Sickle cell disease (SCD), an inherited blood disorder that primarily affects individuals of African descent, is associated with serious medical complications as well as numerous socialenvironmental risk factors. These social-environmental factors are linked to long-standing social inequities, such as financial hardship and racial discrimination, both of which impact cognitive and behavioral functioning in youth. Previous research on the relationship between socialenvironmental risk and psychological functioning has primarily relied on non-modifiable, unidimensional measures of socioeconomic status (SES), such as income and parental education, as a proxy for social-environmental risk. The current study aimed to address the limitations associated with typical SES-type measures by comparing the unique and shared association of SES and more targeted and modifiable social-environmental factors (e.g., parent and family functioning) with specific areas of cognitive and behavioral adjustment in pediatric SCD. Seventy children ages 4 to 8 years old and their parents completed measures of social-environmental risk and psychological adjustment. Exploratory factor analysis indicated parent and family functioning measures were largely independent from SES. Parent and family functioning predicted phonological processing and ADHD symptoms above and beyond SES alone. In addition, the predictive ability of social-environmental risk factors appears to vary by genotype severity for measures of social functioning and math problem solving ability. Future studies are needed to explore more specific and well-supported models of modifiable social-environmental risk and the relative impact of social-environmental risk on cognitive and behavioral functioning.

Objective Studies of early child development in sickle cell disease (SCD) have found modest associations between disease-related risks and developmental status in infants and toddlers, but such associations are evident by early elementary school. We screened four-year-old children with SCD using two screening strategies to assess if biomedical risk factors for neurologic disease are related to developmental screening outcomes at this intermediate age. Methods Seventy-seven four-year-old children with SCD (M = 4.5 years, SD = 0.3 years) completed developmental screenings at routine hematology visits using child testing (Fluharty Preschool Speech and Language Screenings Test, 2nd edition) and parent-report (Ages and Stages Questionnaire, 2nd edition) procedures. Genotype and other biomedical variables were coded from medical records. Results Children with higher-risk SCD genotypes (n = 52) showed lower performance than children with lower-risk genotypes (n = 25) on a measure related to neurologic disease risk in older children (syntactic processing); genotype risk was also related to rates of positive screenings on parent-reported developmental milestones (52% positive screenings in high-risk genotypes versus 12% in low-risk genotypes). Screening outcomes were also related to transcranial Doppler ultrasound findings assessing cerebral blood flow. Conclusions Developmental screening at age four years may be a useful target age for identifying preschoolers with sickle cell-related neurodevelopmental concerns. Parent-report of developmental milestones and behavioral testing each may have a role in screening for children in need of follow-up services to address potential neurodevelopmental effects from SCD.

:Background:Developmental delay occurs frequently in sickle cell disease (SCD). Psychosocial and biomedical factors contribute to delays, but most studies have not examined the timing of risk factors and developmental delay. We examined sociodemographic and biomedical factors to evaluate whether risks of developmental delay differed across 2 developmental periods.Methods:We examined Ages and Stages Questionnaire, second edition (ASQ-2), outcomes in 2-year-olds (n = 100) and 4-year-olds (n = 101) with SCD. ASQ-2 data were obtained from routine developmental screenings administered as part of health care between 2009 and 2016 at a single hematology clinic. Medical record reviews were used to identify sociodemographic and biomedical factors associated with positive screenings for developmental delay.Results:Two-year-olds with positive ASQ-2 screenings (n = 32; 32%) were less likely to have private health insurance or to have been in formal daycare and more likely to have a severe SCD genotype. Four-year-olds with positive screenings (n = 40; 40%) were more likely to have a severe SCD genotype or an abnormal transcranial Doppler ultrasound (TCD) examination indicating high stroke risk. The strength of the association between positive screenings and insurance status, severe genotypes, and TCD examinations differed across the 2 age groups. Domain-level outcomes on the ASQ-2 also differed across the 2 age groups.Conclusion:The cross-sectional data indicate biomedical and psychosocial risks are related to developmental delay, but the association with specific risk factors differs across age.

Adolescent and young adult (AYA) survivors of cancer have diverse psychosocial and medical needs, including those related to fertility and sexual health. Much of the focus of care around issues such as fertility and sexual health tends to be filtered through a biomedical lens. However, it is essential that health care providers assess and support AYA survivors using a biopsychosocial and contextual framework to ensure the most comprehensive and accurate understanding of AYA survivor needs, especially those related to psychosexual health. A trusting relationship between the multi-disciplinary medical team and the AYA survivor that allows for open discussion about the physical and psychosocial components of sexual health is key to providing best care and outcomes.

Pain episodes occur for many preschoolers with sickle cell disease (SCD), but little is known about parent perceptions of managing pain episodes in young children. We surveyed parents of young children with SCD who had managed pain episodes in the past year to assess their management and satisfaction with their strategies, challenges of pain management, and interest in additional education. Parents were recruited from health maintenance visits at a SCD specialty clinic. Forty-two of 51 parents (82%) of 2- to-6-year-olds reported managing pain over the past year. Parents who had managed pain primarily reported using medications. These parents reported at least moderate satisfaction with current management strategies and resources. At least one-third of parents found each facet of pain management queried as at least somewhat challenging. Identifying when their child was in pain, encouraging functional activities, and managing irritable behavior were reported as most challenging. Parents of young children with SCD reported interest in additional pain management education, which could promote better parent and child coping skills.

Objective The aim of this study was to assess how fatigue is related to mood among youth with sickle cell disease (SCD) by evaluating if the cognitive appraisal of stress moderates the impact of fatigue on emotional functioning consistent with the Risk-and-Resistance Model of Chronic Illness. Methods Daily diaries assessing fatigue (Numerical Rating Scale), pain intensity (Numerical Rating Scale), mood (Positive and Negative Affect Schedule for Children), and cognitive appraisal of stress (Stress Appraisal Measure for Adolescents) were collected from 25 youth with SCD (ages 11–18 years) for 8 consecutive weeks resulting in 644 daily diaries for analyses. Results When measured concurrently, higher fatigue was associated with higher negative mood controlling for pain and prior-night sleep quality. Fatigue predicted next-day negative mood through its interaction with primary and secondary appraisal of stress, consistent with stress appraisal as a protective factor. A similar pattern was observed for pain, which, like fatigue, is a common SCD-related stressor. Conclusion Fatigue and negative mood are inter-related when concurrently assessed, but their temporal association in SCD suggests that mood changes are not an inevitable sequalae of increased fatigue; fatigue influenced subsequent levels of negative mood, but only in the presence of less adaptive cognitions about stress; specifically, a higher perceived threat from stress and a lower belief in the ability to manage stress. The results suggest specific cognitive targets for reducing the negative impact of fatigue on mood in SCD.

Youth with sickle cell disease (SCD) experience disease effects including vaso-occlusive pain crises, poor sleep quality, and fatigue. The present study examines how sleep quality and pain medications impact fatigue in youth with SCD. Daily diaries assessing pain, fatigue, sleep quality, mood, and use of pain medications from 25 youth with SCD ages 11 to 18 years were collected for eight consecutive weeks. Poor sleep quality predicted increases in next-day fatigue levels while controlling for pain and mood. Sleep quality did not moderate the existing temporal relationship between pain and next-day fatigue established by Reinman et al. (2019) as predicted. Non-opioid medications affected ratings of next-day fatigue but opioid medications did not. Sleep quality appears to play an important role in predicting next-day fatigue levels and may be an important target for intervention. Pain medication use did not substantially contribute to prospective fatigue levels among youth, but requires further study.