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Sickle cell disease (SCD) was once a disease of childhood because of a limited life expectancy. Due to medical advances, it is now common for people with SCD to live into adulthood. Funding and resources for adults with SCD, however, remain limited. Adult patients would benefit from increased access to medical care, mental health care services, and workforce development. The Indiana Sickle Cell Consortium, a group of medical providers and community-based organizations, worked closely with people living with SCD and their family members to create a campaign advocating for state funding for programs for adults with SCD. This campaign culminated with the passage of a bill that provides $250,000 in funding for program development for adults with SCD. The bill also directs the Indiana Department of Health to carry out a needs assessment for people with SCD in Indiana. However, continued efforts are needed to reduce health disparities for people with SCD. The Indiana Sickle Cell Consortium will continue advocacy efforts in future legislative cycles and bring attention to the health inequities that affect people with SCD.

Newborn screening (NBS) follow-up programs for infants with sickle cell disease (SCD) are highly variable among states. Initiated in 2009, Sickle SAFE, the NBS follow-up program for infants with SCD in Indiana, follows infants through home visits and phone contact. The current study assessed the attainment rates for recently published quality indicators of pediatric SCD care for Sickle SAFE participants. Using retrospective data, we determined the proportion of children who received transcranial Doppler (TCD) screening, influenza, and pneumococcal vaccination and were prescribed hydroxyurea. We calculated the mean age at confirmatory testing, time to receipt of penicillin prophylaxis, and mean age when genetic counseling was offered. One hundred ninety-eight children born with SCD in Indiana between July 1, 2009 and June 30, 2017 were followed for at least 1 year. While 97.5% received at least one dose of conjugated pneumococcal vaccine, vaccination with the 23 valent pneumococcal vaccine varied by location (county) of care (Allen: 14.3%, Lake: 26.7%, St. Joseph: 40.0%, Marion: 73.3%). Overall TCD screening rate for eligible children was 53%; TCD screening rate varied widely by location of care (Lake: 25% vs. Marion: 63.8%). Similarly, hydroxyurea prescribing practices varied significantly by location of care (p

Community health workers are increasingly recognized as useful for improving health care and health outcomes for a variety of chronic conditions. Community health workers can provide social support, navigation of health systems and resources, and lay counseling. Social and cultural alignment of community health workers with the population they serve is an important aspect of community health worker intervention. Although community health worker interventions have been shown to improve patient-centered outcomes in underserved communities, these interventions have not been evaluated with sickle cell disease. Evidence from other disease areas suggests that community health worker intervention also would be effective for these patients. Sickle cell disease is complex, with a range of barriers to multifaceted care needs at the individual, family/friend, clinical organization, and community levels. Care delivery is complicated by disparities in health care: access, delivery, services, and cultural mismatches between providers and families. Current practices inadequately address or provide incomplete control of symptoms, especially pain, resulting in decreased quality of life and high medical expense. The authors propose that care and care outcomes for people with sickle cell disease could be improved through community health worker case management, social support, and health system navigation. This report outlines implementation strategies in current use to test community health workers for sickle cell disease management in a variety of settings. National medical and advocacy efforts to develop the community health workforce for sickle cell disease management may enhance the progress and development of “best practices” for this area of community-based care.

Background: The Sickle Care Coordination, OutReach, Education Program (SCORE) was created in 2017 to improve access to hematology care and care coordination services for children with sickle cell disease (SCD) in an urban community in Lake County, Indiana. Although Lake County has the second highest prevalence of SCD in Indiana, it lacks a pediatric hematologist, and the closest pediatric hematology practice is 65 miles away. Newborn screening data from 2009-2017 showed that only 25% of children in this underserved area received Transcranial Doppler (TCD) and only 25% received the 23 valent pneumococcal vaccine, compared with 70% for both in other regions of Indiana. The goal of the SCORE clinic is to increase TCD screening and vaccination rates as well as increase the number of children who are prescribed hydroxyurea (HU) at maximum tolerated dosing (MTD). Methods: Quarterly SCORE outreach clinics were established in Lake County, Indiana in August 2017, which is 145 miles Northwest of our center. A multi-disciplinary team (social worker, sickle cell educators, nursing staff) accompanies the pediatric hematologist and physician assistant to clinic. Vaccines are administered in SCORE clinic and laboratory tests are drawn on site. Same day TCD screening was established in May 2018. The state's electronic vaccine record was queried to determine which SCD-specific vaccines the children needed prior to each clinic. Education about SCD, HU, and needed screening tests like TCD was provided by members of the multi-disciplinary team. Results: One hundred thirty-one visits have been made to the 10 SCORE outreach clinics that have been held since August 2017. Forty children have received hematology care in the SCORE clinic; 35 (87.5%) children have had at least 2 visits. Twenty-two children were eligible for HU; sixteen (72.7%) were already taking HU at the time of their first visit to SCORE outreach clinic (mean dose prior to first SCORE visit: 18.6 + 5.9 mg/kg/day). Mean HU dose increased significantly after being followed by the SCORE clinic team (26.5 mg/kg/day, p=0.00002). The remaining 6 children initiated HU after receiving care in the SCORE outreach clinic. Nineteen children were eligible for TCD screening since their first SCORE clinic visit, and fourteen (73.7%) children completed the screening. One hundred twenty-one vaccines have been given in clinic to date: 100% of children who were eligible for the 23 valent pneumococcal, hepatitis A and B vaccines have received them. Seasonal influenza vaccine was given to 76.9% of eligible children. Forty-six of the 54 (85.2%) Menveo doses that were due were given, and 80% of Bexsero doses that were due were given. Conclusion: The SCORE program has increased access to pediatric hematology care for children with SCD in an urban, underserved community in Indiana. In the first 2 years of the program, TCD screening rates and pneumococcal vaccination rates have increased to the level seen in other regions of the state. Children who received care in the SCORE outreach clinic had a significant increase in their HU dosing. Outreach clinics can increase access to specialty care in underserved areas. Disclosures Meier: CVS Caremark: Consultancy. OffLabel Disclosure: Hydroxyurea prescription in children less than 2 years of age.

