The Willow Glen Resident

Group aids victims of illness linked to diet drugs

By Annette Ha

Marcie Long smiles sheepishly, then describes, with a hint of excitement in her voice, strolling through Vallco Fashion Park. "I must have walked about a mile that day," she figures.

Not too long ago, the 40-year-old Willow Glen resident couldn't walk 200 feet without gasping for breath. The culprit was pulmonary hypertension, or PH, a progressive fatal lung disorder that has received considerable publicity in the past six months because of its connection to two highly popular diet drugs.

Dexfenfluramine, which is sold as Redux, and fenfluramine, one-half of the popular diet drug duo known as fen-phen, were recently pulled off the market after the FDA announced that they may trigger heart-valve problems, as well as PH.

In cases like Long's, when the disease occurs without a known cause, it is referred to as primary pulmonary hypertension, or PPH.

Shirley Craig, spokesperson for the nonprofit Pulmonary Hypertension Association, emphasized how uncommon the disease is and estimated that Northern California has about 40 cases at the most. "This is an extremely rare disorder," she said.

Two years ago, Long and her husband started a support group in their Willow Glen home where she and other PPH sufferers could share their feelings and help one another deal with the disorder. She was able to locate two others living in San Jose, and now the average meeting has swelled to between 20 and 25, depending on attendance. Some come from as far away as Fairfield for the monthly meetings, because it is the only PPH support group in Northern California.

"They've become almost like a second family," said Almaden resident Betty Harris, whose daughter died earlier this year after a six-year battle with PPH. "Having a support group made a great deal of difference [in our lives]."

With the new diet drugs, Long expects her group will continue to grow.

The PHA added 600 new members this year alone, bringing the nationwide total to 1,100. Craig said the increase is likely due to those who may have contracted the disease after using the diet drugs.

PPH acts on the blood vessels in the lungs, restricting or closing them until the heart has to work twice as hard to pump blood through the remaining vessels, a condition that eventually becomes life-threatening. The progression of the disease may be slowed by a variety of expensive drugs that fight to keep the vessels open, but a heart/lung or lung transplant is the only option left once these therapies are no longer effective.

Long, who owes her second wind to a new set of lungs transplanted just four months ago, hopes that the media spotlight on the diet drugs will lead to a greater public awareness of PPH and to her Willow Glen support group. Group members are sending out press releases, educational fliers and a newsletter in preparation for National Pulmonary Hypertension Awareness/Support Group Week, Nov. 1 through 8. The goal is to educate local communities about the disorder and to tell sufferers where to get help.

When Long was diagnosed back in 1991, there was no group in the Bay Area to turn to.

"I searched for information everywhere--medical libraries, universities. I made endless phone calls," she said. "I'm sure I made a nuisance of myself." Because PPH was so uncommon, it was known only within the medical community, and there was little public information available.

Fellow support-group member Harris said she could find very little information about the disease when her daughter, Lori, was diagnosed in 1991. They were also unable to locate other people in Northern California who had it. Discovering Long and being a part of the Willow Glen support group was a turning point in Lori's life, said her mother. "It was the best thing that could have happened. She knew she wasn't alone with the disease."

Her daughter's death in February showed Harris just how important the group had become to her as well. "I got flowers and lots of phone calls. Everyone [in the group] was very supportive." Harris, a retired Wells Fargo sales manager, continues to be active in the group. "I've formed very close attachments with others," she said.

Members pool information on the latest medical breakthroughs, the different side effects of the many treatments, the correct dosage of drugs to take and how to cope with the day-to-day reality of PPH.

Both Harris and Long hope the diet-drug controversy will have the positive side effect of increasing public awareness of PPH's symptoms.

Shortness of breath, fatigue, dizzy spells, fainting, chest pain and fluid retention are all early warning signs of PPH, which tends to strike women, especially after pregnancy, at a much higher rate than men.

According to Craig, a common misconception among both medical professionals and the patients themselves is that those who display many of the above symptoms are simply imagining things.

"[People with PPH] complain and complain, and they're sent off with anti-depressants," said Craig. "They're very often misdiagnosed."

Others may assume they're out of shape.

"Lori thought she just needed to lose weight," said Harris. Long recalls a similar feeling. "I just kept hitting the treadmill because my doctor told me to get more exercise."

Considering that the average life expectancy after diagnosis is only three to five years, with an additional five years after transplant, the Willow Glen group is eager to get the word out.

"The more people are aware of PPH's symptoms," said Long, "the earlier they can get on the drugs, and the longer they might have to live."

For more information about primary pulmonary hypertension, call PHA at 713/688-7543

[ Back to Contents Page | Willow Glen Resident Home Page | Archives ]