Publication Date: Friday, August 09, 2002
@pullquote:"This was life, I told myself, and you just have to make it work. I was trying to be normal but all I could do was work 8-12 hours a day, come home and sleep. I took naps at the traffic lights."
Toni Fourte, on dealing with primary pulmonary hypertension
Beating the odds
Beating the odds
(August 09, 2002)
By Diana Reynolds Roome
Alert face, bright smile, smart red outfit: This is not the way you expect someone to look when they are fighting a chronic, life-threatening disease.
"I have a bad time in disabled parking spots," chuckles Toni Fourte, making a mock-pained face. "And if I get in the handicapped line at an airport or ... oh my goodness! If a disability doesn't show, people say things. I'm not in a wheelchair, but I am much sicker than many who are. You can never tell what's going on in a person's life."
A lot has been going in Fourte's life since 1997, though when she moved to Mountain View with her husband two years before, her plans did not include being diagnosed with primary pulmonary hypertension (PPH) and told she had just over two years to live.
An accountant with a degree from Michigan State University, Fourte had been looking forward to increasing her qualifications and expanding her career in California. She was working 14 to 16 hours a day and thriving on it, playing racquetball and swimming on weekends, and working out at the gym every day. She was fit, ambitious and thoroughly absorbed in the demands and pleasures of a busy life.
So when she embarked on an MBA program at Santa Clara University in July 1997, she was surprised to notice shortness of breath and a mysterious blackness at the corner of her eyes upon reaching her third floor classroom. By August she was struggling to get to the second floor. By September, she could barely make the trip from her car to the building.
"I thought I was tired," recalls Fourte. "I was working full time, managing a company's accounts, studying for an MBA and doing everything fast, fast, fast."
It was only when she couldn't take ten steps without stopping and heaving that she finally had to admit something was seriously amiss. The urgent care doctors couldn't find anything wrong. But they were worried enough to call her next day and advise her to come in again. At that time there was a major national alert about the use of a weight-loss pill called Fenfluramine (Fen-Phen), which was known to be causing a form of secondary pulmonary hypertension. The doctors wondered if she had been taking it. As someone who kept her weight down by exercising rigorously, Fourte was a little insulted. But the doctors had hit on something.
PPH is notoriously difficult to diagnose but fortunately, Fourte's primary care doctor had recently given her a check-up including an EKG, and noticed a huge discrepancy between that baseline result and those showing up now.
Eventually, Fourte was told she had primary pulmonary hypertension, a rare (around 2 in a million) blood vessel disorder of the lung with no known cause and a dismal survival rate. At El Camino Hospital she underwent an operation to insert a catheter into her chest that could deliver regular doses of a complex medication to keep the blood vessels between heart and lung functioning. Without it, she would experience increasing shortness of breath, chest pain, dizziness and fainting. A pouch with the medication and pump, like a small tape recorder, was permanently attached to her side.
At 37 years old, she heard that her life expectancy was 2.8 years. She could not expect live to see the Millennium or her own 40th birthday.
Fourte shouldered the blow by returning to work as soon as possible.
"I was determined to incorporate this into what I was doing -- it seemed just one more thing to deal with. This was life, I told myself, and you just have to make it work. I was trying to be normal but all I could do was work 8-12 hours a day, come home and sleep. I took naps at the traffic lights."
Things were not made easier by the difficulty of getting ready for work each morning. Showering is a major challenge, as the catheter must not get wet because of danger of infection. Any change in the medication's temperature can make it ineffectual, and a person with PPH cannot survive more than 15 minutes without the medications.
Infection of any kind is extremely dangerous, and the house has to be kept immaculately clean and hygienic -- not easy for someone who is chronically exhausted. The meds have to be mixed, then chilled, a job her husband, Bill, learned to do during three days of training.
She also found, to her surprise, that she had to help others cope with the news of her illness. "You get to know how much you're loved," she says. But at the same time, she decided she would not tell people unless there was a good reason. Their reactions were too unpredictable and sometimes too emotional.
After ten months of struggling, someone at her support group said, "Your job has now changed to keeping yourself alive." It was a big revelation, says Fourte. As a result she quit her job. "With a chronic disease, you only have so much energy in a day. I finally realized that the person I was had gone away, that I was going to have to reinvent myself."
Gradually, she began to change "Why me?" to "Why not me?"
"This is a new ballgame, so I don't see it as life-limiting. I have to get with the program. If not I would go absolutely crazy and self-pitiful."
Her husband was scared, figuring she would be bored out of her mind. Typically, she threw herself into new challenges. She wanted to understand more about the disease, so she helped organize a conference on pulmonary hypertension in California (she and others with PH did not want to fly to the national convention because flying can be dangerous to people who already have potential breathing problems). She was profiled for the Pulmonary Hypertension Association brochure. She even invented a special PH version of Jeopardy, with dos and don'ts and categories of medicine, to help educate people about the disease. With time on her hands (probably for the first time ever), she turned to competitive needlepoint and started to win local and national contests.
"I went from business to artsy crafts and design. Oh, who's this?" she jokes, "I didn't even know she was in there!"
Not content with all this, she decided to go back and finish her MBA, encouraged by her cardiologist, Robert Naylor at El Camino Hospital. She started studies again in 2000 and graduated in 2001.
Now she has beaten the survival odds, her doctors don't talk about a prognosis in the same way. Treatment of PPH has come a long way in the five years since Fourte was diagnosed. Though her medications are cumbersome, she has learned to manage them and is grateful that the medication pump became available only a year before her diagnosis. Before that people usually died within two or three years.
A new drug is now available in pill form, which provides another option if she needs it, though she is reluctant to face the inevitable challenges a very different medication could bring.
The possibility of a lung transplant offers a longer-term solution if not a permanent cure. Though hopeful of her chance to receive one in a year or so (she's on the waiting list at Stanford Hospital), Fourte is also ambivalent about the outcome.
"My issue is quality of life, rather than quantity," she says. "After a transplant, the average person has to take 40-60 pills a day. It's another set of problems. As long as I can take care of myself and my needs, I'd say give those lungs to someone who needs them more."
She would love to go bicycling as she used to, to swim and travel (most of all to Egypt), but is grateful that Mountain View is a place where the climate and medical facilities offer her the best possible quality of life in the circumstances. She is reassured by the fact that the city emergency department came out to visit another woman with PH, to make sure they knew what to do in an emergency.
On a daily basis, Fourte deals with exhaustion, frustration, pain (much of which arises from side effects of the drugs) and the imminence of death. "People say, well anyone could be knocked down by a bus, but this is very different," says Fourte. Yet she is guided by faith and a kind of fatalism that has taught her to appreciate everything she has more than ever.
"My death is not my decision," she says. "I could catch a cold and die any day. I really used to think that my life and achievements had something to do with me, but that's wrong," she says with a wry laugh. "I've found out that our time here is not really of our own making."
For more information on Pulmonary Hypertension and to find local support groups, go to: www.phassociation.org