Hemophilia Activities List Questionnaire Detects Functional Limitations in Young Adults with Hemophilia A Prior to Physical Examination.

Abstract

Abstract 1300Poster Board I-322 BackgroundMeasurement of joint range of motion (ROM) identifies hemophilia patients with arthropathy, but may not reflect functional limitations. Methods to detect early functional decline have not been well-studied, but may be important in improving the care of patients with hemophilia. MethodsFunctional status was determined by Hemophilia Activities List (HAL), a self-assessment questionnaire. Normalized scores were calculated & included activities of upper extremities (UE), basic activities of lower extremities (LE), complex activities of LE, leisure activities & summary score. Scores range from 0-100, with 0 representing poor functional status & 100 the best functional status. Joint range of motion (ROM) was measured by goniometry by a single investigator. Overall joint index is calculated as previously described. (Soucie et al, Blood 2004; 103: 2467) Percent of overall joint ROM limitation is calculated as (patient joint index score/normal joint index score) X 100. T-test was performed to assess statistical significance. Results45 adults with Hemophilia A were studied, 33 severe and 12 mild. Mean age was 41, range 21-65. ROM limitations & functional status differed based on disease severity and age. ROM limitations increased and functional status decreased with age among patients with severe hemophilia A (Table), but there was no change in these parameters with age in persons with mild hemophilia (data not shown). Patients with severe hemophilia had decreased functional score in all areas, with lowest score in complex LE activities. The ability to perform basic & complex tasks of LE is decreased in severe hemophilia patients in the 3rd-4th decade when compared to a cohort with mild hemophilia (p<0.001), even though there is no significant decline in LE joint ROM limitations (20% vs 15%, p=0.21).TableDifferences in ROM Limitations and Functional Score between Severe and Mild Hemophilia PatientsMild Age 20-65 n=12Severe Age 20-39 n=15Comparison to Mild p-valueSevere Age 40-69 n=18Comparison to Severe age 20-39 p-valueJoint Bleeds/6 months0110.00290.4ROM LimitationsTotal ROM12%18%0.0131%0.002UE ROM9%16%0.00928%0.004LE ROM15%20%0.2140%0.002Functional Score (HAL)Summary95690.0005550.047Upper Extremities97780.009700.21Basic Lower Extremities95640.0004480.05Complex Lower Extremities88560.001290.002Leisure Activities95670.00154.14 ConclusionsSelf-reported functional limitations are common in severe hemophilia. Functional decline in tasks of the LE begins as early as the third decade of life, & occurs before significant limitations in joint ROM are detected. Therefore a self-reported questionnaire can be useful in detection of early disability in adults with hemophilia. Future studies should focus on early detection and treatment interventions to prevent further functional decline. DisclosuresNo relevant conflicts of interest to declare.