Disaster Preparedness in Families With Children With Special Health Care Needs.

Children with special health care needs (CSHCN) may present unique challenges for disaster preparedness. This study's objective was to determine the impact of a disaster supply starter kit intervention on preparedness for families of CSHCN. The study was a 1-group pre-post cohort design with consecutive enrollment at the Arkansas Children's Hospital Medical Home Clinic. Pre- and postintervention survey findings were compared using McNemar's test. Of the 249 enrolled, 223 completed the postsurvey. At presurvey, 43% had an Emergency Information Form, compared with 79% at postsurvey (P < .001). At presurvey, 18% had a disaster kit, compared with 99.6% at postsurvey, and 44% added items. Of the 183 respondents who did not have a disaster kit at presurvey, 99% (n = 182) had a disaster kit on postsurvey, and 38% (n = 70) added items. An inexpensive educational disaster supply starter kit may increase preparedness. Further investigation on sustainability and dissemination to other populations is needed.

Evaluation of Anaphylaxis Management in a Pediatric Emergency Department.

OBJECTIVE: In 2006, the National Institute of Allergy and Infectious Disease established evidence-based treatment guidelines for anaphylaxis. The purpose of our study was to evaluate provider adherence to guidelines-based management for anaphylaxis in a tertiary care pediatric emergency department (ED). METHODS: Retrospective chart review was conducted of patients (0-18 years) presenting to the Arkansas Children Hospital ED from 2004 to 2011 for the treatment of anaphylaxis using International Classification of Diseases, Ninth Edition, codes. Multiple characteristics including demographics, clinical features, allergen source, and anaphylaxis management were collected. Fisher exact or χ tests were used to compare proportion of patients treated with intramuscular (IM) epinephrine in the preguideline versus postguideline period. Relative risk (RR) statistics were computed to estimate the ratio of patients who received self-injectable epinephrine prescription and allergy follow-up in the preguideline and postguideline groups. RESULTS: A total of 187 patients (median [range] age, 7 [1-18] years; 67% male; 48% African American) were evaluated. Food (44%) and hymenoptera stings (22%) were commonly described culprit allergens, whereas 29% had no identifiable allergen. Only 47% (n = 87) received epinephrine in the ED and 31% (n = 27) via the preferred IM route. Comparing postguideline (n = 126) versus preguideline (n = 61) periods demonstrated increase in the usage of the IM route (46% postguideline vs 6% preguideline; risk ratio (RR), 7.64; 95% confidence interval [CI], 2.04-46.0; P < 0.001). Overall, 61% (n = 115) of the patients received self-injectable epinephrine upon discharge, and there were no significant differences between the groups (64% postguideline vs 56% preguideline, P = 0.30). Postguideline patients were more likely to receive a prescription compared with preguideline patients (64% postguideline vs 56% preguideline; RR, 1.15; 95% CI, 0.89-1.55; P = 0.30). Only 45% (n = 85) received an allergy referral. Postguideline patients were more likely to receive an allergy referral than preguideline patients (48% postguideline vs 41% preguideline; RR, 1.16; 95% CI, 0.81-1.73; P = 0.40). CONCLUSIONS: Provider use of IM epinephrine has improved since anaphylaxis guidelines were published. However, more provider education is needed to improve overall adherence of guidelines in a tertiary care pediatric ED.

Clinical characteristics and epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) in children with cystic fibrosis from a center with a high MRSA prevalence.

BACKGROUND: We describe the clinical characteristics and epidemiology of methicillin-resistant Staphylococcus aureus (MRSA) in children with cystic fibrosis (CF) from the U.S. CF center with the highest MRSA prevalence. METHODS: Medical records of children with CF were retrospectively reviewed from 1997-2009. MRSA clinical isolates from 2007-2009 were analyzed by polymerase chain reaction and pulsed field gel electrophoresis. RESULTS: The prevalence of MRSA was 1% in 1997 and 49% in 2009. Fifty-five children (26%) had persistent MRSA infection. Sixty-eight percent of MRSA isolates were hospital-associated (HA) MRSA, of which 52% were pulsed-field type USA 100. Ninety-three percent of HA MRSA isolates were clindamycin resistant. Twelve children acquired MRSA before 1 year of age, 83% of whom were hospitalized prior to acquisition of MRSA. Ten of 11 sibling pairs carried indistinguishable MRSA strains. Children with persistent MRSA were hospitalized more often (P = .01), required inhaled medications more frequently (P = .01), and had higher rates of Pseudomonas aeruginosa coinfection (P < .001). CONCLUSION: MRSA prevalence in children with CF is increasing, and most children are infected with HA MRSA. Exposure to health care facilities and gastrointestinal surgeries may facilitate early acquisition of MRSA. Siblings carry indistinguishable MRSA strains, indicating household transmission of MRSA. Children with persistent MRSA had worse pulmonary morbidity. Coinfection with MRSA and P aeruginosa is likely associated with further increased pulmonary morbidity.

