Hemophilia Joint Health Score Score Research Articles

Functional limitations in Romanian children with haemophilia: further testing of psychometric properties of the Paediatric Haemophilia Activities List

Children with haemophilia often experience limitations in activities of daily life. Recently the Paediatric Haemophilia Activities List (PedHAL) has been developed and tested in Dutch children with intensive replacement therapy. The psychometric properties of the PedHAL in children not receiving intensive replacement therapy are not known. The objective was to gain further insight into the psychometric properties of the PedHAL and to study the functional health status of Romanian children and adolescents with haemophilia. Children attending to the rehabilitation centre of Buzias in Romania were sampled consecutively. Construct validity of the PedHAL was evaluated by concurrent testing with objective and subjective measures of physical function and functional ability. Reproducibility was tested by a 3-day test-retest by intraclass correlation coefficient (ICC) and limits of agreement (LOA). Responsiveness to rehabilitation was assessed by Haemophilia Joint Health Score (HJHS) and PedHAL. Twenty-nine children with severe (n = 25) or moderate (n = 4) haemophilia participated. Mean age was 13.2 years (SD 4.0). Median score of the PedHAL was 83.5 (IQR 47.9-90.5). The PedHAL correlated moderately with HJHS (rho = -0.59), Functional Independence Score in& Haemophilia (rho = 0.65) and Child Health Questionnaire-physical function (rho = 0.40) and not with Child Health Questionnaire-mental health, Child Health Questionnaire-behaviour and 6MWT. Test-retest reliability was good (ICC = 0.95). LOA was 17.4 points for the sum score. HJHS scores improved slightly after rehabilitation, whereas PedHAL scores did not change. In general, construct validity and test-retest reliability were good, test-retest agreement showed some variability. Therefore, currently the PedHAL may be more appropriate for research purposes than for individual patient monitoring in clinical practice.

Habitual physical activity in Dutch children and adolescents with haemophilia

For patients with haemophilia, a physically active lifestyle is important to maintain musculoskeletal health and to prevent chronic diseases, such as cardiovascular disease. Therefore, we studied physical activity levels, in Dutch children and adolescents with haemophilia as well as its association with aerobic fitness and joint health. Forty-seven boys with haemophilia (aged 8-18) participated. Physical activity was measured using the Modifiable Activity Questionnaire (MAQ) and was compared with the general population. Aerobic fitness was determined using peak oxygen uptake (VO(₂peak)). Joint health was measured using the Haemophilia Joint Health Score (HJHS). Associations between physical activity, joint health and aerobic fitness were evaluated by correlation analysis. Subjects were 12.5 (SD 2.9) years old, had a Body Mass Index (BMI) of 19.5 (SD 3.1; z-score 0.5) and a median HJHS score of 0 (range 0-6). Cycling, physical education and swimming were most frequently reported (86%, 69% and 50% respectively). Children with severe haemophilia participated significantly less in competitive soccer and more in swimming than children with non-severe haemophilia. Physical activity levels were similar across haemophilia severities and comparable to the general population. VO(₂peak) kg⁻¹ was slightly lower than healthy boys (42.9 ± 8.6 vs. 46.9 ± 1.9 mL kg⁻¹ min⁻¹; P = 0.03). Joint health, aerobic fitness and physical activity showed no correlation. Dutch children with haemophilia engaged in a wide range of activities of different intensities and showed comparable levels of physical activity to the general population. Aerobic fitness was well preserved and showed no associations with physical activity levels or joint health.

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.

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.