Musculoskeletal problems in persons with inhibitors: How do we treat?

Abstract

Summary. Inhibitors are a serious complication, considerably increasing the morbidity, mortality and cost of treatment in this patient group [1]. The challenge of treating people with haemophilia (PWH) with inhibitors can be met by a well‐coordinated multidisciplinary team specialized in haemophilia. Each treatment centre must run a screening programme to detect inhibitors within their population and develop protocols to treat these patients. The treatment centre in Buenos Aires developed a screening programme that tests all our patients twice a year, ensuring early detection of inhibitors and early treatment of complications. In 2006, we analysed the quality of life (QOL) of non‐inhibitor patients and compared it with inhibitor patients detected by this programme and found no differences in QOL measured by the SF36 questionnaire and no differences in school absenteeism [2]. When diagnosis of the inhibitor does not come from a screening programme, its presence is suspected upon a lack of response to conventional replacement therapy for musculoskeletal bleeding, losing the ‘golden moment’ of treatment. This complication is much more serious when facing a traumatic bleed. In this situation, the lack of early diagnosis can lead to permanent damage or even death. Due to the cost of bypassing factors and the lack of experience of the medical team in the treatment of patients with inhibitors, many treatments that would improve the QOL of patients are instituted in an insufficient manner. Therefore, patients with haemophilia and inhibitors are often untreated or undertreated in their community. Orthopaedic surgeons and physiotherapists play a key role in the treatment of these patients and should be included in therapeutic decision making and most specifically in the postoperative treatment of patients with haemophilia and inhibitors. It is important that these patients have quick access to a trained therapeutic team in order to obtain an early diagnosis and treatment plan to prevent the evolution of the pathological process. Early treatment is cost‐effective in maintaining and improving the QOL of patients. Experience in patients with haemophilia and inhibitors is not very extensive. Today, this situation is changing, with several treatment centres beginning to perform surgeries in these most complex patients, giving them a chance to improve their QOL. This article presents the experience of experts from various fields involved in treating patients with inhibitors from a developed and developing world perspective.

Manual therapy in the treatment of patients with hemophilia B and inhibitor

“…For patients with inhibitors, whose frequency of bleeding is higher and who reach adulthood in more disabling musculoskeletal conditions, coordination of the interventions among the entire healthcare team is critical. As a step prior to orthopedic surgery, the combined effort of hematologists and physiotherapists is essential to delay surgery as much as possible, maintaining the patient’s functionality and perception of quality of life [ 19 ].…”

Comprehensive care of the patient with haemophilia and inhibitors undergoing surgery: practical aspects

“…More recently, Caviglia et al . recommended the following for optimal perioperative dosing of rFVIIa and aPCC: for rFVIIa, 120–180 µg kg −1 preoperatively followed by 90 µg kg −1 every 2 h postoperatively, and for aPCC, 100 U kg −1 preoperatively followed by 75–100 U kg −1 postoperatively, to a maximum of 200 U kg −1 .…”

“…Satisfactory intraoperative haemostasis was achieved utilizing the higher initial rFVIIa dosing endorsed by this protocol in 13 procedures performed in five comprehensive haemophilia care centres in the United Kingdom and Ireland [13]. More recently, Caviglia et al [34] recommended the following for optimal perioperative dosing of rFVIIa and aPCC: for rFVIIa, 120-180 µg kg À1 preoperatively followed by 90 µg kg À1 every 2 h postoperatively, and for aPCC, 100 U kg À1 preoperatively followed by 75-100 U kg À1 postoperatively, to a maximum of 200 U kg À1 .…”

Recommendations on multidisciplinary management of elective surgery in people with haemophilia

“…Close collaboration and communication among the MDT members and the patient/family is vital throughout . Surgery in inhibitor patients remains the most challenging area and should only be conducted at an HTC by an experienced, specialized MDT …”

“…1 Surgery in inhibitor patients remains the most challenging area and should only be conducted at an HTC by an experienced, specialized MDT. [5][6][7][8][9][10][11][12][13][14][15] The importance and organization of the MDT has been discussed in depth elsewhere 6,8,15 ; however, a gap can exist between best practice perspectives and practical application. With this in mind, a multidisciplinary panel of specialists was convened to provide practical recommendations on the application of the established principles of multidisciplinary management of elective surgery and the comprehensive care model for PWH.…”