Cerebral oximetry with cerebral blood volume index in detecting pediatric stroke in a pediatric ED.

BACKGROUND: Despite pediatric stroke awareness and pediatric stroke activation systems, recognition and imaging delays along with activation inconsistency still occur. Reliable objective pediatric stroke detection tools are needed to improve detection and activations. Regional cerebral oxygen saturation (rcso2) with cerebral blood volume index (CBVI) can detect abnormal cerebral physiology. OBJECTIVE: To determine cerebral oximetry in detecting strokes in stroke alert and overall stroke patients. METHOD: Left rcso2, right rcso2, and rcso2 side differences for stroke, location, and types were analyzed. RESULTS: Compared with stroke alert (n = 25) and overall strokes (n = 52), rcso2 and CBVI were less than those in nonstrokes (n = 133; P < .0001). Rcso2 side differences in stroke alert and overall strokes were greater than in nonstrokes (P < .0001). Lower rcso2 and CBVI correlated with both groups' stroke location, left (P < .0001) and right rcso2 (P = .004). Rcso2 differences greater than 10 had a 100% positive predictive value for stroke. Both groups' rcso2 and CBVI side differences were consistent for stroke location and type (P < .0001). For both groups, left rcso2 and CBVI were greater than those of the right (P < .0001). Hemorrhagic strokes had lower bilateral rcso2 and CBVI than did ischemic strokes (P < .001). CONCLUSIONS: Cerebral oximetry and CBVI detected abnormal cerebral physiology, stroke location, and type (hemorrhagic or ischemic). Rcso2 side differences greater than 10 or rcso2 readings less than 50% had a 100% positive predictive value for stroke. Cerebral oximetry has shown potential as a detection tool for stroke location and type in a pediatric stroke alert and nonalert stroke patients. Using cerebral oximetry by the nonneurologist, we found that the patient's rcso2 side difference greater than 10 or one or both sides having less than 50% rcso2 readings suggests abnormal hemispheric pathology and expedites the patient's diagnosis, neuroresuscitation, and radiologic imaging.

Goal-Directed Resuscitative Interventions During Pediatric Interfacility Transport.

OBJECTIVES: This article reports results of the first National Institutes of Health-funded prospective interfacility transport study to determine the effect of goal-directed therapy administered by a specialized pediatric team to critically ill children with the systemic inflammatory response syndrome. We hypothesized that goal-directed therapy during interfacility transport would decrease hospital length of stay, prevent multiple organ dysfunction, and reduce subsequent ICU interventions. DESIGN: Before-and-after intervention trial. SETTING: During interfacility transport of critically ill patients by a specialized pediatric transport team, back to a tertiary care children's hospital. PATIENTS: Before-and-after intervention trial. DESIGN: Interfacility pediatric transport patients, age 1 month to 17 years, with systemic inflammatory response syndrome. INTERVENTIONS: Prospective data were collected on all pediatric interfacility transport patients with systemic inflammatory response syndrome transported by the Angel One Transport team at Arkansas Children's Hospital. A 10-month data collection period was followed by institution of a goal-directed resuscitation protocol. Data were subsequently collected for 10 additional months followed by comparison of pre- and postintervention groups. All transport personnel underwent training with didactics and high-fidelity simulation until mastery with goal-directed resuscitation was achieved. MEASUREMENTS AND MAIN RESULTS: All transport patients were screened for systemic inflammatory response syndrome using established variables and 235 (123 preintervention and 112 postintervention) were enrolled. Univariate analysis revealed shorter hospital stay (11 ± 15 d vs 7 ± 10 d; p = 0.02) and fewer required therapeutic ICU interventions in the postintervention group (Therapeutic Intervention Scoring System-28 Scores, 19.4 ± 6.8 vs 17.3 ± 6.6; p = 0.04). ICU stay and prevalence of organ dysfunction were not statistically different. Multivariable analysis showed a 1.6-day (95% CI, 1.3-2.03; p = 0.02) decrease in hospital stay in the postintervention group. CONCLUSIONS: This study suggests that goal-directed therapy administered by a specialized pediatric transport team has the potential to impact the outcomes of critically ill children. Findings from this study should be confirmed across multiple institutions, but have the potential to impact the clinical outcomes of critically ill children with systemic inflammatory response syndrome.

Variation in child health care utilization by medical complexity.

