title: Health-related quality of life and adherence to hydroxyurea in adolescents and young adults with sickle cell disease.
abstract: 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.
  
  
  
  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).
  
  
  
  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) (rs= 0.34, P = 0.04) and mean corpuscular volume (MCV) (rs= 0.42, P = 0.01) and inversely correlated with fatigue (rs= -0.45, P = 0.01), depression (rs= -0.3, P = 0.08), and social isolation (rs= -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).
  
  
  
  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.
authors.affiliation: Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.|Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.|Department of Pediatrics, Division of Hematology and Oncology, Zagazig University Faculty of Medicine, Zagazig, Egypt.
authors.forename: Sherif M
authors.initials: SM
authors.lastname: Badawy
authors.affiliation: Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.|Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
authors.forename: Alexis A
authors.initials: AA
authors.lastname: Thompson
authors.affiliation: Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.|Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Jin-Shei
authors.initials: JS
authors.lastname: Lai
authors.affiliation: Department of Medical Social Sciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Frank J
authors.initials: FJ
authors.lastname: Penedo
authors.affiliation: Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.|Stanley Manne Children's Research Institute, Ann & Robert H. Lurie Children's Hospital, Northwestern University Feinberg School of Medicine, Chicago, Illinois.
authors.forename: Karen
authors.initials: K
authors.lastname: Rychlik
authors.affiliation: Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, Illinois.|Division of Hematology, Oncology and Stem Cell Transplant, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois.
authors.forename: Robert I
authors.initials: RI
authors.lastname: Liem
mesh_terms: D000293:Adolescent; D000328:Adult; D000755:Anemia, Sickle Cell / Q000188:drug therapy*; D002648:Child; D003430:Cross-Sectional Studies; D005260:Female; D006801:Humans; D006918:Hydroxyurea / Q000008:administration & dosage* / Q000009:adverse effects; D008297:Male; D055118:Medication Adherence; D011788:Quality of Life; D055815:Young Adult
chemical_list: D006918:Hydroxyurea
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