Use of Latent Class Analysis to Predict Intensive Care Unit Admission and Mortality in Children with a Major Congenital Anomaly.

OBJECTIVE: To define major congenital anomaly (CA) subgroups and assess outcome variability based on defined subgroups. STUDY DESIGN: This population-based cohort study used registries in Denmark for children born with a major CA between January 1997 and December 2016, with follow-up until December 2018. We performed a latent class analysis (LCA) using child and family clinical and sociodemographic characteristics present at birth, incorporating additional variables occurring until age of 24 months. Cox proportional hazards regression models estimated hazard ratios (HRs) of pediatric mortality and intensive care unit (ICU) admissions for identified LCA classes. RESULTS: The study included 27 192 children born with a major CA. Twelve variables led to a 4-class solution (entropy = 0.74): (1) children born with higher income and fewer comorbidities (55.4%), (2) children born to young mothers with lower income (24.8%), (3) children born prematurely (10.0%), and (4) children with multiorgan involvement and developmental disability (9.8%). Compared with those in Class 1, mortality and ICU admissions were highest in Class 4 (HR = 8.9, 95% CI = 6.4-12.6 and HR = 4.1, 95% CI = 3.6-4.7, respectively). More modest increases were observed among the other classes for mortality and ICU admissions (Class 2: HR = 1.7, 95% CI = 1.1-2.5 and HR = 1.3, 95% CI = 1.1-1.4, respectively; Class 3: HR = 2.5, 95% CI = 1.5-4.2 and HR = 1.5, 95% CI = 1.3-1.9, respectively). CONCLUSIONS: Children with a major CA can be categorized into meaningful subgroups with good discriminative ability. These groupings may be useful for risk-stratification in outcome studies.

Recurrent Intensive Care Episodes and Mortality Among Children With Severe Neurologic Impairment.

Importance: Children requiring care in a pediatric intensive care unit (PICU) are known to have increased risk of subsequent mortality. Children with severe neurologic impairment (SNI)-who carry neurologic or genetic diagnoses with functional impairments and medical complexity-are frequently admitted to PICUs. Although recurrent PICU critical illness episodes (PICU-CIEs) are assumed to indicate a poor prognosis, the association between recurrent PICU-CIEs and mortality in this patient population is poorly understood. Objective: To assess the association between number of recent PICU-CIEs and survival among children with severe neurologic impairment. Design, Setting, and Participants: This population-based retrospective cohort study used health administrative data from April 1, 2002, to March 31, 2020, on 4774 children born between 2002 and 2019 with an SNI diagnosis code in an Ontario, Canada, hospital record before 16 years of age and a first PICU-CIE from 2002 to 2019. Data were analyzed from November 2021 to June 2023. Exposure: Pediatric intensive care unit critical illness episodes (excluding brief postoperative PICU admissions). Main Outcome and Measures: One-year survival conditioned on the number and severity (length of stay >15 days or use of invasive mechanical ventilation) of PICU-CIEs in the preceding year. Results: In Ontario, 4774 children with SNI (mean [SD] age, 2.1 [3.6] months; 2636 [55.2%] <1 year of age; 2613 boys [54.7%]) were discharged alive between 2002 and 2019 after their first PICU-CIE. Ten-year survival after the initial episode was 81% (95% CI, 79%-82%) for children younger than 1 year of age and 84% (95% CI, 82%-86%) for children 1 year of age or older; the age-stratified curves converged by 15 years after the initial episode at 79% survival (95% CI, 78%-81% for children <1 year and 95% CI, 75%-84% for children ≥1 year). Adjusted for age category and demographic factors, the presence of nonneurologic complex chronic conditions (adjusted hazard ratio [AHR], 1.70 [95% CI, 1.43-2.02]) and medical technology assistance (AHR, 2.32 [95% CI, 1.92-2.81]) were associated with increased mortality. Conditional 1-year mortality was less than 20% regardless of number or severity of recent PICU-CIEs. Among children with high-risk PICU-CIEs, 1-year conditional survival decreased from 90% (95% CI, 89%-91%) after the first PICU-CIE to 81% (95% CI, 77%-86%) after the fourth PICU-CIE. Conclusions and Relevance: This cohort study of children with SNI demonstrated a modest dose-dependent association between PICU-CIEs and short-term mortality. These data did not support the conventional wisdom that recurrent PICU admissions are associated with subsequent high mortality risk.

