J Haem Pract 2014; 1(2):J Haem Pract 2014; 1(2): 15-20. doi: 10.17225/jhp00019

Authors: Elsbeth Müller-Kägi*, Manuela Albisetti, Andrea L Koppitz

Elsbeth Müller-Kägi*
Division of Haematology, University Children’s Hospital, Steinwiesstrasse 75, CH-8032 Zürich, Switzerland.

Manuela Albisetti
Division of Haematology, University Children’s Hospital, Steinwiesstrasse 75, CH-8032 Zürich, Switzerland.

Andrea L Koppitz
Zurich University of Applied Science ZHAW, Technikumstrasse 71, CH-8401 Winterthur, Switzerland.


Adolescents experience important changes in their physical, emotional, social and behavioural development. It is known that adolescents wish to be accepted by their peers, strive for independence and are prone to experiment. The challenge for adolescents with haemophilia is the need for taking responsibility for managing their illness and learning to comply with recommended treatment. This study aimed to investigate the process of adherence to treatment in adolescents with haemophilia.


The authors state that they had no interests that might be perceived as posing a conflict or bias.
The authors wish to thank all participants, and Brigit Brand, senior physician of the Division of Haematology at the University Hospital Zurich, for supporting this study. We also thank Professor Erika Froehlicher, Professor Emeritus Department of Physiological Nursing, Department of Epidemiology & Biostatistics, Schools of Nursing and Medicine at University of California San Francisco, and Dr Markus A. Müller for their editing of the English manuscript. The first author thanks Jan Devay, MSc, PharmD, from CSL Behring, for their support with an unrestricted grant for a Master’s of Science in Nursing.
EMK performed the research. EMK and ALK designed the research study. All three authors analysed the data and wrote the paper.


  1. Brackmann HH, Schwaab R, Oldenburg J, Schramm W. Klinische Anwendung plasmatischer und rekombinanter Gerinnungsfaktoren. Bremen: Uni-med; 2003
  2. Karp S, Riddel P. Bleeding disorders. In P. Jackson, J. A. Vessey & N. A. Schapiro (Eds.), Primary care of the child with a chronic condition (5th ed.). St. Louis: Mosby; 2010, pp. 243-261.
  3. Berntorp E. Joint outcomes in patients with haemophilia: the importance of adherence to preventive regimens. Haemophilia 2009; 15(6): 1219-1227.
  4. Lindvall K, Colstrup L, Wollter IM, et al. Compliance with treatment and understanding of own disease in patients with severe and moderate haemophilia. Haemophilia 2006; 12(1): 47-51.
  5. Manco-Johnson MJ, Abshire TC, Shapiro AD, et al. Prophylaxis versus episodic treatment to prevent joint disease in boys with severe hemophilia. N Engl J Med 2007; 357(6): 535-544.
  6. Bill S, Knight Y. Adolescence. In F. Valentine & L. Lowes (Eds.), Nursing care of children and young people with chronic illness. Oxford UK: Blackwell; 2007, pp. 203-233.
  7. Du Treil S, Rice J, Leissinger CA. Quantifying adherence to treatment and its relationship to quality of life in a well-characterized haemophilia population. Haemophilia 2007; 13(5): 493-501.
  8. Nazzaro A-M, Owens S, Hoots W, Larson KL. Knowledge, attitudes, and behaviors of youths in the US hemophilia population: Results of a national survey. American Journal of Public Health 2006; 96(9): 1618-1622.
  9. Vermeire E, Hearnshaw H, Van Royen P, Denekens J. Patient adherence to treatment: three decades of research. A comprehensive review. J Clin Pharm Ther 2001; 26(5): 331-342.
  10. Bill S, Hodges B. Transitional care. In F. Valentine & L. Lowes (Eds.), Nursing care of children and young people with chronic illness. Oxford UK: Blackwell; 2007, pp. 234-255.
  11. De Moerloose P, Urbancik W, Van Den Berg HM, Richards M. A survey of adherence to haemophilia therapy in six European countries: results and recommendations. Haemophilia 2008; 14(5): 931-938.
  12. Khair K. Minimizing joint damage: the role of nurses in promoting adherence to hemophilia treatment. Orthopaedic Nursing 2010; 29(3): 193-202.
  13. Llewellyn CD, Miners AH, Lee CA, et al. The illness perceptions and treatment beliefs of individuals with severe haemophilia and their role in adherence to home treatment. Psychology & Health 2003; 18(2): 185-200.
  14. Schrijvers LH, Uitslager N, Schuurmans MJ, Fischer K. Barriers and motivators of adherence to prophylactic treatment in haemophilia: a systematic review. Haemophilia 2013; 19(3): 355-361.
  15. Füessl HS, Middeke M. Anamnese und klinische Untersuchung (4th ed.). Stuttgart: Thieme; 2010.
  16. World Health Organization WHO. Adherence to long-term therapies: evidence for action. Geneva; WHO; 2003. Retrieved from
  17. Coulter A. Engaging patients in healthcare. Maidenhead, Berkshire: Open University Press; 2011.
  18. Law M, Finkelman S, Hurley P, et al. Participation of children with physical disabilities: relationships with diagnosis, physical function, and demographic variables. Scandinavian Journal of Occupational Therapy 2004; 11(4): 156-162.
  19. Khair K, Meerabeau L, Gibson F. Self-management and skills acquisition in boys with haemophilia. Health Expect 2013; doi: 10.1111/hex.12083
  20. Dodd M, Janson S, Facione N, et al. Advancing the science of symptom management. J Adv Nurs 2001; 33(5): 668-676.
  21. Helgeson VS, Reynolds KA, Siminerio L, et al. Parent and adolescent distribution of responsibility for diabetes self-care: links to health outcomes. J Pediatr Psychol 2008; 33(5): 497-508.
  22. Holman H, Lorig K. Patient self-management: a key to effectiveness and efficiency in care of chronic disease. Public Health Rep 2004; 119(3): 239-243.
  23. KyngAs HA, Kroll T, Duffy ME. Compliance in adolescents with chronic diseases: a review. J Adolesc Health 2000; 26(6): 379-388.
  24. Rankin SH, Duffy Stallings K, London F. Patient education in health and illness (5th ed.). Philadelphia: Lippincott Williams & Wilkins; 2005.
  25. Redman BK. Patientenedukation: Kurzlehrbuch für Pflege- und Gesundheitsberufe (S. Umlauf-Beck, Trans. 2nd ed.). Bern: Huber; 2009.
  26. Charmaz K. Constructing Grounded Theory: a practical guide through qualitative analysis. Los Angeles: Sage; 2006.
  27. Polit DF, Beck CT, Hungler BP. Lehrbuch Pflegeforschung. Methodik, Beurteilung und Anwendung (S. Bartholomeyczik, Trans. 1. ed.). Bern: Huber; 2004.
  28. Khair K. Supporting adherence and improving quality of life in haemophilia care. Br J Nurs 2013; 22(12): 692.
  29. Coulter A. Patient engagement - what works? The Journal of Ambulatory Care Management 2012; 35(2): 80-89.
  30. Lincoln YS, Guba EG. Naturalistic inquiry. Beverly Hills: Sage; 1985.
  31. Schweizerische Akademie der Medizinischen Wissenschaften SAMW. Forschung mit Menschen: Ein Leitfaden für die Praxis. Basel: Schwabe; 2009.

The Journal of Haemophilia Practice is published by Haemnet.

Haemnet is a registered charity that brings together and gives a voice to haemophilia nurses, physiotherapists and allied health care professionals, providing forums for collaborative research, educational activities and support.