Hemophilia Joint Health Score Research Articles

Utility of the Haemophilia Joint Health Score in study of episodically treated boys with severe haemophilia A and B in Lithuania

Musculoskeletal outcome remains the major hallmark of haemophilia. The purpose of the study was to assess joint status using a new musculoskeletal assessment tool in children with haemophilia and describe the development of haemophilic arthropathy during childhood and puberty focussing on the age of remarkable changes. The prospective study involved Lithuanian patients aged 4-17 years with severe haemophilia A and B, no signs of inhibitors and treatment on-demand. Patients were subdivided into two groups according to actual age. Group I patients were 4-9 years and group II patients 10-17 years of age. The musculoskeletal status was measured using the Haemophilia Joint Health Score (HJHS). We report on 20 patients with a mean age of 11.5 years (SD 4.3, range 4-17.2 years). The mean HJHS score was 24.5 (SD 14.5, range 5-50). The most affected joints were ankles, followed by knees and elbows. Mean HJHS score in age group I (n = 7) was 11.6 (SD 6.5); in group II (n = 13) the score was significantly higher - mean 31.5 (SD 12.8) (P = 0.0002). Ankles, knees and elbows were significantly more impaired based on the HJHS scores in older patients as compared with younger ones. The HJHS appears to be a useful tool in evaluating musculoskeletal outcome of patients receiving treatment on-demand. Children > or =10 years of age had significantly higher HJHS scores as a sign of progressing haemophilic arthropathy. We conclude that the most aggravating development of haemophilic joint damage seems to occur from the age of 10 and onwards.

Linking the world with training and research for improving haemophilia care

Linking the world with training and research for improving haemophilia care

Outcome Assessment in Hemophilia: A Pilot Study on Correlation of Different Instruments.

Hemophilia is a rare disease, requiring life-long treatment with expensive clotting factor concentrates. Outcome assessment in hemophilia is important to establish both results of treatment, and compare between strategies. Since the 1980s, hemophilia specific assessment tools were available only for radiological (Pettersson score) and clinical (orthopedic joint score) joint damage. Recently, the HAL (Hemophilia Activities List) was developed to assess functional limitations, and the orthopedic joint score was adapted into the HJHS (hemophilia joint health score) to assess more subtle joint damage.To assess correlation of instruments for outcome assessment, a pilot study was conducted in 22 young adults with hemophilia (15 severe, 5 moderate, 2 mild). Joint damage (Pettersson score, HJHS), physical function (self-reported: HAL, performance-based: Figure 8 test; time taken to walk around two pilons at a distance of 5 meter at maximum speed in a pattern of figure 8), Health Related Quality of Life (SF36), and utility (EQ5D) were measured.Median age was 17.8 years (range 14–30), 15 (68%) patients were treated with early prophylaxis, including all but one patient with severe hemophilia who had recently stopped taking prophylaxis. Overall outcome was good, with median HJHS score of 3.5 (range 0–30; max 140, optimum 0), median HAL score of 100 (range 62–100; max 100= optimum), and median figure 8 test 4.9 sec (range 4.2–7.4). Recent (< 2 years of testing) Pettersson scores were available in only 11 patients (9 with severe, 2 with moderate hemophilia); the median score was 3 (range 0–28, max 78, optimum=0), the median physical component score of the SF36 was 53 (range 24–62), median utility (EQ5D) was 1.0 (range 0.7–1.0, optimum=1.0).Spearman's correlations for EQ5D, and Pearson's correlations for other instruments are shown in the table.StructuralFunctionalQuality of LifeHJHSPetterssonHALFig 8SF 36EQ5DHJHS1Pettersson0.861HAL−0.61−0.261Fig 80.510.51−0.561SF 36−0.73−0.410.81−0.541EQ5D−0.37−0.040.76−0.290.681The HJHS scores showed good correlation with the Pettersson score and the physical component score of the SF36. Correlation with functional outcome assessment was only moderate, while the HAL showed a high correlation with both SF36 and EQ5D. In conclusion, the HJHS is a promising instrument for structural assessment, but functional assessment using the HAL showes higher correlation with Quality of Life and utility. For full assessment of results of treatment and to compare different treatment strategies, functional assessment, in addition to structural assessment, is warranted.

Hemophilia joint health score reliability study

Measurement of joint health is critically important when assessing children with haemophilia. Few measures exist; they lack sensitivity to small changes, don't account for normal development and were never formally validated. To address these concerns, the Hemophilia Joint Health Score (HJHS) was developed by modifying existing scores. To test the inter-observer and test-retest reliability of the HJHS. Using a fully factorial design, four physiotherapists (from Canada, the United States and Sweden) examined eight boys with severe haemophilia A on two consecutive days using the HJHS. The boys ranged in age from 4-12 years and presented with variable joint damage. Six index joints (elbows, knees and ankles) were assessed on 11 impairment items including swelling, flexion and extension loss and gait. Concordance was measured by the intra-class correlation co-efficient. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest of 0.89. This study is the first in a series to assess the psychometric properties of the HJHS, a promising new measure of joint health in boys with haemophilia.

Recent developments in clinimetric instruments

Assessment of impairment and function is essential in order to monitor joint status and evaluate therapeutic interventions in patients with haemophilia. The improvements in the treatment of haemophilia have required the development of more sensitive tools to detect the more minor dysfunctions that may now be apparent. This paper outlines some of the recent developments in this field. The Haemophilia Joint Health Score (HJHS) provides a systematic and robust measure of joint impairment. The MRI Scoring System has been designed to provide a comprehensive scoring system combining both progressive and additive scales. The Functional Independence Score for Haemophilia (FISH) has been developed to assess performance of functional activities and can be used in conjunction with the Haemophilia Activities List (HAL) which provides a self report measure of function. It is recommended that both measures are evaluated as these tools measure different constructs. Further refinement and testing of the psychometric properties of all of these tools is in progress. More widespread use of these tools will enable the sharing of data across the world so promoting best practice and ultimately enhancing patient care.

Physical therapy and imaging outcome measures in a haemophilia population treated with factor prophylaxis: current status and future directions.

Routine infusions of factor VIII to prevent bleeding, known as prophylaxis, and other intensive therapies are being more broadly applied to patients with haemophilia. These therapies differ widely in replacement product usage, cost, frequency of venous access and parental effort. In order to address residual issues relating to recommendations, implementation, and evaluations of prophylaxis therapy in persons with haemophila, a multinational working group was formed and called the International Prophylaxis Study Group (IPSG). The group was comprised of haemophilia treaters actively involved in studies of prophylaxis from North America and Europe. Two expert committees, the Physical Therapy (PT) Working Group and the Magnetic Resonance Imaging (MRI) Working Group were organized to critically assess existing tools for assessment of joint outcome. These two committees independently concluded that the WFH Physical Examination Scale (WFH PE Scale) and the WFH X-ray Scale (WFH XR Scale) were inadequately sensitive to detect early changes in joints. New scales were developed based on suggested modifications of the existing scales and called the Haemophilia Joint Health Score (HJHS) and the International MRI Scales. The new scales were piloted. Concordance was measured by the intra-class correlation coefficient of variation. Reliability of the HJHS was excellent with an inter-observer co-efficient of 0.83 and a test-retest value of 0.89. The MRI study was conducted using both Denver and European scoring approaches; inter-reader reliability using the two approaches was 0.88 and 0.87; test-retest reliability was 0.92 and 0.93. These new PT and MRI scales promise to improve outcome assessment in children on early preventive treatment regimens.