A randomized controlled trial of oral antipyretic treatment to reduce overheating during exercise in adults with multiple sclerosis.

BACKGROUND: Some people with multiple sclerosis (pwMS) avoid exercise due to overheating. Evidence from a variety of cooling treatments shows benefits for pwMS. OBJECTIVE: Conduct a randomized controlled trial of antipyretic treatment before exercise in pwMS. METHODS: Adults over age 18 diagnosed with relapsing-remitting MS reporting heat sensitivity during exercise were randomly assigned to one of six sequences counterbalancing aspirin, acetaminophen, placebo. At each of three study visits separated by ≥ one week, participants received 650-millograms of aspirin, acetaminophen, or placebo before completing a maximal exercise test. Primary outcomes were body temperature change and total time-to-exhaustion (TTE), secondary outcomes were physiological and patient-reported outcomes (PROs). RESULTS: Sixty participants were enrolled and assigned to treatment sequence; 37 completed ≥ one study visit. After controlling for order effects, we found that body temperature increase was reduced after aspirin (+ 0.006 ± 0.32 degrees Fahrenheit, p < 0.001) and after acetaminophen (+ 0.31 ± 0.35; p = 0.004) compared to placebo (+ 0.68 ± 0.35). TTE after aspirin (331.6 ± 76.6 s) and acetaminophen (578.2 ± 82.1) did not differ significantly from placebo (551.0 ± 78.4; p's > 0.05). Aspirin benefited all secondary outcomes compared to placebo (all p's < 0.001); acetaminophen showed broadly consistent benefits. CONCLUSION: These results support antipyretic treatment as effective for reducing overheating during exercise in pwMS and failed to support antipyretics for increasing TTE in the context of a maximal exercise test. Benefits were shown for physiological markers of exercise productivity and PROs of fatigue, pain, and perceived exertion.

Severe Respiratory Disease Among Children With and Without Medical Complexity During the COVID-19 Pandemic.

Importance: Severe respiratory disease declined during the COVID-19 pandemic, partially due to decreased circulation of respiratory pathogens. However, the outcomes of children with higher risk have not been described using population-based data. Objective: To compare respiratory-related hospitalizations, intensive care unit (ICU) admissions, and mortality during the pandemic vs prepandemic, among children with medical complexity (CMC) and without medical complexity (non-CMC). Design, Setting, and Participants: This population-based repeated cross-sectional study used Canadian health administrative data of children aged younger than 18 years in community and pediatric hospitals during a pandemic period (April 1, 2020, to February 28, 2022) compared with a 3-year prepandemic period (April 1, 2017, to March 31, 2020). The pandemic period was analyzed separately for year 1 (April 1, 2020, to March 31, 2021) and year 2 (April 1, 2021, to February 28, 2022). Statistical analysis was performed from October 2022 to April 2023. Main Outcomes and Measures: Respiratory-related hospitalizations, ICU admissions, and mortality before and during the pandemic among CMC and non-CMC. Results: A total of 139 078 respiratory hospitalizations (29 461 respiratory hospitalizations for CMC and 109 617 for non-CMC) occurred during the study period. Among CMC, there were fewer respiratory hospitalizations in both 2020 (rate ratio [RR], 0.44 [95% CI, 0.42-0.46]) and 2021 (RR, 0.55 [95% CI, 0.51-0.62]) compared with the prepandemic period. Among non-CMC, there was an even larger relative reduction in respiratory hospitalizations in 2020 (RR, 0.18 [95% CI, 0.17-0.19]) and a similar reduction in 2021 (RR, 0.55 [95% CI, 0.54-0.56]), compared with the prepandemic period. Reductions in ICU admissions for respiratory illness followed a similar pattern for CMC (2020: RR, 0.56 [95% CI, 0.53-0.59]; 2021: RR, 0.66 [95% CI, 0.63-0.70]) and non-CMC (2020: RR, 0.22 [95% CI, 0.20-0.24]; RR, 0.65 [95% CI, 0.61-0.69]). In-hospital mortality for these conditions decreased among CMC in both 2020 (RR, 0.63 [95% CI, 0.51-0.77]) and 2021 (RR, 0.72 [95% CI, 0.59-0.87]). Conclusions and Relevance: This cross-sectional study found a substantial decrease in severe respiratory disease resulting in hospitalizations, ICU admissions, and mortality during the first 2 years of the pandemic compared with the 3 prepandemic years. These findings suggest that future evaluations of the effect of public health interventions aimed at reducing circulating respiratory pathogens during nonpandemic periods of increased respiratory illness may be warranted.

