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.

Vitamin D deficiency and comorbidities in children with sickle cell anemia.

Vitamin D deficiency is known to be common among patients with sickle cell anemia (SCA). Vitamin D levels were measured in 139 children (aged 7.9 to 15.1 years) to study its association with SCA morbidities; severe deficiency <10 ng/mL was present in 64.0% and only 2.2% were sufficient (>30 ng/mL). Vitamin D levels were associated with pulmonary function (forced expiratory volume in 1 second [FEV(1)]), but not associated with either rates of acute pain or acute chest syndrome episodes. Further studies are needed to be able to compare outcomes in those with deficiency to those with sufficiency, as well as to treating patients with SCA with vitamin D to better establish a possible link, if any, between vitamin D and SCA morbidity.