April 19, 2012

‘Head rush’ disorder makes life a daily balancing act

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Angela Pascale’s chronic condition makes her feel like she gets a head rush every time she stands up.

‘Head rush’ disorder makes life a daily balancing act

Angela Pascale says living with Postural Tachycardia Syndrome (POTS) is like having a sand timer of finite energy. But while others may have a whole gallon of sand in their day, she has a mere thimbleful.

POTS is a chronic condition with a constellation of symptoms that tend to be worse when standing – heart palpitation, lightheadedness, shortness of breath, nausea and dizziness. It’s like experiencing a “head rush” every time you stand up.

The diagnostic criteria for POTS is a rapid heart rate increase of 30 beats per minute or more upon standing, which lasts for 6 months or longer, and a constellation of symptoms that can include extreme fatigue, mental changes commonly called “brain fog,” and skin changes, such as red or purple discoloration in the feet.

POTS is a dysfunction of the autonomic system, the body’s hard drive that controls everything behind the scenes. It affects a half million Americans, 80 percent of them female, often of childbearing age. Some patients improve over time, but many, like Pascale, must learn to live with a new normal.

“People who are well can think of their life on a 1-10 scale. I’m on a 1-5 scale. I never get over 5. My good day is a day you would feel like you were getting sick – tired, weak, everything off,” she said.

Pascale was in her early 20s, living in her own condo and working in Nashville’s music industry when she began having rapid heart rate episodes and trouble staying awake. She was missing a lot of work because she couldn’t even get out of bed. Like many POTS patients, Pascale bounced from doctor to doctor trying to find an explanation.

David Robertson, M.D.

David Robertson, M.D.

Satish Raj, M.D., MSCI

Satish Raj, M.D., MSCI

“A lot of doctors tell you you’re crazy. You come in with 50 symptoms that are not related and of course they’ll think you’re a hypochondriac,” she said.

She finally received her POTS diagnosis from the Cleveland Clinic in 2005 at age 24 and came to the Vanderbilt Autonomic Dysfunction Center for two weeks of clinical research.

“Vanderbilt has the best team, and the clinical research helped me so much,” Pascale said. “For two weeks they poked and prodded, but they also taught me about my body and how symptoms could be signs for something imminent. I began to see the patterns, which gave me more awareness and self-control of my body.”

David Robertson, M.D., Elton Yates Professor of Medicine, Pharmacology and Neurology and director of the Clinical Research Center, said nearly every week a POTS patient is undergoing tests to see which drugs or regimens will help alleviate symptoms.

“There is broad variety with this illness,” Robertson said. “Some may get better but in many, the progress is slow. Our treatments are inadequate and it can be a very long haul. We need more research.”

The cause of POTS is unknown. Some patients develop the condition around puberty, while others present after what appears to be a viral illness. A Vanderbilt genetic study found an anomaly in the norepinephrine transporter, which plays a role in heart rate regulation, but most patients do not have that genetic mutation.

One theory holds that POTS patients have less “squish” in their blood vessels, so circulation is not as effective. Another theory posits that these patients are severely deconditioned and will improve with an exercise regimen.

Satish Raj, M.D., MSCI, assistant professor of Medicine and Pharmacology and associate director of the Masters of Science in Clinical Investigation program, is conducting studies of the regulation of the hormone aldosterone, which helps retain sodium and fluid. Seventy percent of POTS patients have low blood volume, and a synthetic version of aldosterone may normalize blood volume.

Anecdotally, symptoms seem to improve with a high sodium diet and patients are often prescribed salt tablets, but there is not yet scientific evidence to support this.

“One thing we’d like to do better is what we call endophenotyping. You have this overall group of POTS but there are different ways they got there. So how can we identify those subgroups to come up with more rational therapies?” Raj said.

Now living in Austin, Texas, with her cousin and his two young children, Pascale knows how to carefully allot her thimbleful of energy each day.

She gets eight-nine hours of sleep and takes naps, drinks 80 ounces of water, eats small meals and avoids sugar and caffeine. If she wants to go out with friends in the evening, she has to spend all day with her feet up, resting. She scoots around the kitchen on a stool to preserve her energy while she cooks. She’ll pass out after standing for seven minutes, so she’s constantly fidgeting in any line she waits in.

Angela Pascale’s condition keeps her working from home creating custom artwork such as the Vanderbilt Autonomic Dysfunction Center’s holiday card.

Angela Pascale’s condition keeps her working from home creating custom artwork such as the Vanderbilt Autonomic Dysfunction Center’s holiday card.

“I feel like an 80-year-old now and wonder how I’ll feel later on. I’m definitely more grounded and appreciative, but I would have enjoyed being a careless 25-year-old,” she said.

“A lot of people with POTS go through depression. It’s a big loss in life. You lose the person you were. It just creeps into your life and poisons everything.”

She uses her talent for art to work from home drawing custom invitations, greeting cards and logos. Using pencil and marker and then enhancing the work in Photoshop, Pascale creates colorful and whimsical drawings with a hint of melancholy. Last Christmas, she created the Vanderbilt Autonomic Dysfunction Center’s holiday card, featuring Santa having a tilt test, used to diagnose POTS.

Pascale has accepted her new normal but can’t help but wonder how it came to be.

“The biggest frustration is not knowing why we got sick. You can’t help but think it was something you did. People always ask when I got sick and how I got sick and I don’t know the answer to any of those.”

Robertson, however, is sure that more research will eventually bring more answers.

“I think it’s a problem that will be treatable. But right now we have to recognize that in some patients this disease takes a heavy toll and and lasts for years. We just have to understand it better. I’m optimistic over the long haul.”

See more of Pascale’s artwork at www.angelapascale.com.