Intensity of end-of-life care among children with life-threatening conditions: a national population-based observational study.

BACKGROUND: Children with life-threatening conditions frequently experience high intensity care at the end of life, though most of this research only focused on children with cancer. Some research suggests inequities in care provided based on age, disease type, socioeconomic status, and distance that the child lives from a tertiary hospital. We examined: 1) the prevalence of indicators of high intensity end-of-life care (e.g., hospital stays, intensive care unit [ICU] stays, death in ICU, use of cardiopulmonary resuscitation [CPR], use of mechanical ventilation) and 2) the association between demographic and diagnostic factors and each indicator for children with any life-threatening condition in Canada. METHODS: We conducted a population-based retrospective cohort study using linked health administrative data to examine care provided in the last 14, 30, and 90 days of life to children who died between 3 months and 19 years of age from January 1, 2008 to December 31, 2014 from any underlying life-threatening medical condition. Logistic regression was used to model the association between demographic and diagnostic variables and each indicator of high intensity end-of-life care except number of hospital days where negative binomial regression was used. RESULTS: Across 2435 child decedents, the most common diagnoses included neurology (51.1%), oncology (38.0%), and congenital illness (35.9%), with 50.9% of children having diagnoses in three or more categories. In the last 30 days of life, 42.5% (n = 1035) of the children had an ICU stay and 36.1% (n = 880) died in ICU. Children with cancer had lower odds of an ICU stay (OR = 0.47; 95% CI = 0.36-0.62) and ICU death (OR = 0.37; 95%CI = 0.28-0.50) than children with any other diagnoses. Children with 3 or more diagnoses (vs. 1 diagnosis) had higher odds of > 1 hospital stay in the last 30 days of life (OR = 2.08; 95%CI = 1.29-3.35). Living > 400 km (vs < 50 km) from a tertiary pediatric hospital was associated with higher odds of multiple hospitalizations (OR = 2.09; 95%CI = 1.33-3.33). CONCLUSION: High intensity end of life care is prevalent in children who die from life threatening conditions, particularly those with a non-cancer diagnosis. Further research is needed to understand and identify opportunities to enhance care across disease groups.

Location of death among children with life-threatening conditions: a national population-based observational study using the Canadian Vital Statistics Database (2008-2014).

