Elective Surgery Safe in Australia for Hemophilia, Bleeding Disorders

Complication rates for elective surgeries in people with inherited bleeding disorders like hemophilia are low in Australia, reflecting good safety in their performance despite guidelines not always being followed, a study reported.

Use of a specialty hemophilia treatment center (HTC) for surgery, and use of factor replacement therapy were among guidelines sometimes ignored or altered, its investigators found, highlighting a need to review current policies and practices.

The study, “Elective surgery in patients with inherited bleeding disorders: A retrospective analysis,” was published in the journal Haemophilia.

Inherited bleeding disorders, which including hemophilia A, hemophilia B, von Willebrand disease and rare bleeding disorders (RBDs), are thought to affect up to 1% of the general population. Because each is marked by blood failing to clot properly, they can be particularly problematic for surgery.

Treatment to prevent bleeding is typically given during and after a surgical procedure to avoid complications.

According to published guidelines, surgery should be performed in a HTC that has specialized healthcare teams and laboratory staff for needed tests. Guidelines also indicate the appropriate clotting factor replacement doses that should be used at the time of surgery.

How well these guidelines, meant to help in managing these patients during surgery, are put into practice is relatively unknown.

Researchers assessed outcomes of adults with inherited bleeding disorders given approved clotting factor replacement for elective surgery done between 2000 and 2018. They also evaluated if HTCs were being used, and if patients received appropriate treatment.

Data drew on requests, made between 2000 and 2018, for clotting factor replacement for people with hemophilia and other bleeding disorders undergoing elective surgery. These data came from a registered database, called Supply Tracking Analysis Reporting System (STARS), managed by Australian Red Cross Lifeblood that distributes clotting factors in that country.

This database holds information on patients’ age, diagnosis, weight, baseline factor levels, hospital where the surgery was performed, type of surgical procedure, bleeding complications, and the total factor replacement supplied.

For surgeries with a minor bleeding risk, up to five days of factor replacement is recommended. When the procedure is associated with a major bleeding risk, factor replacement for the first seven days after the surgery is recommended, followed by an additional seven days as clinically indicated.

A total of 1,065 elective surgeries were conducted on 571 patients, with 211 people undergoing more than one procedure. Most patients had von Willebrand disease (45.2%), followed by hemophilia A (43.5%), hemophilia B (9.7%), and rare disorders (1.6%).

The most common surgeries included knee replacements, unspecified dental extractions, wisdom tooth extraction, hip replacements, and births (excluding cesarean sections).

Bleeding complications occurred in 14 (1.3%) surgeries and 19 patients (1.8%) received clotting factors for longer than the standard duration after a surgery. According to researchers, this data represented a potential bleeding complication rate of 3.10%.

Around 50% of surgeries were conducted in a HTC. Among the procedures carried out elsewhere, 57% were performed in public hospitals and 43% in private hospitals.

Statistical analysis revealed that patients with severe hemophilia A and B, as well as those with von Willebrand disease type 2M and type 3 VWD were more likely to have their elective surgery in a HTC, compared with patients with milder von Willebrand disease forms (types 1, 2A, 2N, and unspecified).

Patients were also more likely to have surgery in a HTC if it was dental (70%) or orthopedic (66%). Conversely, surgeries involving nuclear medicine (25%), cardiology (30%), and urology (30%) were less likely to be carried out in a hemophilia specialty center.

Reported bleeding complications were not related to the use of a non-HTC. However, 65% of additional coagulation factor requests were made for patients who underwent surgery in a non-HTC setting.

Women were 2.2 times more likely than men to have elective surgery in a non-HTC facility, analysis found. With each subsequent year, procedures were also 1.2 times more likely to be performed in a non-HTC.

About 48% of hemophilia A and B patients received a cumulative factor dose within the recommended guidelines. Among patients whose doses were out of this range, 19% received lower and 11% higher doses than recommended.

The median number of doses given von Willebrand patients (a mix of factor VIII and VWF complex called Biostate) in a surgery with major bleeding risk was much lower than that recommended. These patients with type 3 disease, a more severe form, received doses more in line with recommendations. Most — 90% — of people with type 1 received doses below recommended guidelines, compared with 40% of type 3 patients.

Among rare bleeding disorder patients with FXI deficiency, 46% received the recommended dose, 36% a lower dose and 18% doses higher than those recommended.

“This study is the first to examine a large population of patients with a range of inherited bleeding disorder diagnoses undergoing all elective surgical procedures,” the investigators wrote.

“Low complication rates demonstrate that elective surgery in Australia is being safely performed in patients with inherited bleeding disorders; however, noncompliance with published guidelines exists highlighting areas of practice and policy discrepancies that warrant further exploration,” they concluded.