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Heather Emmert'slife is filled with typical teen-age things, and a slew of medicines to keep her boy from rejecting her double lung transplant. Sentinel Photo by Dan Coyro

By PEGGY TOWNSEND

SENTINEL STAFF WRITER

Until two months ago, 16-year-old Heather Emmert had about as much a chance of being a cheerleader as she had of sprouting wings and flying.

It wasn’t because Heather was a bad student (she got straight A’s) or because of the earrings she had in her eyebrow and belly button (plenty of teen-age girls have them).

Heather had cystic fibrosis and Heather was dying.

But next year, her senior year at Aptos High School, Heather will likely be out in front of the crowds, cheering for her teams in a crisp blue-and-white skirt and sweater.

That’s because a 15-year-old Stockton boy gave up his lungs when he died; and in a dramatic seven-hour operation, they were sewed into Heather’s chest.

The surgery means that Heather will have a shot at a few more years of life. That she will probably make it to her senior prom, to the cheerleading squad where she’s been promised a spot, and to college.

Sitting in a sparsely furnished apartment across from Lucile Packard Children’s Hospital in Stanford, where she is recovering from the double lung transplant, Heather says she’s already done one thing she’s wanted to do her whole life.

She laughed without coughing.

A killer

Cystic fibrosis is a genetic disease that affects 30,000 people in the United States.

It makes the body produce mucus as thick and sticky as rubber cement.

The mucus interferes with digestion and makes cystic fibrosis patients vulnerable to lung, bronchial and sinus infections.

Over time, scar tissue builds up in the lungs, making it hard to breathe.

Thirty years ago, the median survival age for cystic fibrosis patients was 15, says Dr. Preston Campbell, medical director for the Cystic Fibrosis Foundation.

That meant half of everyone who had cystic fibrosis was dead by that age.

But advances in drugs and treatment protocols have pushed the median survival age up to 32, he says, and advances are on the horizon.

Still when a patient’s lungs begin to fail, the best hope is major surgery, a double lung transplant.

In 2000, the latest year for which statistics are available, there were 65 pediatric lung transplants in the United States — about half of them for patients with cystic fibrosis, says Dr. Geoffrey Kerland, medical director of the lung transplant program at Children’s Hospital of Pittsburgh.

After the first year, about 80 percent of those will still be alive.

A salty kiss

Heather was 8 months old when she was diagnosed with cystic fibrosis.

"It was just a motherly thing," says her mom, Sue Emmert, of how she discovered her daughter’s illness. "I thought: There is just not something right with this child."

For one thing, little Heather was not gaining weight. She had bruises all over her body, and when Sue kissed her on the forehead, it tasted salty (a classic warning sign of cystic fibrosis).

Sue and her husband took Heather to Stanford, where she was eventually diagnosed with the disease.

"We didn’t know what that was," Sue says.

The doctors sent the Emmerts home with medicine for Heather and a new routine.

Four times a day, they would cup their hands like karate fighters and pound on Heather’s back to loosen the mucus that filled her airway and lungs.

Little Heather hated the pounding she called "pitty-pats." It left her back as sore as if she’d been in a fight.

She also hated the constant coughing.

"It’s like when you have your worst case of bronchitis," her mom Sue says. "That’s what she lived with her whole life.

"Her cough sounded like it was coming out of her toes."

Heather tried to live her life as normally as possible, but the disease meant that Heather spent a lot of time in the hospital.

There she became close with other cystic fibrosis kids, especially a boy named Adam.

Heather and Adam and a few other kids would turn the hospital into their hangout whenever they were there together.

They would roam the halls in between treatments, toss spaghetti to see if it stuck to the ceiling and rig the door to the hospital roof so they could sneak up there at night.

More than a few times, the hospital had to call security to go find them.

A year ago, Adam failed to make it onto the transplant list and died.

He was 17.

Supply and demand

The first double lung transplant was done in 1987.

In 2000 there were 134 of them.

The problem, says Campbell of the Cystic Fibrosis Foundation, is that there were some 600 patients put on the lung transplant list that same year.

That means, some people may die before they get their new lungs.

The reason is not just a shortage of donors, but medical technology as well.

A lung is only viable for about eight hours after it has been removed from a donor’s body.

If a donor dies too far away from a transplant hospital, the organ may be useless for transplantation.

‘Am I that sick?’

Heather was a freshman in high school when doctors told her they wanted to put her on the list for a double lung transplant.

Her hospitalizations had been getting more frequent and more severe. A year earlier, her weakened lung had collapsed as she put on a sweater.

Still it was a shock when doctors told Heather she needed to decide whether to go ahead with the transplant.

"I thought: Am I that sick?" she remembers.

"I was angry. I thought: This isn’t fair. I shouldn’t have to think about this at age 14."

Deciding whether or not to get a lung transplant is a balancing act.

Since less than half of young lung transplants will be alive after five years, according to statistics, patients don’t want to be put on the transplant list when they are still healthy enough to survive with cystic fibrosis for a few more years.

But if patients wait too long, they may be too ill to get a transplant.

