Self-reported activity of Swedish persons with haemophilia: Change over 2.5 years

Abstract

LAY ABSTRACTCompared with the very recent past, persons with haemophilia currently living in Sweden have the opportunity to have prophylactic treatment to avoid frequent bleeding episodes in their joints and muscles. This makes it easier to live an active life. Using a questionnaire on self-reported activity, younger adults reported fewer difficulties than older persons. Over time the older persons reported increased difficulties in some of the more complex activities, such as playing games, sports, and going on active holidays. One reason could be that the older persons had no medical treatment when they were young so they had arthropathy due to bleeding in their childhood, perhaps combined with problems due to ageing. It is important to capture the person's own perspective of their abilities at their annual check-up.Objectives: To describe self-reported activity using the Haemophilia Activity List (HAL) for Swedish adults with haemophilia and to detect any changes over time. Method: The HAL was sent to the adult population with haemophilia A and B, moderate and severe form, living in Sweden (n = 260). Participants completed the HAL and a questionnaire on sociodemographic and medical information. From a previous study cohort, 61 persons had responded twice to the HAL. The investigated group was divided into early and later treatment onset groups with regard to access to medication. Results: The response rate was 50%. There was a significant difference (p < 0.001) between the early and later treatment groups in all domains in HAL. When analysing HAL "question by question" from the 2 reported time-points, the most prominent outcome was that the reported ability in activities was stable over time at the group level, except for participants who had no access to the clotting factor early in life. They reported greater limitations in some of the activities in the challenging domain "leisure activities and sport". Conclusion:The early treatment group reported a significantly better ability in all activities compared with the late treatment group.

Shortening the Haemophilia Activities List (HAL) from 42 items to 18 items

“…The HAL scores in the current data (median HAL sum: 65.0) were comparable to HAL scores in PWH from Jamaica (median: 66.1) and Brazil (weighted mean: 66.4), but lower than HAL scores in PWH from the United Kingdom (median: 80) and the Netherlands (median: 96) 18–21 . In Sweden, PWH with a later onset of treatment showed a median HAL sum score of 56, compared to a median of 98 in PWH with early treatment 22 . Therefore, the HAL short may still include some items with ceiling effects when used in populations with less limitations in activities and participation.…”

“… 18 , 19 , 20 , 21 In Sweden, PWH with a later onset of treatment showed a median HAL sum score of 56, compared to a median of 98 in PWH with early treatment. 22 Therefore, the HAL short may still include some items with ceiling effects when used in populations with less limitations in activities and participation.…”

Effectiveness of physical exercise on postural balance in patients with haemophilia: A systematic review

“…Self-perceived functionality and the risk of falls are also important variables as part of understanding the degree of affectation that bleeding causes in the joints and consequently in their stability. 14,[56][57][58] Future studies on balance should examine these measures in PWH.…”

“…In future studies, it would be interesting to carry out a specific description of the affected joints with or without arthropathy, in order to know more precisely the effect of the exercise on balance regarding to the grade of arthropathy. Self‐perceived functionality and the risk of falls are also important variables as part of understanding the degree of affectation that bleeding causes in the joints and consequently in their stability 14,56–58 . Future studies on balance should examine these measures in PWH.…”

Manual therapy effectively decreases the frequency of joint bleeding improves joint health and reduces pain in hemophilic elbow arthropathy: A prospective cohort study

“…The primary outcome of this study was frequency of bleeding. The bleeding rate in patients with haemophilic arthropathy may be overestimated due to the difficulty of differentiating between pain caused by arthropathy and acute bleeding (20). The principal researcher provided the patients included in the study with a self-report to record any bleeding (joint and muscle) experienced during the intervention or follow-up period.…”