Abstract
Introduction: Sickle cell disease (SCD) is a hereditary hemoglobinopathy characterized by abnormal hemoglobin S that polymerizes to result in vaso-occlusive episodes (VOEs), chronic hemolysis, and progressive end organ dysfunction, ultimately reducing quality of life and life expectancy. Hydroxyurea (HU), the mainstay disease-modifying therapy, improves clinical outcomes and survival in SCD. Timely initiation is critical for disease control, decreasing rates of VOEs, hospitalizations, morbidity, and mortality. SCD is a low prevalence condition in British Columbia (BC), Canada, but rates are rising due to immigration. Newly relocated residents living with SCD often face delays in accessing HU due to system constraints. Currently, HU is a limited-coverage drug for SCD in BC. Patients must either pay directly for their medication, have private coverage through employment, or enroll in a publicly funded, deductible-based medication coverage plan which requires filing an income tax return and providing an income statement. Additionally, physicians must complete an approval request for coverage. In contrast, HU is listed as a Class I benefit for cancer indications which eliminates direct costs to patients and the need for private insurance or approval forms.This study aims to characterize the causes and consequences of delayed HU initiation in patients with SCD after relocating to BC.
Methods: Patients with SCD who were new to the Adult Red Cell Disorders Program of BC between January 1, 2022 to February 28, 2025 were identified. Health records were retrospectively reviewed to identify baseline patient characteristics, baseline disease characteristics, date of first HU use in BC, reasons for HU delays, and complications prior to initiation. Outcomes included acute care utilization and disease-related morbidity prior to HU initiation. Descriptive analyses were applied.
Results: A total of 57 patients were seen in initial consultation at the Adult Red Cell Disorders Program of BC during the study timeframe. Median age at time of consultation was 28 years old (range, 18 – 66 yrs). Most commonly, patients were female (61.4%) and of African descent (78.9%). Few were refugees (10.5%). The predominant genotype was Hb SS (75.4%), followed by Hb SC (21.1%). Concurrent alpha-thalassemia was present in 21.1%. Of the 57 patients, just over half (52.6%) had no prior HU use before relocating to BC. Of the 43.9% with prior use, approximately half (21.1%) discontinued treatment upon relocation due to lack of access. The average interval between arrival in BC and first hematology consultation was 1 year and 8 months, and the average time from consultation to HU initiation was 14 months. In total, 64.9% experienced a delay in HU initiation (defined as not initiating/continuing HU upon arrival), while only 26.3% did not experience a delay. For 8.8% of patients, a delay was not applicable either because there was a medical contraindication (1 patient) or it was not clinically indicated (4 patients). The most common causes of delay included lack of timely referral to a hematologist due to a lack of access to a referring/primary care physician (40.5%), and delays in obtaining medication financial coverage (29.7%). Among the 35 who experienced delays, 24 (68.6%) experienced a SCD complication prior to HU initiation including 51 VOEs requiring healthcare contact and 12 episodes of acute chest syndrome, among others. This led to 14 emergency department visits and 40 hospital admissions. No deaths occurred during the study period. Overall, the healthcare cost of acute care utilization for sickle cell disease complications prior to HU initiation greatly outweighed the cost of the medication.
Conclusions: Patients with SCD who relocate to BC commonly experience delays in HU initiation due to referral lags to hematologists and systemic impediments in obtaining financial coverage. As a result, patients experience high rates of avoidable complications of SCD resulting in significant morbidity and acute care utilization. Efforts should be made to improve access by addressing system-level barriers that lead to delays, thereby improving patient health outcomes and reducing healthcare costs. Addressing this disparity would advance equity, bridging the gap between patients with SCD and those with malignancies, and ensuring that access to an essential medication is determined by clinical need, not diagnosis or systemic bias.