So doctors look at a patient’s health and blood type, the length of time it may take to get an organ, and how fast the patient’s lungs appear to be failing.

"Talk about the art and science of medicine," says Dr. Richard Whyte, Heather’s transplant surgeon and head of the Division of Thoracic Surgery at Stanford.

"Well, this is the art of it."

"I kept thinking: What if I get the transplant too early?" Heather says, hanging her legs over the arms of a chair at the apartment across from the hospital and sipping from a Dr. Pepper.

She wears jeans and a T-shirt. Her fingernails are painted navy blue.

"I didn’t want to get it too early.

"But if I didn’t get a transplant, I would have just kept deteriorating until ... until."

Heather pauses.

"Until the end."

She decided to go ahead with the surgery.

On May 6, her mother got the call.

Beginning and end

The doctor said a 15-year-old boy had been killed in Stockton and his lungs were available for transplant.

"Oh my god, oh my god," Heather remembers saying when her mother told her the news.

Then she began to cry.

"I thought: This is major surgery, and there’s a very good chance I could die," says Heather.

"I thought, this could be the end ... or this could be the beginning."

While a transplant team headed to Stockton to see if the donor’s lungs were suitable for transplanting into Heather’s body, Heather, her mother and a friend started over the Santa Cruz Mountains to Lucile Packard Children’s Hospital.

By 9 that night, they got the word the lungs were headed to the hospital and the operation would begin at 9:30.

When it was time, Heather lay back on the gurney and nurses wheeled her toward the operating room.

"I told her that I was very proud of her, that this was a new beginning," Sue says, remembering how she watched the nurses wheel her daughter through two big gray doors that opened with a whoosh.

How she watched the doors slowly close and block Heather from view.

"I just collapsed and started crying then," Sue says.

"I just kind of lost it."

A matter of timing

Doing a double lung transplant on a cystic fibrosis patient requires as much precision as defusing a bomb.

On one hand, it usually takes a lot of time to remove a lung from someone with cystic fibrosis, says Whyte, Heather’s transplant surgeon.

"Things are quite inflamed. There are a lot of adhesions between the lung and the chest wall."

On the other hand, a surgeon only has a five- to seven-hour window of time after the lungs are removed from the donor before they can’t be used any more.

"So you have a two- to three-hour travel time and two lungs to do, and it starts to add up, and your eight hours could get eaten up pretty quickly," Whyte says.

Some surgeons start removing a lung before the new organ arrives.

Not Whyte.

"The risk of something happening (like an auto accident) are low, but things could happen," he says.

Whyte waits until the new organs are in the room before he takes out the first lung.

When Heather’s new lungs arrived, Whyte removed Heather’s right lung and sewed in the one that had belonged to the Stockton boy.

Then he did the same on the left.

The operation was done while Heather was hooked up to a heart-lung machine to keep her alive.

When they shut off the machine, Heather had been on the table for seven hours.

Even though no one from the hospital will tell Heather who her donor was, a relative used the internet to find out the donor’s name and what happened to him.

"Some people saw a picture of him and said he looks just like me," says Heather, as an afternoon cartoon show plays unwatched on the television.

"It was very weird.

"It was like he was in the wrong place at the wrong time, but it was the right time and place for me."

A balance beam

"The biggest problems, aside from the early issues, are organ rejection and infection," says Whyte, the surgeon of transplant patients.

Doctors have to suppress the transplant patient’s immune system so the body does not reject the organ.

But they can’t suppress the immune system so much that infection sets in and kills the patient either.

"I put it to them that they walk a balance beam with infection on one side and rejection on the other," Whyte says.

Heather will have to take medication for the rest of her life.

While lung transplants prolong life for cystic fibrosis patients, the future is still uncertain.

Survival rates are below 50 percent after five years, in part because a chronic kind of organ rejection continues despite the immune suppression drugs.

Whyte said advances are being made in organ rejection and preserving organs for longer than eight hours so more transplants can be done.

But when a lung transplant fails, there is very little for a patient to fall back on. Only six percent of lung transplants are done on people whose first transplant failed.

The best results require a patient to take their medications daily and follow the protocols.

"This is not for the faint of heart," says Kerland of Children’s Hospital of Pittsburgh. "This is for people who don’t mind hard work."

What she wants

Heather doesn’t mind the work.

She doesn’t mind swallowing pills and getting tests.

Sitting in the Stanford apartment on a warm July day, Heather says she’s looking forward to graduation, to the senior trip to Disneyland, to going to college back east — and to something she thought she might not live long enough to do.

For the past few years, Heather has wanted to get a tattoo of a rose, the symbol for cystic fibrosis.

But she had to be 18 to get one.

The way doctors were talking before the transplant, she thought she might not make it.

"I wanted it to be a rose," Heather says, "with the initials of my friends who have passed away written in the vines."

A way to remember those who didn’t get the second chance Heather got.

Now, she says, she just might grow old enough to get one.

For information about cystic fibrosis, visit www.cff.org.

Contact Peggy Townsend atptownsend@santa-cruz.com.

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