A long‐term study of hemophilic arthropathy of the knee joint on a program of factor VIII replacement given at time of each hemarthrosis

Abstract

Because there is little available data to demonstrate whether demand therapy is adequate to prevent progression of hemophilic arthropathy, a cohort of 64 patients with severe Hemophilia A (Factor VIII level less than 1%) and no inhibitor were studied with respect to the progression of knee arthropathy over a period of 6 years. Both degree of disease in flexion and extension and progression of disease were rated on an arbitrary scale. At the outset of the study, the majority of knees were either not restricted or mildly restricted as to motion. Of those knees that were not restricted at the outset, 96% remained not restricted. Of the knees that were mildly restricted, only 58% remained stable or improved over the 6 years, and of the knees that were moderately to severely restricted, 75% improved or remained stable. It thus appears that a demand therapy program can maintain normal range of motion in a knee joint. However, if the patient's joint has progressed to mild disease, the ability to maintain or improve the joint with demand therapy appears to be decreased.

Analgesic use and pain coping among patients with haemophilia

“…The other kinds of pain described by participants were consistent with previous reports that haemophilic arthritis caused by repeated joint bleeding is an important cause of morbidity in haemophilia [36]. The other kinds of pain described by participants were consistent with previous reports that haemophilic arthritis caused by repeated joint bleeding is an important cause of morbidity in haemophilia [36].…”

“…A second issue concerned the distinctiveness of bleeding pain and other kinds of pain. The other kinds of pain described by participants were consistent with previous reports that haemophilic arthritis caused by repeated joint bleeding is an important cause of morbidity in haemophilia [36]. Many of the other kinds of pain that were reported probably originated in previous bleeding episodes, and patients with haemophilic arthropathy may sometimes have difficulty distinguishing between a bleed and arthritic pain.…”

Hemophilia treatment in historical perspective: a review of medical and social developments

“…It has been suggested that early treatment of all joint bleedings (at the first sign, and at least within 2 h after onset) may also be highly effective [1,24,25,34]. This remains an important issue, since prophylactic treatment usually implies a large increase in clotting factor consumption.…”

North American prophylaxis studies for persons with severe haemophilia: background, rationale and design

“…Aronstam et al reported that resolution of joint haemorrhage was not dependent upon factor replacement dose in normal joints but required higher dosing in target joints [10]. Brettler et al conducted a similar 6-year observational study of 64 children and adults treated with a slightly higher dose of FVIII (25 U kg )1 ) and liberal use of repeat infusions at 24 h [18]. Brettler et al conducted a similar 6-year observational study of 64 children and adults treated with a slightly higher dose of FVIII (25 U kg )1 ) and liberal use of repeat infusions at 24 h [18].…”

“…Ekert et al reported on a 5-year observational study in which one-third of 24 children treated for haemarthroses with a single infusion of factor VIII (FVIII) at 20 U kg )1 developed decreased range of motion, implying that this dosage was inadequate for prevention and/or that children are at increased risk of joint damage [17]. Brettler et al conducted a similar 6-year observational study of 64 children and adults treated with a slightly higher dose of FVIII (25 U kg )1 ) and liberal use of repeat infusions at 24 h [18]. In the Brettler cohort, 96% of patients who entered the observational period with normal knee range of motion maintained normal values.…”