You have to admire someone who can study for the bar exam without leaving her bedroom. While most aspiring lawyers haunt law libraries, Tishisa Braziel (pronounced "Brazil," like the country) would listen to lectures on CD and read books in her bed. She wanted to spend more time out of the house, but her sick lungs and heart just wouldn't let her. "The whole time I was doing it, I couldn't get Descartes out of my mind," she says. "He was famous for working in bed."

Braziel, a resident of Richmond, Virginia, has primary pulmonary hypertension, a rare and potentially fatal disease that strains her heart and hampers her breathing. For a while, it also kept her from looking more than two weeks into the future. She feels much better these days --well enough to get out of bed for long stretches -- and, thanks to successful treatments, she now plans as far ahead as any other 33-year-old. She recently passed the bar exam, and now she hopes to become a tax lawyer for the IRS, retire with a government pension, and travel the world. Most of all, she plans on staying alive.

There was a time when people with primary pulmonary hypertension didn't have much to look forward to. The disease occurs when the main artery in the lungs begins to narrow, forcing the heart to work extra hard to keep blood flowing. The blood pressure in the artery rises, and both the lungs and heart became gradually weaker. Without treatment -- and sometimes even with treatment -- the condition eventually leads to heart failure and death.

Nobody knows what causes primary pulmonary hypertension. In fact, the term "primary" is doctor code for "something that happens for no apparent reason." Braziel can't say for sure why she got this one-in-a-million disease, but she has her suspicions. She spent the early 1990s in the army, and she was stationed in Turkey during Operation Provide Comfort, a mission that supported Kurdish refugees. She suspects she may have been exposed to something during her service that set her disease in motion.

Without a doubt, her symptoms began to emerge while she was still in uniform. After serving in Turkey, she was stationed at a base in Germany. The daily exercise drills should have kept her in top shape, but she felt like she was sliding downhill. One day, she fell out of formation during a run and promptly passed out. Her sergeant sent her to sick call, but doctors couldn't find anything wrong with her. It would become a recurring theme.

After four years in the army, Braziel returned to civilian life at Mary Baldwin College in Staunton, Virginia. Nobody was asking her to run in formation and shout cadences, and it was easy for her to forget about her physical troubles. She got out of breath walking up the hills on campus, but she told herself she was just out of shape. Things got better when she started law school, mainly because the campus at the University of Virginia is relatively flat.

The relief was only temporary. Braziel took a year off from school, and by the time she returned, her symptoms were impossible to ignore. "I couldn't walk 10 feet without doubling over for air," she says. "I thought to myself, 'I'm only 28-years-old. Nothing should make me feel like this.' " She went to the Student Health Center, where she was diagnosed with asthma. When asthma medications didn't clear up her symptoms, the doctor strongly implied that her problem was mainly in her head. She kept coping with her "asthma," but life got increasingly difficult. One day, she had to climb a flight of 20 stairs at the university library. She got extremely dizzy at the top and probably would have fallen backwards if someone hadn't grabbed hold of her arm.

It was time to get serious. Braziel promptly scheduled an appointment with a pulmonologist at the University of Virginia. The specialist at first suspected chronic bronchitis or emphysema -- relatively common diseases that block air flow in the lungs -- and the first series of tests showed nothing out of the ordinary.

Perplexed, the pulmonologist decided to put Braziel through one more simple test. He placed an oxygen sensor on her finger and asked her to take about 15 steps down the hall. As Braziel walked, her oxygen saturation level plunged to 56, the type of number one would expect to see in a person who was near drowning. "They told me everything was fine, but they started going crazy," Braziel says. "They put me in a wheelchair and wouldn't let me take another step."

After a battery of more tests, the pulmonologist finally made the proper diagnosis. When he shared the news with Braziel in her hospital room, he gave no hint of the gravity of the situation. "He was so calm I thought primary pulmonary hypertension must be something like asthma," Braziel recalls. She got the real story when a friend from law school gathered a huge binder of information on the disease and brought it to her.

Several medications can relieve primary pulmonary hypertension, but no single approach works for every patient. Finding the right treatment usually requires persistent trial-and-error. Braziel's pulmonologist wanted to put her on Flolan (prostacyclin), a drug that is delivered to the heart through a catheter in the chest. Patients must carry a Walkman-sized pump with them at all times. The drug is generally very effective and many patients quickly get used to the pump and the catheter, but Braziel decided to get a second opinion.

After a two-month wait, an expert at Columbia University was finally able to take her case. As part of a series of tests, the pulmonologist gave Braziel calcium channel blockers -- a common type of oral blood pressure medicine -- while monitoring the pressure in her lungs with a computer. As soon as the drugs hit her system, the pressure dropped immediately. Braziel later learned that only 10 to 20 percent of people with primary pulmonary hypertension respond to calcium channel blockers. She would be one of the lucky ones to have a cheap, easy-to-take, relatively effective treatment for her disease. "I've been totally blessed," she says.

Braziel feels much better than she has in years. She used to be attached to an oxygen tank 24 hours a day; now she only needs extra oxygen when she has a cold or flu. She used to be practically confined to her bed; now she can go to the grocery store and do many chores around the house When she lands her job as a tax lawyer, she expects to be able to keep normal office hours, although she may need short afternoon naps.

As grateful as she feels, her life is far from easy. She takes 10 pills a day, including calcium channel blockers, diuretics (drugs that reduce the water retention caused by calcium channel blockers), and potassium supplements (to replace the nutrients flushed out by the diuretics.) The medications keep her disease in check, but they also make her tired and dizzy. The Veterans Administration pays for a large portion of her medical bills, but she still faces a lifetime of high healthcare costs. And unless treatments improve dramatically, she knows she'll never be as physically active as she'd like.

For Braziel -- and for many other women with her disease -- the biggest disappointment is that she can never have a child. Pregnancy would put too much strain on her already weakened heart, and she would almost certainly die before a baby could be delivered. "When they told me that, I literally started shaking," she says. She had always planned on having kids, but now she's envisioning a family of one. "I'll have to be enough," she says. If that's the way it works out, it will be a remarkable family: one Army veteran, one tax lawyer, and, most of all, one survivor.

-- Chris Woolston, MS, is a health and medical writer with a master's degree in biology. He is a contributing editor at Consumer Health Interactive and was a staff writer at Hippocrates, a magazine for physicians. His reporting on occupational health for CHI earned him an award from the Northern California chapter of the Society of Professional Journalists.

References

First published October 30, 2003
Last updated November 4, 2008
Copyright © 2003 Consumer Health Interactive

or find more on:

Back to top of page