BACKGROUND: Patterns in location of death among children with life-threatening conditions (e.g., cancer, genetic disorders, neurologic conditions) may reveal important inequities in access to hospital and community support services. We aimed to identify demographic, socioeconomic and geographic factors associated with variations in location of death for children across Canada with life-threatening conditions. METHODS: We used a retrospective observational cohort design and the Canadian Vital Statistics Database to identify children aged 19 years or younger who died from a life-threatening condition between Jan. 1, 2008, and Dec. 31, 2014. We used multivariable logistic regression to determine predictors of in-hospital death for children aged 1 month to 19 years, and for neonates younger than 1 month. RESULTS: Overall, 13 115 decedents younger than 19 years had life-threatening conditions. Of 5250 children and 7865 neonates, 74.2% and 98.1%, respectively, died in hospital. Among children, we found a higher proportion of hospital deaths in the lowest (v. highest) income quintile (odds ratio [OR] 1.59, 95% confidence interval [CI] 1.28-1.97), and a lower proportion among children living more than 400 km (v. < 50 km) from a pediatric hospital (OR 0.73, 95% CI 0.65-0.86). Compared with Ontario, hospital death was most common in Quebec (OR 1.38, 95% CI 1.14-1.67) and least common in British Columbia (OR 0.43, 95% CI 0.34-0.53). Compared with an oncologic cause of death, all causes except neurologic and metabolic conditions had significantly higher odds of dying in hospital. INTERPRETATION: In addition to demographics, we identified socioeconomic and geographic differences in location of death, suggesting potential inequities in access to high-quality care at the end of life. Health care policies and practices must ensure equitable access to services for children across Canada, particularly at the end of their life.

Clinical characteristics of children with severe neurologic impairment: A scoping review.

OBJECTIVE: The aim of this study is to extrapolate the clinical features of children with severe neurologic impairment (SNI) based on the functional characteristics and comorbidities described in published studies. METHODS: Four databases were searched. We included studies that describe clinical features of a group of children with SNI (≥20 subjects <19 years of age with >1 neurologic diagnosis and severe functional limitation) using data from caregivers, medical charts, or prospective collection. Studies that were not written in English were excluded. We extracted data about functional characteristics, comorbidities, and study topics. RESULTS: We included 102 studies, spanning 5 continents over 43 years, using 41 distinct terms for SNI. The terms SNI and neurologic impairment (NI) were used in 59 studies (58%). Most studies (n = 81, 79%) described ≥3 types of functional characteristics, such as technology assistance and motor impairment. Studies noted 59 comorbidities and surgeries across 10 categories. The most common comorbidities were related to feeding, nutrition, and the gastrointestinal system, which were described in 79 studies (77%). Most comorbidities (76%) were noted in <10 studies. Studies investigated seven clinical topics, with "Gastrointestinal reflux and feeding tubes" as the most common research focus (n = 57, 56%). The next most common topic, "Aspiration and respiratory issues," included 13 studies (13%). Most studies (n = 54, 53%) were retrospective cohorts or case series; there were no clinical trials. CONCLUSIONS: Despite the breadth of described comorbidities, studies focused on a narrow set of clinical topics. Further research is required to understand the prevalence, clinical impact, and interaction of the multiple comorbidities that are common in children with SNI.

Physician home visits in Ontario: a cross-sectional analysis of patient characteristics and postvisit use of health care services.

BACKGROUND: It is unknown how much of current physician home visit volume is driven by low-complexity or low-continuity visits. Our objectives were to measure physician home visit volumes and costs in Ontario from 2005/06 to 2018/19, and to compare patient characteristics and postvisit use of health care services across home visit types. METHODS: This was a retrospective cross-sectional study using health administrative data. We examined annual physician home visit volumes and costs from 2005/06 to 2018/19 in Ontario, and characteristics and postvisit use of health care services of residents who received at least 1 home visit from any physician in 2014/15 to 2018/19. We categorized home visits as palliative, provided to a patient who also received home care services or "other," and compared characteristics and outcomes between groups. RESULTS: A total of 4 418 334 physician home visits were performed between 2005/06 and 2018/19. More than half (2 256 667 [51.1%]) were classified as "other" and accounted for 39.1% ($22 million) of total annual physician billing costs. From 2014/15 to 2018/19, of the 413 057 home visit patients, 240 933 (58.3%) were adults aged 65 or more, and 323 283 (78.3%) lived in large urban areas. Compared to the palliative care and home care groups, the "other" group was younger, had fewer comorbidities, and had lower rates of emergency department visits and hospital admissions in the 30 days after the visit. INTERPRETATION: About half of physician home visits in 2014/15 to 2018/19 were to patients who were receiving neither palliative care nor home care, a group that was younger and healthier, and had low use of health care services after the visit. There is an opportunity to refine policy tools to target patients most likely to benefit from physician home visits.

