Fighting for life: 14-year-old Palmer girl is fighting cystic fibrosis while awaiting a living lung transplant

Meghan Gonski appears to be a normal 14-year-old girl. She loves horses, art, and, of course, chatting online. She excitedly shows her sketch book and painted ceramics, and is proud of all the horse posters tacked up in her room.

On her computer is a collection of photographs she has taken as the official team photographer for her sister's comp soccer team. They're good -- really good, actually -- much better photographs than a 14-year-old normally takes.

And then Meghan coughs. And coughs again. And coughs again. And you realize Meghan is much more courageous than the average 14-year-old, because Meghan is literally fighting for every breath she takes.

Meghan is only 14, but for the last 11 years she has been grasping for every breath, as cystic fibrosis fills her lungs with mucus.

Some of the harsh medications she's taken have obliterated her hearing, forcing her to learn American Sign Language. She's up to the challenges, though. In fact, she's now teaching American Sign Language to others.

Through everything, Meghan remains incredibly upbeat and cheerful. She has a smile on her face and a twinkle in her eye. You'd never guess what she's been through.

Meghan's best hope is a living lung transplant -- one in which two donors give up lobes of their lungs, which are then put together and transplanted into Meghan's body. The Gonskis already have one donor ready. They need another before it's too late to matter.

A sick 3-year-old

When she was just a toddler, Meghan continually got sick. At first, her parents, Jim and Pat Gonski, thought she was just like any other toddler trying to fight off colds.

After a cycle of antibiotics, Meghan would be fine for a couple of weeks, but then she'd get sick again. Finally, the Gonskis took her to an Anchorage doctor, and almost immediately, cystic fibrosis was suspected.

"We always noticed that when we kissed Meghan's forehead, she always tasted salty," Jim Gonski said. "That was a big sign of cystic fibrosis. They did a sweat test on Meghan, and while we were waiting for the results, I started reading up on cystic fibrosis, and Meghan certainly had a lot of the symptoms."

The diagnosis came back, and it wasn't good. Cystic fibrosis, a genetic disease that ultimately causes mucus to build in the lungs to the point of suffocation, was a battle Meghan would face for the rest of her life.

Fighting for breath

Meghan has spent a good portion of her life tucked away in hospitals, shuttling to and from doctor's appointments and, most importantly, fighting to breathe. While other kids her age played soccer and enjoyed slumber parties, Meghan traveled to places like Denver and St. Louis, trying to find a treatment option that would ease her struggle to breathe.

"Our first hospital stay was when she was three," Pat Gonski said. "It was pretty rough."

During her hospital visits, doctors would pump Meghan full of antibiotics, trying to rid her lungs of mucus. Following the treatments, she acts like a kid again. If there's one thing doctors have learned, it's that you can't keep Meghan down for long.

"She wasn't sick and had lots of energy. She would be riding around the hospital on a tricycle," Jim Gonski said with a smile. "It was great to see her so alive, but you are there in the hospital, and it's hard to keep a toddler from running around all the time.

Initially, the family didn't spend all its time in the hospital. But through the years, the stays have become more and more frequent.

"We went 18 months between hospital stays at first, then down to 12 months, six months, three months," Pat Gonski said. "Second grade and sixth grade were really tough years for Meghan. She was in the hospital all the time."

Following her sixth-grade year, Meghan and her family went to Denver, where she had one of the five lobes of her lung removed, because, essentially, it was compacted with mucus.

Any resemblance of a normal teen-age life is gone for Meghan. She gets to school when her health allows, and it is a big challenge since she lost all of her hearing.

"Meghan doesn't have a normal childhood life," Jim Gonski said.

The Gonskis have two other children -- Brandon is entering his first year of college and Katie is 12. Family time is hard to come by, the Gonskis said.

"It's hard to give all the kids equal time. A lot of the time with Meghan is medical time," Pat Gonski said. "It's hard. Real hard."

Meghan has turned to art as a release, and she always has been fascinated by horses.

In mid-August, the Gonski family went to California to spend a week at a horse ranch as part of the Wish Upon the North Star wish-granting program. Meghan also loves talking on MSN. Following the transplant, she is looking forward to being able to play the flute again, as music is another of her passions.

"I really like the computer," she said. "I like talking on Messenger with other people."

Meghan's doctor, Dr. Dion Roberts of Anchorage, recently went to Ireland for a conference on cystic fibrosis. He brought Meghan two posters.

"He brought me a big poster, and then a framed one from Ireland," Meghan said proudly. "They are very nice."

Throughout the years, Meghan's condition has constantly been monitored. Her lung capacity slowly diminished at first. Lately, however, it has gone quickly.

"The pulmonary function test measures the volume of your lungs. One of the 'magic numbers' is 60, when you qualify for the transplant list. Meghan was going down slowly, but then last year she did a nosedive. She started falling fast -- 60, 40, 30, then 20 percent," Jim Gonski said. "Last November she was at 28 percent when we were in St. Louis Children's Hospital. Now she's down to around 19 percent."

