Comprehensive Sickle Cell Disease Management Is Associated with Superior Grade Attainment and High School Graduation Rates

Background: Sickle cell disease (SCD) in adolescents and young adults has been associated with low high school graduation rates. Complications of the disease may decrease school attendance, academic performance, and grade attainment. The graduation rate for patients with SCD enrolled in the Cooperative Study of Sickle Cell Disease (CSSD) was 71%. Poor educational outcomes might be due to a number of factors including socioeconomic status or days spent in the hospital due to illness. Neurological complications of SCD may also contribute. Stroke and microvascular ischemic events are detrimental to cognitive function and influence academic achievement and grade attainment. Patient and practitioner adherence to best practices, such as those described in the NHLBI 2014 guidelines vary widely, and the association of best practices with graduation rates has not been studied. We compared high school graduation rates of patients at Akron Children's Hospital Sickle Cell Disease Program (ACHSCDP) with national and local rates.

Methods: We conducted a retrospective chart review of patients born between 1994 and 2000 who received care for all forms of SCD at ACHSCDP. Patients were identified through an onsite registry. Thirty-two patients were identified for chart review. We defined successful high school attainment as graduation from 12^th grade, or having completed 11^th grade by July 2018 and enrolled to begin 12^th grade in Fall 2018, before or at 19 years of age. We determined successful high school attainment for patients at Akron Children's Hospital and assessed factors associated with success.

Results: Patients born from 1994 to 2000 with sickle cell disease (SS, SC, SBeta-0, SBeta-+) were analyzed (n=32). Two patients transferred out of ACHSCDP prior to age 14 and were not included in our analysis. Of 30 patients studied, 14 were male and 16 were female. Twenty-six patients (86.7%) had graduated or were starting senior year as of July 2018. Of the 26 successful students, 12 (46.2%) had either an IEP or 504 (federally defined accommodation plan) in place. None of the 4 unsuccessful students had an IEP, however 1 student had a 504. Of the 26 successful students, 13 had HbSS, 1 had HbS/B ^o thal, 5 had HbS/B ⁺ thal, and 7 had HbSC. All 4 unsuccessful students had HbSS. Of the 26 successful students, clinical data from 4 years before graduation or 3 years before completion of the 11^th grade revealed that the students were hospitalized an average of 5.77 days per year and visited the emergency department an average of 1.38 times per year. Of the 4 unsuccessful patients, data collected 4 years before the age of 19 revealed that the patients were hospitalized an average of 5.85 days per year and visited the emergency department an average of 1.37 times per year. Only one patient had a documented stroke, and this child successfully graduated from high school. Of the 26 successful students, 18 (69.2%) were enrolled in college after finishing high school and 3 students were in their senior year of high school.

Discussion: In this cohort, a high percentage of patients (86.7%) graduated or were on track to graduate from high school. In the United States, approximately 90% of patients with SCD are African American. All patients analyzed here were African American. With this in mind, ACHSCDP patients' graduation rate compares favorably to estimated graduation rates across the US general population (84%), the U.S. African America population (76%), the U.S. sickle cell patient population (71%), the Ohio general population (84%), and the Ohio African American population (68%).

The higher graduation rate among children at the ACHSCDP is associated with a multidisciplinary care model that prioritizes optimal hematologic care adhering to evidence based best practices including stroke risk screening and prevention, and use of hydroxyurea. In addition, team members assess for educations status and disease specific limitations at each encounter. A social worker advocates for educational needs. An outreach nurse makes school visits to educate staff and assist with implementation of federal accommodation plans. A teacher is available during inpatient stays to provide education in coordination with the patients' school. With comprehensive care, SCD is not a barrier to successful grade attainment, and academic distress in SCD patients should prompt re-evaluation of the patient's holistic medical care.