A Strong Bond

Good friends were brought together by a serious vascular disorder often overlooked in women

Brigitte Santella remembers telling her husband that she’d rather die than feel like this.

Up until her 40s, the Chesapeake, Va., nurse had been healthy. Then out of the blue in 2005, her blood pressure started to spike.

In 2006, a series of symptoms followed — stomach pain, nausea, dizziness and a pounding heart so severe she couldn’t work and ended up in the emergency room a few times.

On one of those ER visits, she met two women who knew exactly what she was going through.

At 6 a.m. on Feb. 15, 2009, Maureen Womack showed up at Santella’s bedside. The two women had talked on the phone a month before but this was the first time they had met face-to-face. A few hours later, Womack left to check on her pet and came back with Mary Hayes.

“It was like angels being sent to me,” says Santella.

The three women bonded instantly; all close in age, all mothers who worked in the medical field, all living in Chesapeake.

But what brought them together was a very similar nightmare: life with fibromuscular dysplasia (FMD).

Not as Rare as Once Thought

Although considered a rare disease, it is possible up to 10 million Americans have FMD, although many never experience any symptoms, says UVA’s Alan Matsumoto, M.D., an FMD expert. About 85 percent of known cases have affected women under 50 and girls, although men and boys can have it too.

In severe cases, as with Hayes, it can cause a stroke. It can also lead to heart attack.

With FMD, there is an abnormal development or growth of cells in the walls of arteries, causing the vessels to narrow or bulge. Tell-tale signs of the disease are an artery that looks like a string of beads and a roaring sound (or bruit) that can be heard with a stethoscope.

It most often affects the renal and carotid arteries (that supply blood to the kidneys and brain respectively), but almost any artery can be involved. The causes are unknown. Mechanical and hormonal factors may play a role. There also may be a genetic component as it can run in families.

Hayes’ Family Connection

Hayes’ sister has FMD and her mother died from it in 2004. FMD almost took Hayes’ life as well. After she went into congestive heart failure in 2004, it took quite a while, she shares, before doctors figured out that her heart problems were stemming from a renal artery that was almost 90 percent blocked. FMD also closed up her carotid artery, leading to a stroke and ending her career as a respiratory therapist.

Once FMD is uncovered, symptoms can be controlled with blood pressure medication and regular angioplasties where doctors insert a balloon to open up a blocked artery.

Santella, Hayes and Womack have had their fair share of angioplasties. And all take medication to keep their blood pressure in check. They also share a deep gratitude for the expertise they’ve found at UVA. They’ve all had an angioplasty performed by Matsumoto, an interventional radiologist, and regularly make the 3-hour drive to see him.

Matsumoto is one of several FMD experts at UVA, including two pediatric nephrologists who specialize in treating children with the condition.

UVA has a long history of treating FMD. In fact, in 1978 a UVA interventional radiologist, Charles J. Tegtmeyer, M.D., changed the course of medical history when he performed the nation’s first balloon angioplasty — a procedure that has either improved or saved the lives of countless people with FMD and other vascular conditions. (Dr. Tegtmeyer died in 1996.)

While some doctors are not familiar with the condition and often overlook it, Matsumoto and his colleagues know it well.

“Hypertension and stroke are the more common symptoms of FMD,” Matsumoto says. “But someone can also experience intestinal symptoms, a choking sensation and even anxiety like when you’ve had too much coffee. Brigitte, Mary and Maureen all had these peculiar symptoms and at one time it was implied to them that it was all in their head.”

Womack’s Harrowing Year

For several months during 2007, Womack went to the ER eight times, not to visit a friend but rather because her blood pressure would spike whenever she stood up.

She remembers that year well: “I couldn’t go anywhere. I couldn’t drive. It felt like a noose was around my neck. My right arm would go numb. My speech was slurred. I really thought I was going to stroke. Then it would pass after a couple of minutes.”

Hearing these symptoms and reviewing tests that showed Womack’s arteries to be clear, Matsumoto referred her to his colleague Dearing Johns, M.D., a cardiologist.

Four weeks later, Womack’s blood pressure was under control and her symptoms were gone. The key was that Johns closely monitored Womack (even having her call whenever her blood pressure went up) so Johns could quickly adjust the medication and dose until finding just the right mix.

“Dr. Johns worked her miracle,” Womack says.

A Shared Gratitude

Remarkably, despite such a frightening disease that springs up almost without warning, Womack retains a sense of humor.

“I call it 'arteries with an attitude,' she jokes.

No doubt, her humor, family and new-found friends all help. As a volunteer for the advocacy group Fibromuscular Dysplasia Society of America, she reaches out to other women with FMD and when they’re close enough (like Hayes, Santella and one of her co-workers) she sends them to UVA.

“I know I would not be here today if it weren’t for them,” Womack says of her UVA doctors.

She adds: “The big thing with Dr. Matsumoto and Dr. Johns is they believed me. They listened to my symptoms even though my blood pressure would sometimes be normal in their office but high when I got out to my car.”

Hayes and Santella, too, are grateful for Matsumoto.

“I love him. He’s wonderful. He really cares,” says Mary Hayes.

“He’s a great listener,” adds Santella.

Santella is also thankful to the Fibromuscular Dysplasia Society; it put her in touch with Womack, then Hayes and ultimately UVA. A week after she registered on the society’s website, she got her first of many calls from Womack. “She told me all my symptoms were due to FMD. I was doubtful. I questioned why I had GI, cardiac and musculoskeletal problems if the culprit was a renal artery problem. But Maureen gave me hope. It’s been a blessing to have Maureen and Mary to talk to.”