Rate of Food Insecurity Among Households with Children with Sickle Cell Disease is Above the National Average.

OBJECTIVES: Despite studies demonstrating the negative impact of food insecurity on health in children, limited research has been done to assess the prevalence and sequelae of food insecurity in sickle cell disease (SCD). We tested the hypothesis that food insecurity is common in children with SCD and is associated with increased SCD morbidity. METHODS: Between May and November 2017, we conducted a single-center cross-sectional study using the previously validated, self-administered, US 18-item household food security survey module and the 9-item youth (12-17 years old) food security survey module during regular outpatient clinic visits. We also included the incidence of vaso-occlusive pain or acute chest syndrome requiring hospitalizations in the year before the questionnaire. RESULTS: A total of 75 caregivers and 24 children completed the surveys. The median age of the children was 10.4 years (interquartile range 5.5-15.3), 46.7% were boys. The rate of household food insecurity was 21.3% (16 of 75). Among the 24 children who completed the youth survey, 45.8% were classified as food insecure. Discordance occurred between caregivers' and children's assessment of food insecurity. A total of 81.8% (9 of 11) children reported being food insecure, whereas their caregivers reported to be food secure. The incidence for pain and acute chest syndrome in the year pre-enrollment was not different between food-secure and food-insecure children (59.3 and 43.8/100 patient-years, P = 0.54; 8.5 and 12.5/100 patient-years, P = 0.49, respectively). CONCLUSIONS: In a tertiary care medical center in Tennessee, one in five households with children with SCD were assessed as food insecure, with a substantial discordance between caregiver and child assessment of food insecurity.

Integrated psychology support and comprehensive cognitive evaluation improves access to special education services for children with sickle cell disease.

BACKGROUND: Children with sickle cell disease (SCD) are at risk for cognitive deficits. Limited data describe whether comprehensive cognitive evaluation improves education resources and whether caregivers perceive the assessment as beneficial. We tested our two hypotheses: (a) an integrated comprehensive cognitive evaluation program in children with SCD results in increased special education services allocation; and (b) caregivers will value comprehensive cognitive evaluation services provided. PROCEDURE: In a tertiary care medical facility, as part of quality improvement project, in a before-and-after evaluation between March 2011 and July 2014, we examined the impact of targeted comprehensive cognitive evaluation on change in special education services. We also evaluated the caregiver's perception regarding the utility of the provided services. RESULTS: A total of 21% (42 of 196) students (median age 11 years, range 3-18) with SCD were referred for cognitive assessment due to overt stroke (n = 11), silent stroke (n = 14), or concerns about cognitive or academic functioning without evidence of strokes (n = 17). At baseline, 45.2% received special education services and after the comprehensive cognitive evaluation 86.7% received special education services (P < 0.001). Among 33 caregivers who completed the survey, 97% reported that the assessment was helpful and 60% indicated that assessment led to beneficial changes for their children at school. CONCLUSION: Education advocacy coupled with comprehensive cognitive assessment in students with SCD improved access to special education services, and caregivers uniformly endorse this service as having added value.

Clustering of end-organ disease and earlier mortality in adults with sickle cell disease: A retrospective-prospective cohort study.

Chronic end-organ complications result in morbidity and mortality in adults with sickle cell disease (SCD). In a retrospective-prospective cohort of 150 adults with SCD who received standard care screening for pulmonary function abnormalities, cardiac disease, and renal assessment from January 2003 to 2016, we tested the hypothesis that clustering of end-organ disease is common and multiple organ impairment predicts mortality. Any end-organ disease occurred in 59.3% of individuals, and 24.0% developed multiple organ (>1) end-organ disease. The number of end-organs affected was associated with mortality (P ≤ .001); 8.2% (5 of 61) of individuals with no affected end-organ, 9.4% (5 of 53) of those with 1 affected organ, 20.7% (6 of 29) of those with 2 affected end-organs, and 85.7% (6 of 7) with 3 affected end-organs died over a median follow up period of 8.7 (interquartile range 3.5-11.4) years. Of the 22 individuals who died, 77.3% had evidence of any SCD-related end-organ impairment, and this was the primary or secondary cause of death in 45.0%. SCD-related chronic impairment in multiple organs, and its association with mortality, highlights the need to understand the common mechanisms underlying chronic end-organ damage in SCD, and the urgent need to develop interventions to prevent irreversible end-organ complications in SCD.

Secondary benefit of maintaining normal transcranial Doppler velocities when using hydroxyurea for prevention of severe sickle cell anemia.

In a retrospective cohort study, we tested the hypothesis that when prescribing hydroxyurea (HU) to children with sickle cell anemia (SCA) to prevent vaso-occlusive events, there will be a secondary benefit of maintaining low transcranial Doppler (TCD) velocity, measured by imaging technique (TCDi). HU was prescribed for 90.9% (110 of 120) of children with SCA ≥5 years of age and followed for a median of 4.4 years, with 70% (n = 77) receiving at least one TCDi evaluation after starting HU. No child prescribed HU had a conditional or abnormal TCDi measurement. HU initiation for disease severity prevention decreases the prevalence of abnormal TCDi velocities.