Stress and the Home Environment in Caregivers of Children with Sickle Cell.

OBJECTIVE: Caregivers of young children with chronic illnesses are at high risk for elevated levels of stress and mental health symptoms. This study examined stress and mental health symptoms as well as the socioeconomic status (SES) and home environments of a cohort of caregivers of infants and toddlers with sickle cell disease (SCD). METHODS: Forty-two caregivers of infants and toddlers (aged 1-34 months) with SCD completed the Brief Symptom Inventory (BSI) and Parent Stress Index (PSI). The Home Observation for Measurement of the Environment (HOME) was used to assess family living environments. RESULTS: Compared to test norms, caregivers reported high levels of situational/demographic life stress [mean difference (MD) 5.7, p = .003] and child distractibility/hyperactivity (MD 3.62, p = .001) on the PSI. However, no significant differences in psychological symptoms of distress were noted on the BSI. Caregivers scored significantly lower than norms on PSI subdomains of acceptability (MD -1.88, p = .03), competence (MD -3.11, p = .002), depression (MD -3.94, p < .001), and the overall parent domain (MD -12.55, p = .005). Significant correlations were found between PSI scores and the HOME and between SES and the HOME. CONCLUSION: Caregivers of infants and toddlers with SCD experience elevated levels of life stress but, in turn, endorse high acceptance of their child and self-competence in parenting. Although life stress may be high in this population, symptoms of psychological distress were not identified. Caregivers reporting elevated life and illness-specific stressors may benefit from environmental supports and interventions.

Validation of the fine motor subtest of the Bayley-III with children with sickle cell disease using Rasch analysis.

BACKGROUND: Children with sickle cell disease (SCD) are at risk for fine motor (FM) delays; however, screening for FM impairments is not common among young children with SCD. The Bayley Scales of Infant and Toddler Development, Third Edition (Bayley-III) is the most commonly used performance-based developmental assessment. We aim to determine if the FM subtest of the Bayley-III is structured hierarchically in accordance with development and comprehensively evaluates FM development in children with SCD. METHODS: Bayley-III assessments were completed between October 2009 and December 2013. The Bayley-III FM screening test, a shorter and more rapid method of assessing for FM impairments, was not directly administered to participants. Screening test scores were calculated from full Bayley-III scores. RESULTS: Rasch analysis was performed using WINSTEPS. Sixty children with SCD were included in the final Rasch model. The Rasch-generated Wright map, which jointly positions items and persons on the same latent trait, illustrated that the FM items were slightly skewed towards more challenging items, indicating more difficult items may be overrepresented. High item separation values were reported (17.4), and item outfit statistics were less than 1.7. More than one third of items demonstrated overfit, indicating possible item redundancy. The FM subtest and the screening test, a shorter and faster method of assessing skills, were highly correlated (r = 0.993, p < 0.001). CONCLUSION: The Bayley-III FM subtest is structured hierarchically, aligning with motor development, and comprehensively evaluates FM development in children with SCD. The test could be improved by reordering items, removing overfitting items and modifying screening test items to capture all ranges of development. The screening test is comprehensive and has high potential clinical utility among children with SCD.

Implementation of an educational intervention to optimize self-management and transition readiness in young adults with sickle cell disease.

