Frailty hits nearly 1 in 4 older hemophilia patients, UK study finds

Nearly a quarter of hemophilia patients aged 50 and older in a prospective screening program at a large UK hemophilia center met criteria for frailty, a study found.

All patients with frailty had other health conditions, and nearly 80% were taking five or more regular medications. Hemophilic arthropathy, or joint damage caused by repeated bleeding, appeared to contribute substantially to impaired mobility and frailty in some patients, particularly those with more pronounced frailty, the researchers said.

Falls were also reported in about one-quarter of those considered frail.

The findings highlight “the high prevalence of frailty” in people with hemophilia aged 50 and older and the need for hemophilia care teams to identify which patients are frail and establish pathways for further assessment and intervention, the researchers wrote.

The study, “Frailty assessment in people with haemophilia,” was published in the Journal of Thrombosis and Haemostasis.

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Hemophilia is caused by deficiencies in clotting proteins needed to control bleeding. Hemophilia A results from missing or defective factor VIII (FVIII), while hemophilia B is caused by reduced or dysfunctional factor IX (FIX).

Advances in hemophilia treatment have substantially improved survival, meaning more people with hemophilia are now living longer. As a result, age-related health conditions are becoming increasingly relevant in hemophilia care.

Frailty refers to a reduced ability of the body to cope with physical or medical stresses and reflects declines in overall physiological reserve. Although it becomes more common with age, frailty can also occur earlier in people living with chronic diseases. It is associated with poorer outcomes, including a greater likelihood of hospitalization and death.

Recognized features of frailty include falls, impaired mobility, cognitive problems, incontinence, and polypharmacy, or use of multiple medications. Identifying frailty can enable clinicians to implement interventions such as medication reviews, exercise programs, physiotherapy, and more comprehensive geriatric assessments.

Researchers at the Oxford Haemophilia and Thrombosis Centre set out to determine the prevalence of frailty among people with hemophilia after incorporating routine frailty screening into clinic appointments. They also examined health problems that might contribute to frailty and the actions clinicians took when they were identified.

Their analysis included people with hemophilia A or B who were registered at their center and were at least 50 years old. Of 116 eligible patients, 111 (all men, median age 62; 80% with hemophilia A) had a clinical frailty scale (CFS) score recorded and were included in the analysis.

The CFS ranges from 1, indicating a very fit person, to 9, indicating severe dependence and terminal illness. The researchers used a score of 4 or higher to define frailty; a score of 4 reflects symptoms that limit daily activities without requiring help from others.

Overall, 27 patients (24%) met the frailty threshold. Among the 66 patients aged 50-64, 12 (18%) were frail. Eleven of these had severe hemophilia, and one had moderate disease.

Among the 45 patients aged 65 and older, 15 (33%) were frail, with cases spanning mild, moderate, and severe hemophilia.

The researchers noted that the 33% rate among patients aged 65 and older was substantially higher than the roughly 10% prevalence of frailty previously reported in the general UK population of that age. The 18% rate among those aged 50-64 also suggested that frailty may develop relatively early in some people with hemophilia.

All 27 patients with frailty had at least one additional health condition besides hemophilia, and 41% had five or more additional conditions. The most common were hepatitis C, affecting 81%, high blood pressure (78%), and hemophilic arthropathy (74%).

A total of 78% were prescribed at least five regular medications. Falls had been documented for 26% of patients with frailty, while 11% had significant immobility.

The researchers examined eight patients with CFS scores of 5-7, indicating moderate to severe frailty, in greater detail. Hemophilic arthropathy was considered a major contributor to reduced mobility and daily functioning in five of these patients. The three without substantial arthropathy had mild hemophilia, but other serious conditions limited their function.

Scores for a separate Timed Up-and-Go test, which measures how quickly a person can rise from a chair, walk three meters, turn around, return, and sit down, were available for 47 patients. Most completed the test within the conventionally normal time, including some who met the CFS definition of frailty. This led the researchers to suggest that the test may be less sensitive than the broader CFS assessment for this population.

Seventy-eight percent of those identified as frail were sent for review by a hemophilia physiotherapist, and 74% had their primary care physicians notified.

“To our knowledge, this is the first report of frailty measured prospectively [over time],” in people with hemophilia, the scientists wrote.

The team said the study had some limitations, including a small number of patients, limited use of the mobility test, and the lack of systematic screening for all frailty syndromes.

“Further studies are required to determine the long-term implications of high frailty scores … and understand which aspects of haemophilia” most contribute to frailty, they wrote.