Adherence to Hydroxyurea in Children With Sickle Cell Anemia
Adherence to Hydroxyurea in Children With Sickle Cell Anemia
Medication non-adherence is a true public health problem. Despite advancements in the
 molecular understanding of disease and improvements in therapy, patient health outcomes will
 not improve unless patients take prescribed medications regularly. Decreasing the gap between
 efficacious and effective therapy for patients with SCD is an essential research agenda.
 Hydroxyurea has been shown to be safe and efficacious in children and infants. However, the
 effectiveness of the prophylaxis depends on adherence to the recommended regimen. Medication
 adherence in SCD has previously been found to be sub-optimal in patients taking penicillin,
 desferoxamine, and pain medication. Adherence to HU has been studied to some extent in
 children with SCD. Based on estimates of adherence in other chronic illness we expect
 approximately 50% of patients to be >80% adherent with their HU administration. There is no
 gold standard for improving adherence to treatment. There have been a few attempts in the SCD
 population to improve adherence. These include a day camp to promote education about
 desferoxamine and peer support, a combination of a slide-show about SCD and it complications,
 weekly phone calls by the clinic social worker and a calendar, and a seven-phase educational
 program. Given the striking improvements in the peripheral blood smear findings of patients
 with SCD on HU therapy, with reduction in the numbers of sickled cells, we hypothesize that
 viewing the peripheral blood smear of patients with poor adherence to HU compared to a blood
 smear of someone on HU can be used to improve adherence in non-adherent patients.

 We will conduct a randomized trial between the intervention of regularly showing children and
 their parents the peripheral blood smear and standard care, including reminders of the
 importance of compliance and review of complete blood count parameters, including WBC, MCV,
 and Hgb concentration. The outcome measures will be increase in hemoglobin concentration and
 %HbF and increase in perceived QOL. QOL will be measured with age-appropriate and
 parent/proxy PedsQL™. Medication adherence will also be monitored throughout the study with
 pharmacy prescription refills, physician assessment, and self-report via a visual analogue
 scale. Adherence estimates, hemoglobin concentration, %HbF and QOL will be measured at
 baseline, 3 months and 6 months.
Inclusion Criteria:

 -  Sickle cell anemia

 -  on Hydroxyurea for at least six months

 -  age 2-17.9 years

 Exclusion Criteria:

 -  none