BACKGROUND: The transition from the pediatric setting to adult care is a well-described period of morbidity and mortality for persons with sickle cell disease (SCD). We sought to measure the feasibility and effectiveness of providing skill-based educational handouts on improving self-management and transition readiness in adolescents with SCD. METHODS: This was a single-center study in which participants completed a self-assessment, the Adolescent Autonomy Checklist (AAC), to assess transition readiness and self-management skills at baseline. After results were reviewed by the study coordinator, participants were provided with skill-based handouts on noted areas of deficit. The AAC was subsequently completed at a follow-up visit. All data were stored electronically and transferred into SAS for statistical analyses. RESULTS: Sixty-one patients completed the AAC at baseline and postintervention. At baseline, patients reported needing the most help with skills in money management, living arrangements, vocational skills, and emergency and healthcare skills. Postintervention, statistically significant improvements (P < 0.05) occurred in skills related to laundry, housekeeping, healthcare, and sexual development. A regression model exploring the time to follow-up showed that most improvements could not be attributed to maturation alone. CONCLUSION: This study showed that educational handouts are a readily implementable and well-accepted intervention among adolescents with SCD who identify challenges with skills necessary to successfully transition to adult care. Distinguishing which transition needs are best improved with this type of intervention will help to strengthen the multidisciplinary approach necessary to support adolescents and young adults with SCD as they matriculate to adult care.

Transition Needs of Adolescents With Sickle Cell Disease.

OBJECTIVE: This article describes how adolescents with sickle cell disease (SCD) perceive their ability to perform everyday tasks required for transition to adult health care and independent living. METHOD: The Adolescent Autonomy Checklist (AAC) was adapted to include skills associated with managing SCD (AAC-SCD) and was administered to adolescents during clinic visits. Participants indicated "can do already" or "needs practice" for 100 activities in 12 categories. RESULTS: Of 122 patients, the percentage of adolescents who needed practice was greatest in living arrangements (38.7%), money management (35.8%), vocational skills (29.6%), and health care skills (25.5%). We found a significant effect of age and of cerebrovascular injury on the percentage of those who reported "needs practice" in multiple categories. We found no effect of gender and limited effect of hemoglobin phenotype on any skill category. CONCLUSION: Findings support the need for educational intervention to improve transition skills in adolescents with SCD.

Activity competence among infants and toddlers with developmental disabilities: Rasch analysis of the Infant Toddler Activity Card Sort (ITACS).

BACKGROUND: Development is rapid in the first years of life. Developmental delays appearing during this critical period have the potential to persist throughout the child's life. Available standardized assessments for this age record a child's ability to successfully complete discrete skills but fail to capture whether the child incorporates those skills into daily routines that are meaningful to the child and family. The Infant Toddler Activity Card Sort (ITACS) is a newly developed photograph-based early intervention tool to measure the participation-related concept of activity competence using caregiver report. The purpose of the present study was to use Rasch analysis to determine if ITACS items comprehensively measure the construct of child activity competence. RESULTS: A total of 60 child/caregiver dyads participated. The dichotomous caregiver-reported responses (present vs. absent) on the 40 individual ITACS items were used in Rasch analysis, and three iterations of the model were completed. The final model included 51 child/caregiver dyads and 67 ITACS assessments with a good spread of individual ability measure (6.47 logits). All items demonstrated adequate infit except for "sleeping" (range 0.68-1.54). Five items (sleeping, eating at restaurants, brushing teeth, crawling, and interact with pets) demonstrated high Mean Square (MNSQ) outfit statistics and one (take a bath) demonstrated low MNSQ outfit. ITACS items demonstrated a good spread of item difficulty measures (6.27 logits), and a clear ceiling was observed. Three activity items (smiling, breastfeeding, and playing with adults) were rarely endorsed as concerns. The activities most likely to be reported as challenging were "crying/communicating" and "going to school". Person and item reliability statistics were adequate (0.79 and 0.80, respectively). The separation between individuals and between items were adequate to good (1.96 and 1.99, respectively). CONCLUSIONS: Findings indicate that ITACS items are measuring a unidimensional construct--activity competence in early childhood. The Rasch analysis of caregiver responses suggest that some activities are more likely to be considered challenging and may be important targets for intervention. These results provide evidence to further validate the ITACS as a caregiver report measure and support its use in the early intervention setting to facilitate caregiver driven goal development.

Psychometric Properties of the Infant Toddler Activity Card Sort.

