Modifiable factors related to transition readiness in adolescents and young adults with epilepsy.

OBJECTIVE: To characterize transition readiness in adolescents and young adults (AYAs) with epilepsy and validate the social-ecological model of AYA readiness to transition (SMART) in a sample of AYAs with epilepsy. METHODS: This cross-sectional study included typically developing youth with epilepsy 13-25 years old seen in a comprehensive epilepsy center. Adolescents and young adults completed measures of transition readiness (Transition Readiness Assessment Questionnaire; TRAQ), epilepsy knowledge, epilepsy self-management, developmental factors, and emotional and behavioral functioning. Adolescents and young adults also completed a measure of their relationship quality with healthcare providers. Caregiver report was included when available. RESULTS: Participants included 82 AYAs (Mage = 17.3 ±â€¯2.8; 86.6% White Non-Hispanic, 53.7% females) with epilepsy. Transition Readiness Assessment Questionnaire scores (M = 3.33, SD = 0.86) were correlated with modifiable and non-modifiable factors: age (r = 0.66, p < 0.001), income (r = -0.23, p = 0.04), AYA cognitive problems (r = 0.24, p = 0.03), AYA knowledge (r = 0.31, p = 0.005), AYA expectations (r = 0.26, p ≤ 02), AYA inattention (r = -0.24, p = 0.03), AYA executive dysfunction (r = 0.25, p = 0.02), caregiver-reported AYA odd behaviors (r = -0.25, p = 0.036), and caregiver-reported AYA communication problems (r = 0.25, p = 0.04). Transition Readiness Assessment Questionnaire scores were higher in AYAs who had seen adult providers for general healthcare issues (e.g., primary care), but were not related to other demographic (e.g., minority status, insurance), medical (years since diagnosis, type of epilepsy, polytherapy, seizure frequency), developmental (e.g., adaptive skills, cognitive functioning) or emotional/behavioral factors (e.g., skills, relationship with the provider, psychosocial functioning). Linear regression including variables significantly correlated with the outcome of transition readiness (F (7, 59) = 9.70, p < 0.001) explained 54% of the variance. Specifically, age was the only significant model predictor. SIGNIFICANCE: Transition readiness in AYAs with epilepsy was predicted by non-modifiable (e.g., age) and correlated with modifiable factors (e.g., knowledge, psychosocial/cognitive functioning). Providers must better prepare patients prior to transfer, and future research should use an epilepsy-specific measure of transition readiness to identify targets for intervention.

Quality of life improves with integrated behavioral health services in pediatric new-onset epilepsy.

The current study compared differences in health-related quality of life (HRQOL) between youth with new-onset epilepsy with and without elevated psychological symptoms at time of epilepsy diagnosis within an integrated behavioral health and epilepsy service. Patients received both behavioral health and epilepsy care during clinic visits. A retrospective chart review was conducted between July 2011 and December 2015. Caregivers completed the Behavior Assessment System for Children-2: Parent Rating Scale (BASC-2: PRS) to assess psychological symptoms at the diagnostic visit, along with completing the Pediatric Quality of Life Inventory (PedsQL™ 4.0) at the diagnostic visit and each subsequent epilepsy clinic visit during the first year of treatment. Latent growth curve modeling was used to identify HRQOL changes over the first year of treatment. Health-related quality of life was significantly lower for youth with elevated psychological symptoms at diagnosis and over the first year of treatment compared with those without psychological symptoms. For those with elevated internalizing, inattention, withdrawal, and atypical symptoms at diagnosis, greater HRQOL improvements were detected over the first year of treatment compared with those without elevated psychological symptoms at the diagnostic visit. Within integrated behavioral health and epilepsy routine care, targeted psychological interventions can improve HRQOL over the first year of treatment, particularly for those with premorbid psychological symptoms.

Antiepileptic drug behavioral side effects and baseline hyperactivity in children and adolescents with new onset epilepsy.

