The minute Pedro Estalilla came home from his job as a database administrator one day in 2018, his wife, Diana, a registered nurse, and daughter Ashley knew something was wrong. He was pale and in pain, and they found that his blood pressure was extremely high.
At the emergency room near the couple’s home in Tulare, California, doctors determined that Estalilla, who has type 2 diabetes, was having a mild heart attack. Because he also has
Five hospitals turned him down, and 18 hours later, Estalilla was still waiting. By the time he was finally airlifted to the University of California, San Francisco Medical Center (UCSF Health), his mild heart attack had become major.
“It’s like they say: It felt like an elephant was sitting on my chest,” says Estalilla, 55. “I thought, ‘This can’t be the end of me. But if this is it, I need to say my goodbyes.’”
Doctors rushed him to the hospital’s catheterization lab, where they opened the artery that was stopping blood flow to his heart and inserted stents to keep it open, all while treating and monitoring his bleeding disorder. Fortunately, he survived the scare and spent a week in the hospital while his doctors figured out the best postoperative treatment for him. He now focuses on
Cases like Estalilla’s are becoming more common among the bleeding disorders community. Advances in treatment and improved blood and factor product safety have allowed people with bleeding disorders to better manage their conditions and live longer lives.
Now, they are facing health risks rarely encountered in the past. “With every success comes new problems,” says Andrew D. Leavitt, M.D., director of UCSF’s program for noncancerous blood disorders and co-director of the UCSF Hemophilia Treatment Center. “Now, we’re dealing with disorders of being in your 50s and 60s that we never used to deal with because we didn’t have patients in their 50s and 60s.”
Growing Risks and Challenges
Doctors used to think that hemophilia protected the heart because the blood is naturally “thin.” But that’s not entirely the case. Rates of heart disease are rising for people with bleeding disorders, although they are slightly less likely to die from cardiac causes than people whose blood clots normally.
Like everyone else, people with bleeding disorders become more prone to heart disease risk factors such as high blood pressure, high cholesterol, and obesity as they age — and sometimes at higher rates than in the general population. For instance, the prevalence of hypertension in adults with hemophilia is 49%, compared with about 32% in the population at large, according to a
Rates of obesity are also growing among people with bleeding disorders, research shows. “Joint disease from past bleeds leads to limited activity, which can lead to weight issues,” Leavitt says. (Frustratingly, a high body mass index results in limited range of motion and increased pain, making it harder to exercise and lose weight to improve heart health.)
When heart attacks, strokes, and other serious cardiac events happen, doctors must weigh the need for anticoagulant and antithrombotic treatment against the clotting factors required to treat hemophilia. Balancing those competing risks requires significant coordination between hematologists and cardiologists, Leavitt says.
“The challenge becomes finding a product to maintain their levels high enough that they can tolerate all of the anticoagulant and antiplatelet drugs needed,” Leavitt says. The good news: “We now have easier ways, including long-acting factor, to maintain higher levels longer to have that nice balance between giving new treatments related to the heart disease and providing a level of safety against bleeding due to their hemophilia.”
Be alert to these common symptoms, and always call 911 if you suspect a heart attack.
- Chest discomfort: Uncomfortable pressure, squeezing, fullness, or pain in the center of the chest that lasts for more than a few minutes, or that goes away and comes back
- Discomfort in other areas of the upper body: Pain or discomfort in one or both arms, the back, neck, jaw, or stomach
- Shortness of breath
- Other signs: Sweating, nausea, or lightheadedness
Prevention Is Key
Doctors prefer to prevent serious events such as heart attacks and strokes from happening in the first place, however, says Jacob Mayfield, M.D., chief cardiology fellow at the University of Washington in Seattle. “Things like a healthy diet and exercise are important because those are the things that will help normalize risk,” he says.
To fend off cardiovascular disease, experts suggest following these steps:
Eat a Plant-Based Diet
Mayfield recommends two dietary approaches for good heart health. “The Mediterranean diet is what I recommend to most patients,” he says. The DASH diet, which stands for dietary approaches to stop hypertension, is similar and particularly helpful for people with high blood pressure.
