INTRODUCTION: Haemophilia is a hereditary haemorrhagic disorder characterized by deficiency or dysfunction of coagulation factors. Recurrent joint and muscle bleeds lead to progressive musculoskeletal damage. Haemophilia affects patients physically but also socially and psychologically. Traumatic experiences, chronic stress and illnesses can lead to mental disorders, but many persons with haemophilia maintain a highly positive outlook. AIM: To explore qualitatively which coping mechanisms persons with haemophilia use and in what way they help them to live with their diagnosis. METHODS: We recruited five adults with haemophilia and conducted semi-structured face-to-face interviews. Transcripts were analysed using interpretative phenomenological analysis (IPA). RESULTS: Two core themes emerged from the analysis: social support as an external factor and resilience as an internal factor of coping with the disease. Persons with haemophilia usually need help with health-related complications, and this affects the social support they require. Their wider support network tends to involve family and friends but also healthcare professionals and other specialists. This network provides practical help but also functions as an important psychological protective factor. An unexpected finding was that persons with haemophilia want not only to receive support but are also keen to offer support to others. CONCLUSION: These findings can help identify persons who provide most support to people suffering from haemophilia. Haemophilic centres should include in their teams psychologists and social workers and offer individual and group therapy to their clients, group meetings for friends and families of persons with haemophilia, provide learning resources to teachers aiming to incorporate children with haemophilia in their peer group, and organize Balint groups for physicians, psychologists and other healthcare professionals.

1st Department of Internal Medicine Faculty of Medicine Comenius University Bratislava Bratislava Slovak Republic

Department of Paediatric Haematology Haemophilia Comprehensive Care Centre Children's University Hospital Brno and Masaryk University Brno Czech Republic

Institute of Psychology Czech Academy of Sciences Praha Czech Republic

Olomouc University Social Health Institute Palacky University Olomouc Olomouc Czech Republic

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Peyvandi F, Jayandharan G, Chandy M, et al. Genetic diagnosis of haemophilia and other inherited bleeding disorders. Haemophilia. 2006;12(3):82‐89. PubMed

Peyvandi F, Garagiola I, Young G. The past and future of haemophilia: diagnosis, treatments, and its complications. Lancet. 2016;388(10040):187‐197. PubMed

Franchini M, Mannucci PM. Hemophilia A in the third millennium. Blood Rev. 2013;27(4):179‐184. PubMed

Oldenburg J. Optimal treatment strategies for hemophilia: achievements and limitations of current prophylactic regimens. Blood Rev. 2015;125(13):2038‐2044. PubMed

Whelan SF, Hofbauer CHJ, Horling FM, et al. Distinct characteristics of antibody responses against factor VIII in healthy individuals and in different cohorts of hemophilia A patients. Blood. 2013;121(6):1039‐1048. PubMed

CNHP . Czech national haemophilia programme. 2018 annual report. https://www.cnhp.cz/index.php?pg=registr–vysledky–2018. Accessed October 7, 2019.

Giangrande PLF, Hermans C, O'Mahony B, et al. European principles of inhibitor management in patients with haemophilia. Orphanet J Rare Dis. 2018;13(1):66. PubMed PMC

Balkaransingh P, Young G. Novel therapies and current clinical progress in hemophilia A. Ther Adv Hematol. 2018;9(2):49‐61. PubMed PMC

Schrijvers LH, Uitslager N, Schuurmans MJ, et al. Barriers and motivators of adherence to prophylactic treatment in haemophilia: a systematic review. Haemophilia. 2013;19:355‐361. PubMed

Hanley J, McKernan A, Creagh MD, et al. Guidelines for the management of acute joint bleeds and chronic synovitis in haemophilia. A United Kingdom Haemophilia Centre Doctors' Organisation (UKHCDO) guideline. Haemophilia. 2017;23(4):511‐520. PubMed

Holcomb JB. Methods for improved hemorrhage control. Crit Care. 2004;8(Suppl 2):57‐60. PubMed PMC

Lee CA, Kessler CM, Varon D, et al. The destructive capabilities of the synovium in the haemophilic joint. Haemophilia. 1998;4(4):506‐510. PubMed

Leslie R, Manno C. Modern management of haemophilic arthropathy. Br J Haematol. 2007;136(6):777‐787. PubMed

Manco‐Johnson MJ, Pettersson H, Petrini P, et al. Physical therapy and imaging outcome measures in a haemophilia population treated with factor prophylaxis: current status and future directions. Haemophilia. 2004;10(4):88‐93. PubMed

