops and carbon monoxide transfer factor, arterial blood gas analysis, and physiological assessment of both thoracic muscle function and upper airways obstruction. Results -The mean nadir of oxygen saturation during sleep was 75% (95% confidence interval 69% to 81%). A mean of 3-4% of total sleep duration was spent at an oxygen saturation level below 85%.

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 c 2 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%

Children with medical technology dependency (MTD) require a medical device to compensate for a vital body function and substantial nursing care. As such, they require constant high-level supervision. Respite care provides caregivers with a temporary break, and is associated with reduced stress; however, there are often barriers. The study utilizes mixed methodology with the National Survey of Children with Special Health Care Needs (NS-CSHCN) and semistructured interviews with state-wide care coordinators to understand the gap for respite care services. Fifty-nine percent of parents who needed respite care received none. Parents of older children with MTD were more likely to report respite needs. Care coordinators described that home health shortages created barriers to respite care utilization, and the lack of respite care can lead to hospital readmission. Although respite care is a vital resource to support families of children with MTD, it is infrequently available, which can have severe consequences.

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ObjectiveChildren with medical technology dependence (MTD) are frequently readmitted to the hospital. However, due to their medical fragility, it is often difficult to untangle the root causes for readmissions to identify the most effective preventive approaches. We sought to explore environmental and family factors driving hospital readmissions for children with MTD.DesignSemi‐structured, in‐person interviews were conducted with state‐wide care coordinators for children with MTD in Illinois with at least 1 year of experience. Interview topics related to children with MTD transitioning from hospital‐to‐home, essential supports for living in the community, and factors which influenced and prevented hospital readmission. The interview guide served as an initial codebook which was iteratively modified as themes emerged.ResultsFifteen care coordinators with on average 6.6 years of experience were interviewed. They described that lack of home nursing was one of the primary drivers of readmissions due to parental exhaustion and lack of medical expertize in the home. Unavoidable medical admissions, a lack of a plan for emergencies, and home environmental factors also contributed to readmissions.ConclusionHospital readmission is an expected occurrence for children with MTD, yet still may be substantially reduced through consistent, quality home health nursing to bolster family capacity and allow for respite from constant caregiving. Improved incentives for the home health workforce to increase manpower would be ultimately offset by reduced hospitalizations for children with MTD. Additionally, more research is needed to understand which home nursing structures and skills optimally support families in the reality of manpower scarcity.

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.

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