Medication Adherence Among Pediatric Patients With Sickle Cell Disease: A Systematic Review

Abstract

OBJECTIVES: Describe rates of adherence for sickle cell disease (SCD) medications, identify patient and medication characteristics associated with nonadherence, and determine the effect of nonadherence and moderate adherence (defined as taking 60%-80% of doses) on clinical outcomes. METHODS:In February 2012 we systematically searched 6 databases for peer-reviewed articles published after 1940. We identified articles evaluating medication adherence among patients ,25 years old with SCD. Two authors reviewed each article to determine whether it should be included. Two authors extracted data, including medication studied, adherence measures used, rates of adherence, and barriers to adherence. RESULTS:Of 24 articles in the final review, 23 focused on 1 medication type: antibiotic prophylaxis (13 articles), iron chelation (5 articles), or hydroxyurea (5 articles). Adherence rates ranged from 16% to 89%; most reported moderate adherence. Medication factors contributed to adherence. For example, prophylactic antibiotic adherence was better with intramuscular than oral administration. Barriers included fear of side effects, incorrect dosing, and forgetting. Nonadherence was associated with more vaso-occlusive crises and hospitalizations. The limited data available on moderate adherence to iron chelation and hydroxyurea indicates some clinical benefit. CONCLUSIONS:Moderate adherence is typical among pediatric patients with SCD. Multicomponent interventions are needed to optimally deliver life-changing medications to these children and should include routine monitoring of adherence, support to prevent mistakes, and education to improve understanding of medication risks and benefits. Pediatrics

Adherence to hydroxyurea, health-related quality of life domains, and patients’ perceptions of sickle cell disease and hydroxyurea: a cross-sectional study in adolescents and young adults

“…Nevertheless, hydroxyurea utilization and adherence remain suboptimal in this population [13–18]. Barriers to hydroxyurea adherence include forgetfulness, fear of side effects (e.g.…”

Outcomes of referrals to Child Protective Services for medical neglect in patients with phenylketonuria: Experiences at a single treatment center

“…1.7 Problems with adherence to medical management recommendations are not limited to PKU and are seen in many other chronic childhood diseases, including asthma [12], human immunodeficiency (HIV) [13], sickle cell [14], and leukemia [15]. Non-adherence in chronic disease, including PKU, can be observed in any or all of three areas: participation in health care (e.g.…”

Study protocol for a randomized controlled trial to assess the feasibility of an open label intervention to improve hydroxyurea adherence in youth with sickle cell disease

“…Medication nonadherence is common across chronic pediatric conditions [21–28], especially in populations with social, economic, linguistic and cultural barriers [18, 29–34]. The consequence of uneven HU use is that patients fail to maintain personal best HbF over time [9, 20, 21, 28, 35].…”

“…The consequence of uneven HU use is that patients fail to maintain personal best HbF over time [9, 20, 21, 28, 35]. Poor adherence to HU may occur frequently in this age group, especially outside of clinical trials [9, 20, 28].…”

“…The consequence of uneven HU use is that patients fail to maintain personal best HbF over time [9, 20, 21, 28, 35]. Poor adherence to HU may occur frequently in this age group, especially outside of clinical trials [9, 20, 28]. Older adolescents and young adults with SCD utilize health care at the highest levels of all age groups [36–38], and may be protected from cumulative disease burden by stable HU use starting in childhood [8], underscoring the imperative of establishing self-management of HU prior to adulthood.…”