True grit: The story of Cailyn Fowler (Part 2 of 3)

Living with CF

While the effects of ivacaftor are fast, they're not instantaneous, so Cailyn goes about her normal routine.

It is a Wednesday, so she goes to dance class. The doctors have told her to find a physical activity that she enjoys, and for Cailyn, dancing is it. She takes five different types of classes (including clogging and lyrical dance), and today, she spends five hours there, fielding more excited questions from her peers and instructors.

Here at class, she becomes once again indistinguishable from her peers, just another happy teen learning the latest dance technique. But when Cailyn goes home, her life becomes very different.

Understanding how cystic fibrosis attacks the body gives insight into how difficult it can be for Cailyn to try to have a normal life.

In many cells throughout the body, there are tiny proteins called CFTR, which stands for Cystic Fibrosis Transmembrane cell conductance Regulator.

The proteins are gatekeepers in each cell. They allow water and salt to pass through the cell walls.

If the surface of, say, a lung cell is too dry, the CFTR will allow water to pass through the cell walls to the surface. This creates a healthy, thin flow of mucus to wash away bacteria and obstructions.

But in people who suffer from CF, there is a genetic mutation that stops the gatekeeper from passing water and salt through the cells. As a result, the mucus that flows along the inside of a person's lungs becomes thick and sticky. Instead of flushing bacteria out, it traps it and allows it to grow.

The thick, sticky mucus that characterizes CF wreaks havoc on a person's body.

It gums up a variety of vital processes. There can be chronic infection and pain in the sinuses; the liver and reproductive organs can be damaged. The pancreas is affected in a way that makes it difficult to digest food and absorb nutrients. The intestines become blocked, and there can be abnormally salty sweat.

But worst of all is the lungs.

"They get thick, sticky secretions in their lungs, and that damages the lungs," says Corrigan. The mucus makes it difficult for Cailyn to draw breath sometimes. Corrigan says that, when someone dies from CF, it's usually because of respiratory failure.

Cailyn has experienced all of these symptoms to some extent. She experiences frequent and severe stomach pain ("My stomach's been bad lately," she says soberly). She has a terrible time maintaining her 83 pounds, let alone gaining enough weight for the doctors to consider her healthy.

Then there is the coughing, often subtle, but always there. At times, she coughs up blood, or vomits.

"Sometimes it pops the blood vessels in my lungs," says Cailyn.

The coughing goes on constantly, all day long, and even in Cailyn's sleep.

"The coughing wakes me up," says Cindy. "A lot of time, she sleeps right through it."

"I haven't been [sleeping through it]," says Cailyn.

"No," agrees Cindy. "Lately you haven't been."

And then there is a constant parade of other symptoms that can come and go. With mucus-coated lungs, she is more susceptible to colds and other forms of infection.

"Recently, she had to take the ambulance from the culinary arts class because her vitamin K levels were so low her blood wasn't clotting," says Cindy. "It's always something."

As bad as this staggering collection of ailments is, Cailyn's treatments are as frustrating for her as the symptoms.

To begin with, Cailyn has to go to the hospital regularly. Despite all of her efforts to treat her CF, the disease overwhelms her body's defenses.

"She has to go into the hospital for what they call a CF cleanout," says Cindy. "When she gets out, she's pretty much back to new, until ... in three or four months, she's back at square one."

Every time this happens, Cailyn spends a couple of weeks at a time in the CF Clinic in Portland.

Corrigan estimates that Cailyn spends about a month out of every year of her life being treated in the hospital.

In all, that means that the 17-year-old has been in the clinic for nearly a year and a half of her life.

"It's prison," says Cailyn. She says that she would rather do anything than just sit in her patient room all day, every day.

"As with any kid that's a frequent flier in a place like a hospital, she's comfortable there," says Corrigan. "However, you're removed from your regular life as a high school girl. It's hard to be a teenager and have a chronic illness. She's a teenager."

Cindy says that, because of the risk of infections, Cailynn is essentially confined to her room at the hospital.

"When she goes out of her room, just for like exercise for physical therapy, she can go around the unit 16 times without touching anything, and then she has to go back to her room. Sixteen times, that's a mile," says Cindy.

Even when she's at home, life is not a picnic for people with CF. First, there are the 37 pills she has to take every day, for various reasons.

Then there's the feeding tube. Cailyn's flat stomach has a small plastic tube sticking out, just several inches to the left of her bellybutton.

It's unobtrusive, and serves a vital purpose, but she hates it all the same.

Every night, the tube is connected to a bag that feeds five cans of a thick, chalky, calorie-laden formula into her body.

"It's real thick, like a sludge," says Cindy.

They give her the equivalent of 3,000 calories a day, enough to keep an obese person four times her weight rolling in fat, but the CF ensures that her starving body will absorb only a fraction of the nutrients.

This is the first thing Cailyn mentions when she dares to talk about life on ivacaftor, a life that is not dictated by CF.

"Then I can just get my tube out. I want it out so bad."

"She still won't be able to take the tube out for a year," says Cindy, providing a gentle reminder to a girl for whom a year must seem like a lifetime. "If and when she gains weight, she'll have to maintain that weight for a year."

That doesn't stop Cailyn from dwelling on the details. Her hopes, she has admitted, are up.

"Then they drop cans off," she says. "Then I'd take four, then I'd take three cans a night. Then I'd take two."

She sounds like a younger child describing Christmas.

"It's going to be at least a year," repeats Cindy.

Aside from being joined to five cans of the loathsome formula every night, Cailyn doesn't like the tube itself. It hurts, it limits what she can do physically, and it provides a constant physical reminder that there is a difference between her and the other people around her.

When other girls were hosting weekend-long slumber parties, it was difficult for Cailynn to participate.

"It's annoying," she says. "Sometimes at dance, I can't do some of the stuff that they do. It makes it bleed."

Then there is the Nebulizer, a machine that Cailyn uses to inhale medicinal vapors designed to break up the mucus in her lungs.

While she's inhaling air from the Nebulizer, she wears a vibrating vest, which looks like a clunky blue flotation jacket with air tubes that run to a unit the size and shape of a microwave.

When she's feeling well, Cailyn has to wear the vest for 40 minutes every day. It shakes her chest cavity, which helps to break up the mucus.

That's on a good day.

On a bad day, she undergoes three 40-minute sessions with the vest.

Between that, and physical therapy, it can be difficult for Cailynn to find any time for living life.

"She has a good hour and a half of treatments that she's doing at home every day," says Corrigan.

When she's undergoing treatments or tube-based feedings, Cailynn likes to read and watch tv to help pass the time.

She likes books and television shows about people with extraordinary powers. Werewolves and vampires, teenagers with wings or telekinetic powers – these are the figures that populate her imagination, while she herself sits in her room, sucking air from her Nebulizer and tucked into her vibrating vest, her 83 pounds chained to a nutrient bag by her feeding tube.

Click here for Part 3