Experts Define Recommendations for Best Surgery Management Practices in Hemophilia Patients

A panel of multidisciplinary experts have developed recommendations for applying best practice guidelines in the planning and managing of surgery in hemophilia patients.

The guidelines were outlined in the consensus review, “Recommendations on multidisciplinary management of elective surgery in people with haemophilia,” published in the journal Haemophilia.

Hemophilia is a bleeding disorder estimated to affect 20,000 people in the U.S. The recommended treatment for patients with severe hemophilia is the replacement of the deficient clotting factor for that type of hemophilia.

These patients are at a greater risk of bleeding-related complications, particularly when undergoing surgery. About 80 percent of bleeding episodes in hemophilia are in the joints — mainly in the knees, elbows, and ankles. As a result, joint replacement surgery is a common procedure for these patients.

It is highly recommended that any surgery — from planning to the procedure itself to the postoperative period — should be managed by a specialized and experienced multidisciplinary team in a hemophilia treatment center.

In these centers, patients have access to high-level laboratory facilities and specialist medical support. The multidisciplinary teams are usually made up of a hematologist, nurse, orthopedic surgeon who specializes in disorders of the bones and joints, anesthetist, physiotherapist, laboratory specialist, dentist, pharmacist, and social worker and/or psychologist.

With careful planning, most surgical procedures can be safely carried out in hemophilia patients, even those who have developed antibodies against clotting factor therapy.

However, despite the existence of well-established medical guidelines in these cases, their practical application may fall short.

To address this, a multidisciplinary panel of experts composed of three hematologists, three orthopedic surgeons, three physiotherapists, two hemophilia nurses, and a dentist developed recommendations for the practical application of already-established principles of multidisciplinary surgical care in hemophilia.

Recommendations were defined for each period of surgical management: preoperative planning, during surgery, postoperative recovery, and physical medicine and rehabilitation — in the case of orthopedic surgery.

The specialists pointed out that clear communication between all members of the multidisciplinary team, as well as between the team and the patient, are essential. They recommended the appointment of a medical team coordinator, not only to facilitate these communications but, most importantly, to ensure all critical steps are efficiently performed.

Careful planning of the surgery, including a dental examination, due to the increased risk of complications related to the teeth; a physical assessment and prehabilitation, which is physical and/or lifestyle preparation designed to improve a patient’s recovery time following surgery; laboratory testing; and the development of a therapeutic plan to stop potential bleeding and to manage pain, was considered crucial for the procedure’s success.

Rehabilitation should begin shortly after surgery, paying special attention to the management of pain and bleeding.

The authors noted that the skill and experience of a multidisciplinary team are critical for performing safe, effective surgery in hemophilia patients. Institutions that do not have these specialized and experienced teams should “seek practical advice on how to maximize their available facilities and appropriate decision‐making in PWH [hemophilia],” they wrote.

In addition, they strongly recommend that any surgery in hemophilia patients with antibodies against clotting factor replacement treatment should only take place at a specialized hemophilia treatment center and be performed by an experienced multidisciplinary team.