Addressing the issues of complex care from the perspective of people with haemophilia

Czech version

Authors: K. Ratajová ¹; J. Blatný ²; I. Poláčková Šolcová ^1,4; M. Bohůn ³; Z. Meier ¹; T. Horňáková ¹; R. Brnka ^1,5; P. Tavel ¹
Authors‘ workplace: Univerzita Palackého v Olomouci, Institut sociálního zdraví Univerzity Palackého v Olomouci, Olomouc ¹; Oddělení dětské hematologie, FN Brno a Masarykova Univerzita, Brno ²; Český svaz hemofiliků, Praha ³; Psychologický ústav Akademie věd České republiky, Praha ⁴; První interní klinika, Lékařská fakulta Univerzity Komenského v Bratislavě ⁵
Published in: Transfuze Hematol. dnes,26, 2020, No. 3, p. 196-200.
Category: Original Papers

Overview

Introduction: Haemophilia is a hereditary disease characterised by shortage or dysfunction of specific proteins involved in blood coagulation. Repeated bleeds into joints and muscles lead to severe and progressive damage to these tissues. Adults with haemophilia face a wide range of problems associated with their condition. Analysis of interviews undertaken as part of a pilot study and focusing on social support, resilience and quality of life of haemophilia sufferers revealed some important and problematic aspects of the system of care for these people.

Goals: To identify problematic areas in the care for persons with haemophilia as well as issues viewed as challenging by our respondents, persons suffering from haemophilia. Methods: Five adult men suffering from haemophilia an residing in the Czech Republic participated in our study. We conducted semi-structured in-depth interviews focusing on the quality of life of persons with haemophilia. Interviews were then analysed according to the issues that arose.

Results: Problematic areas in the system of haemophilia care were assessed based on respondents’ views and divided into (1) haemophilia treatment, (2) haemophilia awareness and (3) financing of haemophilia treatment and health haemophilia-associated impairments.

Conclusion: The issue of establishing an optimal system of care for persons with haemophilia is highly complex. This study’s findings could contribute towards improving care for haemophilia sufferers. Information on shortcomings of currently available care coming from patients themselves could moreover serve as feedback for patients’ organisations, state institutions and members of specialised haemophilia teams. Our findings should be developed and extended by further studies in the future.

Keywords:

haemophilia – care – treatment – information – qualitative analysis

Sources

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

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

3. 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.

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

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

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

7. 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.

8. 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.

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

10. 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.

11. Trail S. Physical and psychosocial challenges in adult hemophilia patients with inhibitors. J Blood Med. 2014;5:115–122.

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

13. Tashakkori A, Teddlie C. (eds.) Handbook of mixed methods in social & behavioral research. Thousand Oaks, CA: Sage; 2003.

14. Guest G, MacQueen KM, Namey EE. Introduction to Thematic Analysis. In: Guest G, MacQueen KM, Namey EE. Applied Thematic Analysis. Lodon: Sage, 2012;3–21.

15. Braun V, Clarke V. Using thematic analysis in psychology. Qualitative Res Psychol. 2006;3(2):77–101.

16. Smejkal P, Blatný J, Hluší A, et al. Konsenzuální doporučení Českého národního hemofilického programu (ČHNP) pro diagnostiku a léčbu pacientů s hemofilií. Transfuze Hematol Dnes. 2017;23(1):82–99.

17. Leung L. Validity, reliability and generalizability in qualitative research. J Med Primary Care. 2015;4(3):324–327.

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