Patient-Related Outcomes of Elective Major Orthopedic Surgery in Severe Hemophilia Patients: A Review of the Literature and a Proposed Agenda for Research.
Authors: Botteman Marc F. , Ballal Rahul , Joshi Ashish V. 
Published: October 2019

Abstract
Objectives: Severe hemophilia patients experience frequent spontaneous joint hemorrhages, which lead to severe pain and swelling, reduced flexion and function, and ultimately debilitating arthropathy. Elective major orthopedic surgery (e.g., total joint replacement) in patients with advanced arthropathy is increasingly considered to reduce pain, improve function, and reduce joint bleeds. This analysis reviewed the impact of surgery on quality of life and other humanistic outcomes in hemophilia patients with advanced arthropathy.
Materials and Methods: A systematic search of the published literature was conducted to identify studies reporting on the humanistic and patient-related outcomes of major orthopedic surgery in patients with hemophilia and advanced arthropathy. Studies were included if they reported quality of life (QOL), pain, functional status and work and productivity outcomes. Wherever possible, data were extracted and normalized across various scoring systems (WOMAC, WFH, HSS, Cleveland Rating, and Knee Society) for comparisons across studies.
Results: A total of 32 studies were examined. Among those, only one included a formal, validated QOL instrument (WOMAC) with pre/post surgery data. This study documented dramatic improvements in objective and patient perceived QOL measures. Of the other 31 studies, 4 provided sufficient pre/post surgery data to facilitate a quantitative assessment of other key humanistic and patient-related outcomes. In 50 patients pooled across these four studies, all (100%) had moderate or severe pain in the targeted joint prior to surgery. After surgery 86% (43/50) reported no pain at follow up. Improvements were also reported regarding functional status, where 100% of 16 cases pooled across two studies had severe or moderate loss of function prior to joint surgery and 31% (5/16) regained full function upon follow up. Similar, if not better, results were reported in studies that reported only average scores across pain and function parameters. It was found that joint pain was the most prevalent complaint prior to surgery and was the primary indication for surgery. Anecdotal evidence from the 32 studies indicates that improvements in pain and function following surgery were so significant in some patients that they were able to rejoin the workforce and take part in recreational activities, including sports. There were several reports of patients who were unable to walk long distances or were confined to wheelchairs before surgery who became able to walk several blocks unaided following surgery.
Conclusions: Major elective orthopedic surgery in hemophilia patients with advanced arthropathy is one of the most effective medical interventions, with success rates in terms of pain reduction over 80%. However, the broader impact of surgery on QOL outcomes, which appears excellent, remains to be better documented. To that effect, the systematic use of a set of validated instruments to be administered before and after surgery is recommended to help document the outcomes of these surgeries.