Objective To demonstrate the feasibility of performing a noninvasive, molecular-based red blood cell (RBC) antigen test on infants and very young children with sickle cell disease as part of a statewide newborn screening follow-up program. Study design A prospective pilot project was conducted using a noninvasive buccal swab and test kit to perform DNA-based, extended RBC phenotyping in 92 children participating in a newborn hemoglobinopathy screening follow-up program. Reported data include the extended panel of antigens detected by molecular analysis compared with unaffected population estimates. Results Molecular-based RBC antigen testing was successful, with extended RBC typing generated for all subjects. Molecular testing detected several rare negative or rare positive phenotypes, demonstrating the utility of obtaining an extended antigen panel. Conclusion This study demonstrates the feasibility of performing antigen testing on buccal swab specimens from children with sickle cell disease as part of a newborn screening follow-up program with the aim of allowing specific unit matching to prevent alloimmunization with RBC transfusions. The general applicability of testing may be limited by a lack of uniform insurance coverage for buccal swab testing, however.

While newborn screening for sickle cell disease (SCD) has improved survival of affected infants through early prescription of prophylactic penicillin and SCD education for the parents, newborn screening follow-up programs are highly variable among states. The novel statewide newborn screening program in Indiana, Sickle SAFE (Screening, Assessment, Follow-up, Education), was started in 2009 and followed infants through the first year of life until 2013 when the program expanded to provide follow-up for the first 5 years of life. Sickle SAFE ensures timely notification and education of families of affected patients and links them to a hematologist. An initial home visit is scheduled and coordinated by the Sickle SAFE Program Coordinator within one month of receipt of abnormal screening results. After the initial home visit, coordinators maintain phone contact with families regularly throughout the enrollment period. An analysis of Georgia Medicaid claims published in 2016 reported that 47.2% of children between 2 and 3 years of age had screening transcranial Doppler (TCD) and 73.6% received PPSV23 (Pneumovax). The current study aims to assess the rates of attainment for recently published quality indicators of pediatric SCD care for children enrolled in Sickle SAFE. A retrospective study was initiated to determine the proportion of children enrolled in Sickle SAFE who received TCD screening between 2 and 3 years of age (HbFS only) and influenza and pneumococcus [PCV (Prevnar) and PPSV23] vaccination. The mean age at confirmatory testing and receipt of penicillin prophylaxis (goal ≤ 2 months) as well as the mean age of the affected infant when families were offered genetic counseling (goal ≤ 6 months) were calculated. From 2009-2012, all positive newborn screens for any sickle hemoglobinopathy were reflexively sent for confirmatory DNA testing. From 2013 onward, only HbFS newborn screens were sent for confirmatory DNA testing. Children who were enrolled in Sickle SAFE less than two years of age were excluded from the PPSV23 and TCD analyses. A total of 141 children were born with SCD and enrolled in Sickle SAFE for at least one year between July 1 2009 and June 30 2015. The majority (56.7%) had HbSS, 32.6% had HbSC, 9.9% were compound heterozygotes for HbS and beta thalassemia and the remaining 0.7% had another sickle hemoglobinopathy. The mean length of follow-up was 2.7 ± 1.0 years. 55.3% were female and 78.7% were African American. 87.2% were publicly insured. Mean age for all patients born with SCD to have confirmatory testing was significantly shorter when confirmatory DNA testing was sent for all affected infants compared to when it was limited to only infants with HbFS [40.3 ±14.1 days (2009-2012) vs. 127.8 ± 173.1 days (2013-2015), p= 1.5E-6]. While the age at confirmatory testing did not differ for infants with HbFS results between the two periods [45.9 ± 14.1 days (2009-2012) vs. 45.8 ± 14.0 days (2013-2015), p=0.98], infants with results other than HbFS were significantly older when confirmatory testing was performed after the process change in 2013 [40.3 ± 14.1 days (2009-2012) vs. 225.3 ± 221.4 days (2013-2015), p=2.6E-6]. Mean age at which genetic counseling was offered to all families with affected children was 26.8 ± 9.9 (range 9-60) days. Mean age at receipt of first dose of penicillin was 28.6 ± 15.0 (range 5-62) days for infants with HbFS, and 29.5 ± 20.1 (range 5-142) days for those with other sickle hemoglobinopathies (p=0.78). 86.5% of enrollees received at least one influenza vaccine while 95.0% had received at least one dose of PCV. Over two-thirds (69.1%) of children with HbFS had TCD screening between the ages of two and three years. 77.3% of Sickle SAFE enrollees who were followed for more than 2 years received PPSV23. Newborn screening for SCD allows for supportive care aimed at reducing morbidity and mortality. Reflex DNA confirmatory testing is an important part of this quality care, regardless of sickle genotype, and our data shows that confirmatory testing is delayed when it is not reflexively performed for all affected infants. The intensive follow-up provided through the Sickle SAFE program increases adherence to quality standards for comprehensive care, as evidenced by a higher rate of TCD screening compared to published data. Education of state departments of health for improved funding and support of programs like Sickle SAFE may help to improve outcomes for affected children. Disclosures No relevant conflicts of interest to declare.