Planning and undertaking elective surgery in people with haemophilia (PWH) is most effective with the involvement of a specialist and experienced multidisciplinary team (MDT) at a haemophilia ...treatment centre. However, despite extensive best practice guidelines for surgery in PWH, there may exist a gap between guidelines and practical application. For this consensus review, an expert multidisciplinary panel comprising surgeons, haematologists, nurses, physiotherapists and a dental expert was assembled to develop practical approaches to implement the principles of multidisciplinary management of elective surgery for PWH. Careful preoperative planning is paramount for successful elective surgery, including dental examinations, physical assessment and prehabilitation, laboratory testing and the development of haemostasis and pain management plans. A coordinator may be appointed from the MDT to ensure that critical tasks are performed and milestones met to enable surgery to proceed. At all stages, the patient and their parent/caregiver, where appropriate, should be consulted to ensure that their expectations and functional goals are realistic and can be achieved. The planning phase should ensure that surgery proceeds without incident, but the surgical team should be ready to handle unanticipated events. Similarly, the broader MDT must be made aware of events in surgery that may require postoperative plans to be changed. Postoperative rehabilitation should begin soon after surgery, with attention paid to management of haemostasis and pain. Surgery in patients with inhibitors requires even more careful preparation and should only be undertaken by an MDT experienced in this area, at a specialized haemophilia treatment centre with a comprehensive care model.
Ageing successfully with haemophilia: A multidisciplinary programme Boccalandro, E.; Mancuso, M. E.; Riva, S. ...
Haemophilia : the official journal of the World Federation of Hemophilia,
January 2018, 2018-Jan, 2018-01-00, 20180101, Letnik:
24, Številka:
1
Journal Article
Recenzirano
Introduction
Persons with haemophilia (PWH) born before the middle 1970s have spent a substantial part of their lives without the benefits of replacement therapy, that became available on a relative ...large scale only during the 1970s. As a consequence, this group of PWH, although still relatively young, suffers from musculoskeletal and functional problems that are typical of old people.
Methods
We report herewith the short‐term results of a project based upon a multidisciplinary training programme led by a physiotherapist and an occupational therapist, that was implemented over a period of 12 months in 40 patients with severe or moderate hemophilia A or B born before the middle 1970s and regularly followed‐up at a comprehensive haemophilia treatment centre in Italy. The project was aimed to provide information and skills in order to empower the older PWH carrying physical handicaps and functional limitations that had resulted from the inadequate management in their early ages, and to enable them to cope more efficiently with their crippling disease and prevent further disabilities.
Results and Conclusions
The comparison of the data obtained before and after the 12‐month programme found marginal improvements, but the purpose of this programme was indeed to offer a blueprint for the future. In this respect, the level of satisfaction for the programme was very high and we expect that it will be implemented long‐term by our older PWH.
Summary
Recombinant factor VIII (rFVIII) products provide a safe and efficacious replacement therapy for prevention and treatment of bleeding episodes in patients with haemophilia A. The present ...investigations from the multinational, open‐label guardian™ clinical trials assessed the haemostatic response of turoctocog alfa (NovoEight®), a rFVIII product, in patients with severe haemophilia A (FVIII ≤ 1%) undergoing surgery. All patients had a minimum of 50 exposure days to any FVIII product prior to surgery and no history of inhibitors. A total of 41 procedures (13 orthopaedic, 19 dental and 9 general) were performed in 33 patients aged 4–59 years. Of the 41 procedures, 15 were major surgeries in 13 patients and 26 were minor surgeries in 21 patients. The success rate for haemostatic response was 100% (success was defined as ‘excellent’ or ‘good’ haemostatic outcome). Turoctocog alfa consumption on the day of surgery ranged from 27 to 153 IU kg−1. The mean daily dose declined over time, while retaining adequate FVIII coverage as measured by trough levels. Overall, no safety issues were identified. No thrombotic events were observed and none of the patients developed FVIII inhibitors. In conclusion, the present results show that turoctocog alfa was effective in controlling blood loss by obtaining a sufficient haemostatic response in patients with severe haemophilia A undergoing surgery.
Background
While there is substantial literature addressing the principles of general management of haemophilia, literature on perioperative management of haemostasis is scarce.
Objective
The aim of ...this study was to better understand perioperative management among congenital haemophilia B patients (without inhibitors) and to gain insights into real‐world surgical practices.
Method
A systematic literature review, with an emphasis on haemophilia B, was conducted using EMBASE®, Medline® and the Cochrane Library. Studies from 1974 to June 2015 were accessed, and 132 studies were eligible for the full‐study review. An international expert panel with five haematologists and one surgeon reviewed the resulting literature and provided further insights.
Results
The literature review revealed that documented experience in the perioperative management of bleeding risk in haemophilia B patients is relatively scarce. Therefore, the review was amended to provide a comprehensive overview of the perioperative management for haemophilia A and B patients; the expert panel applied a particular focus to haemophilia B. Several gaps were identified in the literature including the lack of consensus on defining surgery in terms of bleeding risk, optimal factor levels during surgery and lack of robust evidence on surgical outcomes. The ensuing discussions with the expert panel provided validation of some of the results from the systematic literature review and proposed future directions for perioperative management. Suggestions included collaboration with haemophilia treatment centres (HTCs) to collect real‐world data on perioperative management, establishing the need for optimal factor level monitoring practice, and the appropriate adoption of extended half‐life products in clinical settings.
