Don’t tune out news stories about strokes just because you have hemophilia and think you’re protected. A 2011 study published in Haemophilia showed that adult men with hemophilia were twice as likely to develop heart disease, which puts you at risk for stroke and heart attack, as non-Hispanic white males. Further, if you have hemophilia B or inhibitors and use prothrombin complex concentrates, you are at increased risk for clot formation. It’s time to tune in and take charge.
A stroke results from a sudden interruption of blood flow to the brain. Starved of oxygen carried in the blood, brain cells can die. The American Stroke Association (ASA) lists three main types of strokes: hemorrhagic, ischemic and transient ischemic. Hemorrhagic strokes comprise about 13% of all strokes. They occur when a weakened artery in the brain leaks or ruptures, spilling blood into brain tissue. The blood puts pressure on brain cells, causing damage. This is the most common type of stroke in people with hemophilia, says Annette von Drygalski, MD, PharmD, director of the Hemophilia and Thrombosis Treatment Center at the University of California San Diego.
Ischemic strokes account for about 87% of all strokes. They result when an artery to the brain is narrowed or blocked. The main contributor is atherosclerosis, a buildup of fatty deposits on blood vessel walls called plaque. A blood clot at the site is called a thrombosis. One that breaks free from the artery and travels in the bloodstream where it lodges in another site is called an embolism.
Transient ischemic attacks (TIAs), or mini-strokes, differ from other strokes in their duration. Most are short, lasting one to five minutes. But they should be taken seriously. About one-third of people who experience a TIA sustain a stroke within a year, warns the ASA.
Ramping up risk
The ASA reports that nearly 800,000 Americans have strokes each year, three-quarters of them for the first time. Strokes are the fourth-leading cause of death and impairment in the US. Almost half of those who survive a stroke have cognitive defects six months later.
Patients with hemophilia should know their risk of sustaining a stroke. For the general population, the main risk factors are hypertension (high blood pressure), smoking, being overweight or obese, and having diabetes mellitus. Other contributors include high cholesterol, lack of physical activity, eating a high-fat, high-sodium diet, alcohol abuse, stress and cardiovascular disease.
However, some patients with hemophilia are at an alarming risk of stroke. “For patients with severe hemophilia, the risk of dying from stroke is approximately 40-fold higher than for the general age-matched male population,” says von Drygalski. Hypertension pushes the risk even higher, she warns.
Work with your primary care physician or cardiologist to address your risk factors. Getting your high blood pressure under control is imperative. It’s the main contributor to strokes and the most easily managed risk factor.
Women with bleeding disorders whose heavy menstrual bleeding (menorrhagia) is controlled by hormonal contraceptives should be aware of the associated risk of strokes. A 2012 study published in the New England Journal of Medicine that followed 1.6 million Danish women for 15 years showed that the safest hormonal contraceptives were those containing progestin only. “Birth control pills that contain estrogen derivatives have the highest risk of blood clots,” von Drygalski says. The researchers cited the Mirena® intrauterine device (IUD) as being safe, an option that is often recommended for women with bleeding disorders (See “Contraceptive Helps Decrease Bleeding in Women” http://www.hemaware.org/story/contraceptive-helps-decrease-bleeding-women). Skin patches and vaginal rings were significantly riskier, though.
In addition, oral contraceptives containing estrogen and progestin increased risks of heart attack and stroke twofold. Older age was also a factor, as were smoking and hypertension. The FDA recommends that women over 35 years old who smoke should not use combination hormonal contraceptives. “The risks and benefits of oral contraceptives for a woman with a bleeding disorder should be discussed with the prescribing physician or gynecologist,” advises von Drygalski.
When it comes to a stroke, time is ticking. The sooner the person gets to the hospital, the quicker the stroke can be managed. For the general population, the clot-busting drug called tissue plasminogen activator (t-PA), if administered within three hours of the stroke, can minimize long-term damage and speed recovery. But t-PA increases the risk of further bleeding. “It may not be appropriate for people with bleeding disorders,” von Drygalski says.
Other treatments may include low-dose aspirin or antiplatelet medications. Their use depends on the type and severity of your bleeding disorder, the type and severity of the stroke, personal risk and family history of strokes, and the use of prophylactic clotting factor replacement, says von Drygalski. She stresses that treatment is individualized and should be based on expert opinion, requiring careful analysis of your personal risks and benefits. “That’s why it is important to obtain care from hematologists experienced in hemostasis/thrombosis, preferentially specialized in adult care,” she says.
Damage and repair
Recovery from a stroke depends on many factors, including its location and how much brain tissue was affected. The following stroke complications can be temporary or permanent: numbness or paralysis, blurred or lost vision, impaired or lost speech, chewing and swallowing difficulties, and loss of bladder and bowel control. That’s also true if the person experiences memory loss, a change in emotional state and/or depression.
Rehabilitation to regain verbal and motor skills may involve speech therapy, physical therapy and occupational therapy.
In October 2014, the American Heart Association and ASA published new and revised guidelines in the journal Stroke online, the first update since 2011. Among the more than 30 new recommendations are lifestyle changes. “Those include weight loss, smoking cessation, a moderate amount of physical activity and dietary changes to include more fruit and vegetables,” says von Drygalski.
The guidelines also recommend measuring your blood pressure at home regularly, using a device with a cuff. Women who are prone to migraine headaches with aura (visual disturbances) are advised to only take oral contraceptives that contain no estrogen.
Further, the guidelines mentioned revamping your diet. They encourage people to adopt the Mediterranean diet, supplemented with nuts. Because salt contributes to high blood pressure, patients are encouraged to lose the salt shaker, but increase their potassium intake. It’s important to read product labels and choose foods that are low in sodium and salt. The joint guidelines also encourage people to get screened for sleep apnea, a temporary lapse in breathing during sleep, that can cause loss of oxygen to the brain and body.
The loss of control that can result from a stroke is daunting. But you can stay in the driver’s seat if you reduce your risk factors and manage your blood pressure.