Research priorities for children with neurological impairment and medical complexity in high-income countries.

AIM: To identify the highest-priority clinical research areas related to children with neurological impairment and medical complexity among clinicians and caregivers. METHOD: A modified, three-stage Delphi study using online surveys and guided by a steering committee was completed. In round 1, clinicians and family caregivers suggested clinical topics and related questions that require research to support this subgroup of children. After refinement of the suggestions by the steering committee, participants contributed to 1 (family caregivers) or 2 (clinicians) subsequent rounds to develop a prioritized list. RESULTS: A diverse international expert panel consisting of 49 clinicians and 12 family caregivers provided 601 responses. Responses were distilled into 26 clinical topics comprising 126 related questions. The top clinical topics prioritized for research were irritability and pain, child mental health, disorders of tone, polypharmacy, sleep, aspiration, behavior, dysautonomia, and feeding intolerance. The clinician expert panel also prioritized 10 specific research questions. INTERPRETATION: Study findings support a research agenda for children with neurological impairment and medical complexity focused on addressing clinical questions, prioritized by an international group of clinicians and caregivers.

Validation of Neurologic Impairment Diagnosis Codes as Signifying Documented Functional Impairment in Hospitalized Children.

OBJECTIVE: To assess the performance of previously published high-intensity neurologic impairment (NI) diagnosis codes in identification of hospitalized children with clinical NI. METHODS: Retrospective study of 500 randomly selected discharges in 2019 from a freestanding children's hospital. All charts were reviewed for 1) NI discharge diagnosis codes and 2) documentation of clinical NI (a neurologic diagnosis and indication of functional impairment like medical technology). Test statistics of clinical NI were calculated for discharges with and without an NI diagnosis code. A sensitivity analysis varied the threshold for "substantial functional impairment." Secondary analyses evaluated misclassified discharges and a more stringent definition for NI. RESULTS: Diagnosis codes identified clinically documented NI with 88.1% (95% confidence interval [CI]: 84.7, 91) specificity, and 79.4% (95% CI: 67.3, 88.5) sensitivity; negative predictive value (NPV) was 96.7% (95% CI: 94.8, 98.0), and positive predictive value (PPV) was 49% (95% CI: 42, 56.1). Including children with milder functional impairment (lower threshold) resulted in NPV of 95.7% and PPV of 77.5%. Restricting to children with more severe functional impairment (higher threshold) resulted in NPV of 98.2% and PPV of 44.1%. Misclassification was primarily due to inclusion of children without functional impairments. A more stringent NI definition including diagnosis codes for NI and feeding tubes had a specificity of 98.4% (95% CI: 96.7-99.3) and sensitivity of 28.6% (19.4-41.3). CONCLUSIONS: All scenarios evaluated demonstrated high NPV and low-to-moderate PPV of the diagnostic code list. To maximize clinical utility, NI diagnosis codes should be used with strategies to mitigate the risk of misclassification.

ASPIRE trial: study protocol for a double-blind randomised controlled trial of aspirin for overheating during exercise in multiple sclerosis.

