Close observation and frequent joint examinations are key to avoiding the development of joint disease among pediatric patients with hemophilia, according to a study published in Haemophilia.
In hemophilia, joint health is a key indicator of quality of life. Because recurrent bleeding often results in the deterioration of joint health, clinicians are working to identify optimal methods for early detection and treatment. The authors of the study sought to assess the age-specific incidence of acute joint disease in a single hemophilia treatment center based on consecutive ultrasound follow-ups for 5 years.
This prospective study recruited 48 patients with hemophilia (36 with hemophilia A, 12 with hemophilia B) who were 20 years of age or younger. They must have been seen at the pediatric hemophilia treatment center at the Hiroshima University Hospital in Japan. The research team had access to detailed data about their disease, including treatment and any prophylaxis initiated, history of joint bleeding, and levels of physical activity. These 48 patients underwent consecutive ultrasound scans annually from 2015 to 2020.
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Six joints were evaluated in the study: the left and right elbow, left and right knee, and left and right ankle. Ultrasound scans of these joints were evaluated by two radiologists and one orthopedist. X-rays were also performed every year. Musculoskeletal assessments were conducted by trained physiotherapists using the Hemophilia Joint Health Score, version 2.1.
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Forty-two patients received standard half-life prophylaxis and 2 received extended half-life prophylaxis after it was introduced in 2016. Twenty of the 42 patients switched from standard to extended half-life products during the study. In addition, 3 switched from factor prophylaxis to emicizumab, whereas 2 received a combination of both.
Ultrasound scans revealed that most patients under the age of 10 had no joint disease. However, the incidence and severity of joint disease increased with age after 10 years. Joint abnormalities discovered via ultrasound corresponded 100% with X-ray findings. The most affected joint was the ankle. Four patients had a cumulative Hemophilia Joint Health Score score of 4 or more, and 36 had a cumulative score of less than 3.
The researchers found that the routine joint evaluation and frequent assessment of pharmacokinetic profiles performed at their hemophilia treatment center resulted in better avoidance of joint disease compared with other institutions (P <.001).
“Our findings revealed the efficacy of consecutive joint examinations using ultrasound in detecting early alterations in joint status in individual cases,” the authors wrote. “Further studies are required to achieve zero joint bleeding among patients with hemophilia.”
Reference
Mizoguchi Y, Tani C, Aizawa M, et al. Age-specific incidence of joint disease in paediatric patients with haemophilia: a single-centre real-world outcome based on consecutive US examination. Haemophilia. Published online August 28, 2023. doi:10.1111/hae.14848
