Search Results (4)

This study evaluates the health-related quality of life (HRQoL) among patients with hemophilia A currently undergoing prophylactic treatment at the Hemophilia Center of Northern Greece. Using the Haem-A-QoL questionnaire, we assessed various HRQoL dimensions in a cohort of 29 adult male patients, analyzing the impact of age, disease severity, and treatment regimens. The results revealed that younger patients (18–30 years old) exhibited significantly better overall HRQoL scores (total score of 25.36) compared to older age groups (37.81 for the 31–45 group and 43.71 in the 45+ group), particularly in the physical health (29.16 vs. 48.43 vs. 58.57) and mental well-being domains (25 vs. 37.11 vs. 41.07). Interestingly, moderate hemophilia patients reported lower HRQoL (42.31) than those with severe form (34.85), suggesting unique challenges in managing their condition. The ’Sports/Free Time’ domain had the highest scores (65.81), indicating significant limitations in physical activities in the everyday lives of affected individuals. However, better outcomes were observed in the mental dimension (36.09), work/study (34.88), family planning (10.68), and relationships aspects (16.67), where our cohort reported very low scores compared to similar studies, indicating a significantly better quality of life in these domains. These findings highlight the importance of personalized psychosocial support and targeted interventions to address the specific needs of hemophilia patients, particularly in enhancing physical activity opportunities and managing the psychological burden of moderate hemophilia. The study contributes valuable insights into the HRQoL of hemophilia patients and underscores the necessity for tailored approaches to improve patient outcomes across all dimensions of life. Full article

Background/Objectives: Hemophilia A is an X-linked recessive illness produced by a deficiency of coagulation factor VIII. This study aimed to evaluate serum vitamin D in hemophilic pediatric patients and its correlation with joint health and quality of life. Methods: This case-control study was performed on ninety children under the age of 18 years old and separated into two groups: study group of 45 children with hemophilia A and control group of 45 healthy children at an outpatient pediatric hematology clinic at the Beni-Suef University hospitals. Results: Serum vitamin D levels were significantly lower in hemophilia A patients than in controls (p < 0.001). The level of serum vitamin D was deficient in 38 (84.4%), insufficient in 4 (8.8%) and sufficient in 3 (6.6%) in the study group while deficient in 8 (17.7%), insufficient in 16 (35.5%) and sufficient in 21 (46.6%) in the control group. Total hemophilia joint health score (HJHS) had a significant negative correlation with serum total calcium (R = −0.31, p = 0.038) and serum vitamin D level (R = −0.974, p < 0.001) while also positively correlated with alkaline phosphatase (R = 0.834, p < 0.001). A quality-of-life index that is specific to total hemophilia (Haemo-Qol/Haem-A-QoL) had a significant positive correlation with total hemophilia joint health score (HJHS) (R = 0.934, p < 0.001) and negatively correlated with serum vitamin D level (R = −0.924, p-value lower than 0.001), alkaline phosphatase (R = 0.842, p < 0.001), and severity of hemophilia (R = 0.67, p < 0.001). Conclusions: patients with hemophilia A had lower vitamin D levels than healthy controls. The severity of vitamin D deficiency is related positively to (HJHS) hemophilia and quality of life hemophilia cases according to Haemo-QoL. Full article

Haemophilia presents a significant challenge to the quality of life of affected individuals. Evaluating the health-related quality of life (HRQoL) of people with haemophilia (PwH) provides a valuable mean of assessing their perception of overall care outcomes, while also identifying influential factors across various age and condition severity demographics. This observational retrospective study determined the HRQoL of 100 adult PwH in Northern Greece through comprehensive analysis and interpretation of their HRQoL levels, particularly in domains concerning their physical, emotional, and mental well-being, obtained through the Haem-A-QoL index questionnaire. Disease severity and young age were significantly associated with the administration of prophylactic treatment (84.2% of patients with severe haemophilia and 65.2% of patients aged 18–30). The mean Haem-A-QoL score was 40.11 ± 17.38, with the lowest HRQoL observed in the 46–60 age group (46.16), and the highest in the ≥61 age groups (35.16). Notably, the ‘Sports/Leisure’ and ‘Physical Health’ domains exhibited the highest scores, in contrast to ‘Family Planning’ and ‘Relationships/Sexuality’. Individuals with mild haemophilia recorded the lowest mean score (39.38), while those with a severe condition exhibited the highest (41.23). Age, disease severity, and physical activity emerged as primary determinants significantly affecting HRQoL outcomes. Full article

Hemophilia A is an X-linked coagulopathy, where there is a deficit in the production of the coagulation factor VIII. Even though there is a higher incidence of hemophilia A than of hemophilia B, it is still considered a rare disease, as its incidence is of 1 in 10,000 people born. We have applied three questionnaires regarding quality of life: Haem-A-QoL, Haemo-SYM and EQ-5D-5L to 101 adult patients with hemophilia A, which were separated into two groups: on-demand and prophylactic treatment. The results showed a relatively young lot, with medium and high education, but with a sedentary lifestyle and are pensioners. They also seem to have moderate mobility issues but, overall, a good quality of life. The quality of life in our studied lot is relatively good, but a more diverse lot is advised. Full article