Hemophilic arthropathy is one of the conditions most associated with arthrofibrosis and loss of range of motion. Progressive fibrosis of synovium leads to pain, spasm, and shortening of muscles, ...resulting in joint contractures and restriction of joint motion. It is common to see even young children with severe loss of motion of elbows, knees and ankles. Treatment should be primarily by physiotherapy, splintage, and corrective devices. The late or severe cases may require surgical correction in the form of soft‐tissue procedures, osteotomy and especially joint replacement
Summary
Patients with von Willebrand disease (VWD) may need orthopaedic surgery because of disabling chronic arthropathy due to recurrent joint bleeding. They may also require this surgery ...independently of their haemostasis disorder. Knowledge regarding the management of orthopaedic surgery in VWD is limited. Description of management of orthopaedic surgery in patients with VWD, based upon retrospective data collection and analysis of 32 orthopaedic procedures carried out over a period of 33 years in 23 patients was the aim of this study. Of 32 procedures, six were minor (three hand surgery, one foot surgery, two others) and 26 were major (seven joint replacements, nine arthroscopic procedures, two foot surgery, eight others). Twenty‐two procedures were performed using replacement therapy with plasma‐derived concentrates containing both factor VIII (FVIII) and von Willebrand factor (VWF). Two procedures in patients with acquired von Willebrand syndrome (AWVS) were performed using FVIII‐VWF concentrates associated with intravenous immunoglobulins, or desmopressin plus tranexamic acid. Seven procedures were performed using desmopressin alone and one using intravenous immunoglobulins in AVWS. Bleeding complications occurred in seven procedures (22%). In one patient, an anti‐VWF antibody was diagnosed after surgery. Anticoagulant prophylaxis of venous thromboembolism was implemented in four cases only and in two instances there was excessive bleeding. In conclusion, control of surgical haemostasis was achieved in most patients with VWD undergoing orthopaedic surgery. The control of haemostasis combined with an adequate surgical technique and early post‐operative rehabilitation are warranted for the successful performance of orthopaedic surgery in VWD, which requires the involvement of specialized haemophilia centres.
Summary
Knowledge regarding the management of orthopaedic surgery in patients with rare bleeding disorders (RBDs) is limited. Retrospective data collection and analysis of 35 orthopaedic procedures ...(6 minor and 29 major) carried out in 22 patients with RBD between 1982 and 2013. These surgeries were performed using heterogeneous regimens of hemostatic therapy, except for seven procedures performed with no hemostatic treatment in four patients with mild factor deficiency. Of the 28 procedures carried out with hemostatic treatment, nine (32%) were performed using replacement therapy with dosages of concentrates of the deficient factor aimed to achieve perioperative plasma levels judged to be compatible with hemostasis; three (11%) using factor replacement therapy associated with fresh frozen plasma (FFP); four (14%) using recombinant activated factor VII; four (14%) using virus inactivated plasma alone; three (11%) using virus inactivated plasma associated with desmopressin; one (4%) using FFP alone; and four (14%) procedures using tranexamic acid alone. Bleeding complications occurred in 7 of 35 procedures (20%) involving five patients. Prophylaxis of venous thromboembolism was performed only in one case with no excessive bleeding, but two patients not on thromboprophylaxis developed superficial thrombophlebitis. A satisfactory control of hemostasis was achieved in most patients. In some of those characterized by mild factor deficiency (FVII, FXI) hemostatic treatment could be avoided in some instances. The control of hemostasis combined with an adequate surgical technique is needed for the successful outcome of orthopaedic surgery in RBDs that requires the involvement of specialized haemophilia centres.
Integrated postural analysis in children with haemophilia Boccalandro, E.; Pasta, G.; Mannucci, P. M. ...
Haemophilia : the official journal of the World Federation of Hemophilia,
March 2014, Letnik:
20, Številka:
2
Journal Article
Recenzirano
Summary
The maintenance of a correct posture in haemophilic boys might contribute to prevent joint bleeds, chronic pain and dysfunction. This single‐centre study was aimed at evaluating whether or ...not postural alterations are more common in haemophilic than in non‐haemophilic boys and whether they are related to the orthopaedic status. Posture and balance were investigated in boys with severe/moderate haemophilia (cases) and in age‐matched non‐haemophilic peers (controls). Thirty‐five cases (89% with haemophilia A: 74% with severe disease) were included in the study and compared with 57 controls. Posture was evaluated on digital pictures of anterior, lateral and posterior views of the habitual standing position. Balance was examined with a portable force platform with eyes open and closed. The trajectory of the total body centre of force (CoF) displacement over the platform was computed by multiple planes obtaining different measures: sway area, velocity, acceleration and body loads. The joint status of cases was assessed with the Haemophilia Joint Health Score. Cases were more disharmonic than controls (52% vs. 26% in controls; P = 0.04), swayed significantly less and more slowly than controls (P < 0.05 for several parameters of CoF displacement) revealing stiffness of the musculoskeletal system. However, they were able to maintain their stance within a similar sway area. Haemophilic boys have more postural disharmonies than non‐haemophilic peers, hence a global evaluation of the orthopaedic status should include also balance and posture examination to identify early dysfunction and establish a tailored physical or rehabilitation programme.
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