Older age ‘unknown territory’ for patients with bleeding disorders

People with bleeding disorders including haemophilia are living longer than ever before, meaning new strategies are needed for their care and ongoing treatment, a new Australian report finds.

Australia is entering “unknown territory” as the first generation of patients to experience the impact of ageing on bleeding disorders enter their senior years – with some patients living into their 80s and 90s as a result of improved treatments, according to the Haemophilia Foundation of Australia report.

HFA developed the report, Getting Older with a Bleeding Disorder: a needs assessment, after consultation with people in the bleeding disorder community, specialist haematologists and other relevant organisations, to assess the future needs of older people with bleeding disorders including haemophilia and Von Willebrand Disease (VWD).

Australian Bleeding Disorders Registry (ADR) data show that as of June 2019, there were more than 6300 Australians living with bleeding disorders – including a significant number aged ≥ 65 years with mild haemophilia, VWD and Factor X1 deficiency and a few over 95.

Relatively young people with bleeding disorders also often experienced complications that were usually associated with growing older, such as joint and muscle damage, arthritis, pain and mobility problems, the report found.

Data from the PROBE (Patient Reported Outcomes Burden and Experiences) Australian Study showed that challenges and concerns faced by older people with bleeding disorders included mobility, joint and dexterity problems, losing independence, financial concerns nursing homes being unable to provide adequate care and difficulties with treatment.

Many people with bleeding disorders were living with hepatitis C and some had HIV acquired from treatment products during the early days of the epidemics.

“As they entered their senior years, they were concerned they were reaching a tipping point where the health conditions of ageing combined with complications of their bleeding disorder could be too difficult for them to manage, even with their usual determination and positive approach,” the report authors said.

One haematologist interviewed for the report said: “Lot of patients have got complex multi-system disorders. This has an impact on their quality of life, which is compounded by the bleeding disorder, which needs regular treatment,”

Another haematologist commented that other health care services may sometimes overlook contacting the Haemophilia Treatment Centre (HTC) for management advice when older people with bleeding disorders consulted with them about health conditions unrelated to their bleeding disorder.

“This could have significant implications for their health if bleeding complications and interactions with their bleeding disorder treatments were not taken into consideration,” the report cautioned.

Haematologists working in HTCs also expressed concern that care of older people with bleeding disorders and their complications required close liaison between the GP and the HTC, and this could  be difficult if there was not a strong relationship with a specific GP or a lack of continuity of care.

And haematologists faced challenges such as infusions and vein care becoming “increasingly problematic” as patients got older.

Difficulties including fragile or hidden veins, collapsed veins due to hydration, problems with dexterity in the hands and vision problems meant people could have multiple attempts to infuse, sometimes leading to avoidance or delay of treatment. The problems could prompt some patients to switch from prophylaxis back to on-demand treatment, which could have negative impacts on their health.

New and emerging therapies could help to address those challenges, the report suggested.

For example, Extended Half-Life (EHL) clotting factor concentrates for Factor VIII and Factor IX have enabled clinicians to eliminate bleeding episodes entirely in some people with haemophilia. The extended half-life reduced the frequency of infusions from 2-4 times a week to 1-2 times a week in haemophilia A, and from 1-2 times a week to every 10-14 days in haemophilia B.

Non-clotting factor concentrates such as emicizumab, fitusiran and concizumab also had the advantage of being given subcutaneously every 1-4 weeks for prophylaxis.

The HFA outlined wide-ranging recommendations, including ensuring Haemophilia Treatment Centres (HTCs) were resourced to coordinate the many services and programs needed to support patients’ needs as they grow older, so their care was better integrated.

It urged better preventive dental care for patients, access to new and emerging therapies that require less frequent infusions, and further research to better understand the impact of ageing on people with bleeding disorders, including on arthritis, heart disease and hypertension.

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