The Infant Toddler Activity Card Sort (ITACS) is a new measure that engages caregivers in goal development by measuring participation in early childhood. Initial evaluation of the ITACS is required to assess its psychometric properties. To evaluate test-retest reliability, concurrent validity, and between-group differences of the ITACS. Caregivers (N = 208) of children 0-3 years, who were typically developing (TD) or had a developmental delay (DD), completed the ITACS and Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT) with repeat assessment after 2 weeks later (n = 117). The ITACS demonstrated moderate concurrent validity with the PEDI-CAT. Children with DD had significantly more activity concerns on the ITACS than TD. Adequate test-retest reliability was not achieved. The ITACS is a useful tool for identifying caregiver concerns in activity engagement; interventions can be tailored to address concerns and increase participation in childhood routines.

The Infant Toddler Activity Card Sort: A Caregiver Report Measure of Children's Occupational Engagement in Family Activities and Routines.

Early intervention (EI) therapy services aim to address family-centered goals to facilitate young children's development and participation in meaningful life activities. Current methods to evaluate children in EI assess discrete developmental skills but provide little information on the child's ability to successfully incorporate that skill in everyday life. Furthermore, traditional measures have limited opportunity for parent report, despite parent engagement being a critical component for successful implementation of EI services. A measure that involves parents in the therapeutic process to track family-driven EI goals related to a child's occupational engagement in meaningful family activities and routines is desperately needed. Using a qualitative design and conventional content analysis, we identified 41 distinct activities of infant-toddlers from a cohort of 23 caregivers. Activity items were matched with photographs and validated with experts in pediatric rehabilitation using the Delphi method. The resulting 40 activity/photograph pairs were used to develop a new measure of pediatric occupational engagement, the Infant Toddler Activity Card Sort (ITACS).

Increasing Educational Attainment in Adolescents with Sickle Cell Disease.

Adolescents with sickle cell disease (SCD), a chronic condition primarily impacting African Americans, experience challenges graduating high school. Understanding demographic, educational, and community-level correlates of disease is critical to creating effective interventions for these youths. This study aimed to examine 1) educational attainment for adolescents with SCD, 2) neighborhood correlates of their education, and 3) feasibility of a pilot to increase General Education Diploma (GED) class enrollment. Findings suggest demographic characteristics influence educational attainment. Improving educational attainment in adolescents with SCD requires understanding risk beyond disease severity. Identifying risk and protective neighborhood-level factors can inform interventions to improve educational attainment. Feasibility of programming to increase GED enrollment should be further investigated.

Effects of labor contractions on catecholamine release and breathing frequency in newborn rats.

Plasma catecholamines in newborn rats (0-2 hr old) were analyzed following vaginal birth, cesarean section with simulated labor contractions, or cesarean section without labor contractions. Upon delivery, pups were exposed to key elements of the rat's natural birth process, that is, umbilical cord occlusion, tactile stimulation, and cooling. Only pups exposed to actual or simulated labor showed an immediate rise in norepinephrine and epinephrine. Initial postpartum respiratory frequencies were higher in vaginal than in cesarean delivered pups and, in all groups, inversely correlated with catecholamine titers, suggesting respiratory distress or transient tachypnea at lower catecholamine levels. These findings establish a rat model for analyzing effects of labor on neonatal adaptive response during the transition from prenatal to postnatal life.

Feasibility of a Community-Based Sickle Cell Trait Testing and Counseling Program.

BACKGROUND: Sickle cell trait (SCT) screening is required at birth in the United States; however, adults rarely know their SCT status prior to having children. PURPOSE: Assess feasibility of a community-based SCT education and testing intervention. METHODS: Participants were recruited from eight community sites to complete an educational program and offered a hemoglobin analysis. A genetic counselor met individually with participants to discuss lab results. RESULTS: Between July 14, 2010 and May 31, 2012, 637 participants completed the educational program. Five hundred seventy (89.5%) provided a blood sample, and 61 (10.9%) had SCT or other hemoglobinopathies. The genetic counselor met with 321 (56.3%) participants. CONCLUSIONS: Community-based SCT testing shows initial feasibility and may increase the number of individuals who know their trait status.