García‐Dasí M, Torres‐Ortuno A, Cid‐Sabatel R, et al. Practical aspects of psychological support to the patient with haemophilia from diagnosis in infancy through childhood and adolescence. Haemophilia. 2016;22(5):e349‐e358. PubMed

Holstein K, Eifrig B, Langer F. Relationship between haemophilia and social status. Thromb Res. 2014;104(1):53‐56. PubMed

Bottos AM, Zanon E, Sartori MT, Girolami A. Psychological aspects and coping styles of parents with Haemophilic child undergoing a programme of counselling and psychological support. Haemophilia. 2007;13(3):305‐310. PubMed

Thornburg CD, Duncan NA. Treatment adherence in haemophilia. Patient Prefer Adherence. 2017;11:1677‐1686. PubMed PMC

Trail S. Physical and psychosocial challenges in adult haemophilia patients with inhibitors. J Blood Med. 2014;5:115‐122. PubMed PMC

Färber F, Rosendahl J. The association between resilience and mental health in the somatically ill. Dtsch Arztebl Int. 2018;115(38):621‐627. PubMed PMC

Beeton K, Neal D, Lee C. An exploration of health‐related quality of life in adults with haemophilia – a qualitative perspective. Haemophilia. 2005;11(2):123‐132. PubMed

Canclini M, Saviolo‐Negrin N, Zanon E, et al. Psychological aspects and coping in haemophilic patients: a case–control study. Haemophilia. 2003;9(5):619‐624. PubMed

Binnema M, Schrijvers LH, Bos R, Schuurmans MJ, Fischer K. Coping in adult patients with severe haemophilia. Haemophilia. 2014;20(4):513‐518. PubMed

Lester S. An Introduction to Phenomenological Research. Taunton: Stan Lester Developments; 1999.

Brocki JM, Wearden AJ. A critical evaluation of the use of interpretative phenomenological analysis (IPA) in health psychology. Psychol Health. 2006;21(1):87‐108.

Tashakkori A, Teddlie C, eds. Handbook of Mixed Methods in Social & Behavioral Research. Thousand Oaks, CA: Sage; 2003.

Alase A. The interpretative phenomenological analysis (IPA): a guide to a good qualitative research approach. Int J Educ Lit Stud. 2017;5(2):9‐19.

Frohlich DO. The social support model for people with chronic health conditions: a proposal for future research. Soc Theor Health. 2014;12(2):218‐234.

Miller TA, DiMatteo MR. Importance of family/social support and impact on adherence to diabetic therapy. Diabetes Metab Syndr Obes. 2013;6:421‐426. PubMed PMC

Usta YY. Importance of social support in cancer patients. Asian Pac J Cancer Prev. 2012;13(8):3569‐3572. PubMed

Southwick SM, Vythilingam M, Charney DS. The psychobiology of depression and resilience to stress: implications for prevention and treatment. Annu Rev Clin Psychol. 2005;1(1):255‐291. PubMed

Inagaki TK, Orehek E. On the benefits of giving social support: when, why, and how support providers gain by caring for others. Curr Dir Psychol Sci. 2017;26(2):109‐113.

Rutter M. Resilience in the face of adversity: protective factors and resistance to psychiatric disorder. Br J Psychiatry. 1985;147:598‐611. PubMed

Harandi TF, Taghinasab MM, Nayeri TD. The correlation of social support with mental health: a meta‐analysis. Electron Physician. 2017;9(9):5212‐5222. PubMed PMC

Masten AS, Reed MJ. Resilience in development In: Snyder CR, Lopez SJ, eds. Handbook of Positive Psychology. New York: Oxford University Press; 2002:117‐131.

Gheslagh RG, Sayehmiri K, Ebadi A, et al. Resilience of patients with chronic physical diseases: a systematic review and meta‐analysis. Iran Red Crescent Med J. 2016;18(7):e38562. PubMed PMC

Santavirta N, Björvell H, Solovieva S, et al. Coping strategies, pain, and disability in patients with hemophilia and related disorders. Arthritis Care Res. 2001;45(1):48‐55. PubMed

Ridder D, Geenen R, Kuijer R. Psychological adjustment to chronic disease. Lancet. 2008;372(9634):246‐255. PubMed

Connelly LM. Trustworthiness in qualitative research. Medsurg Nursing. 2016;25(6):435‐436. PubMed