Health-related quality of life and adherence to hydroxyurea in adolescents and young adults with sickle cell disease

Abstract

Background: Complications related to sickle cell disease (SCD) result in significant declines in health-related quality of life (HRQOL). While hydroxyurea reduces SCD complications, adherence remains suboptimal. The study's objectives were to assess the feasibility of Internet-based electronic assessment of HRQOL in SCD clinic and to examine the relationship between HRQOL and hydroxyurea adherence in adolescents and young adults (AYAs) with SCD.

Procedure: A cross-sectional survey was administered on tablets to 34 AYAs (12-22 years old) in a SCD clinic from January through December 2015. Study measures included Patient Reported Outcomes Measurement Information System (PROMIS^® ) computerized adaptive testing and ©Modified Morisky Adherence Scale 8-items (©MMAS-8).

Results: Participants (59% male, 91% Black) had median age of 13.5 (range 12-18) years. Ninety-one percent completed PROMIS® measures electronically in the clinic, meeting our feasibility criterion of ≥85% completion rate. ©MMAS-8 scores positively correlated with fetal hemoglobin (HbF) (r_s = 0.34, P = 0.04) and mean corpuscular volume (MCV) (r_s = 0.42, P = 0.01) and inversely correlated with fatigue (r_s = -0.45, P = 0.01), depression (r_s = -0.3, P = 0.08), and social isolation (r_s = -0.78, P = 0.02). Low ©MMAS-8 scores, indicating poor adherence, were associated with worse fatigue (P = 0.001) and trended toward significance for pain (P = 0.07) and depression (P = 0.06). Homozygous hemoglobin S disease patients with low HbF (<16%) had worse social isolation (P = 0.04) and those with low MCV (<102 fl) reported worse fatigue (P = 0.001), pain (P = 0.01), mobility (P = 0.01), and social isolation (P = 0.04).

Conclusions: HRQOL assessment in the SCD clinic is feasible. SCD patients with low hydroxyurea adherence and/or low HbF or MCV levels had worse HRQOL scores, particularly fatigue. Future prospective studies examining the relationship between HRQOL and hydroxyurea adherence are warranted.

Keywords: PROMIS®; adherence; adolescents; compliance; health-related quality of life; hydroxyurea; patient-reported outcomes; patient-reported outcomes measurement information system; sickle cell anemia; sickle cell disease; young adults.