Adherence to prophylaxis and bleeding outcome in haemophilia: A multicentre study

Liesbeth H. Schrijvers*, Marlene Beijlevelt - van der Zande, Marjolein Peters, Janske Lock, Marjon H. Cnossen, Marieke J. Schuurmans, Kathelijn Fischer

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Prevention of bleeding and joint damage in severe haemophilia is dependent on adherence to prophylactic replacement therapy. The aim of this study was to assess adherence to prophylaxis, including associations with age, bleeding and clotting factor consumption (CFC). In three Dutch haemophilia centres, semi-structured interviews about adherence to prophylaxis in the previous 2 weeks were conducted with patients or parents of a child with haemophilia. Patients were classified, according to pre-specified definitions, as adherent, sub-optimally adherent or non-adherent based on missing, timing, and dose of infusions. Association of annual bleeding rates, mean CFC, person performing the infusion (parents verus patients) with adherence categories were analysed. Overall, 241 patients with haemophilia using prophylaxis were studied. Parents were more adherent (66%; n = 48/73) than patients (43%; n = 72/168). Sub-optimal adherence occurred in 29% of parents and 37% of patients and was characterized by changes in timing of infusion (mostly from morning to evening), while missing

Original languageEnglish
Pages (from-to)454-460
JournalBritish Journal of Haematology
Volume174
Issue number3
DOIs
Publication statusPublished - Aug 2016

Keywords

  • Adherence
  • Chronic
  • Hemophilia
  • Prophylaxis
  • Von Willebrand

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