Joint problems in hemophilia A are common despite prophylaxis
Researchers surveyed 120 doctors about their patients' joint health
Almost two-thirds of men with moderate or severe hemophilia A have joint problems that cause pain and reduce their quality of life, even when they’re on factor replacement therapy to reduce the frequency of bleeding episodes, a real-world study shows.
“Overall, the data indicate that joint problems still represent a substantial burden in the care of [hemophilia A],” the researchers wrote. The study, “Assessment of joint health in patients receiving prophylaxis for haemophilia A: a cross-sectional survey in five European countries,” was published in BMJ Open.
Like other types of hemophilia, hemophilia A impairs the blood’s ability to clot properly due to the lack of a clotting protein, causing frequent and excessive bleeding. When bleeding occurs into the joints, they can swell and become damaged and less mobile.
Real-world data on joint health in people with hemophilia A are limited, however, leading researchers to launch a multinational survey that gathered answers from 120 doctors on 351 patients across France, Germany, Italy, Spain, and the U.K. between February 2020 and May 2021.
Prevalence of joint problems
All the patients were men, ages 18 or older, who were receiving preventive therapy, or prophylaxis, to reduce the frequency of bleeding episodes caused by moderate or severe hemophilia A. None had developed inhibitors, that is, neutralizing antibodies that target the replaced clotting protein and can make therapy less effective.
Of the 351 patients, 209 (59.5%) had joints affected by hemophilia A, while 142 (40.5%) didn’t. Patients with joint problems were a mean 5.7 years older than those without joint problems (34.7 vs. 29 years). It was also more common for those with joint problems to have a history of inhibitors (19.6% vs. 9.2%).
Pain or discomfort was reported more frequently by those with joint problems than by those without them (85.7% vs. 53.3%). Patients with joint problems were also more likely to be prescribed pain medication, such as paracetamol, nonsteroidal anti-inflammatory drugs, or opioids (73.2% vs. 60.6%).
These patients had lower scores on a health status measure called EuroQol Visual Analogue Scale (65.5 vs. 81.1 points), which records a patient’s self-rated health from best to worst, lower scores indicating worse overall health. They also had more difficulty performing everyday activities (74.3% vs. 26.7%).
Up to half the patients with joints affected by hemophilia A had a history of synovitis, an inflammation of the membrane that lines the joints (49.8%) or hemophilic arthropathy, a complication marked by pain and limited joint movement (48.4%). More than a third (35.4%) had joint surgery and nearly as many (24.9%) used at least one walking aid.
The data, which reflect clinical practice in some countries in Europe, indicate men with moderate or severe hemophilia A without inhibitors have joint problems despite prophylaxis that “represent a clinically relevant burden,” wrote the researchers, who noted about joint problems mainly described as mild or moderate, “early identification/monitoring and management of affected joints should be an important consideration to help prevent long-term joint morbidity [disease].”
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