OBJECTIVE: To examine baseline psychological functioning and antiepileptic drug (AED) behavioral side effects in new onset epilepsy and determine, by age, whether baseline psychological functioning predicts AED behavioral side effects 1 month following AED initiation. METHODS: A retrospective chart review was conducted between July 2011 and December 2014 that included youths with new onset epilepsy. As part of routine interdisciplinary care, caregivers completed the Behavior Assessment System for Children, 2nd Edition: Parent Rating Scale to report on baseline psychological functioning at the diagnostic visit and the Pediatric Epilepsy Side Effects Questionnaire to identify AED behavioral side effects at the 1-month follow-up clinic visit following AED initiation. Children (age = 2-11 years) and adolescents (age = 12-18 years) were examined separately. RESULTS: A total of 380 youths with new onset epilepsy (Mage  = 8.9 ± 4.3 years; 83.4% Caucasian; 34.8% focal epilepsy, 41.1% generalized epilepsy, 23.7% unclassified epilepsy) were included. Seventy percent of youths had at-risk or clinically elevated baseline psychological symptoms. Children had significantly greater AED behavioral side effects (M = 25.08 ± 26.36) compared to adolescents (M = 12.36 ± 17.73), regardless of AED. Valproic acid demonstrated significantly greater behavioral side effects compared to all other AEDs, with the exception of levetiracetam. Higher hyperactivity/impulsivity at baseline significantly predicted higher AED behavioral side effects 1 month after AED initiation in both age groups. SIGNIFICANCE: Younger children seem to be more prone to experience behavioral side effects, and these are likely to be higher if youths with epilepsy have baseline hyperactivity/impulsivity. Baseline psychological screening, specifically hyperactivity, can be used as a precision medicine tool for AED selection.

Validation of the PedsQL Epilepsy Module: A pediatric epilepsy-specific health-related quality of life measure.

OBJECTIVE: To validate a brief and reliable epilepsy-specific, health-related quality of life (HRQOL) measure in children with various seizure types, treatments, and demographic characteristics. METHODS: This national validation study was conducted across five epilepsy centers in the United States. Youth 5-18 years and caregivers of youth 2-18 years diagnosed with epilepsy completed the PedsQL Epilepsy Module and additional questionnaires to establish reliability and validity of the epilepsy-specific HRQOL instrument. Demographic and medical data were collected through chart reviews. Factor analysis was conducted, and internal consistency (Cronbach's alphas), test-retest reliability, and construct validity were assessed. RESULTS: Questionnaires were analyzed from 430 children with epilepsy (Mage = 9.9 years; range 2-18 years; 46% female; 62% white: non-Hispanic; 76% monotherapy, 54% active seizures) and their caregivers. The final PedsQL Epilepsy Module is a 29-item measure with five subscales (i.e., Impact, Cognitive, Sleep, Executive Functioning, and Mood/Behavior) with parallel child and caregiver reports. Internal consistency coefficients ranged from 0.70-0.94. Construct validity and convergence was demonstrated in several ways, including strong relationships with seizure outcomes, antiepileptic drug (AED) side effects, and well-established measures of executive, cognitive, and emotional/behavioral functioning. SIGNIFICANCE: The PedsQL Epilepsy Module is a reliable measure of HRQOL with strong evidence of its validity across the epilepsy spectrum in both clinical and research settings.

Understanding adherence to noninvasive ventilation in youth with Duchenne muscular dystrophy.

Duchenne muscular dystrophy (DMD) is an X-linked, progressive neuromuscular disorder that results in chronic respiratory insufficiency and subsequently failure requiring noninvasive ventilation (NIV). Adherence to NIV in neuromuscular disorders and related barriers are poorly described. The aim of the current study was to assess NIV adherence, adherence barriers, and identify psychosocial predictors of adherence in young boys with early DMD-related sleep disordered breathing and recommended nocturnal NIV. This cross-sectional study included 42 youth with DMD with prescribed nocturnal NIV, and their caregivers. Caregivers and youth completed questionnaires assessing adherence barriers, psychosocial symptoms (eg, anxiety and depressive symptoms), and stress. Medical information pertinent to cardiopulmonary health and neurologic status at both enrollment and initiation of NIV was reviewed. Adherence to NIV, defined as percent days used and days used ≥4 hours/day was 56.1 ± 38.7% and 46.2 ± 40.6%, respectively. Average duration of use on days worn was 5.61 ± 4.23 hours. NIV usage was correlated with the severity of obstructive sleep apnea but not cardiopulmonary variables. Mask discomfort was the most commonly reported adherence barrier followed by behavioral barriers (eg, refusing to use). Multiple regression analyses revealed that internalizing behaviors (eg, anxiety and depressive symptoms) and total adherence barriers significantly predicted NIV adherence. Adherence to NIV in DMD is poor and similar to other pediatric chronic diseases. Our data suggest interventions targeting adherence barriers and patient internalizing symptoms may improve adherence to NIV in DMD.