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.

Student perspectives on managing sickle cell disease at school.

OBJECTIVE: The study objective was to identify the perceptions of children with sickle cell disease (SCD) in the school environment. METHODS: Semistructured interviews (N = 14) were conducted with pediatric patients ages 6 to 10 who attended Metro Nashville Public Schools. These participants were recruited through the Vanderbilt Sickle Cell Disease Clinic. Participants were asked about the perceived efficacy of their teachers to (1) understand SCD; (2) communicate with students regarding SCD; (3) handle an SCD-related pain episode in school; and (4) identify methods to compensate for school absenteeism associated with an SCD diagnosis. Content analysis identified underlying themes. RESULTS: Five themes emerged that highlighted the perceptions and recommendations of children with SCD in the school environment: (1) perceptions that allow students to prevent SCD from limiting their school experience; (2) administrator actions to alleviate challenges associated with SCD; (3) communication about SCD; (4) how SCD interferes with school activities; and (5) ways students advocate for themselves. Students also provided four areas of recommendations for school personnel: (1) ways teachers can help with school activities; (2) make-up work for school absences; (3) empowering students with SCD; and (4) helping with SCD episodes at school. CONCLUSIONS: Students with SCD advocated strongly for their needs at school to attain their education. However, they perceived school personnel to lack knowledge about SCD management. This could be overcome with a handbook specific for teachers of students with SCD that could address each of the five themes.

Feasibility of implementing mobile technology-delivered mental health treatment in routine adult sickle cell disease care.

Sickle cell disease (SCD) is a severe hemoglobinopathy characterized by acute and chronic pain. Sufferers of the disease, most of whom are underrepresented minorities, are at increased risk for mental health disorders. The purpose of this study is to test the acceptability and implementation of a computerized cognitive behavioral therapy (cCBT) intervention, Beating the Blues, to improve depression, anxiety, and pain in patients with SCD. Adults with SCD and significant symptoms of depression (Patient Health Questionnaire [PHQ-9] score ≥ 10) or anxiety (Generalized Anxiety Disorder Scale [GAD-7] score ≥ 10) were eligible to participate and be randomized to either receive eight sessions of cCBT with care coach support or treatment as usual. Participants reported daily pain and mood symptoms using a mobile diary app. Depression, anxiety, and pain symptoms were assessed at 1, 3, and 6 months. Thirty patients were enrolled: 18 to cCBT, and 12 to control. The cCBT intervention was feasible to implement in clinical settings and acceptable to participants. Patients in the cCBT arm reported a marginally greater decrease in depression at 6 months (-3.82, SE = 1.30) than those in the control group (-0.50, SE = 1.60; p = .06). There were no significant effects of treatment on anxiety; however, cCBT was associated with improved daily pain reported via a mobile diary app (p = .014). cCBT, delivered via mobile device, is a feasible strategy to provide mental health care to adults living with SCD. cCBT was acceptable to the target population; was able to be implemented in real-world, nonideal conditions; and has the potential to improve patient-reported outcomes.

Risk Factors for 30-Day Readmission in Adults with Sickle Cell Disease.

BACKGROUND: Readmission to the hospital within 30 days is a measure of quality care; however, only few modifiable risk factors for 30-day readmission in adults with sickle cell disease are known. METHODS: We performed a retrospective review of the medical records of adults with sickle cell disease at a tertiary care center, to identify potentially modifiable risk factors for 30-day readmission due to vasoocclusive pain episodes. A total of 88 patients ≥18 years of age were followed for 3.5 years between 2010 and 2013, for 158 first admissions for vasoocclusive pain episodes. Of these, those subsequently readmitted (cases) or not readmitted (controls) within 30 days of their index admissions were identified. Seven risk factors were included in a multivariable model to predict readmission: age, sex, hemoglobin phenotype, median oxygen saturation level, listing of primary care provider, type of health insurance, and number of hospitalized vasoocclusive pain episodes in the prior year. RESULTS: Mean age at admission was 31.7 (18-59) years; median time to readmission was 11 days (interquartile range 20 days). Absence of a primary care provider listed in the electronic medical record (odds ratio 0.38; 95% confidence interval, 0.16-0.91; P = .030) and the number of vasoocclusive pain episodes requiring hospitalization in the prior year were significant risk factors for 30-day readmission (odds ratio 1.30; 95% confidence interval, 1.16-1.44; P <.001). CONCLUSION: Improved discharge planning and ensuring access to a primary care provider may decrease the 30-day readmission rate in adults with sickle cell disease.

Cognitive deficits are associated with unemployment in adults with sickle cell anemia.

An estimated 25-60% of adults with sickle cell disease (SCD) are unemployed. Factors contributing to the high unemployment rate in this population are not well studied. With the known risk of cognitive deficits associated with SCD, we tested the hypothesis that unemployment is related to decrements in intellectual functioning. We conducted a retrospective chart review of 50 adults with sickle cell anemia who completed cognitive testing, including the Wechsler Adult Intelligence Scale-IV, as part of standard care. Employment status was recorded at the time of testing. Medical variables examined as possible risk factors for unemployment included disease phenotype, cerebral infarction, and pain frequency. The mean age of the sample was 30.7 years (range = 19-59); 56% were women. Almost half of the cohort (44%) were unemployed. In a multivariate logistic regression model, lower IQ scores (odds ratio = 0.88; p = .002, 95% confidence interval, CI [0.82, 0.96]) and lower educational attainment (odds ratio = 0.13; p = .012, 95% CI [0.03, 0.65]) were associated with increasing odds of unemployment. The results suggest that cognitive impairment in adults with sickle cell anemia may contribute to the risk of unemployment. Helping these individuals access vocational rehabilitation services may be an important component of multidisciplinary care.

Coping with the pediatric anogenital exam.

PURPOSE: To focus on children's and adult guardians' anticipatory and acute attempts to cope with the pediatric anogenital exam. METHODS: A sample of 40 girls (ages 8-15) and a separate sample of 109 adult guardians who accompanied children to the medical clinic rated their use of specific coping strategies on a 5-point Likert scale both pre- and postexam. FINDINGS: Overall, participants reported relatively high levels of active coping strategies and relatively low levels of passive coping responses throughout the medical encounter. Effects of situational and dispositional variables on the use of specific coping strategies also were found. CONCLUSIONS: Data provided indirect evidence of the inherent strengths and self-righting tendencies of those dealing with the exceptional circumstances surrounding allegations of child sexual abuse. Such data suggest the value of identifying individuals' coping resources and options to facilitate successful completion of the medical evaluation and provide therapeutic benefit to patients and families.