Both eating plans feature generous quantities of fruits and vegetables, lean protein such as fish and poultry, low-fat dairy, healthy oils, whole grains, beans, nuts, and seeds, with sparing amounts of salt, sugar, and saturated fat. The Mediterranean diet emphasizes heart-friendly olive oil.
Get Moving
Official exercise guidelines call for 30 minutes of moderately intense physical activity five times a week. But, says Mayfield, “everyone’s a little different. The most important thing is to set small, incremental goals for yourself.”
If you are just getting started, walk for five minutes three times a week, then increase the goal after a month. “You’re going for sustainable changes,” Mayfield says. Swimming is another option that is easy on the joints.
Try to Keep a Healthy Weight
Healthy eating and regular exercise are both key to dropping excess pounds. Losing weight may sound overwhelming, but shedding just 5% to 10% of your body weight can improve your blood pressure, cholesterol, and blood sugar levels,
Kick the Habit
Quitting smoking does wonders for every aspect of your health, but particularly for the heart and lungs. “It can be challenging, when someone has a chronic disease, to give up things that provide pleasure,” Mayfield says. “But quitting can really extend your life.”
Educate Your Provider
It’s crucial to have a relationship with a primary care or internal medicine doctor to monitor your overall health, particularly heart disease risk factors such as cholesterol, blood pressure, blood sugar, and weight, Mayfield says.
But keep in mind that “many providers have a bias that people with bleeding disorders are unlikely to have thrombotic complications like heart attacks or stroke,” he says. “Patients need to remind their provider that people with bleeding disorders are living longer and that it’s important to try to prevent cardiovascular disease.”
Jennifer DeGlopper, 57, a digital marketing consultant in Punta Gorda, Florida, who has hemophilia B and von Willebrand disease, had to prompt her primary care doctor to take a closer look at her rising cholesterol and blood pressure readings.
“As I got older, I thought maybe it’s time to do something about it,” says DeGlopper, who has a family history of heart problems and was just a year shy of her father’s age when he died from a heart attack. She ended up seeing a cardiologist, which is a reasonable option if you have significant risk factors. She is now on both blood pressure and cholesterol-lowering drugs.
Take Prescribed Medications
Thankfully, antihypertensive and cholesterol-lowering medications such as statins are generally fine to take if you have a bleeding disorder, Mayfield says. Use them as directed to get the most benefit.
But it may be a different story if you need antiplatelet drugs (blood thinners), such as aspirin or clopidogrel (Plavix), or anticoagulant drugs, which could trigger additional bleeding. “That’s where it gets complicated for people who have bleeding disorders,” Mayfield says. “It really has to be an individualized approach. If someone has a relatively low-risk atherosclerotic process, we may not give them any antiplatelet therapy at all.”
Whether you are trying to wrangle troublesome risk factors or manage an ongoing heart issue, make sure that specialists are working together on your dual health challenges, Leavitt stresses. “Everybody is different, and very close interactions between your hematologist and cardiologist are essential,” he says. “This is definitely not plug-and-play.”
Rick Starks took up tai chi, the ancient practice of meditative movement, after he had a heart attack 10 years ago. The 68-year-old former paramedic in Haigler, Nebraska, grew up before the newest therapies for treating his hemophilia B had been developed. After years of practicing martial arts — and sustaining multiple injuries and bleeds — he found himself overweight and in too much pain to exercise.
“Both ankles were pretty much destroyed so bad that I could hardly walk a couple blocks,” says Starks, who also had both hips replaced in his 30s. “I became a couch potato because I couldn’t do anything.”
After his heart crisis, Starks started watching tai chi videos and then took classes online. His weight dropped to 200 pounds, and he went off his blood pressure medication. Soon, he was teaching tai chi at National Hemophilia Foundation meetings and elsewhere, inspiring others to follow his lead.
“The gentle movement strengthens your body and increases your flexibility,” Starks says. “Then there’s the meditative part of it that helps all those things that stress you out during the day. Right now, I feel like there’s no reason I couldn’t live to be 100.”