Abstract 1300

Poster Board I-322

Background

Measurement 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.

Methods

Functional 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.

Results

45 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).

Table.

Differences in ROM Limitations and Functional Score between Severe and Mild Hemophilia Patients

Mild 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-value
Joint Bleeds/6 months 0 11 0.002 9 0.4 
ROM Limitations      
Total ROM 12% 18% 0.01 31% 0.002 
UE ROM 9% 16% 0.009 28% 0.004 
LE ROM 15% 20% 0.21 40% 0.002 
Functional Score (HAL)      
Summary 95 69 0.0005 55 0.047 
Upper Extremities 97 78 0.009 70 0.21 
Basic Lower Extremities 95 64 0.0004 48 0.05 
Complex Lower Extremities 88 56 0.001 29 0.002 
Leisure Activities 95 67 0.001 54 .14 
Mild 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-value
Joint Bleeds/6 months 0 11 0.002 9 0.4 
ROM Limitations      
Total ROM 12% 18% 0.01 31% 0.002 
UE ROM 9% 16% 0.009 28% 0.004 
LE ROM 15% 20% 0.21 40% 0.002 
Functional Score (HAL)      
Summary 95 69 0.0005 55 0.047 
Upper Extremities 97 78 0.009 70 0.21 
Basic Lower Extremities 95 64 0.0004 48 0.05 
Complex Lower Extremities 88 56 0.001 29 0.002 
Leisure Activities 95 67 0.001 54 .14 
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Conclusions

Self-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.

Disclosures

No relevant conflicts of interest to declare.

Topics:
hemophilia a, physical examination, young adult, range of motion, hemophilias, functional status, early diagnosis, goniometry, joint disorders, severity of illness

Author notes

*

Asterisk with author names denotes non-ASH members.

© 2009 by The American Society of Hematology
2009
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