Multiple joint procedures in haemophilia: benefit of self-reported activities

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This paper presents a long-term follow-up (mean 6 years) of three cases, providing insight into individual changes in self-reported activities of persons with haemophilia (PWH) who underwent multiple joint procedures (MJP). The procedures include one bilateral ankle pan arthrodesis by means of an ankle arthrodesis nail (case 1) and two times bilateral total knee arthroplasty (TKA), both type Genesis II (cases 2 and 3). MJP are defined as any combination of total hip arthroplasty (THA), total knee arthroplasty (TKA) and ankle arthrodesis (AA) during one hospital admission (one session or staged). The results of MJP need meticulous long-term follow-up, including tools with the capacity to provide detailed measurements for levels of activity. Measurements performed before and after surgery included active range of motion (AROM), the Hemophilia Activities List (HAL) sub-scores for basic and complex lower extremity and the McMaster Toronto Arthritis patient disability questionnaire (MACTAR). Pain scores were only available post-surgery and were by means of a visual analogue scale (VAS), 0 indicating no pain and 10 maximum pain. Post-operative VAS scores were 0.3 on average (range 0-1), indicating only minimal pain. Pre-operative pain was the actual indication for MJP. However, it was measured with a different score, the WFH score. Regarding post-operative AROM, case 1 showed a decreased ankle plantar flexion averaging 15˚ (range 10-20˚) and dorsiflexion averaging 7.5˚ (range 5-10˚), results expected after surgical fixation of the tibiotalar joint. Cases 2 and 3 showed an increased active flexion of both knee joints, on average 6° (range 5-10˚), and extension of 11° (range 0-20˚). From these two cases the HAL showed improvement in both basic (Δ 40 and Δ 30) and complex lower extremity (Δ 40 and Δ 3,5) activities. When considering the standard deviations of the HAL normalised total score of 18, it can be appreciated that these improvements are clinically relevant (effect size Δ 1). The MACTAR showed individual progress, with emphasis on walking, standing, riding a bicycle and walking stairs. These MJP cases provide insight on the benefits of self-reported activities, as well as the relationship between body and activity of the ICF. For the long-term follow-up of this very specific population, self-reported activities should be included.

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