Children with medical complexity (CMC) have multiple specialty need, technology dependence, and high health care utilization. The objective of this study is to profile types of pediatric health care utilization and costs by increasing levels of medical complexity. This is a cross-sectional study of the 2007, 2008 and 2009 Full-Year Data Sets from the Medical Expenditure Panel Survey. Medical complexity was defined by a higher number of positive items from the five question children with special health care needs (CSHCN) Screener. CMC were defined by ≥ 4 positive screener items. Outcomes included the number of inpatient, outpatient, and emergency department visits, associated costs and diagnoses, and reported satisfaction. ICD-9 codes were grouped by Clinical Classifications Software. Of 27,755 total study subjects ≤ 17 years, 4,851 had special needs and 541 were CMC. Older age, male gender, white/non-Hispanic race/ethnicity, and public insurance were all associated with medical complexity (all p < 0.001). CMC had an annual mean of 19 annual outpatient visits ($616) and 0.26 inpatient visits ($3,308), with other significant cost drivers including home health ($2,957) and prescriptions ($2,182). The most common reasons for non-CSHCN and less-complex CSHCN outpatient visits were viral illnesses, while the main reasons for CMC visits were for mental health. Compared to families without CSHCN, those with CMC have, on average, lower satisfaction with health care (8.4 vs. 8.9 out of 10, p < 0.001). Health care models for CMC should account for mental health conditions that may be driving high numbers of outpatient encounters.

Outcomes of children treated with tracheostomy and positive-pressure ventilation at home.

BACKGROUND: Long-term outcomes for children who survive on tracheostomy and positive-pressure ventilation (TPPV) at home are not well known. METHODS: A retrospective review of 20 years of clinical data at a single institution was performed. Outcome measures included 5-year survival, decannulation rate, and neurocognition. RESULTS: A total of 91 children were categorized under neuromotor dysfunction (52%), chronic lung disease (29%), and congenital anomalies (20%). The 5-year survival rates for these categories were 89% (95% confidence interval [CI] = 80%-99%), 76% (95% CI = 57%-100%), and 94% (95% CI = 83%-100%), respectively. Overall, the 5-year decannulation rate was 25% (95% CI = 14%-35%), with children with chronic lung disease having the highest rate (51%). It was found that 14% were extremely delayed in neurocognition. CONCLUSION: Most children on TPPV at home survive beyond 5 years, and a significant number are decannulated. Primary care physicians and communities should be prepared to accommodate the increasing number of children on TPPV at home.

Pharmacogenetic predictors of methylphenidate dose-response in attention-deficit/hyperactivity disorder.

OBJECTIVE: Because of significant individual variability in attention-deficit/hyperactivity disorder (ADHD) medication response, there is increasing interest in identifying genetic predictors of treatment effects. This study examined the role of four catecholamine-related candidate genes in moderating methylphenidate (MPH) dose-response. METHOD: Eighty-nine stimulant-naive children with ADHD 7 to 11 years old participated in a randomized, double-blind, crossover trial of long-acting MPH. Parents and teachers assessed each child's response on placebo and three MPH dosage levels using the Vanderbilt ADHD rating scales. Children were genotyped for polymorphisms in the 3' untranslated region of dopamine transporter (DAT), exon 3 on dopamine receptor D(4) (DRD4), codon 158 on catechol-O-methyltransferase, and the adrenergic α(2A)-receptor promoter. Linear mixed models evaluated gene, dose (milligrams per kilogram per day), and gene-by-dose effects on inattentive and hyperactive-impulsive domain outcomes. RESULTS: The most statistically significant gene-by-dose interactions were observed on hyperactive-impulsive symptoms for DRD4 and DAT polymorphisms, with participants lacking the DAT 10-repeat allele showing greater improvements in symptoms with increasing dose compared with 10-repeat carriers (p = .008) and those lacking the DRD4 4-repeat allele showing less improvement across MPH doses compared with 4-repeat carriers (p = 0.02). CONCLUSIONS: This study suggests that DAT and DRD4 polymorphisms may be associated with individual variability in MPH dose-response, although further research in larger samples is required to confirm these findings and their clinical utility. CLINICAL TRIAL REGISTRATION INFORMATION: Response Variability in Children with Attention-Deficit/Hyperactivity Disorder (ADHD); http://www.clinicaltrials.gov; NCT01238822.

Phthalate exposure and precocious puberty in females.

OBJECTIVE: To determine whether phthalate exposure is associated with precocious puberty in girls. STUDY DESIGN: This was a multicenter cross-sectional study in which 28 girls with central precocious puberty (CPP) and 28 age- and race-matched prepubertal females were enrolled. Nine phthalate metabolites and creatinine were measured in spot urine samples from these 56 children. RESULTS: Levels of 8 of the 9 phthalate metabolites were above the limit of detection (LOD) in all 56 subjects. Mono (2-ethylhexyl) phthalate (MEHP) was below the LOD in 25/56 samples (14 subjects with precocious puberty and 11 controls). No significant differences between the children with CPP and the controls in either absolute or creatinine-normalized concentrations of any of the 9 phthalate metabolites were measured. CONCLUSIONS: Although phthalates may be associated with certain other toxicities in humans, our study suggests that their exposure is not associated with precocious puberty in female children.