The evaluation of the quality of life in patients with haemophilia

Joanna Owsianowska


Introduction: Haemophilia is a genetic disease. Bleeding, pain, temporary or long-term lack of physical activity, and even the risk of disability could have a negative impact on the quality of life.

The aim of the paper is to evaluate the quality of life of patients with haemophilia, and to provide answers to the following questions: 1. What is the quality of life of patients with haemophilia? 2. Which sphere of life is given the highest and which is given the lowest score? 3. Is there a connection between the sociodemographic data, the health situation and the quality of life?

Materials and methods: In total 163 adults diagnosed with haemophilia who are mainly treated at home and living in different parts of Poland were included in the survey. All sociodemographic data were collected using an author-designed questionnaire. The quality of life was measured using the WHOQOL-100 questionnaire proposed by World Health Organization.

Results: All surveyed patients were males diagnosed with haemophilia A, which is consistent with the epidemiology of the disease and the fact that haemophilia A is more frequent in Poland than haemophilia B. Most of the males (144) were diagnosed with severe haemophilia, 18 with moderate and only 6 with mild haemophilia. What is more, 126 (75%) respondents had type C hepatitis.

Conclusions: In the field of 6 life spheres measured with the use of the WHOQOL-100 questionnaire the highest score – 14.66 – was gained in the sphere of spirituality, religion and personal beliefs. The lowest score – 12.99 – was gained in the physical sphere, mostly concerning pain and feelings of discomfort. The outcome of 12.89 was gained in the environmental sphere. After detailed analysis of the researched material there was a conclusion made: 1. A group of men with inborn haemophilia demonstrate an average quality of life, comparable to the level of life of sick people living in other European countries. 2. The researched group give the highest estimate to the spiritual sphere of life, religion and personal beliefs, while the physical and environmental spheres were given the lowest estimate. 3. The degree of seriousness of haemophilia is of core importance when it comes to the level of life. Type C hepatitis and the status of pensioner is also a serious matter.


haemophilia; disability; quality of life

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White GC, Rosendaal F, Aledort LM, Luster JM, Rothschild C, Ingerslev J. Definitions in haemophilia.

Recommendations of the Scientific Subcommittee on Factor VIII and Factor IX of the Scientific and Standarization Committee of the International Society on Thrombosis and Haemostasis. Thromb Haemost 2001;85(3):560.

Plug J, Mauser-Bunschoten EP, Bröcker-Vriends AH, van Amstel HK, van der Bom JG, van Diemen-Homan JE, et al. Bleeding in carriers of hemophilia. Blood 2006;108(1):52-6. doi: 10.1182/blood-2005-09-3879.

Windyga J. Hemofilie – postępy w diagnostyce i leczeniu. Acta Haematol Pol 2010;41(2):183-99.

Windyga J. Skazy krwotoczne. Warszawa: PZWL; 2006. p. 70-7.

Bolton-Maggs PH. Optimal haemophilia care versus the reality. Br J Haematol 2006;132(6):671-82. doi: 10.1111/j.1365-2141.2005.05952.x.

Narodowy Program Leczenia Chorych na Hemofilię i Pokrewne Skazy Krwotoczne na lata 2012–2018. (5.05.2016).

Łopaciuk S. Zaburzenia krzepnięcia krwi. In: Dmoszyńska A, Robak T, editors. Podstawy hematologii. Lublin: Czelej; 2003. p. 373-82.

Amador-Medina LF, Vargas-Ruiz AG. Hemophilia. Rev Med Inst Mex Seguro Soc 2013;51(6):638-43.

Mannuccio Mannucci P, Franchini M. Present and future challenges in the treatment of haemophilia: a clinician’s perspective. Blood Transfus 2013;11 Suppl 4:77-81. doi: 10.2450/2013.012s.

Amstermark J, Dolan G, Hilberg T, Jimenéz-Yuste V, Laffan M, Lassila R, et al. Managing haemophilia for life: 4th haemophilia global summit. Haemophilia 2014;20 Suppl 5:1-20. doi: 10.1111/hae.12468.