INTRODUCTION: The many benefits of exercise for persons with multiple sclerosis (MS) are well established, yet patients often refrain from exercise due to overheating and exhaustion. The present randomised controlled trial tests aspirin (acetylsalicylic acid (ASA)) as a convenient method to prevent overheating and improve exercise performance in persons with MS. The effects of ASA are compared with those of acetaminophen (APAP) and placebo. METHODS AND ANALYSIS: Participants are seen for a laboratory maximal exercise test on 3 separate days separated by at least 1 week. At each session, body temperature is measured before oral administration of a standard adult dose (650 mg) of ASA, APAP or placebo. One hour after drug administration, participants perform a maximal ramp test on a cycle ergometer. Primary outcomes are (a) time to exhaustion (that is, time spent cycling to peak exertion) and (b) body temperature change. Crossover analyses will include tests for effects of treatment, period, treatment-period interaction (carryover effect) and sequence. ETHICS AND DISSEMINATION: Ethical approval was granted by the institutional review board at Columbia University Irving Medical Center (reference: AAAS2529). Results of the trial will be published in peer-reviewed scientific journals and presented at national and international conferences. Neurologists, physiatrists, primary care physicians and physiotherapists are important stakeholders and will be targeted during dissemination. Positive trial results have the potential to promote aspirin therapy, an inexpensive and readily available treatment, to reduce overheating and allow more persons with MS to benefit from exercise. TRIAL REGISTRATION NUMBER: NCT03824938.

Caregiver Decisional Conflict Before and After Consultation About Gastrostomy Tube Placement.

OBJECTIVES: Families describe decision-making about gastrostomy tube (g-tube) placement as challenging. We measured caregiver decisional conflict before and after initial g-tube consultation to evaluate the potential benefit of a decision aid and feasibility in testing it. METHODS: Families presenting for initial consultation about g-tube placement completed the decisional conflict scale (DCS) at 1 or 2 of 3 time points: before consultation, after consultation, and after viewing a video. The decision support consultation was a 2-hour structured meeting with a pediatric hospitalist, nurse practitioner, and dietitian that was focused on clarifying the indication, feasibility, safety, and family values around tube placement. The video described decision-making and lived experiences of families with tube feeding. RESULTS: We measured the decisional conflict of 61 caregivers. Preconsultation decisional conflict scores were high (mean = 38.7), but there was substantial variation between families (SD = 19.4). Baseline scores did not vary between clinically relevant subgroups. Postconsultation DCS scores were lower (17.9 and SD = 13.5 for consult alone; 12.7 and SD = 13.2 for consult with video). Three caregivers (7.7%) of families had residual decisional conflict scores >37.5, the threshold conventionally associated with decision delay. CONCLUSIONS: Measuring decisional conflict among caregivers deciding about pediatric g-tube is feasible during the clinical encounter. Residual decisional conflict after our institution's current decision support consultation model (with or without an additional video) was low, so development of an additional structured decision aid is not warranted. Further study of preconsult DCS variability across different clinical subgroups may help identify families benefiting from additional decisional support.

Collaborative International Research in Clinical and Longitudinal Experience Study in NMOSD.

Objective: To develop a resource of systematically collected, longitudinal clinical data and biospecimens for assisting in the investigation into neuromyelitis optica spectrum disorder (NMOSD) epidemiology, pathogenesis, and treatment. Methods: To illustrate its research-enabling purpose, epidemiologic patterns and disease phenotypes were assessed among enrolled subjects, including age at disease onset, annualized relapse rate (ARR), and time between the first and second attacks. Results: As of December 2017, the Collaborative International Research in Clinical and Longitudinal Experience Study (CIRCLES) had enrolled more than 1,000 participants, of whom 77.5% of the NMOSD cases and 71.7% of the controls continue in active follow-up. Consanguineous relatives of patients with NMOSD represented 43.6% of the control cohort. Of the 599 active cases with complete data, 84% were female, and 76% were anti-AQP4 seropositive. The majority were white/Caucasian (52.6%), whereas blacks/African Americans accounted for 23.5%, Hispanics/Latinos 12.4%, and Asians accounted for 9.0%. The median age at disease onset was 38.4 years, with a median ARR of 0.5. Seropositive cases were older at disease onset, more likely to be black/African American or Hispanic/Latino, and more likely to be female. Conclusions: Collectively, the CIRCLES experience to date demonstrates this study to be a useful and readily accessible resource to facilitate accelerating solutions for patients with NMOSD.

