Clinical and radiographic scores in haemophilic arthropathies: how well do these correlate to subjective pain status and daily activities?

Abstract

Haemophilic patients who reached adulthood before the establishment of prophylactic treatment frequently show multiple and substantial arthropathies. The aim of this study was to determine to what extent haemophiliac's subjective impairment due to arthropathies correlates with objective clinical and radiographic parameters. By means of a questionnaire and a visual analogue scale, we consulted 79 haemophiliacs concerning their joint-pain status, how these were treated and to what extent their daily activities had been affected. Using a scoring system suggested by the Advisory Committee of the World Federation of Haemophilia, clinical evaluation was performed. Radiographs of 60 patients were assessed by means of the Petterson scale. The results were statistically compared. We found a significant correlation between pain intensity and clinical pathology as well as between pain intensity and radiographic joint damage for both knees and for the right ankle. The number of painful joints correlated well with the number of clinically/radiographically affected joints. The more pronounced the objective damage to joints, the more frequently patients claimed to have constant pain, depressive episodes and a dependency on pain-relieving medication. The more pronounced the objectively assessed damage to the knee and ankle joint, the higher the likelihood that the patient suffers from severe joint pain and reduction of activity. Treatment of painful symptoms from arthropathies is often insufficient. Scores and questionnaires may help to define the haemophiliacs pain status more clearly, thereby offering a possibility of assessment and long-term observation.

Surface electrical stimulation of the quadriceps femoris in patients affected by haemophilia A

“…The mean values of both legs reached a 32.76% and a 30.56% according to the Pettersson's and Gilbert's scores, respectively. These values are in agreement with those reported by Wallny et al [3], who stated that knees suffer from a larger number of injuries, which can be aggravated due to the fact that they also support the body weight.…”

Physical and psychosocial challenges in adult hemophilia patients with inhibitors

“…Studies have confirmed that patients with inhibitors experience greater ROM limitations and joint pain at an earlier age than those without inhibitors 3,4,15. Patients with high-titer inhibitors clearly demonstrate worse clinical and radiological joint scores than patients without inhibitors, and a three-fold increased risk of disability,3,4,12 due to more rapidly progressive joint disease 2,11,16…”

“…Hemophilic arthropathy is an ongoing cumulative process that eventually results in damaging joint effects 8–11. The long-term effects on joints include limited range of motion (ROM), deformity, crippling disability, and chronic pain 3,4,12–14.…”

Participation and risk‐taking behaviour in sports in children with haemophilia

“…The elbow, knee and ankle joint were chosen to measure because these joints are the frequently affected joints in haemophilia [7,8]. Children were asked to actively stretch or bend the joint maximally without interference by the investigator.…”

“…To measure healthrelated quality of life (HR-QoL), the Haemo-QoL Index was used [15]. The Haemo-QoL Index is the short-form of the Haemo-QoL questionnaire, which was specifically developed for use in children with haemophilia (aged [4][5][6][7][8][9][10][11][12][13][14][15][16] and demonstrated good psychometric properties [16,17]. Due to the extensive nature of the original Haemo-QoL questionnaires (64-77 items), a short-form was chosen.…”