title: Promoting self-management and adherence during prophylaxis: evidence-based recommendations for haemophilia professionals.
abstract: BACKGROUND
  Throughout life, a patient with severe haemophilia is confronted with many treatment-related challenges. Insight into self-management and non-adherence could improve the quality of care for these patients. The aim of this study was to provide an overview of the current evidence on self-management and adherence to prophylaxis in haemophilia.
  
  
  METHODS
  Based on series of studies and published literature, aspects of treatment were explored: learning and performing self-infusion, achieving self-management skills in adolescence, adherence issues and coping with haemophilia. Evidence-based and age-group-specific recommendations for haemophilia professionals were formulated.
  
  
  RESULTS
  Nearly, all severe haemophilia patients and parents were able to perform self-infusion and the quality level of infusion skills was acceptable. Learning self-infusion was generally initiated before the onset of puberty and full self-management was obtained 10 years later. Adherence was defined using a Delphi consensus procedure and was determined by skipping, dosing and timing of infusions. Adherence levels varied according to age, with highest levels in children (1-12 years) and the lowest among 25-40 years. Adherence to prophylaxis was acceptable (43%), yet 57% of the population struggled with prophylaxis. Qualitative research showed that the position of prophylaxis in life is the main driver of adherence. This position is influenced by acceptance and self-management skills. Regarding coping with haemophilia, the majority of patients used a problem-focused approach.
  
  
  CONCLUSIONS
  Self-management and adherence to prophylaxis vary during the life span. Acceptance of the disease and self-management skills were important aspects that may require tailored professional support.
authors.affiliation: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.
authors.forename: L H
authors.initials: LH
authors.lastname: Schrijvers
authors.affiliation: Nursing Science, Faculty of Health Care, University of Applied Science, Utrecht, the Netherlands.|Nursing Science, University Medical Center Utrecht, Utrecht, the Netherlands.
authors.forename: M J
authors.initials: MJ
authors.lastname: Schuurmans
authors.affiliation: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, the Netherlands.|Julius Centre for Health Sciences and Primary Care, University Medical Center, Utrecht, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Fischer
mesh_terms: D000223:Adaptation, Psychological; D000367:Age Factors; D003029:Coagulants / Q000627:therapeutic use*; D005169:Factor VIII / Q000627:therapeutic use*; D006467:Hemophilia A / Q000188:drug therapy*; D006801:Humans; D055118:Medication Adherence; D010819:Physician's Role; D036301:Qualitative Research; D012648:Self Care
chemical_list: D003029:Coagulants; D005169:Factor VIII
keywords: compliance; coping; nurse; prophylaxis; psycho-social; self-infusion