Decades ago most hemophilia patients died young.
Advances in treatment have led to them living about as long as the general population, which means that for the first time, doctors are facing the challenge of caring for aging hemophiliacs.
A key problem is that in the days before treatment breakthroughs, patients experienced hemophilia-related organ and other damage that can pose problems for doctors treating them today.
Researchers studying the issue concluded that a multifaceted, integrated approach is the best way to care for aging hemophiliacs.
Their review, “Progress in the contemporary management of hemophilia: The new issue of patient aging,” was published in the European Journal of Internal Medicine.
Before the hemophilia treatment breakthroughs that began four or five decades ago, patients “had a very short life expectancy” of 20–30 years,” the researchers wrote. “Many of them died at a young age due to life-threatening bleeding episodes. Today, this clinical picture has dramatically improved.”
As hemophiliacs get older, they face many of the same age-related health problems others do.
But older hemophiliacs “suffer from the consequences of the comorbidities [other medical conditions] that developed when their treatment was at the same time poorly available and unsafe,” the researchers said.
This means that some age-related problems are likely to show up in hemophiliacs earlier than in the population at large.
- musculoskeletal problems – loss of joint and muscle function and chronic pain that can impair daily activities and worsen quality of life.
- cardiovascular disease – risk factors most frequently seen in hemophilia patients include hypertension, or high blood pressure, and being overweight.
- viral infections such as HIV and hepatitis – these conditions imply treatment with antiviral drugs, which may affect patients’ kidneys and liver.
- chronic kidney disease – this condition causes hypertension. Because of hemophilia patients’ deficient blood clotting ability, it could increase their risk of a brain hemorrhage.
- chronic pain – this condition also implies treatments that could adversely affect hemophilia patients.
- cancer – chemotherapy can cause loss of white blood cells, which could worsen hemophilia patients’ tendency to bleed.
These six age-related conditions require therapies that can affect hemophilia patients’ bleeding disorders and treatments. This makes it harder for doctors to manage their treatments.
“Issues related to the intake of multiple drugs are likely to be particularly cogent in older” hemophilia patients, “who often have some degree of pre-existing impairment of such organs critical for drug metabolism and excretion as the liver and kidney,” the researchers wrote.
“With this background, a new challenge is to implement a comprehensive approach aimed to provide optimal care for the aging [hemophilia patients], uniquely characterized by the long-term consequences of a lifelong bleeding disorder superimposed on the problems generally experienced by older people.”
The researchers said managing older patients’ disease requires an “integrated evaluation of functional, mood, cognitive and therapeutic domains.” Doctors from different specialties — including internists, geriatricians and clinical pharmacologists — should cooperate to optimize patient care, the team said.