A clinical profile of hemophilia patients and assessment of their quality of life in Western Uttar Pradesh, India: An observational study

Abstract

Objective: To describe the clinical profile of hemophiliac patients and their quality of life (QoL) in Western Uttar Pradesh. Materials and Methods: This was an observational cross-sectional study. Patients of all age groups with hemophilia admitted in the medicine and pediatrics ward for factor/fresh frozen plasma transfusion or having history of such type of transfusion were consecutively enrolled. A detailed clinical history was elicited from the accompanying parent/guardian. The HAEMO-QoL questionnaire was used for the assessment of QoL of the patients. Results: Out of the total 71 hemophilia patients, 66.2% (51/77) were >18 years of age. Type A hemophilia was observed in 88.3% (68/71) patients. Majority of the patients had severe hemophilia (80.5%). The family history and joint involvement were present in 58.4% and 77.9% of the patients, respectively. Knee joint was observed to be as the target joint among 57.1% of the patients. 76.6% of the patients had joint swelling, resulting in compromised joint movement. Bleeding occurred in soft tissues and joints in 62.3% and 15.6% of the patients, respectively. The patients had poor QoL scores. Overall, hepatitis B and C infection were observed in 6.5% and 9.1% of the patients, respectively. Orthophysiotherapy was needed in 22/26 (84.6%) patients. Conclusion: The severe type of haemophilia Type A was more common than mild and moderate types. The study suggests that important determinants for QoL in haemophilia patients are availability of factor concentrate from the clinical perspective and social and psychological support to ensure a holistic care approach.