Health-related quality of life in adult patients with hemophilia, Riyadh, Saudi Arabia

Background: Despite a reduction in mortality rates, hemophilia patients continue to suffer the consequences of repeated bleeding episodes. Assessment of health-related quality of life (HRQoL) attempts to determine how different life aspects are affected and helps guide future decision-making. AIMS: The aim of the study was to assess HRQoL in adult patients with hemophilia managed at King Khalid University Hospital (KKUH), Riyadh and to identify factors affecting HRQoL. Settings and Design: An observational cross-sectional study was conducted at KKUH in Riyadh from September 2014 to April 2015. Subjects and Methods: Sixty-one patients followed up and treated at KKUH from 2000 to 2015 were enrolled in the study. Patients were asked to complete the Arabic version of the Hemophilia-Specific QoL (HAEMO-QoL-A) Questionnaire. Results: Forty-Nine hemophilic patients completed the HAEMO-QoL-A questionnaire. Of these, 81.6% had hemophilia A, with a mean age of 29.84 years. The most common target joint was the knee (89.7%). The majority used on-demand treatment (55.1%). The HAEMO-QoL-A mean total score was 99.89. “Treatment concerns” were the most impaired dimension, whereas the “work/school, family, and social life” dimension was least affected. Patients receiving on-demand treatment had the lowest average bleeding events (8.22, P = 0.007). Conclusion: Frequent bleeding events, prophylactic treatment, and presence of clotting factor inhibitors negatively impact the HRQoL of patients with hemophilia

Effect of Poloxamer 188 vs Placebo on Painful Vaso-Occlusive Episodes in Children and Adults With Sickle Cell Disease: A Randomized Clinical Trial.

Key PointsQuestionCan poloxamer 188, an agent that is reported to reduce blood viscosity and cell-cell interactions, effectively reduce the duration of vaso-occlusive episodes (painful crises) in hospitalized patients with sickle cell disease? FindingsIn this randomized clinical trial that included 388 children and adults with sickle cell disease, treatment with poloxamer 188 vs placebo resulted in mean time to last dose of parenteral opioids during vaso-occlusive episodes of 81.8 vs 77.8 hours, a difference that was not statistically significant. MeaningAmong patients with sickle cell disease, poloxamer 188 did not significantly shorten the duration of painful vaso-occlusive episodes

Effect of poloxamer 188 vs placebo on painful vaso-occlusive episodes in children and adults with sickle cell disease : a randomized clinical trial

Key PointsQuestionCan poloxamer 188, an agent that is reported to reduce blood viscosity and cell-cell interactions, effectively reduce the duration of vaso-occlusive episodes (painful crises) in hospitalized patients with sickle cell disease? FindingsIn this randomized clinical trial that included 388 children and adults with sickle cell disease, treatment with poloxamer 188 vs placebo resulted in mean time to last dose of parenteral opioids during vaso-occlusive episodes of 81.8 vs 77.8 hours, a difference that was not statistically significant. MeaningAmong patients with sickle cell disease, poloxamer 188 did not significantly shorten the duration of painful vaso-occlusive episodes