Chronic ankle pain in hemophilia significantly affects quality of life

UK study urges pain management for patients to ensure better outcomes

Lindsey Shapiro, PhD avatar

by Lindsey Shapiro, PhD |

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People with moderate-to-severe hemophilia and ankle joint disease reported significant impairments in life quality and foot and ankle function, according to a recent U.K. study.

Chronic pain emerged as a significant predictor of these patient-reported outcomes, as did the presence of inhibitors — neutralizing antibodies that can lower the efficacy of treatments — in the blood.

Ankle pain should be monitored in the clinic, according to researchers, who noted that “further research is needed to provide robust evidence in clinical practice for the use of … interventions that may improve pain and prevent the decline in ankle joint health across haemophilia.”

The study, “Decline in health-related quality of life and foot and ankle patient reported outcomes measures in patients with haemophilia and ankle haemarthropathy,” was published in the Journal of Foot and Ankle Research.

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Uncontrolled internal bleeding in hemophilia can cause permanent joint damage

In more severe cases of hemophilia, uncontrolled internal bleeding can cause permanent joint damage, a condition known as hemophilic arthropathy. Symptoms can include swelling, tightness, and joint pain.

The most affected joints are usually the ankles, knees, and elbows, but the ankles are particularly problematic, according to researchers. In the long term, changes in the ankle joint structure can affect muscle control and, in turn, the daily lives of patients.

Arthropathy — and the pain and disability that come with it — can contribute to a significant decline in  quality of life.

Here, researchers investigated the specific impact of ankle arthropathy on the quality of life of adults with moderate-to-severe hemophilia A or B living in the U.K.

A total of 243 participants were enrolled across 18 U.K. sites from April 2017 to August 2019. All participants had a confirmed diagnosis of ankle arthropathy, and most had severe hemophilia A (75.7%).

Participants completed a questionnaire relating to quality of life, other foot and ankle outcome measures, pain, and demographics. A healthcare professional collected details about disease characteristics, treatments, and ankle and joint health.

Health-related quality of life, as assessed by the HAEMO-QoL-A questionnaire, was generally poor across disease severities. On a scale of 0 to 100, with 100 being the best possible health, patients with severe hemophilia scored an average of 35.3, and those with moderate disease scored a 35.8.

Likewise, scores on the Manchester-Oxford Foot Questionnaire (MOXFQ) — foot and ankle version — reflected poor function and pain in the ankles, as well as negative effects on social interactions.

On a scale of 0 (no pain) to 10 (pain as bad as you can imagine), severe hemophilia patients reported a score of 5.0 over the last six months, and those with moderate disease had a score of 5.5.

Among those who gave information about pain medication, 56.2% said they did not use it regularly.

Clinician-rated hemophilia joint health scores for the ankle indicated moderate to severe levels of arthropathy in both groups, according to the researchers.

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Pain appears to be significant driver of life quality declines

While the study included a small number of patients, and its results should be “interpreted with caution,” findings nonetheless indicate that the impacts of ankle arthropathy on life quality are significant and “equivalent across disease types and severity.”

Pain appeared to be a significant driver of life quality and MOXFQ declines, emerging as an independent predictor of these outcomes in a final statistical analysis.

Pain management is critical for patients living with hemophilia to ensure better outcomes, the team noted, especially in light of the fact that more than half of the participants said they weren’t using pain medications.

The presence of inhibitors also predicted patient-reported outcomes. Inhibitors have been previously associated with increased arthropathy, hospitalizations, and poor life quality.

There is thus a need to “closely monitor inhibitors to prevent or delay the development of ankle [hemophilic arthropathy],” the researchers wrote.