‘Problem joints’ linked to worse clinical outcomes in hemophilia A
Chronic pain seen in patients with problems in 2 or more joints: Study
People with severe hemophilia A who have two or more self-described problem joints — a person-centric characterization of joints based on underlying joint damage, pain, and mobility impairment, regardless of bleeding — have worse clinical outcomes, including more chronic pain, a new study from researchers in Europe has found.
The study also showed that bleed-related hospitalizations and bleeding episodes were more common among hemophilia A patients with such problem joints.
According to the researchers, the data show that the “presence and number of problem joints was associated with clinical burden … in people with moderate or severe [hemophilia A].”
Noting that “diagnosing and managing joint morbidity [disease symptoms] is critical,” the team wrote that the “use of person-centric characterizations of joint morbidity may improve analysis of long-term outcomes and lead to improvements in future [hemophilia] care.”
The study, “An assessment of burden associated with problem joints in children and adults with moderate or severe haemophilia A: analysis of the CHESS-Paediatrics and CHESS II cross-sectional studies,” was published in the Orphanet Journal of Rare Diseases.
Clinicians’ understanding of problem joints is ‘lacking’
Hemophilia A, the most common form of hemophilia, occurs when blood fails to clot properly due to the lack or malfunction of a clotting protein called factor VIII, or FVIII. As a result of poor clotting, people with hemophilia experience heavy and prolonged bleeding episodes.
A hallmark sign of hemophilia, especially in severe cases, is bleeding into the joints. Joint bleeds and subsequent inflammation can cause damage and lead to joint disease.
Researchers and clinicians often assess joint morbidity based on the presence of persistent bleeding, deeming junctures to be so-called target joints if they have three or more spontaneous bleeds in a six-month period.
However, joint issues occur for many hemophilia patients in the absence of persistent bleeding, leading researchers to argue that important aspects of joint health are being overlooked with the overemphasis on bleeding.
“As underlying joint damage, pain, and mobility impairment can all be present without clinical recording of persistent bleeds, these target joint definitions are likely to lead to underdiagnosis of joints otherwise worthy of clinical attention,” the researchers wrote.
In light of this, the concept of a problem joint has emerged, with patient communities defining such problem joints as junctures with chronic pain or mobility impairments, with or without persistent bleeding.
But according to the researchers, clinicians’ “understanding of [hemophilic] joint morbidity through the lens of the person-centred ‘problem joint’ definition is lacking” — which led to this study.
“Given the importance of diagnosing and managing joint morbidity through the lifespan, we conducted an analysis on a subset of adult and [pediatric] patients with the aim of [analyzing] the holistic burden of problem joints,” the team wrote.
The analysis used data from 703 children and adolescents and 468 adults with moderate or severe hemophilia A without inhibitors, or neutralizing antibodies against FVIII.
Worse clinical outcomes included more hospitalizations
Having one or more problem joints was more common among adults than for children and adolescents (44% vs. 14%).
Overall, 39% of adults with moderate hemophilia A and 47% of adults with severe hemophilia A had one or more problem joints. In contrast, 11% of pediatric patients with moderate hemophilia A and 16% of children and adolescents with severe hemophilia had one or more problem joints.
Chronic pain was common in people with severe hemophilia who had two or more problem joints.
Nearly all — 93% of the total — pediatric patients with severe hemophilia and two or more problem joints reported moderate or severe chronic pain. More than half (69%) of adults with severe hemophilia with two or more problem joints experienced moderate or severe chronic pain.
These findings may underline the importance of detecting problem joints in order to offer treatment and lifestyle strategies that can help to improve the effectiveness [of] treatment, adherence, functionality, and pain management … [in] people with [hemophilia A].
In addition to pain, hemophilia patients with problem joints had worse clinical outcomes and were less physically active, the study found.
Also, adult hemophilia patients with problem joints were more likely to experience bleeding episodes.
Regardless of underlying disease severity, pediatric hemophilia patients were more likely to require hospitalization if they had problem joints. The researchers also found that bleed-related hospitalization in the previous year, as well as lifetime bleed-related hospitalization, were increased in patients with problem joints. The same tendency was seen among adults.
“These findings may underline the importance of detecting problem joints in order to offer treatment and lifestyle strategies that can help to improve the effectiveness [of] treatment, adherence, functionality, and pain management, with the aim of reducing the impact on the lives and well-being of people with [hemophilia A] and their families,” the researchers wrote.
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