Optimising musculoskeletal care for patients with haemophilia

Carção, Manuel; Hilliard, Pamela; Escobar, Miguel A.; Solimeno, Luigi Piero; Mahlangu, Johnny; Santagostino, Elena

doi:10.1111/ejh.12581

Cited by 11 publications

(12 citation statements)

References 57 publications

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“…Frequent, recurrent and uncontrolled joint bleeding resulting from chronic synovitis leads to persistent pain and joint deformity that may be addressed by radiosynovectomy and orthopaedic surgery, respectively . Delayed referral to an orthopaedic surgeon may result in chronic synovitis and joint damage, requiring more aggressive surgical intervention . Regular monitoring at the HTC of joint status of patients who experience frequent joint bleeds and/or pain can help to identify candidates for orthopaedic surgery and prevent delayed referral.…”

Section: Methodsmentioning

confidence: 99%

“…1,16 Delayed referral to an orthopaedic surgeon may result in chronic synovitis and joint damage, requiring more aggressive surgical intervention. 16 Regular monitoring at the HTC of joint status of patients who experience frequent joint bleeds and/or pain can help to identify candidates for orthopaedic surgery and prevent delayed referral. Although not the focus of this article, fractures, septic joints and compartment syndromes must be treated as an emergency.…”

Section: Principles Of Elective Surgical Planning For the Mdtmentioning

confidence: 99%

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Recommendations on multidisciplinary management of elective surgery in people with haemophilia

et al. 2018

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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.

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Section: Methodsmentioning

confidence: 99%

Section: Principles Of Elective Surgical Planning For the Mdtmentioning

confidence: 99%

Recommendations on multidisciplinary management of elective surgery in people with haemophilia

et al. 2018

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“…Patients with hemophilia who deteriorate to the extent of requiring TKA pose particular challenges to the arthroplasty surgeon, anesthetist and other clinical teams. Such patients should be managed by a multidisciplinary team in a center with experience of managing the perioperative care of patients with hemophilia 45 . Generally, any major surgery in patients without inhibitors is performed under hemostatic cover with bolus or continuous infusion of the relevant factor concentrate to prevent major perioperative bleeding 45 .…”

Section: Total Knee Arthroplasty In Hemophiliamentioning

confidence: 99%

“…Such patients should be managed by a multidisciplinary team in a center with experience of managing the perioperative care of patients with hemophilia 45 . Generally, any major surgery in patients without inhibitors is performed under hemostatic cover with bolus or continuous infusion of the relevant factor concentrate to prevent major perioperative bleeding 45 . Patients with inhibitors are more likely to bleed and have a higher rate of potential complications such as infection; until relatively recently, major orthopedic procedures were only rarely performed in this group due to the potential for major bleeding but new products and protocols have rendered such procedures safer in such patients 46 .…”

Section: Total Knee Arthroplasty In Hemophiliamentioning

confidence: 99%

Evidence-Based Management of the Knee in Hemophilia

Liddle

Rodríguez-Merchán

2017

JBJS Rev

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➢ Hemophilia is among the commonest bleeding disorders encountered in orthopaedic practice and results from an X-chromosome-linked failure of coagulation factors VIII (hemophilia A) or IX (hemophilia B).➢ Morbidity in hemophilia is a result of repeated hemarthroses, and the aim of orthopaedic management is to prevent these, to treat them promptly, and to address the joint pathology that results.➢ Both radiosynovectomy and arthroscopic synovectomy are effective in reducing the degree of synovitis and the number of hemarthroses, although, to our knowledge, there is little evidence that it prevents progression to end-stage arthritis. There is substantial evidence for the safety and effectiveness of radiosynovectomy, and this is the first line of treatment in our practice.➢ In end-stage disease, total knee arthroplasty is challenging as a result of severe contractures and is associated with a higher rate of complications such as infection (7% compared with 1% to 2% in osteoarthritis) and need for blood transfusions and a higher revision rate than that seen in total knee arthroplasty for osteoarthritis.➢ However, many series of total knee arthroplasty for hemophilia have now been published, and excellent results can be gained in terms of pain relief and quality of life.

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“…Physiotherapy and rehabilitation in haemophilic patients are important to return to the normal status of joint motion, to regain the muscle strength, to obtain the optimal functional levels and to improve the patients’ quality of life. 6,7 Primary prophylaxis is the gold standard for treating the haemophilia patients and rehabilitation should always be performed under the cover of prophylactic treatment.…”

Section: Introductionmentioning

confidence: 99%

Musculoskeletal treatment in haemophilia

Atilla

Guney

2019

EFORT Open Reviews

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Haemophilia is a group of coagulation disorders inherited in an X-linked recessive pattern. Nearly three-quarters of all haemorrhages in haemophilia occur in the musculoskeletal system, usually in the large muscles and joints of the lower extremity. While prevention of bleeding with active prophylaxis is the recommended optimal therapy for severe haemophilia, there are many patients suffering from musculoskeletal system complications subsequent to uncontrolled bleeding. Recombinant clotting factor concentrates led to home treatment of acute bleeding episodes as well as allowing for minor and major surgical interventions. Avoiding of further complications by radiosynoviorthesis is the first-line recommendation, and arthroplasty is regarded as the effective salvage procedure for patients presenting with severe disability. Physiotherapy and rehabilitation in haemophilia patients are important to return the normal status of joint motion, to regain the muscle strength, to obtain the optimal functional levels and to improve patients’ quality of life. Cite this article: EFORT Open Rev 2019;4 DOI: 10.1302/2058-5241.4.180068

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Optimising musculoskeletal care for patients with haemophilia

Cited by 11 publications

References 57 publications

Recommendations on multidisciplinary management of elective surgery in people with haemophilia

Recommendations on multidisciplinary management of elective surgery in people with haemophilia

Evidence-Based Management of the Knee in Hemophilia

Musculoskeletal treatment in haemophilia

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