Pb2274 prophylaxis adherence relative to quality of life and anxiety level in turkish patients with severe hemophilia a

Background: For patients with severe hemophilia A, bleeding increases anxiety and reduces quality of life (QoL). Prophylaxis is a therapeutic approach that enhances QoL for these individuals, but compliance is a major issue. Aims: This study investigated adherence to prophylaxis relative to QoL and anxiety level among patients with this condition. Methods: Forty-three patients with severe hemophilia A were stratified by age: 12 children in Group 1 (2-13 years), 17 adolescents in Group 2 (14-21 years), and 14 young adults in Group 3 (>21 years). Regular prophylaxis (RP) 25-40 IU/kg was prescribed three times weekly for 30 patients, and pharmacokinetic (PK) prophylaxis was prescribed for the remaining 13. Quality of life and anxiety level were assessed in parents of Group 1 patients and Group 2/3 patients using the 36-Item Short Form Health Survey (SF36) and the State-Trait Anxiety Inventory (STAI), respectively. The present study was approved by Gazi University ethical committee.Results were analysed using SPSS-15.0 program. Results: All 12 Group 1 patients (100%; nine RP, three PK) and 10 Group 2 patients (59%; all PK) adhered completely to prophylaxis. Seven Group 2 patients (41%) and four Group 3 patients (29%) received irregular prophylaxis. The remaining 10 Group 3 patients (71%) received on-demand treatment. Group 1 patients experienced vascular access problems due to young age. Seven (58%) of them were receiving home treatment. Nine Group 3 patients (64%) were reluctant to receive prophylaxis because they lived a sedentary life and were not participating in sports activities. Median QoL score was lower in Group 3 than in Group 1 parents and Group 2 patients (p<0.05 for both). Median anxiety level was higher in Group 1 parents and Group 3 patients than in Group 2 patients (p<0.05 for both). Summary/Conclusion: The results suggest that PK prophylaxis would improve QoL and reduce anxiety for young adults with severe hemophilia A. The findings also indicate that improved family education about vascular access for home treatment would improve QoL and reduce anxiety for children with this condition.