Quality of life of pediatric patients with haemophilia in the Czech Republic - experiences of one centre
Abstract
Aim of study: The Czech National Haemophilia Programme is focusing not only on the health status and treatment of patients but also on the quality of their life. Examples of patients of our Comprehensive Care Center (CCC) demonstrate how the patients themselves evaluate their health status and quality of life.
Methods and results: 183 questionnaires focusing on quality of life of 72 pediatric patients with haemophilia and 3 with Von Willebrand disease were collected. For small children the questionnaires were filled out by their parents, school children and older patients filled them out themselves.
The highest percentage of questionnaires were filled out by children of age between 12-14 years (22.9%) roughly 15% were done by age groups of 5-7, 15-17 and above 18 years the rest of age groups were negligible. 80.4% of questionnaires were filled out by boys with Haemophilia A the rest by patients with Haemophilia B and VWD. 58.7% of questionnaires were filled out by patients with severe type of Haemophilia. 42.5% were filled out by boys on permanent prophylactic treatment, 49.7% of questionnaires by boys with moderate and mild type of Haemophilia and on demand treatment. 3.9% of questionnaires were filled out by patients with inhibitor. 14 questionnaires from patients with temporary prophylactic treatment were excluded from analysis as a group difficult to evaluate. Therefore the total number of questionnaires was 165.
Responses were evaluated in 3 ways: percentage representation of respective responses, transformation of responses into numeric scale of 0-100 (the higher value the better health condition) and percentiles. Followed parameters of quality of life are rated in patients without inhibitor on numeric scale between 75-95 i.e. very positively.
In a small group of patients with inhibitor the evaluation is significantly worse and oscillates between 30 (sense of physical health) and 95 (satisfaction with treatment). Conclusion: Presented data show that availability of good quality substitution treatment, long term prophylaxis, home treatment and complex care for pediatric patients with Haemophilia in specialized centers brings positive results which are reflected also in positive evaluation of quality of life by patients themselves.