Survival and Health Care Use After Feeding Tube Placement in Children With Neurologic Impairment.

BACKGROUND AND OBJECTIVES: Children with neurologic impairment (NI) often undergo feeding tube placement for undernutrition or aspiration. We evaluated survival and acute health care use after tube placement in this population. METHODS: This is a population-based exposure-crossover study for which we use linked administrative data from Ontario, Canada. We identified children aged 13 months to 17 years with a diagnosis of NI undergoing primary gastrostomy or gastrojejunostomy tube placement between 1993 and 2015. We determined survival time from procedure until date of death or last clinical encounter and calculated mean weekly rates of unplanned hospital days overall and for reflux-related diagnoses, emergency department visits, and outpatient visits. Rate ratios were estimated from negative binomial generalized estimating equation models adjusting for time and age. RESULTS: Two-year survival after feeding tube placement was 87.4% (95% confidence interval [CI]: 85.2%-89.4%) and 5-year survival was 75.8% (95% CI: 72.8%-78.4%). The adjusted rate ratio comparing weekly rates of unplanned hospital days during the 2 years after versus before tube placement was 0.92 (95% CI: 0.57-1.48). Similarly, rates of reflux-related hospital days, emergency department visits, and outpatient visits were unchanged. Unplanned hospital days were stable within subgroups, although rates across subgroups varied. CONCLUSIONS: Mortality is high among children with NI after feeding tube placement. However, the stability of health care use before and after the procedure suggests that the high mortality may reflect underlying fragility rather than increased risk from nonoral feeding. Further research to inform risk stratification and prognostic accuracy is needed.

Decision-making around gastrostomy tube feeding in children with neurologic impairment: Engaging effectively with families.

Children with neurologic impairment may not be able to feed safely or sufficiently by mouth to maintain an adequate nutritional state. Gastrostomy tube (G-tube) feeding is an important, often essential, intervention in such situations. However, many parents and families struggle with the decision to proceed with G-tube feeding. This practice point reviews common reasons for decisional conflict in parents and explores key aspects of life with G-tube feeding. A framework for shared decision-making and the International Classification of Functioning, Disability and Health (ICF) approach are highlighted. Practical recommendations for clinicians on engaging with families for decision-making around this life-changing intervention are provided.

Emerging Methodologies in Pediatric Palliative Care Research: Six Case Studies.

Given the broad focus of pediatric palliative care (PPC) on the physical, emotional, and spiritual needs of children with potentially life-limiting illnesses and their families, PPC research requires creative methodological approaches. This manuscript, written by experienced PPC researchers, describes issues encountered in our own areas of research and the novel methods we have identified to target them. Specifically, we discuss potential approaches to: assessing symptoms among nonverbal children, evaluating medical interventions, identifying and treating problems related to polypharmacy, addressing missing data in longitudinal studies, evaluating longer-term efficacy of PPC interventions, and monitoring for inequities in PPC service delivery.

Survival and Surgical Interventions for Children With Trisomy 13 and 18.

