Elsevier

Thrombosis Research

Volume 127, Supplement 1, January 2011, Pages S10-S13
Thrombosis Research

Regular Article
Clinical challenges within the aging hemophilia population

https://doi.org/10.1016/j.thromres.2010.10.004Get rights and content

Abstract

Older patients with hemophilia face many challenges related not only to hemophilia but also to general comorbidity associated with aging. Patients with hemophilia often have known risk factors for cardiovascular disease, such as hypertension and hepatitis C virus (HCV) infection, which may counteract any protective effects bestowed by the hypocoagulable state. Arthritis and joint disease are common and contribute to disability and pain. The high prevalence of chronic HCV infection has led to an increased risk for liver failure and hepatocellular carcinoma. Renal function and urological disorders are a concern in these patients, and issues related to sexuality are an important but often-overlooked issue. The use of routine procedures for general health maintenance in the elderly (e.g. colonoscopy) can be more complex in patients with hemophilia due to the inherent risk of bleeding, and serious disorders such as malignancy can be overlooked if signs of abnormal bleeding are attributed to hemophilia, rather than to cancer. Prospective studies are needed to address these challenges so that evidence-based guidance can be given to clinicians who treat older patients with hemophilia.

Section snippets

Joint disease

Among older men, those with hemophilia are more likely to have musculoskeletal disease compared to those without hemophilia (Fig. 2) [11]. Specifically, those with hemophilia had significantly more joint instability, flexion contractures, impaired range of motion, axial deformity, muscular atrophy, joint swelling, and chronic synovitis (Fig. 3). Arthritis and joint disease may contribute to inactivity, muscle weakness, and poor balance that can increase the risk of falls and fractures,

Orthopedic issues

Historically, men were not considered at risk of developing osteoporosis, but evidence is now emerging that indicates that it may be a common event, particularly in men with hemophilia [13], [14], [15]. Infection with human immunodeficiency virus (HIV) or hepatitis C virus (HCV) is associated with an increased risk of osteoporosis [13], [14]. Osteoporosis increases the risk of fractures, which are associated with substantial morbidity and mortality in men [16], [17]. Few studies have evaluated

Cardiovascular disease

In a survey of men aged 65–78 years conducted in Italy, the prevalence of hypertension was significantly higher in those with hemophilia than in those without hemophilia (Fig. 2) [11]. However, the prevalence of CVD and hypercholesterolemia was significantly lower in those with hemophilia; these results may have been compounded by treatment.

The prevalence of atherosclerotic disease appears to be similar in patients with and without hemophilia, but results from some studies have suggested that

Liver disease

Infection with HCV is a major cause of liver disease, liver failure, and transplantation, making it a leading cause of death in patients with hemophilia [1]. In most cases, chronic HCV infection leads to hepatitis and some degree of fibrosis [22]. Co-infection with HIV and HCV can accelerate the progression to cirrhosis and liver failure [23]. Among older patients with hemophilia and chronic HCV infection, the prevalence of cirrhosis increases to as high as 60% [24]. Biomarkers of liver disease

Kidney disease

The risk of death due to renal disease is increased substantially in patients with hemophilia compared to the general population [21]. Risk factors for both acute and chronic renal disease include hypertension and HIV infection (although HIV-negative patients may also develop renal disease); additional risk factors for chronic renal disease are advanced age, non-white race, and recent hospital admission for kidney bleeding [29].

While hematuria is a common and often transient symptom in younger

Health maintenance

Traditionally, patients with hemophilia receive most of their care in hemophilia treatment centers. However, clinicians in hemophilia treatment centers may have limited experience with the wide scope of geriatric and health maintenance issues that older patients with hemophilia face. Collaboration with internists and other specialists is therefore critical for providing adequate care to aging patients with hemophilia.

Many issues related to aging and general health maintenance can be

Conclusions

Older patients with hemophilia face a variety of important health issues. Some issues are related to the aging process and have emerged only recently as life expectancy in patients with hemophilia has improved due to treatment advances. In some cases, factors related to hemophilia may increase the risk of developing certain conditions. Frequent bleeding may also mask signs of serious conditions, such as chronic renal disease or malignancy.

The lack of prospective data on the management of other

Conflict of interest

Research support from CSL Behring and Baxter; consultancy for CSL Behring, Baxter, Bayer, Novo Nordisk, Inspiration Biopharmaceuticals.

Role of the funding source

Support for the seminar by CSL Behring.

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