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- Title
Health-related quality of life in patients with haemophilia and inhibitors on prophylaxis with anti-inhibitor complex concentrate: results from the Pro- FEIBA study.
- Authors
Gringeri, A.; Leissinger, C.; Cortesi, P. A.; Jo, H.; Fusco, F.; Riva, S.; Antmen, B.; Berntorp, E.; Biasoli, C.; Carpenter, S.; Kavakli, K.; Morfini, M.; Négrier, C.; Rocino, A.; Schramm, W.; Windyga, J.; Zülfikar, B.; Mantovani, L. G.
- Abstract
Patients with haemophilia A and inhibitors are at high risk for severe bleeding, progression of joint disease and deterioration of health-related quality of life ( HRQoL). To determine the impact of prophylaxis with an activated prothrombin complex concentrate ( aPCC) on HRQoL, HRQoL was assessed using the Short-Form (SF)-36 Health Survey and the EQ-5D questionnaire in subjects ≥14 years participating in a prospective, randomized, crossover study comparing 6 months of aPCC prophylaxis with 6 months of on-demand therapy. Eighteen of 19 patients completed the survey or questionnaire before and after the on-demand therapy and prophylaxis periods. A general trend towards improved HRQoL after prophylaxis was observed for the 18 evaluable patients in all SF-36 dimensions except for vitality/energy and physical functioning. After prophylaxis, 'good responders,' defined as patients experiencing ≥50% reduction in bleeding, exhibited statistically and clinically significant differences in the physical component score ( P = 0.021), role - physical ( P = 0.042), bodily pain ( P = 0.015), and social functioning ( P = 0.036). Similarly, the EQ-5D health profile showed a trend towards improvement after prophylaxis in all evaluable patients. Among the good responders, improvements did not differ from those observed after on-demand treatment. EQ visual analogue scale values were slightly improved following prophylaxis for all evaluable patients and the EQ-5D utility index improved in the good responders only. During prophylaxis, patients missed significantly fewer days from school or work because of bleeding than during on-demand treatment ( P = 0.01). In conclusion, by significantly reducing bleeding frequency in good responders, aPCC prophylaxis improved HRQoL compared with on-demand treatment.
- Subjects
HEMOPHILIACS; QUALITY of life; BLOOD coagulation disorders; HEMOPHILIA treatment; PROTHROMBIN
- Publication
Haemophilia, 2013, Vol 19, Issue 5, p736
- ISSN
1351-8216
- Publication type
Article
- DOI
10.1111/hae.12178