Treatment-related knowledge and skills of patients with haemophilia and their informal caregivers
- 426 Downloads
- 4 Citations
Abstract
Background Replacement therapy in haemophilia plays an important role in the effective management of this rare bleeding disorder and requires a high level of knowledge and practical skills. Objective To evaluate and to compare the knowledge and skills about the medicines and their management among people with haemophilia treated with clotting factor concentrates, and their informal caregivers. Setting Eight Hospital Pharmacies working closely with Haemophilia Care Centres in France. Method In this cross-sectional and multi-centre study, 26-item questionnaire was specifically developed to assess the knowledge and skills. Face-to-face interviews using a questionnaire were conducted with patients and caregivers. Main outcome measure Level of knowledge and skills about disease, treatment and medication management. Results This study included 80 patients and 55 caregivers. Although most interviewees had basic knowledge of the treatment, only 43.7 % knew the effect of clotting factor concentrates on the haemostasis process. Similarly, only 12.7 % of the patients and 30.9 % of the caregivers referred to inhibitors as adverse effects. Despite intensive training for home treatment, 55.7 % reported difficulties with reconstitution or injection. The knowledge required for the responsible management of their medications had not been totally acquired: only 17 participants were indeed familiar with the medication storage conditions; 29 patients and 9 caregivers had already experienced an emergency which they had to treat with no medicine available at home; and 47.4 % of participants had already thrown away an unused drug vial. Conclusion This study shows the need of a regular update and to renew awareness of haemophilia treatment specificities among these populations. The identified needs suggest that we should more invest in educational techniques or therapeutic education programs more focused on medication management.
Keywords
Caregivers France Haemophilia Knowledge Patients Skills TreatmentNotes
Acknowledgments
The authors particularly thank all the patients and caregivers who participated in the study. We also thank the pharmacists who agreed to participate in this study: D. Hettler (Reims), K. E. Toguyeni (Lille), A. C. Gerout (Strasbourg), M. Dufour (Rouen) and E. Pelus (Colmar); as well as the HCCs of Lyon (Louis Pradel Hospital: S. Meunier, A. Lienhart, C. Négrier, A. Garcia, C. Lefranc, M. Gineste, M. Merzaq) and Paris (Cochin Hospital: N. Stieltjes, V. Roussel) for their cooperation and the development of the questionnaire.
Funding
None.
Conflicts of interest
The authors have no conflicts of interest to declare.
Supplementary material
References
- 1.O’Mahony B, Black C. Expanding hemophilia care in developing countries. Semin Thromb Hemost. 2005;31(5):561–8.CrossRefPubMedGoogle Scholar
- 2.Iorio A, Halimeh S, Holzhauer S, Goldenberg N, Marchesini E, Marcucci M, et al. Rate of inhibitor development in previously untreated hemophilia A patients treated with plasma-derived or recombinant factor VIII concentrates: a systematic review. J Thromb Haemost. 2010;8(6):1256–65.CrossRefPubMedGoogle Scholar
- 3.Makris M, Calizzani G, Fischer K, Gilman EA, Hay CRM, Lassila R, et al. EUHASS: the European haemophilia safety surveillance system. Thromb Res. 2011;127(Suppl 2):S22–5.CrossRefPubMedGoogle Scholar
- 4.Plug I, van der Bom JG, Peters M, Mauser-Bunschoten EP, de Goede-Bolder A, Heijnen L, et al. Thirty years of hemophilia treatment in the Netherlands, 1972–2001. Blood. 2004;104(12):3494–500.CrossRefPubMedGoogle Scholar
- 5.Rabiner SF, Telfer MC. Home transfusion for patients with hemophilia A. N Engl J Med. 1970;283(19):1011–5.CrossRefPubMedGoogle Scholar
- 6.Schrijvers LH, Beijlevelt-van der Zande M, Peters M, Schuurmans MJ, Fischer K. Learning intravenous infusion in haemophilia: experience from the Netherlands. Haemophilia. 2012;18(4):516–20.CrossRefPubMedGoogle Scholar
- 7.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):355–61.CrossRefPubMedGoogle Scholar
- 8.Lindvall K, Colstrup L, Wollter I-M, Klemenz G, Loogna K, Grönhaug S, et al. Compliance with treatment and understanding of own disease in patients with severe and moderate haemophilia. Haemophilia. 2006;12(1):47–51.CrossRefPubMedGoogle Scholar
- 9.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):931–8.CrossRefPubMedGoogle Scholar
- 10.Nilson J, Schachter C, Mulder K, Hahn M, Steele M, Hilliard P, et al. A qualitative study identifying the knowledge, attitudes and behaviours of young men with mild haemophilia. Haemophilia. 2012;18(3):e120–5.CrossRefPubMedGoogle Scholar
- 11.Simmons GM, Frick N, Wang A, Miller ME, Fragueiro D. Identifying information needs among children and teens living with haemophilia. Haemophilia. 2014;20(1):1–8.CrossRefPubMedGoogle Scholar
- 12.Khair K, Meerabeau L, Gibson F. Self-management and skills acquisition in boys with haemophilia. Health Expect. 2015;18(5):1105–13.CrossRefPubMedGoogle Scholar
- 13.Magli-Barioz D, Ounnoughène N, Paugy P, Quarrè M-C, Vassilief D, Lopez I, et al. Evaluation of the impact of information about treatment-related risks in patients receiving blood-derived or recombinant medications. Haemophilia. 2004;10(5):572–81.CrossRefPubMedGoogle Scholar
- 14.El Dakhakhny AM, Hesham MA, Hassan TH, El Awady S, Hanfy MM. Impact of health instructions on improving knowledge and practices of haemophilia A adolescents: a single centre experience. Haemophilia. 2014;20(4):e260–6.CrossRefPubMedGoogle Scholar
- 15.Mulders G, de Wee EM, Vahedi Nikbakht-Van de Sande MCVM, Kruip MJHA, Elfrink EJ, Leebeek FWG. E-learning improves knowledge and practical skills in haemophilia patients on home treatment: a randomized controlled trial. Haemophilia. 2012;18(5):693–8.CrossRefPubMedGoogle Scholar
- 16.Lindvall K, Colstrup L, Loogna K, Wollter I, Grönhaug S. Knowledge of disease and adherence in adult patients with haemophilia. Haemophilia. 2010;16(4):592–6.PubMedGoogle Scholar
- 17.Duncan N, Roberson C, Lail A, Donfield S, Shapiro A. A haemophilia disease management programme targeting cost and utilization of specialty pharmaceuticals. Haemophilia. 2014;20(4):519–26.CrossRefPubMedGoogle Scholar
- 18.Furmedge J, Lima S, Monagle P, Barnes C, Newall F. “I don’t want to hurt him”. Parents’ experiences of learning to administer clotting factor to their child. Haemophilia. 2013;19(2):206–11.CrossRefPubMedGoogle Scholar