IMPORTANCE: Trisomy 13 and 18 are genetic diagnoses with characteristic physical features, organ anomalies, and neurodevelopmental disability. Most children with these disorders die shortly after birth, although limited data suggest some children survive longer. Surgeries are controversial, and little evidence is available about outcomes. OBJECTIVE: To describe survival and utilization of any type of surgery among children with trisomy 13 and 18 born over a 21-year period in Ontario, Canada. DESIGN, SETTING, AND PARTICIPANTS: This retrospective cohort study used linked health administrative databases to identify children born in Ontario between April 1, 1991, and March 31, 2012, with a diagnosis code for trisomy 13 or 18 on a hospital record in the first year of life. Survival was calculated from birth and death dates; children living on March 31, 2013, were censored at their last clinical encounter. EXPOSURES: All procedures classified as occurring in an operating room through March 31, 2013, were categorized as major, intermediate, or minor surgeries. MAIN OUTCOMES AND MEASURES: Survival and surgical procedure utilization. RESULTS: The cohorts included 174 children with trisomy 13 (mean [SD] birth weight, 2.5 [0.7] kg; 98 [56.3%] female); and 254 children with trisomy 18 (mean birth weight, 1.8 [0.7] kg; 157 [61.8%] female), with follow-up times of 0 to more than 7000 days. Median (interquartile range [IQR]) survival times were 12.5 (2-195) days for trisomy 13 and 9 (2-92) days for trisomy 18. Mean 1-year survival for trisomy 13 was 19.8% (95% CI, 14.2%-26.1%) and 12.6% (95% CI, 8.9%-17.1%) for trisomy 18. Ten-year survival for trisomy 13 was 12.9% (95% CI, 8.4%-18.5%) and 9.8% (95% CI, 6.4%-14.0%) for trisomy 18. Survival did not change over the study period. Forty-one children (23.6%) with trisomy 13 and 35 children (13.8%) with trisomy 18 underwent surgeries, ranging from myringotomy to complex cardiac repair. Median age at first surgery for trisomy 13 was 92 (IQR, 30.5-384.5) days and for trisomy 18, it was 205.5 (IQR, 20.0-518.0) days. Kaplan-Meier curves showed 1-year survival after first surgery of 70.7% (95% CI, 54.3%-82.2%; n = 23) for trisomy 13 and 68.6% (95% CI, 50.5%-81.2%; n = 29) for trisomy 18. CONCLUSIONS AND RELEVANCE: Among children born with trisomy 13 or 18 in Ontario, early mortality was the most common outcome, but 10% to 13% survived for 10 years. Among children who underwent surgical interventions, 1-year survival was high.

Family Experiences With Feeding Tubes in Neurologic Impairment: A Systematic Review.

BACKGROUND AND OBJECTIVE: Gastrostomy tubes (G-tubes) are frequently used to provide enteral nutrition for children who have neurologic impairment. Understanding the impact of G-tubes from the family's perspective will inform decision-making and improve support from health care providers. This study explored the experiences of families after G-tube placement in children with neurologic impairment. METHODS: We conducted a systematic review of English-language qualitative primary research studies describing family experiences after G-tube placement. Six electronic databases were searched from inception to June 2014. Two authors independently screened and identified relevant studies, evaluated quality of reporting by using the Consolidated Criteria for Reporting Qualitative Research tool, and extracted data. Overarching concepts were developed by using thematic analysis. RESULTS: From 2674 screened abstracts, 84 texts were reviewed, and 13 studies met the inclusion criteria. G-tubes affect the lives of children, parents, and the family unit in many ways, both positive and negative. Improvements and challenges were described for children's health and happiness, for parental caregiving and stress, and for logistics and bonding within the family. G-tube feeding also changed relationships within the family, between the family and the medical system, and between the family and the outside world. Furthermore, experiences varied, with different families framing similar concepts as positive and negative. CONCLUSIONS: G-tube placement has diverse effects on daily life for children with neurologic impairment and their families. Clinicians may use the themes identified in this study to guide conversations with families about their values, experiences, and expectations before and after G-tube placement.

Shared decision-making about assistive technology for the child with severe neurologic impairment.

Shared decision-making is a process that helps frame conversations about value-sensitive decisions, such as introduction of assistive technology for children with neurologic impairment. In the shared decision-making model, the health care provider elicits family values relevant to the decision, provides applicable evidence in the context of those values, and collaborates with the family to identify the preferred option. This article outlines clinical, quality of life, and ethical considerations for shared decision-making discussions with families of children with neurologic impairment about gastrostomy tube and tracheostomy tube placement.