Knowledge of disease and adherence in adult patients with haemophilia

Abstract

Patients with moderate and severe haemophilia are evaluated on a regular basis at their haemophilia centres but patients with mild haemophilia are seen less often because of fewer problems related to their disease. The needs of patients with milder forms of haemophilia, however, are often underestimated, both by the patient and staff at healthcare facilities. This study evaluated the knowledge of disease and adherence to treatment among patients with severe, moderate and mild haemophilia. This was a prospective multicentre study performed in Haemophilia Centres in Scandinavia. A total of 413 (67%) of 612 patients aged >25 years with mild, moderate and severe haemophilia completed a self-administered questionnaire. The mean age of the respondents was 49.7 years (range 25-87 years). Of the 413 respondents, 150 had a mild, 86 had a moderate and 177 had a severe form of haemophilia. A total of 22 (5%) patients did not know the severity of their disease, and 230 (56%) patients knew the effect of factor concentrate in the blood. Of the 413 respondents, 53 (13%) of the cohort never treated a haemorrhage. Patients with mild haemophilia, P </= 0.001, were the least likely to treat a haemorrhage. The relative number of patients who were afraid of virus transmission by factor concentrate was about similar in the three groups, 27% of those with severe haemophilia, 26% with moderate and 24% with mild haemophilia. This study shows that the amount of knowledge among haemophilia patients about their disease and treatment is somewhat limited, and demonstrates the importance of continually providing information about haemophilia and treatment, especially to patients with a mild form of the disease.

‘Just an unfortunate coincidence’: children’s understanding of haemophilia genetics and inheritance

“…Studies of young people and adults with haemophilia have shown that 69% of patients aged 13-25 years [21] and 96% of patients aged over 25 [22] were aware of heredity i.e. a healthy son and carrier daughter.…”

Coping strategies in young and adult haemophilia patients: A tool for the adaptation to the disease

“…Brodin et al also found that middle‐aged patients expressed better adaptation than younger patients. Another variable to take into account is the perception and attitude towards the disease; some authors point out how patients with moderate or mild forms of haemophilia show poorer coping strategies than in severe forms due to the lack of experience and knowledge of symptoms …”

Adherence-Related Issues in Adolescents and Young Adults with Hematological Disorders

“…instructions and effectiveness) [3,48,92,93], perceptions of one's ability to influence health outcomes [94] and confidence in one's ability to meet the specific demands of cancer treatment and recovery [95], are all cognitive and motivational processes with the potential to influence treatment adherence. For example, patients are often not aware of the therapeutic effects of potentially lifesaving treatment which they are prescribed [79]. Decision-making competence [96] and patient involvement in decision-making [97], along with faith in the treatment option and physician [39], are additional barriers to adherence.…”

“…Disease severity [79] and complications [80], along with adverse clinical experiences [76], have a negative impact on adherence.…”