Assessment of musculoskeletal function and mood in haemophilia A adolescents: a cross-sectional study

Abstract

Haemophilia A is characterized by the occurrence of frequent spontaneous intra-articular and intramuscular bleeding. If inadequately treated, it results in progressive damage to joints and muscles leading to crippling deformities and musculoskeletal dysfunction. These complications result in lifelong chronic pain and disability that may greatly affect the patients' mood. We aimed to evaluate the musculoskeletal function in our haemophilia A patients and its correlation to depressed mood in these patients and determine the impact of degree of factor VIII deficiency, different replacement therapy regimens and frequency of hemarthrosis, on both musculoskeletal function and mood. A cross-sectional study was carried out on 50 adolescent haemophilia A patients. Musculoskeletal function was assessed using Functional Independence Score for Hemophilia (FISH) and mood status was assessed using Beck Depression Inventory-Short Form (BDI-SF). The mean FISH score was 23.32 ± 4.69 (range 13-28) and the tasks that obtained lower scores were step climbing, squatting and walking. Of our 50 patients included, 16(32%) were not depressed, 18(36%) were with mild depression, 11(22%) were with moderate depression and 5(10%) were with severe depression. There was a highly significant negative correlation between mean FISH score and mean BDI-SF score (P < 0.001). The better the replacement therapy regimen, the better the musculoskeletal function that could be obtained in haemophilia A patients and the better the mood.

Patient‐reported experience of bleeding events in haemophilia

“…A total of 20 male patients with moderate or severe haemophilia living in the United States completed the in-depth interviews. The sample included 10 adults (mean age = 41 yr; range: 19-52) and 10 adolescents (mean age = 13 yr; range: [12][13][14][15][16][17]. Four participants (2%) had three to five bleeds per month, nine (45%) had 1-2 bleeds per month and seven (35%) had a bleed once every 2 months or less (Table 1).…”

“…Patients, especially adolescents, sometimes expressed feeling depressed due to the fact that they are unable to participate in contact sports because of the likelihood of a bleed occurrence. The association between bleed events and depression in haemophilia was observed in a recent cross-section study of adolescents with haemophilia A that showed that adolescents with lower Functional Independence Score for Haemophilia (FISH) scores were more likely to have Beck Depression Inventory-Short Form (BDI-SF) indicative of depression (13).…”

Pain and structural alterations in knee joints in patients with haemophilia

“…Due to recurrent joint bleedings, various changes in the synovial tissue, the articular cartilage and the subchondral bone are observed . These changes are accompanied by crippling deformities and musculoskeletal dysfunction . Furthermore, a specific motor adaption to pain in the musculoskeletal system of PwH takes place .…”

“…9,10 These changes are accompanied by crippling deformities and musculoskeletal dysfunction. 11 Furthermore, a specific motor adaption to pain in the musculoskeletal system of PwH takes place. 12 There are first insights that a modified movement caused by pain leads to further problems in the long run.…”

Outcome Assessment in Hemophilia

“…The FISH was found to be highly reliable, with a pooled intraclass correlation coefficient of 0.98. In studies from Mexico [46] and Egypt [47], the FISH was found to discriminate the severity of hemophilia, as well as between patients and healthy controls. The FISH had a good correlation with the WFH clinical score (r = −0.61).…”