Working memory and school readiness in preschool children with sickle cell disease compared to demographically matched controls.

Children diagnosed with sickle cell disease (SCD) are at risk of the development of neurobehavioural problems early in life. Specific impairments in executive function skills, including working memory, have been documented in school-aged children with SCD. These executive skills are known to strongly contribute to early academic skills and preparedness for entering kindergarten. This study examined working memory and school readiness in preschool children with SCD compared to a healthy control group matched for race, sex and parent education. A total of 84 patients diagnosed with SCD (61.9% haemoglobin [Hb]SS/HbSß0 -thalassaemia) and 168 controls completed testing. The mean (SD) ages of patients and controls at testing were 4.53 (0.38) and 4.44 (0.65) years respectively. The SCD group performed worse than controls on measures of executive function, working memory and school readiness (p < 0.01; Cohen's D range: 0.32-0.39). Measures of working memory were associated with school readiness after accounting for early adaptive development. Multiple linear regression models among patients diagnosed with SCD revealed that college education of the primary caregiver was positively associated with school readiness (p < 0.001) after controlling for sex, genotype, age and early adaptive development. These results highlight the need to implement school readiness interventions in young children diagnosed with SCD emphasising executive function skills.

Nocturnal Enuresis in Sickle Cell: Sociodemographic, Medical, and Quality of Life Factors.

OBJECTIVE: Nocturnal enuresis is more prevalent in youth with sickle cell disease (SCD) compared to the general population. The purpose of this study is to estimate prevalence of nocturnal enuresis using diagnostic criteria and identify associated sociodemographic, medical, and health-related quality of life (HRQOL) factors. METHODS: Youth with SCD (N = 248; ages 6.00-17.99 years) and their caregivers completed semi-structured interviews and questionnaires. HRQOL was measured using the Pediatric Quality of Life (PedsQL) Inventory. Medical information was abstracted from medical record. We generated multivariable logistic regression models to examine associations between factors and current nocturnal enuresis and nocturnal enuresis occurring any time in the past (lifetime). RESULTS: Among participants (mean age, 11.3 ± 3.6 years; 50.8% male), 21.4% reported current nocturnal enuresis and 46% reported lifetime nocturnal enuresis. Male sex [odds ratio (OR), 2.57; p = .001], difficulty arousing from sleep (OR, 3.57; p < .001), higher school functioning HRQOL (OR, 1.02; p = .014), and higher fetal hemoglobin levels (OR, 1.03; p = .048) were associated with lifetime nocturnal enuresis. Younger age (OR, 1.16; p = .005), higher youth-reported fatigue (OR, 1.01; p = .045), difficulty arousing from sleep (OR, 4.92; p < .001), and higher lactate dehydrogenase levels (OR, 1.00; p = .042) were associated with current nocturnal enuresis. CONCLUSIONS: Nocturnal enuresis is prevalent in youth with SCD and is associated with HRQOL, diminished sleep, greater fatigue, and disease severity markers. Routine assessment of sleep behaviors and fatigue are necessary when treating patients with SCD to understand the impact of nocturnal enuresis on HRQOL.

The Adolescent and Caregiver Sickle Cell Disease Self-management Skills Checklist: Preliminary Reliability and Validity.

Adolescents with sickle cell disease (SCD) need assistance in developing the knowledge and skills that contribute to increased disease self-management and successful transition to adult-based health care. This study evaluated the preliminary psychometric properties of the Self-Management Skills Checklist (SMSC and SMSC-C; Adolescent and Caregiver versions), a measure of perceived SCD-specific knowledge and skills. A retrospective cohort study included 114 adolescents (mean=15.6 y) and their caregivers. We examined internal structure and reliability, score changes over time, and group differences. Cronbach coefficient alphas were 0.79 and 0.74 for caregiver-reported Skills and caregiver-reported knowledge, respectively, and 0.77 and 0.44 for adolescent-reported skills and adolescent-reported knowledge, respectively, indicating good internal consistency for 3 of the subscales. Poor reliability in the adolescent-reported knowledge summary score and factor analysis suggest an interpretation item-by-item, independent of one another. Participant group differences in age and chronic transfusion treatment existed in both summary and subscale scores of the SMSC and SMSC-C. Follow-up administrations of the scales indicated an increase in caregiver-reported skills for their adolescents from time 1 scores (M=3.72±0.83) to time 2 scores (M=3.99±0.63) (t16=2.178, P=0.045). Findings provide preliminary support for the usage of the SMSC and continued development to improve its psychometrics.

Pediatric to Adult Care Transition: Perspectives of Young Adults With Sickle Cell Disease.

Objectives: The aim of this study was to explore perspectives of transition and transition readiness of young adult patients (YAs) with sickle cell disease (SCD) who have transitioned to adult health care. Methods: In all, 19 YAs with SCD (ages 18-30 years) participated in one of three focus groups and completed a brief questionnaire about transition topics. Transcripts were coded and emergent themes were examined using the social-ecological model of adolescent and young adult readiness for transition (SMART). Results: Themes were consistent with most SMART components. Adult provider relationships and negative medical experiences emerged as salient factors. YAs ranked choosing an adult provider, seeking emergency care, understanding medications/medication adherence, knowing SCD complications, and being aware of the impact of health behaviors as the most important topics to include in transition programming. Conclusions: The unique perspectives of YAs can inform the development and evaluation of SCD transition programming by incorporating the identified themes.