Clinical Management Bundle for Patients with Sickle Cell Disease: Improving Completion of Age-Specific Screening Tests and Disease-Modifying Therapy Use

Introduction
Most patients with Sickle Cell Disease (SCD) in the United States do not receive evidence-based preventive care. Disease-modifying therapies (DMT) such as hydroxyurea have been shown to reduce or prevent many complications but are underutilized. Our large urban pediatric medical center follows approximately 950 children and young adults with SCD who require complex health care services and care coordination, including screening for early signs of disease-related complications. Care is provided at one large main campus site and two satellite sites. Preventive care includes urinalysis screening for nephropathy, dilated eye exam screening for retinopathy, and transcranial Doppler (TCD) ultrasound studies for stroke prevention. The SCD team, in conjunction with institutional quality improvement and analytics supports, created a “care bundle” encompassing the four components above as a clinical outcome metric for FY24 (July 2023-June 2024).

Objectives
We hypothesized that involving multiple disciplines and using electronic health record (EHR)-based tracking tools to support the care bundle model would improve rates of age-appropriate annual screenings and DMT prescribing. The aim was to increase composite bundle completion from an annual average of 60.6% for FY23 to > 66% for FY24, among patients with either SCD-SS or SCD-Sβ⁰ thalassemia, age 2.5-21 years, and who were not chronically transfused.

Methods
Bundle component specifications were ensuring TCD completion within 15 months for patients age 2.5-16 years, dilated eye exam and urinalysis completion within 2 years for patients age 11-21 years, and hydroxyurea or voxelotor prescribed at least once during the past 6 or 13 months, respectively, during FY24. Quality improvement methodology and tools, such as driver diagrams and process mapping, were
utilized to improve workflows to support the care bundle. Interdisciplinary teams comprised of key stakeholders from nursing, care coordination/scheduling, ophthalmology, phlebotomy, and radiology collaborated to support this work. Registry-based tracking tools in the EHR were tailored to care team workflows to quickly identify patients and ensure timely screening access. Nursing and scheduling teams provided iterative feedback to adapt the EHR tools for ease of use while prioritizing patient-centered care. Nursing workflow was altered to alert providers when DMTs needed to be prescribed, enhancing provider education and consistency.

Results
Completion of the composite SCD care bundle for the entire targeted population (395 patients on average) increased from 60.6% to 69.9%, exceeding our goal for FY24. All individual bundle components improved: Retinopathy screening increased from 49.5% as of June 2023 to 73.5% as of June 2024, nephropathy screening from 87.6% to 95.9%, stroke screening from 87.4% to 92.5%, and DMT prescriptions from 82.5% to 86.3%, respectively. The data was stratified by child opportunity index (COI) and payor and allowed the team to monitor for significant disparities in bundle completion, which were not observed. However, we did note differences between care sites; the composite bundle
rates increased from 65.5% for FY23 to 74.6% for FY24 at our main campus site, but only increased from 36.8% to 44.7%, respectively, at our satellite sites. In addition, because the SCD care team implemented their new processes for all patients with SCD, the rate of preventive screening completion for patients with other disease types improved as well. For example, for patients with either SCD-SC or SCD-Sβ⁺ thalassemia, retinopathy screening rose from 47.9% as of June 2023 to 68.0% as of June 2024, and nephropathy screening rose from 71.0% to 88.4% over the same time period.

Conclusion
The clinical management bundle successfully increased DMT prescribing rates and improved the rates of retinopathy, nephropathy, and stroke screening among patients with SCD, surpassing our goal. The substantial advances were a result of collaboration across specialties, staff roles, care sites, and the support provided by hospital leadership and institutional quality improvement teams. Future work will explore outcomes related to tighter DMT prescribing guidelines and improving patient adherence, decreasing the disparities in bundle completion rates across care sites, and streamlining next steps related to abnormalities identified on the various preventive screenings.