Youth with Sickle Cell Disease: Genetic and Sexual Health Education Needs.

OBJECTIVES: Assess the need for and interest in a sexual health and sickle cell disease (SCD) inheritance educational program for youth with SCD. METHODS: Using a cross-sectional approach, qualitative data were collected during interviews of youth with SCD between ages 11-19 years from an urban hospital. Inductive and emergent coding was used to identify themes. Frequencies were recorded and analyzed. RESULTS: Thirty-five youth patients were approached; 20 (57%) consented and completed interviews. Half of the participants were adolescent girls. The mean age was 16.9±1.8 years (range 13-19). Of the 20 participants, 100% expressed interest in SCD genetic counseling and/or posed questions regarding SCD inheritance. Nineteen (95%) demonstrated deficits in sexual health knowledge and/or requested sexual health education. Seventeen (85%) actively engaged with educational materials. Twelve (60%) reported engaging in sexual activity, 7 (35%) in risky behaviors, and 3 (15%) reported past sexually transmitted infection (STI) diagnosis. CONCLUSIONS: Youth with SCD demonstrated limited knowledge regarding sexual health and inheritance of SCD. Topics like information-seeking, limited knowledge, and need for social skills training provide evidence for the creation of an educational intervention for this vulnerable population. This program shows initial feasibility for youth with SCD.

Maternal anesthesia via isoflurane or ether differentially affects pre-and postnatal behavior in rat offspring.

Our understanding of prenatal behavior has been significantly advanced by techniques for direct observation and manipulation of unanesthetized, behaving rodent fetuses with intact umbilical connections to the mother. These techniques involve brief administration of an inhalant anesthesic, enabling spinal transection of the rat or mouse dam, after which procedures can continue with unanesthetized dams and fetuses. Because anesthetics administered to the mother can cross the placental barrier, it is possible that fetuses are anesthetized to varying degrees. We compared in perinatal rats the effects of prenatal maternal exposure to two inhalant anesthetics: ether and isoflurane. Fewer spontaneous fetal movements and first postpartum nipple attachments were observed following maternal exposure to ether as compared to isoflurane. Neonatal breathing frequencies and oxygenation did not account for group differences in nipple attachment. Our results provide evidence that the particular inhalant anesthetic employed in prenatal manipulation studies determines frequencies of perinatal behavior.

Hippocampal and visuospatial learning defects in mice with a deletion of frizzled 9, a gene in the Williams syndrome deletion interval.

Wnt signaling regulates hippocampal development but little is known about the functions of specific Wnt receptors in this structure. Frizzled 9 is selectively expressed in the hippocampus and is one of about 20 genes typically deleted in Williams syndrome. Since Williams syndrome is associated with severe visuospatial processing defects, we generated a targeted null allele for frizzled 9 to examine its role in hippocampal development. Frizzled 9-null mice had generally normal gross anatomical hippocampal organization but showed large increases in apoptotic cell death in the developing dentate gyrus. This increase in programmed cell death commenced with the onset of dentate gyrus development and persisted into the first postnatal week of life. There was also a perhaps compensatory increase in the number of dividing precursors in the dentate gyrus, which may have been a compensatory response to the increased cell death. These changes in the mutants resulted in a moderate decrease in the number of adult dentate granule cells in null mice and an increase in the number of hilar mossy cells. Heterozygous mice (the same frizzled 9 genotype as Williams syndrome patients) were intermediate between wild type and null mice for all developmental neuronanatomic defects. All mice with a mutant allele had diminished seizure thresholds, and frizzled 9 null mice had severe deficits on tests of visuospatial learning/memory. We conclude that frizzled 9 is a critical determinant of hippocampal development and is very likely to be a contributing factor to the neurodevelopmental and behavioral phenotype of patients with Williams syndrome.