Life expectancy for adults with sickle cell disease (SCD) has remained unchanged over the past 30 years despite improvements in pediatric SCD care and survival. Young adults who have recently transitioned from pediatric to adult care are at increased risk of death for several reasons. Inadequate preparation for transition to adult care, lack of adult hematologists with SCD expertise, and impaired cognitive function from either overt or silent cerebral infarcts caused by SCD contribute to the increased rate of acute care encounters and deaths in the 18-35 year age group and highlight the need for well-designed transition programs for adolescents and young adults (AYAs) with SCD. Allen County ranks 3rd in SCD incidence among the 92 counties in Indiana, and has 3 board certified Pediatric Hematologist-Oncologists who provide care to children who live in the region. When children "age out" of the pediatric system, there are no adult hematologists willing to accept these individuals into their practice. This leaves a significant number of adults with SCD without a medical home or providers who are comfortable prescribing hydroxyurea (HU) or managing the myriad of SCD complications that can occur in adulthood. A novel partnership between our Pediatric and Adult Hematology practice and an Allen County Family Medicine residency program was initiated to educate Family Medicine residents and attending physicians (FPs) about SCD, including HU. The long term goal is to improve rates of successful transition from pediatric to adult care for AYAs with SCD.

Three online educational modules about SCD (comprehensive care for AYAs with SCD, HU prescribing practices, best practices in transition for AYAs with SCD) were developed and CME-accredited. Prior to the rollout of the online educational modules, an electronic pre-test was distributed to 32 FP residents. The pre-test had 5 questions for each module. Questions about comprehensive care for AYAs covered reproductive health, contraception options for women with SCD, and recommended screening tests for AYAs with SCD. HU questions covered indications for and benefits of HU, dosing and monitoring for side effects. Questions about transition included social and cognitive milestones of adolescent development, the recommended components of a transition program, timing and need for SCD knowledge assessments. The protocol was reviewed by the St Vincent IRB which granted a waiver of consent.

Twenty-four of the 32 (75%) FP residents completed the pre-test knowledge assessment. One third of respondents correctly identified the rationale for HU treatment for SCD, but two-thirds correctly answered the question about the clinical benefits of HU. Half of the FPs selected the correct starting dose of HU for adults, but only one of the 24 (4.2%) respondents were able to correctly identify the age at which HU should be offered to infants with SCD. Three of the 24 (12.5%) FP residents correctly selected the most common cause of death in adults with SCD. Thirty percent correctly identified AYAs between 18 and 21 years of age as a high risk for mortality. Two questions regarding reproductive health were included in the pre-test; one-fifth of FP residents correctly answered the female contraception question, but none answered the priapism question correctly. Twenty-two of 24 (92%) FP residents understood that AYAs with SCD should know the hemoglobinopathy trait status of their partners because of risks of giving birth to an infant with SCD. The majority also correctly answered questions about developmental milestones of adolescents and were aware that AYAs with SCD should be knowledgeable about outpatient pain management techniques.

The majority of FP residents understand the clinical benefits of HU, but need more education about HU's mechanism of action and proper dosing. Additional education is also needed about appropriate contraceptive choices for women with SCD and priapism in men. An overwhelming majority of FPs correctly identified adolescent developmental milestones and understood that AYAs with SCD should have a baseline level of knowledge about their disease and its management. Future directions include comparing knowledge assessment results after the FP residents complete the three educational modules as well as repeating the knowledge assessments annually to assess knowledge retention.


Meier: Pfizer: Research Funding; CVS Caremark: Consultancy.

Author notes


Asterisk with author names denotes non-ASH members.