Validation of a new pediatric joint scoring system from the International Hemophilia Prophylaxis Study Group: Validity of the hemophilia joint health score

Abstract

Objective. Repeated hemarthrosis in hemophilia causes arthropathy with pain and dysfunction. The Hemophilia Joint Health Score (HJHS) was developed to be more sensitive for detecting arthropathy than the World Federation of Hemophilia (WFH) physical examination scale, especially for children and those using factor prophylaxis. The HJHS has been shown to be highly reliable. We compared its validity and sensitivity to the WFH scale. Methods. We studied 226 boys with mild, moderate, and severe hemophilia at 5 centers. The HJHS was scored by trained physiotherapists. Study physicians at each site blindly determined individual and total joint scores using a series of visual analog scales. Results. The mean age was 10.8 years. Sixty-eight percent were severe (93% of whom were treated with prophylaxis), 15% were moderate (24% treated with prophylaxis), and 17% were mild (3% treated with prophylaxis). The HJHS correlated moderately with the physician total joint score (r s ‫؍‬ 0.42, P < 0.0001) and with overall arthropathy impact (r s ‫؍‬ 0.42, P < 0.0001). The HJHS was 97% more efficient than the WFH at differentiating severe from mild and moderate hemophilia. The HJHS was 74% more efficient than the WFH at differentiating subjects treated with prophylaxis from those treated on demand. We identified items on the HJHS that may be redundant or rarely endorsed and could be removed from future versions. Conclusion. Both the HJHS and WFH showed evidence of strong construct validity. The HJHS is somewhat more sensitive for mild arthropathy; its use should be considered for studies of children receiving prophylaxis.

Progression of haemophilic arthropathy in children: a Lithuanian – Danish comparative study

“…This finding is in accordance with the results from several other studies that compared physical joint status in two different treatment groups and demonstrated superiority of prophylaxis over on‐demand treatment strategy in reducing the rate of joint deterioration . However, a direct comparison of clinical joint scores among studies is not possible as the HJHS, used in this study, is a new and recently validated tool .…”

Hemophilia Joint Health Score, Functional Independence Score in Hemophilia, and Pettersson Score in Pediatric Patients With Severe Hemophilia A

“…They also reported that worsening of the HJHS was noticed with increasing age, and the most critical and severe era for the development of hemophilic joint injury begins at the age of 7. Increasing the number of affected joints, the higher the HJHS; the HJHS was significantly positively correlated with the number of affected joints ( r =−0.508, P =0.001) and with the frequency of hemarthrosis ( r =0.389, P =0.001), similar to our findings, previous studies reported weak to moderate correlation between HJHS with the number of affected joints and the severity of hemarthroses 18,19 . In the current study, we observed a significant positive correlation between “patients” age and Pettersson score ( r =0.418, P =0.003).…”

Joint health scores in a haemophilia A cohort from Pakistan with minimal or no access to factorVIIIconcentrate: correlation with thrombin generation and underlying mutation

“…This study investigated musculoskeletal status in individuals with haemophilia A who had minimal access to both FVIII concentrate and multidisciplinary haemophilia care. HJHS and Gilbert score were found to be higher in severe compared with non-severe individuals, consistent with international studies in which cohorts had access to FVIII replacement: a Canadian study investigated 226 boys with mild, moderate or severe haemophilia (both A and B included) and found a higher HJHS in severe individuals [17]; a Dutch study reported the HJHS for 47 cases and concluded that individuals with mild or moderate haemophilia can be expected to have less joint damage [18]. Even though the individuals in those studies were on prophylaxis or on-demand therapy, the severity of haemophilia still played an important role in joint health, as found in our cohort which had minimal access to FVIII concentrate.…”