Families coping with sickle cell disease (SCD) often face heightened psychosocial risk factors, and research in pediatric SCD has often focused more on this area than resiliency factors. The aim of this study was to gain a better understanding of family resiliency in SCD based on caregiver perspectives. A secondary qualitative analysis was conducted with data from a mixed-methods study of caregivers of youth with SCD (n=22). Qualitative analyses involved coding based on 2 resiliency frameworks, organizing coding categories into themes, and systematically reintegrating these themes into a conceptualization that reflected family resiliency. Themes aligned well with the resiliency frameworks and related to family belief systems and meaning-making around SCD (acceptance of SCD, positive attitude, religious faith), family organization and adaptation (flexibility, stability, social supports), and the importance of communication and problem-solving. Study findings emphasize the importance of assessing resilience in families of youth with SCD and suggest the potential clinical benefits of developing psychosocial interventions based on family strengths.

The objective of this systematic review was to assess the relationship between pain (frequency/intensity/duration, impairment, coping) and emotional functioning in pediatric Sickle Cell Disease, and evaluate the state of the literature. Studies were included if they met each of the following criteria: (a) primarily pediatric sample of youth or young adults up to age 21 years with SCD, (b) examined emotional functioning including anxiety and/or depressive and/or internalizing symptoms, and/or affect, (c) examined pain intensity/frequency/duration and/or pain-related impairment and/or pain coping as it relates to emotional functioning, as defined above. Using the established guidelines for systematic reviews, we searched PsycINFO, PubMED, and CINAHL databases for studies published through June 2018. Screening resulted in 33 studies meeting inclusion criteria. Study data were extracted and evaluated for scientific merit, resulting in four studies being removed. 29 studies were included in the final synthesis. Studies provide strongest evidence of a relationship between increased pain frequency and higher depressive and anxiety symptoms. There are moderate-to-strong associations between pain-related impairment and depressive symptoms, and small-to-strong associations between pain-related impairment and anxiety. When examining pain-coping strategies, maladaptive cognitive strategies show the strongest association with emotional functioning. There is a need for more adequately powered, prospective studies based on theoretical frameworks in order to advance our understanding of the relationship between pain and emotional functioning in pediatric SCD.

Objective Families of youth with Sickle Cell Disease (SCD) can face psychosocial adversity including emotional distress, functional impairments, and sociodemographic risk factors. Systematic screening of psychosocial risk can identify families who may benefit from further assessment and evidence-based care. The Psychosocial Assessment Tool (PAT) is a brief caregiver-report screener based on the tri-level Pediatric Psychosocial Preventative Health Model (PPPHM). Methods Findings are presented from the baseline assessment of a longitudinal study validating a Sickle Cell version of the PAT 2.0. Primary caregivers of 136 youth with SCD receiving care through a multidisciplinary SCD clinic in a children’s hospital completed the PAT and validation measures. A subset of 25 caregivers completed the PAT a second time within 3–5 weeks. Results Internal consistency for the total score was strong (α = .87), and for the subscales was moderate to strong (α  =  .74–.94), with the exception of the Family Structure (α  =  .38), Caregiver Beliefs (α  =  .48), and Stress Reactions (α  =  .56) subscales. Test–retest reliability was also strong (r = .86, p < .001). Moderate to strong correlations with all except two criteria measures provided validation for the total and subscale scores. Validation measures varied significantly across the three levels of the PPPHM. Conclusions Results provide support for the reliability and validity of the PAT in SCD. Systematic screening with the PAT can help identify families of youth with SCD at risk for psychosocial problems and potentially help connect them to appropriate services.