Better treatments and comprehensive care provided at hemophilia treatment centers (HTCs) during the past 35 years mean people with hemophilia are now reaching their golden years. While living longer is something to celebrate, it also means facing aging-associated health conditions, such as cancer and kidney disease.
A review of previous studies, conducted by Barbara A. Konkle, MD, a full member of the Puget Sound Blood Center and professor of medicine at the University of Washington School of Medicine in Seattle, and colleagues from across the country, was published in the November 2009 issue of Haemophilia. It identified five co-morbidities that aging patients with hemophilia and their providers need to address—cancer, cardiovascular disease, joint disease, liver disease and renal disease—and provided management tips.
Studies in the US and the Netherlands have shown that cancer is a common cause of death for people with hemophilia. The authors attributed this to overlooked symptoms, such as blood in the urine or stool, and delayed treatment because of bleeding complications.
Patients who are co-infected with HIV are more prone to non-Hodgkin lymphoma, basal cell cancer and Kaposi sarcoma. But the rates have been cut in half in the past decade with the use of highly active antiretroviral therapy (HAART).
The most common cancer in patients with hemophilia and hepatitis C virus is hepatocellular carcinoma, a type of liver cancer. For those at risk for liver cancer, the authors recommended following the practice guidelines of the American Association for the Study of Liver Disease, which include periodic ultrasounds and blood testing for tumor markers.
Patients with hemophilia should follow the American Cancer Society screenings for the general population, including for colonoscopy, the authors wrote.
When it comes to heart health, previous studies of the protective effect of hemophilia have been contradictory. The authors note that older people with hemophilia are often sedentary and overweight, and have high cholesterol and triglycerides—all risk factors for cardiovascular disease. The authors foresee the need for cardiologists to work with HTC staff to coordinate care. Heart-healthy lifestyle changes for the general population apply to this community, too—quitting smoking, starting exercise, eating a low-cholesterol diet and taking medications, if necessary. In cases in which standard heart function tests, such as walking on a treadmill or riding an exercise bike, are too difficult because of advanced joint arthritis, the authors recommended substituting water exercise or an elliptical machine.
Although prophylaxis is common in younger patients with severe hemophilia, it is less common in older men. Because primary prophylaxis is the only known approach to prevent or limit joint disease, older people with hemophilia often suffer from advanced arthropathy. Managing their advanced joint disease, said the authors, takes a multipronged approach that should include pain medications; RICE (rest, ice, compression, elevation), splints, casts and physical therapy; and daily exercise. Less invasive procedures, such as synovectomy, may decrease bleeding frequency and pain in target joints but cannot reverse bone and cartilage damage. Joint replacements can be successful in the right patient. Exercise and maintaining a healthy weight were also recommended.
[Steps for Living: Treatment Basics]
A major cause of death and disability in people with hemophilia is chronic liver disease from hepatitis C infection. People co-infected with HIV tend to experience more rapid development of cirrhosis and liver failure. Currently, the most successful treatment regimen for hepatitis C is a combination of pegylated interferon-alpha and ribavirin. Unfortunately, this regimen fails to clear the virus in up to 50% of patients.
To manage liver disease, the authors recommended liver biopsy, the gold standard in determining the extent of disease and predicting progression. The authors also discussed the use of liver transplantation in certain patients.
According to the authors, the most surprising finding came from a previous survey of adult patients with hemophilia, which found a 50-fold increased risk of dying from kidney disease. The main contributing risk factors were the presence of HIV infection or inhibitors, high blood pressure and kidney bleeding.
The authors recommended the same types of diligent management as in the general population, such as consulting a nephrologist and ordering appropriate tests and kidney biopsy, if needed. They also discussed dialysis and kidney transplant for people with advanced kidney disease.
Although this list of possible issues related to aging may look daunting, the secret to staying healthy as you get older is being proactive. Scheduling routine screenings, doctor visits and exams will help you stay on top of your health concerns.
This study was supported by Baxter Healthcare Corporation.