Stephensen D, Drechsler WI, Scott OM. Biomechanics of lower limb haemophilic arthropathy. Blood Rev 2012;26(5):213-21. doi: 10.1016/j.blre.2012.06.003.

Windyga J. Jak w przyszłości będziemy leczyć hemofilię? Acta Haematol Pol 2008;39(4):651-60.

Batorova A, High KA, Gringeri A. Special lectures in haemophilia management. Haemophilia 2010;16 Suppl 5:22-8. doi: 10.1111/j.1365-2516.2010.02289.x.

Zimmermann R, Staritz P, Huth-Kühne A. Challenges in treating elderly patients with haemophilia: a focus on cardiology. Thromb Res 2014;134 Suppl 1:48-52. doi: 10.1016/j.thromres.2013.10.023.

Franchini M, Frattini F, Crestani S, Bonfanti C. Haemophilia B: current pharmacotherapy and future directions. Expert Opin Pharmacother 2012;13(14):2053-63. doi: 10.1517/14656566.2012.721780.

Markusic DM, Hoffman BE, Perrin GQ, Nayak S, Wang X, LoDuca PA, et al. Effective gene therapy for haemophilic mice with pathogenic factor IX antibodies. EMBO Mol Med 2013;5(11):1698-709. doi: 10.1002/emmm.201302859.

Windyga J. Profilaktyka krwawień u chorych na hemofilię A powikłaną inhibitorem czynnika VIII. Hematologia 2011;2(4):311-7.

Astermark J, Morado M, Rocino A, van den Berg HM, von Depka M, Grin­geri A, et al. Current European practice in immune tolerance induction therapy in patients with haemophilia and inhibitors. Haemophilia 2006;12(4):363-71. doi: 10.1111/j.1365-2516.2006.01296.x.

Colvin BT, Astermark J, Fischer K, Gringeri A, Lassila R, Schramm W, et al. European principles of haemophilia care. Haemophilia 2008;14(2):361-74. doi: 10.1111/j.1365-2516.2007.01625.x.

Astermark J, Rocino A, von Depka M, Van Den Berg HM, Gringeri A, Montovani LG, et al. Current use of by-passing agents in Europe in the management of acute bleeds in patients with haemophilia and inhibitors. Haemophilia 2007;13(1):38-45. doi: 10.1111/j.1365-2516.2006.01403.x.

Franchini M, Mannucci PM. Haemophilia A in the third millennium. Blood Rev 2013;27(4):179-84. doi: 10.1016/j.blre.2013.06.002.

Jaracz K. Jakość życia po niedokrwiennym udarze mózgu: uwarunkowania kliniczne, funkcjonalne, psychospołeczne i czasowe. Poznań: Katedra Pielęgniarstwa Akademii Medycznej im. Karola Marcinkowskiego w Poznaniu; 2002.

Hudak PL, Wright JG. The characteristics of patient satisfaction measures. Spine 2000;25(24):3167-77.

Hunt SM, McKenna SP. Quality of life claims in trials of anti-hypertensive therapy. Qual Life Res 1997;6(2):185-91.

Jończyk H, Reczek A, Brzostek T, Zdziarska J. Jakość życia chorych na hemofilię typu A. Probl Pielęg 2009;17(2):116-22.

Aznar JA, Garcia-Dasi M, Pérez-Alenda S, Marco A, Jaca M, Moret A, et al. Secondary prophylaxis vs. on-demand treatment to improve quality of life in severe adult haemophilia. A patients: a prospective study in a single centre. Vox Sang 2014;106(1):68-74. doi: 10.1111/vox.12066.

Varaklioti A, Kontodimopolus N, Katsarou O, Niakas D. Psychometric properties of the greek Haem-A-QoL for measuring quality of life in Greek haemophilia patients. Biomed Res Int 2014;2014:968081. doi: 10.1155/2014/968081.

von Mackensen S, Gringeri A, Siboni SM, Mannucci PM. Italian Of Haemophilia Centres (AICE). Haemophilia 2012;18(3):345-52.

