Sickle Cell Disease: Addressing Stroke Risk Requires Action Beyond the Clinic

For children with sickle cell disease (SCD), the risk of stroke is a looming and life-altering threat. Yet, despite clear national guidelines, more than half of at-risk children never receive the simple, lifesaving screening that could prevent it.

A new study led by the Department of Pediatrics at UC San Francisco uncovers why improving care within clinics alone isn’t enough—systemic barriers outside the doctor’s office are preventing children from getting the care they need. The solution? A broader, population-level approach that reimagines how care is coordinated across hospitals, insurers, and entire healthcare systems.

A Preventable Risk with Life-Changing Consequences

“Transcranial Doppler (TCD) ultrasound screening identifies patients who are at risk of stroke, allowing clinicians to incorporate regular blood transfusions that reduce the risk by 90%,” said Madhav Vissa, MD, lead author of the study and UCSF Assistant Professor of Pediatrics in the Division of Hematology.

However, many children never make it to the appointment. The TCD test, which often requires a separate trip to a radiology clinic, creates logistical hurdles that families—many already navigating frequent hospitalizations, multiple specialists, and transportation barriers—often struggle to overcome.

The paper, recently published in Pediatric Blood & Cancer, emphasizes that population health approaches, such as developing care coordination programs through partnerships with health plans, are needed alongside in-clinic improvements to create a sustainable difference in stroke risk and health outcomes.

Why Clinic-Based Fixes Aren’t Enough

Despite national organizations issuing guidelines for yearly screening for children with SCD, clinics and patients face a host of difficulties completing the test, which commonly requires a separate appointment with a radiology clinic.

To address this, the Pacific Sickle Cell Regional Collaborative (PSCRC), a group of medical institutions and community-based organizations dedicated to improving SCD care, conducted a quality improvement study to increase TCD completion rates across 15 clinics. The PSCRC sites implemented a variety of interventions, including calling patients before appointments, automated appointment reminders, streamlining scheduling workflows, and providing staff with additional training.

Despite initial progress, the improvements didn’t last. The data suggested that clinic-based interventions alone can’t reach most at-risk children. “These results show that if we want real, lasting change, we need solutions that go beyond the walls of the clinic,” said Vissa.

Solving Challenges Families Face

The strains faced by families navigating SCD may be too complex for clinic-level solutions to address, requiring innovations at the hospital, insurer, and systemic levels. Failures of systems to coordinate multiple appointments, lack of accessibility in scheduling around school and work hours, transportation challenges, frequent hospitalizations, mistrust of the medical system, and complexities with disease management can all lead to low rates for preventative care appointments like TCD screenings.

UCSF Leading the Way

The UCSF Comprehensive Sickle Cell Center in Oakland and San Francisco is helping families overcome these challenges by offering TCD screening directly within the clinic.

“Solving this complex logistical hurdle at UCSF Benioff Children’s Hospital Oakland eliminates the need for a separate trip to a radiology clinic, saving families significant hassle and helping more children get their stroke risk analyzed,” said Vissa, who notes that smaller clinics may not have the resources for in-house TCD screening.

The PSCRC is essential in bringing together healthcare providers, community organizations, and other stakeholders to improve access to high-quality sickle cell care. Since 2014, the UCSF Department of Pediatrics has helped lead the consortium, with psychologist Marsha Treadwell, PhD, and hematologist Elliott Vichinsky, MD, serving as principal investigators. Treadwell, who also serves as regional director for the PSCRC, connected Vissa and the group of UCSF specialists to the clinic leads for the quality improvement collaboration.

 

Recent PSCRC studies on the telehealth needs of patients with SCD and why hydroxyurea therapy is underutilized were led by Treadwell and other UCSF Department of Pediatrics members. The PSCRC also uses telementoring sessions for providers who care for children with SCD, allowing UCSF experts to share knowledge and support those in rural areas or handling complex cases.

Through the PSCRC, pediatric specialists at UCSF can effectively share best practices and inform partnerships to make it easier for children with SCD to get the specialized services and preventative care they need to thrive.

Other authors from the UCSF Department of Pediatrics: Priya Parikh, MD; Neha Bhasin, MD; Marsha Treadwell, PhD; and Naomi Bardach, MD.

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