J Haem Pract 2016; 3(2):1 - 7. doi: 10.17225/jhp00084

Authors: Piet de Kleijn, David Sluiter, Kathelijn Fischer, Charles Vogely, Eveline Mauser-Bunschoten, Roger Schutgens

Piet de Kleijn
Department of Rehabilitation, Nursing Science and Sports and Van Creveldkliniek, University Medical Center Utrecht, The Netherlands. E-mail:

David Sluiter
Department of Rehabilitation, Nursing Science and Sports, University Medical Centre Utrecht, The Netherlands

Kathelijn Fischer
Van Creveldkliniek, University Medical Center Utrecht, The Netherlands

Charles Vogely
Department of Orthopaedics, University Medical Center Utrecht, the Netherlands

Eveline Mauser-Bunschoten
Van Creveldkliniek, University Medical Center Utrecht, The Netherlands

Roger Schutgens
Van Creveldkliniek, University Medical Center Utrecht, The Netherlands


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.


The present study was supported by an unrestricted grant from Bayer B.V., the Netherlands. It is an extension of the studies resulting from the BHAP Caregiver Award in 2011. The authors report no other conflicts of interest. The authors wish to thank Greig Blamey for his assistance with the manuscript.


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