Wojna D, Anwajler J, Mlądzka I, Ostrowska B, Skolimowski T. Wpływ fizjoterapii na stan czynnościowy stawów łokciowych oraz jakość życia chorych na hemofilię. Ortop Traumatol Rehabil 2008;3(6):315-22.

Pollmann H, Klamroth R, Vidovic N, Kriukov AY, Epstein J, Abraham I, et al. Prophylaxis and quality of life in patients with haemophilia A during routine treatment with ADVATE [antyhemofilac factor (recombinant), plasma/albumin-free method] in Germany: a subgroup analysis of the ADVATE PASS post-approval, non-interventional study. Ann Hematol 2013;92(5):689-98.

doi: 10.1007/s00277-013-1678-4.

World Federation of Haemophilia. Report on the Annual Global Survey 2012. http;// (6.11.2014).

Khawaji M, Astermark J, von Mackensen S, Akesson K, Berntorp E. Bone density and health-related quality of life in adult patients with severe haemophilia. Haemophilia 2011;17(2):304-11. doi: 10.1111/j.1365-2516.2010.02423.x.

Lindvall K, von Mackensen S, Berntrop E. Quality of life in adult patients with haemophilia – a single centre experience from Sweden. Haemophilia 2012;18(4):527-31. doi: 10.1111/j.1365-2516.2012.02765.x.

Khawaji M, Astermark J, Berntorp E. Life long prophylaxis in a large cohort of adult patients with severe haemophilia: a beneficial on orthopaedic outcome and quality of life. Eur J Haematol 2012;88(4):329-35. doi: 10.1111/j.1600-0609.2012.01750.x.

Plug I, Paters M, Mauser-Bunschoten EP, de Goede-Bolder A, Heijnen L, Smit C, et al. Social participation of patients with haemophilia in the Netherlands. Blood 2008;111(4):1811-15. doi: 10.1182/blood-2007-07-102202.

Kodra Y, Cavazza M, Schippati A, De Santis M, Armeni P, Arcieri R, et al. The social burden and quality of life of patients with haemophilia in Italy. Blood Transfus 2014;12 Suppl 3:567-75. doi: 10.2450/2014.0042-14s.

Carvalhosa AM, Henrard S, Lambert C, Hermans C. Physical and mental quality of life in adult patients with haemophilia in Belgium: the impact of financial issues. Haemophilia 2014;20(4):479-485. doi: 10.1111/hae.12341.

Szaniawska-Thiel M, Laguna P. O potrzebie pomocy psychologicznej dziecku choremu na hemofilię i jego rodzinie. Psychiatr Pol 2007;41(6):813-25.

Iannone M, Pennick L, Tom A, Cui H, Gilbert M, Weihs K, et al. Prevalence of depression in adults with Haemophilia. Haemophilia 2012;18(6):868-74. doi: 10.1111/j.1365-2516.2012.02863.x.

Farrugia A, Cassar J, Kimber MC, Bansal M, Fischer K, Auserswald G, et al. Treatment for life for severe haemophilia A – A cost-utility model for prophylaxis vs. on-demand treatment. Haemophilia 2013;19(4):228-38. doi: 10.1111/hae.12121.

Fischer K, Hermans C. The European Principles of Haemophilia Care: a pilot investigation of adherence to the principles in Europe. Haemophilia 2013;19(1):35-43. doi: 10.1111/j.1365-2516.2012.02928.x.

Dune T. Sexual expression, fulfilment and haemophilia: reflections from the 16th Australian and New Zealand Haemophilia Conference. Haemophilia 2012;18(3):138-9. doi: 10.1111/j.1365-2516.2011.02743.x.

Parish KL. Sexuality and haemophilia: connections across the life – span. Haemophilia 2002;8(3):353-9.


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