Joint Pain and quality of life in adult haemophilia- a case series

Background: Haemophilia is a hereditary X-linked recessive disorder characterized by the deficiency of factor VIII or IX coagulant activity. Internal and prolonged bleeding into the joint space and muscles are the main symptoms of haemophilia which inadvertently results into haemophilic arthropathy and affects the individual’s life. This study was conducted to gain an insight into the impact of haemophilia on the Quality of Life (QoL) of haemophiliac adults. Methods: This was a case series which included 30 patients, who were screened for the inclusion and exclusion criteria and enrolled in the study. Male patients with Haemophilia A or B who were undergoing treatment at the hospital and in the age group of 18-60 yrs. with complications of Haemophilia such as joint swelling, spontaneous bleeding etc. were included in the study. Patients with and musculoskeletal, neurological or cardiovascular conditions not associated with Haemophilia and other bleeding tendencies such as liver failure, anti- coagulant drugs, etc. were excluded from the study. The outcome measures used were the HAEM- A – QOL, which is an adult version of the quality of life in Haemophilic adults and the Numerical Pain rating scale (NPRS). Results: Total 30 patients were included in this study, the HAEM- A- QOL questionnaire was administered and NPRS was taken. The average age of the patients was 29 years (±3.97) and mean NPRS score was 4.2 (±1.31). 40% of the subjects had elbow as their target joint and rest 60% had knee as their target joint. The Mean and Standard Deviation for various subscales were: 1. Day to Day activities – 56(±6.99) 2. Mood and Feelings – 65.1(±7.32) 3. Work or school life, family life and Social Life – 62.8(±2.17) 4. Haemophilia Treatment – 30.5 (±7.56). Average QOL of Life was 53.6, with standard deviation ±10.46 in adult male haemophilia patients. Conclusion: The present study concludes that there was a moderate affection of the quality of life in these patients. According to the HAEM- A- QOL, the emotional and behavioural aspect was severely affected followed by the Social life and the Activities of Daily living, which were moderately affected. Worries/ concerns related to the treatment were mildly affected.