Enhancing Use of Hydroxyurea In Sickle Cell Disease Using Patient Navigators

The state of Virginia, including the Virginia Department of Health and three academic medical
centers and one federally qualified health center, plans a two-phase demonstration, first of
improvement in the percentage of adults with SCD who are in SCD specialty care (Phase I),
then of improvement in adherence to HU of eligible SCD adults (Phase II). Both phases will
use existing academic SCD providers, and an innovative, multimodal strategy, featuring
specially trained SCD patient navigators (PNs), that addresses barriers to care and to HU
use. In Phase I we will demonstrate the feasibility of utilizing PNs to overcome patient
access barriers to SCD care. In Phase II we will test the efficacy of PNs for overcoming
barriers to acceptance of and adherence with HU therapy. Patients will be randomized to a PN
arm or to a usual care arm. Providers will implement NIH guidelines for HU eligibility and
prescribing in both arms. All HU eligible patients will be offered HU at each clinical visit.
Among patients prescribed HU, if a maximum tolerated dose (MTD, defined in end points) has
not been reached, providers will dose escalate every 8-12 weeks to MTD, rather than to
clinical effect, before declaring treatment success or failure.

This project will be critically important and impactful by demonstrating the feasibility of a
statewide community-based strategy to assist vulnerable SCD adults in obtaining SCD specialty
care and likely prolonging life, a model that other states could adopt.

PHASE I:

Inclusion Criteria:

- Patient Self Report of Sickle Cell Disease (Genotypes: Hb SS, SC, SBoThal, SB+Thal)

- 15 years or older

- Virginia resident

Exclusion Criteria:

-Visited one of a pre-selected list of sickle cell specialists in Virginia within the last
6 months

PHASE II:

Inclusion Criteria:

- Sickle Cell Disease (SCD) patient (Genotypes: SS or SBoThal)

- Eligible for Hydroxyurea (according to NIH guidelines)

- 15 years or older

- Virginia resident

Exclusion Criteria:

- Pregnancy

- Enrollment in scheduled chronic transfusion program

- SCD Genotype: Hb SC and SB+Thal)