Under Pressure

Managing treatment for both hemophilia and high blood pressure
Author: Sarah M. Aldridge, MS

Although you can’t feel it, it can hurt you. High blood pressure, or hypertension, is a condition that should keep us on high alert. “My high blood pressure was picked up as a part of my routine evaluation at my hemophilia treatment center (HTC) in the mid-1980s,” says William R. Buchanan Jr., 68, who has severe hemophilia A and HIV. His reading was 180/120, what the American Heart Association (AHA) considers a hypertensive crisis. “They told me about the risk of stroke,” says the retired US Fish and Wildlife Service employee from Conshohocken, Pennsylvania. “To me, that was the most worrisome thing.”

Just as Buchanan learned, monitoring and managing hypertension should be high among your health priorities when you have hemophilia.

High blood pressure

Blood pressure is measured in millimeters (mm) of mercury. The top number, or systolic pressure, gauges your heart at its peak contraction. The lower number, or diastolic pressure, gauges your heart at rest. The AHA defines high blood pressure as a systolic reading of 140–159 or above, or a diastolic reading of 90–99 or higher. (See “Blood Pressure Stages Table.”)

Risk factors for high blood pressure include age, being overweight, smoking, eating a high-sodium diet and family history. “I was advised to reduce my salt intake,” says Buchanan. And tobacco use. “I was smoking a pack a day, but now I have 6, 7 cigarettes a day,” he says.

Blacks and Hispanics have twice the risk of hypertension as do whites, says the AHA. But that’s not what her study on men with hemophilia revealed, says Annette von Drygalski, MD, PharmD, director of the Hemophilia and Thrombosis Treatment Center at the University of California San Diego. In a 2013 Hypertension study she co-authored, nearly 50% of the 458 men with hemophilia from three diverse hemophilia treatment centers had hypertension vs. 31.7% of their peers without hemophilia. “It was not ethnicity-dependent,” she says. “Everyone had that finding in all age groups.

“High blood pressure also tracked in general with age, body mass index, diabetes and kidney function,” says von Drygalski. However, those risk factors occur at the same rate as in the general public, so more research is needed.

Monitoring and managing

“HTCs and patients equally should start paying attention to their blood pressure early on,” says von Drygalski. For every increase of 20 mm of mercury in systolic blood pressure, the risk of intracranial hemorrhage (brain bleeding) doubles in the general population, she says.

The AHA recommends lifestyle changes to help lower blood pressure. “One is maintaining an ideal body weight,” says Miguel A. Escobar, MD, medical director of the Gulf States Hemophilia Treatment Center in Houston, Texas. “Second is a healthy diet, decreasing the amount of sodium/salt intake.” Another is physical activity. But this can be problematic for older men with joint issues. “We have to work individually with each patient to figure out what they can do,” Escobar stresses. Buchanan can’t do much walking because he needs knee replacement surgery. “I can do isometrics and some resistance exercises,” he says.

Other health issues should be assessed and addressed. “We have to try to find if there are any other risk factors for cardiovascular disease, such as high cholesterol and diabetes, and act on them,” Escobar says. Conversely, because hypertension is a risk factor for heart attack, stroke and kidney disease, these also need to be evaluated in men with hemophilia.

Chronic inflammation from HIV and hepatitis C virus (HCV) can elevate blood pressure. So can some of the medications used to treat these diseases, particularly protease inhibitors. “Patients with HIV and HCV have a worse risk for heart disease,” von Drygalski says.

Effective medications

Becoming a gym rat and banning the salt shaker may not be enough to control your high blood pressure. “I hear too many people say they’re trying a lifestyle change, but it’s not having an effect,” von Drygalski says. “Unless you bring down the blood pressure with medication, sometimes there really is no other rescue.”

Buchanan is on enalapril, an ACE inhibitor that blocks the production of angiotensin, which cause arteries to constrict. He also takes Norvasc® (amlodipine), a calcium channel blocker that relaxes blood vessels. “They’ve kept my blood pressure pretty much in check. Last time it was 120/80 at the HTC,” he says. Other drugs prescribed for hypertension include beta-blockers, which lower the heart rate; statins to get rid of LDL (bad) cholesterol; and diuretics, which help the body eliminate excess salt and water.

“Most of our patients are now treatable,” says von Drygalski. “The closer they can get to this ideal of 120/80, the better.”