Introduction: Sickle Cell Disease (SCD) guidelines recommend that patients on hydroxyurea receive monitoring at least every 2-3 months, but it is unknown if this occurs in clinical practice. This study aimed to determine if patients with SCD at Nationwide Children's Hospital (NCH) had at least four, in-person monitoring visits during a 12-month period and if frequent monitoring was associated with hydroxyurea adherence and clinical outcomes.
Methods: We performed a retrospective analysis of children on hydroxyurea for at least 12 months during 2010-2015. Patients' demographics, laboratory studies, prescriptions, and number of hydroxyurea and acute visits were recorded from their 12-month period that met eligibility criteria. Patients were considered frequently monitored if they had ≥4 hydroxyurea visits and adherent if they had prescriptions for hydroxyurea for ≥80% of the days in their 12-month period.
Results: Seventy-four children met the eligibility criteria and 57 (77%) had frequent monitoring. The most common reason for not obtaining frequent monitoring was missing a scheduled appointment. A greater proportion of frequently monitored patients were adherent to hydroxyurea (66.7% vs. 17.7%, p<0.001) and they had significantly fewer acute visits (median 1 vs. 2 visits, p=0.032) compared to infrequently monitored patients.
Conclusions: Our study shows that most children on hydroxyurea at NCH received frequent monitoring and that it was associated with improved adherence and outcomes. Our results suggest that frequent in-person monitoring could be an opportunity to identify poorly adherent patients. These data inform our next quality improvement initiative that will maximize adherence to these monitoring guidelines.