Background: There have been significant changes in clinical guidelines for sickle cell disease (SCD) over the past two decades, including updated indications for hydroxyurea, transfusions, and iron overload management. In practice however, there are few studies that examine SCD care utilization over time. Methods:We conducted a serial cross-sectional cohort study of pediatric SCD patients from 2004 to 2019 using Georgia Medicaid claims data. For each year, we reported receipt of any transfusion, chronic transfusion, or three or more filled hydroxyurea prescriptions. For children receiving chronic transfusion (six or more annual transfusions), we evaluated iron overload diagnosis, monitoring, and chelation use. Among children with sickle cell anemia (SCA), we examined rates of transfusions and hydroxyurea use.The Cochran-Armitage test was used to assess trend.Results: There were 5316 unique children 2-18 years old with SCD enrolled in Georgia Medicaid from 2004 to 2019. Children receiving any transfusion increased from 2004 to 2010, then stabilized. In SCA patients, chronic transfusions initially increased from 2004 to 2010, then stabilized from 2010 to 2019. For chronically transfused children, monitoring of iron burden and filled chelator prescriptions both increased significantly. Hydroxyurea use in SCA patients increased from 12% to 37%, with increases noted within each age group, most notably from 21% to 60% in the 13-18-year-old cohort. Conclusion:We demonstrated changes in SCD care utilization over time, including increased hydroxyurea use, changes in transfusion rates, and increased attention to iron overload management. While trends in clinical management do follow updates in treatment guidelines, there is still delayed and suboptimal uptake of guideline recommendations in pediatric SCD patients.

Background Children with sickle cell disease (SCD) are at increased risk for bacterial infections including osteomyelitis (OM). Fever and bone pain, key presenting symptoms of OM, are common in SCD, thus complicating diagnosis. We reviewed presentation, imaging features, and microbiologic etiologies of children with SCD treated for OM. Methods The comprehensive SCD clinical database of children and adolescents with SCD followed at a single, large tertiary pediatric center were searched to identify all diagnostic coding for potential cases of osteomyelitis in children ages 6 months to 21 years from 2010 to 2019. Medical charts were reviewed to determine OM diagnostic probability based on radiographic and microbiologic findings and the duration of prescribed antibiotic treatment for OM. Results Review of 3553 patients (18 039 person‐years) identified 20 episodes of probable OM in 19 children. Magnetic resonance imaging (MRI) findings to support OM were definitive in 4/19 (21%), probable in 10/19 (53%), suspected in 5/19 (26%), based on blinded radiologist review. Blood and/or operative cultures from bone and tissue debridement isolated Salmonella species in seven (35%) cases and methicillin‐susceptible Staphylococcus aureus (MSSA) in two (10%). Six patients received antibiotic treatment prior to obtainment of cultures. Of culture‐positive cases, MRI findings for OM were definitive or probable in six of nine (67%), suspected in three of nine (33%). Conclusions Distinction between OM and sickle‐related bone infarct or vasoocclusion is difficult based on imaging findings alone. Early attainment of blood and operative cultures increases the likelihood of identifying and adequately treating OM.

Background Early experience with the COVID-19 pandemic showed disproportionately high morbidity and mortality among individuals with certain chronic medical conditions. Individuals with sickle cell disease (SCD) are at high risk for pulmonary and other complications including acute chest syndrome (ACS) and have high rates of hospitalization from other viral respiratory infections, raising concern that COVID-19 would be associated with higher morbidity, mortality and health care utilization among those with SCD. Public health interventions such as social distancing, avoidance of large group activities, and widespread use of masks have been shown to reduce the transmission of COVID-19 in the general population but have been inconsistently implemented. In Georgia, COVID-19 restrictions, including school closures, were implemented in mid-March, and on-site school instruction was replaced by virtual instruction for the remainder of the school year. At our institution, most routine, non-urgent outpatient clinic visits were cancelled or postponed from mid-March through May in order to minimize COVID-19 exposure risk. Efforts to initiate the use of telemedicine as an alternative to in-person office visits were rapidly instituted. We hypothesized that adherence to public health restrictions, especially sheltering in place, would be high among patients and families with SCD, and sought to measure the impact of COVID pandemic on healthcare utilization in children and adolescents with SCD in the Atlanta area. Methods The SCD Program at Children's Healthcare of Atlanta (CHOA) provides comprehensive outpatient, emergency and inpatient services at 3 locations in metropolitan Atlanta. CHOA's Sickle Cell Clinical Database (SCCD) contains prospectively collected demographic, diagnostic, treatment and other clinical information on all patients with SCD beginning in 2010, including all outpatient clinic, emergency department (ED) and inpatient hospital utilization. To assess the impact of COVID-19 on healthcare utilization, we tracked clinic, ED and inpatient utilization for the 4-month period (March through June) 2020 compared with the same 4-month period in 2018 and 2019. Results The figure shows utilization patterns for each four-month period from 2018-2020. As expected, face to face outpatient clinic visits fell dramatically from February to April 2020 (-25% in March, -64% in April) and then returned to pre-COVID levels by June. The addition of telemedicine visits raised total outpatient visits in June 2020 to above pre-COVID levels. Total utilization during the 4-month period in 2020 were compared to the mean for the same periods in 2018 and 2019. Face to face clinic visits decreased from 2971.5 to 2023 (-32%), ED visits from 1,217 to 687 (-44%), and total inpatient admissions from 699 to 410 (-41%). Admissions with a primary discharge diagnosis of pain decreased from a mean of 407 in 2018-2019 to 173 (-57%), fever/infection from 67.5 to 40 (-41%), and ACS from 101 to 75 (-26%). Patients with chronic pain and/or history of high utilization (>5 admissions in a given year) showed decreases in utilization similar to all other patients. Summary These data describe the significant changes in utilization among pediatric patients with SCD during the COVID-19 pandemic. Face to face outpatient clinic visits decreased during March and April but returned to pre-COVID levels in June. Unexpectedly, ED and inpatient hospital utilization for acute illness decreased dramatically through April and remained low through June. In March there was a significant decrease in the clinic setting due to a large number of cancelled or rescheduled outpatient visits, despite many being rescheduled as telemedicine visits. However, the largest unexpected decrease was seen in emergency department visits and hospitalizations for acute events, specifically fever and pain events. It is also important to note the decreased utilization of patients with chronic pain who are typically high utilizers. During clinic encounters, families mentioned that less stress from school, reduced respiratory infections, and better medication adherence with parents at home, were possible contributors to reduced sickle cell symptoms while sheltering in place. These observations will guide the development of a patient survey with the goal of obtaining qualitative data to explain the reasons for decreased utilization during the pandemic. Figure Disclosures Lane: FORMA Therapeutics: Membership on an entity's Board of Directors or advisory committees; Global Blood Therapeutics: Membership on an entity's Board of Directors or advisory committees.