Meghan is on the transplant list, but the list is far too long, and Meghan's deteriorating condition probably won't allow her to get to the top before cystic fibrosis claims her life. So, instead of turning to a transplant from a cadaver, living donors are going to be needed to save Meghan's life.

Living lung transplant

The next step for the Gonskis is a living lung transplant. Every person's lungs have five lobes -- three on the right and two on the left. Basically, a lower left lobe from one donor is taken, and a lower right lobe from another donor is taken. The two lobes are then combined and then transplanted into the recipient. The process is called a living lobar transplant.

"Actually, it is easy medically, but psychologically and mentally, it is incredibly hard," said Ann Doyle, the living lobar transplant coordinator at Washington University St. Louis Children's Hospital. "When it works, it is an incredible life-affirming thing."

At St. Louis Children's Hospital, the first living lobar transplant was performed in 1994, and that recipient is still alive and now attending college.

Since then, 41 transplants have been performed, and 16 of the patients are still alive. Since 2000, St. Louis Children's Hospital has performed nine living lobar transplants, and seven recipients are still alive. Doyle stressed that transplants from cadavers are still the best alternative, but when that option isn't available, living lobar transplants are looked at.

"We don't use it as our first line of surgery. We use it as, sort of, a rescue you could say," Doyle said. "When we are out of options, we use living lobar transplants as a rescue."

There are several facilities, many of them universities, performing living lobar transplants, but St. Louis Children's Hospital leads the way in pediatric cases. That is where the Gonskis are hoping Meghan will receive her transplant in the near future. Now all they need is a donor.

Donor needed

The Gonskis already have one donor lined up for a living lobar transplant. A second donor is needed, and once a second person is available, the transplant can take place. If a donor were able to get tested and approved this month, the transplant could happen as early as December or January.

According to Doyle, there are a few criteria that must be met by potential donors. First, a donor must be at least 5 feet, 5 inches tall, because Meghan is 5 feet, 1 inch tall. The bigger-sized person has a bigger lobe, giving Meghan a better chance at survival. Secondly, the person must be between the ages of 18 and 50. Thirdly, the person must not have smoked.

"Basically, I'm looking for a healthy person and basic demographics," Doyle said. "Some people think this is rocket science. It's not."

Every potential donor remains anonymous to the Gonskis, because of the intense psychological nature of the transplant.

"Our philosophy is that the closer you are to the family, the harder it is to walk away from the transplant. We push that a donor shouldn't be afraid to back out if they don't want to go through with it," Doyle said.

If someone is interested in donating one of their lobes, that person must first contact Doyle. After an interview, potential donors then must go to St. Louis for three days of testing. If a donor is still identified as being a successful candidate, the transplant process begins. Donors must go to St. Louis for three days prior to the surgery, and then following the procedure, they are in-patients for approximately two weeks.

"One of the biggest things is people getting time off work or being able to invest that much time in being away from home," Jim Gonski said.

All travel, hotel and food costs are covered by the Gonskis, and the procedure itself is covered by insurance.

"We have to be more cautious than casual because there is a big investment," Doyle said.

Following the procedure, there is about a six- to nine-month recovery time for the donors. Doyle said recovery time depends on the person.

"If a person has a sedentary lifestyle and employment, they can go back to work and they won't notice much of a change. They may be out of breath after a flight of stairs instead of two flights, for example," Doyle said. "The big thing people notice is a lack of endurance. You are going to notice that for a while.

"The general public is not going to notice a big difference. With a little bit of exercise and some lifestyle changes, they'll be totally back to normal in six to nine months," she said.

That doesn't mean the procedure is totally without complications.

"One thing donors comment on is a persistent nonproductive cough," Doyle said. "There have been some cases where there's been some permanent nerve damage, some numbness, in the chest area. We are extremely cautious about doing living lobar transplants because we are taking two people into surgery who don't have any medical indicators that they need surgery. We are putting them at risk when they don't need to be put at risk."

Unlike other organs, it isn't imperative that a donor be related to Meghan for their lung to work.

"We've found that there is no significant difference in the rate of rejection for family or nonfamily donors with lungs," Doyle said. "We believe that just because there aren't any family donors available, a child should not be deprived of a transplant. We believe that every child deserves a transplant if donors can be identified."

The family waits

"We haven't had a hospital stay since Meghan has lost her hearing, but it is going to be hard," Pat Gonski said. "The transplant will be extremely difficult to explain through sign language."

The Gonskis are hoping a donor can be identified soon. Meghan's condition has deteriorated quickly, and having the transplant now rather than later will give her the best chance at survival.

"Our position is that Meghan doesn't have a great standard of life right now," Jim Gonski said. "She is on oxygen at night. She needs injections every eight hours. In terms of the transplant, she is as stable now as she is going to be."

The future could be very, very bright for Meghan Gonski. Following the transplant, she is looking forward to a number of things, most notably getting to play her flute again, going back to school and riding horses.

"She is very upbeat and looking forward to getting to do those activities," Jim Gonski said. "She's an upbeat young girl."