ALBERT

Description: Background Sickle cell trait (SCT) affects approximately 8% of African Americans and these individuals are at risk of having children with SCT and sickle cell disease (SCD). Universal newborn screening in the United States detects SCT, but only 16% of individuals of childbearing age with SCT know their status. This is because SCT is not reliably recorded in medical records and SCT may not be consistently or effectively communicated to caregivers. Interventions that increase caregivers' SCT knowledge are important so that individuals with SCT can make informed decisions about their reproductive health. Objectives The objectives of this study were to determine if in-person SCT education with a trained educator improved caregivers' SCT knowledge and to assess caregivers' satisfaction with their SCT education. Methods Nationwide Children's Hospital (NCH) is a SCT referral center in Columbus, OH. At NCH, it is standard that caregivers of infants with SCT who are referred by their primary care providers receive in-person SCT education using visual aids from an educator who has completed a hemoglobinopathy counselor training course. From August 2015-July 2016, we performed a prospective, cross-sectional study of English-speaking caregivers of infants with hemoglobin S-trait who presented for education. Caregivers were excluded if they had SCD or previously received SCT education at NCH. Prior to receiving education, subjects electronically reported their demographic information and completed a health literacy assessment using the Newest Vital Sign (NVS, range 0-6) and an 8-question (true/false, multiple choice) pilot-tested, SCT knowledge assessment (SCTKA) that included items from a published SCT knowledge study and items routinely discussed during NCH SCT education. Lower NVS scores suggest a higher likelihood of limited health literacy. Caregivers with SCTKA scores

Description: Background The2014 National Heart, Lung, and Blood Institute Expert Panel Report recommends that hydroxyurea (HU) be offered to all children with Hemoglobin SS and Sβ0 sickle cell disease (SCD) and that it be considered for children with Hemoglobin SC or Sβ+ who have clinically severe SCD. Describing current HU use among hospitalized children with SCD could identify whether HU is being prescribed to children who may benefit from it and whether HU improves hospitalization outcomes. Objectives The primary aims of this study were to determine the current rate of HU use in hospitalized children and to determine if HU was differentially used in children with clinically severe SCD. Additionally, we examined if hospitalized HU users had improved hospitalization outcomes as measured by fewer deaths, shorter lengths of stay (LOS), fewer intensive care unit (ICU) admissions, and fewer erythrocyte transfusions compared to HU non-users. Methods We performed a retrospective analysis of the Pediatric Health Information System (PHIS) inpatient data, a dataset developed by the Children's Hospital Association. PHIS participating hospitals supply International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) codes, de-identified demographic data, procedure codes, and charge codes for all of their hospitalization discharges. We included children ages 2-18 with SCD discharged between January 1, 2011-September 30, 2014 from the 42 hospitals that provided complete data to PHIS during the study period. Only patients' most recent hospitalization during the study period was analyzed to reflect the current rate of HU use. The following patient data within PHIS were obtained for all eligible discharges: age, gender, HU use, discharge status (dead, alive), ICU admission, asthma diagnosis, total number of ICD-9-CM diagnoses, LOS (days), erythrocyte transfusion, and insurance provider (private, public, unknown). Since the ICD-9-CM codes for patients' SCD genotype are not discreet, we did not use SCD genotype in our analyses. We defined patients as having clinically severe SCD if they had a history of a recent ICU admission (after 2009) or a history of ≥3 hospital admissions in the year preceding their most recent admission. This severity definition was selected because it is used in clinical practice or in clinical trials to identify children with severe SCD and because it could be captured within PHIS. Chi-squared and t-tests were used to compare the two groups, and since this was an observational study, propensity score weighting was used to balance differences in the baseline characteristics between HU users and non-users. Results We identified 2,665 unique children with SCD. Approximately 80% had an inpatient code indicating HU use. The mean age of HU users was 11.1 years (SD ± 4.8), which was younger (p=0.013) than non-users (11.7 years SD ± 4.8). Two HU users and four non-users died during their hospitalization. Significantly more (p

Description: Abstract 2473 Poster Board II-450 Introduction: Children with HbSS or HbSβothalassemia have an 11% risk of stroke before the age of 20 without intervention. Transcranial Doppler ultrasonography (TCD) has been instrumental in detecting children with sickle cell disease (SCD) at high risk of stroke. Yearly TCD screenings are recommended for children with HbSS or HbSβothalassemia between the ages of 2 and 16 years. However, annual TCD compliance rates are very low. Our objective was to complete a qualitative, descriptive analysis of caregiver understanding, opinions, and barriers to their child's annual TCD screenings. Methods: The study consisted of interviewing 28 caregivers of children diagnosed with HbSS or HbSβothalassemia. The interviews were structured around a Health Belief Model (HBM) framework. The HBM describes the interaction of perceived susceptibility, perceived severity, perceived barriers, perceived benefits, cues to action, self-efficacy, demographic factors, and psychosocial factors which together produce the likelihood that an individual will take a recommended health action. The interviews consisted of both open response questions and Likert scale type questions where the caregiver agreed (“5”) or disagreed (“1”) with a statement. Topics covered during the interview included caregiver knowledge of TCD, adherence to screening guidelines, perceived susceptibility of their child to stroke, barriers that may prevent annual TCD screenings, perceived benefits to screening, and self-efficacy. The interviews were audio recorded and were later transcribed and coded. Common themes were extracted from the transcriptions. Results: 61% of the interviewed caregivers reported that their child underwent a TCD in the past year. Many caregivers perceived their child as being at high risk for stroke, as over 60% thought that stroke occurred either “sometimes” or “frequently” in children with SCD. All of the interviewed caregivers saw benefits to TCD screening. We identified seven categories of barriers expressed by caregivers: lack of knowledge, lack of self-efficacy, fear, financial barriers, transportation barriers, missed appointments, and hours of radiology clinic. Three of the most frequently mentioned barriers included lack of knowledge, lack of self-efficacy, and fear of chronic transfusions. Lack of knowledge was reported to be a barrier by 32% of caregivers who reported having very limited knowledge about TCD testing or that they had forgotten the information previously provided to them. 46% of caregivers did not know that TCD screening was an annual test, and only one of the 28 caregivers knew that chronic transfusions would result if the TCD was consistently abnormal. Additionally, only one caregiver knew the age recommendations for screening. Caregivers also seemed to lack self-efficacy, as 18% of caregivers believed they had no role in stroke prevention and 50% did not realize that they could request that their doctor order an annual TCD test. Many caregivers (29%) believed that chronic transfusions could cause their child a lot of problems. Less frequently cited barriers included financial barriers, transportation barriers, missed appointments, and daytime/weekday hours of radiology clinic. Conclusions: The most commonly encountered barrier to TCD screening described by caregivers was the lack of knowledge of the purpose and recommendations for TCD screenings. We propose that health care providers provide ongoing education at each visit to caregivers regarding stroke risk, TCD screening, and the current recommendations for children with SCD. Some of the barriers identified in this study can easily be addressed to increase TCD compliance rates and decrease stroke incidence rates in children with SCD; for example, offering night and weekend radiology hours or identifying transportation issues prior to a TCD appointment and providing transportation when needed. Educating caregivers on the risks and benefits of chronic transfusions in stroke prevention could also prove beneficial in reducing caregiver fear of chronic transfusions. Strengthening the therapeutic alliance between the caregiver, child, and health care team is essential in order to increase caregiver compliance and self-efficacy with TCD screenings and decrease stroke incidence in children with SCD. Disclosures: No relevant conflicts of interest to declare.