Sunday, April 27, 2008

Double lung transplant gives teen hope after a lifetime of struggle

Samantha Moschetta, right, shares a joke with physician assistant Joanne Snyder at Children's Hospital in Pittsburgh. Photo: Bob Donaldson/Post-Gazette

From the Pittsburgh Post-Gazette:

On Good Friday Samantha Moschetta received a double lung transplant.

And days later, for the first time in years, she was able to walk far enough from Children's Hospital in Pittsburgh to buy Italian ice and chai tea.

The 17-year-old Penn Hills girl returned home on a recent Friday as an example of transplant success.

But that success tops off a lifelong struggle with cystic fibrosis that required daily determination to survive the potentially deadly disease. She had to undergo daily life-sustaining treatments from her parents, David and Betsy Moschetta, and significant expenditures of time and energy just to live a burdensome life.

Samantha's future remains uncertain due to the difficulties inherent with lung transplants, coupled with continuing problems that cystic fibrosis imposes on digestion.

But hers is a story of how persistence and determination -- and her absolute refusal to give up -- paid off.

Today Samantha literally breathes more freely than she has for most of her 17 years. It provides her incentive to resume a relatively normal teenage life, including possibly returning to high school and getting a driver's license.

Which is to say that Samantha Moschetta is back in action.

Five months after she was born, Samantha was diagnosed with cystic fibrosis -- an inherited chronic disease that afflicts the lungs and digestive system of about 30,000 children and adults in the United States and 70,000 people worldwide.

A defective gene and its protein product cause the body to produce unusually thick, sticky mucus that clogs the lungs and leads to life-threatening lung infections. It also obstructs the pancreas and stops natural enzymes from helping the body break down and absorb food, the Cystic Fibrosis Foundation states.

In the 1950s, few children with CF lived long enough to attend elementary school. But advances in research and treatments now allow many to live into their 30s and 40s, if not longer.

But Samantha's prognosis was not rosy. She underwent four breathing treatments each day for most of her life to dislodge mucus buildup in her lungs and make it a bit easier to breathe. She's been on a regimen of strong antibiotics and other medications to prevent lung infections promoted by the mucus.

Then she developed diabetes due to mucus in the pancreas that blocked insulin secretion. Lack of insulin, the hormone needed for blood glucose to enter cells, causes blood glucose to build up in the blood to dangerous and even deadly levels.

It added yet another difficult high-maintenance disease to the challenge of survival. She now uses an insulin pump and continues taking pancreatic enzymes to help her digest food properly.

Her problems grew more severe each year. Two years ago she became wheelchair-bound most of the time because she could not breathe well enough to walk. She's been on oxygen at nights since age 13 but had to go on oxygen full time four months ago.

Her father would awake at 4:30 a.m. each morning to pound on her back and sides for an hour and a half to loosen mucus in her lungs, along with three shorter but still intense treatments throughout the day.

She attended Penn Hills schools until eighth grade, then enrolled in a cyber school. "I'm used to it," she said of her limitations. "It doesn't bother me."

Since age 12, she's been on the transplant list. In the case of CF, one can be too sick for a transplant, and such was the case with Samantha, who at 4 feet ,7 inches tall, weighed a mere 58 pounds. Doctors told her she had to gain weight to qualify for a transplant.

Samantha, unconvinced she wanted a transplant, received encouragement from her parents to go through with it. Without the transplant, Mr. Moschetta was sure, his daughter would not survive 2008.

"My appetite was decreasing because I was not feeling well," Samantha said, noting that she burned many calories struggling to breathe.

To gain weight, she had to have nutrition and lipids or fats pumped directly into her stomach and legs.

Her doctors also gave her incentives. Dr. Timothy Murphy, a Children's Hospital pediatric pulmonologist, told her he'd pay her $10 for each pound she gained. He in turn could reduce that total by $10 for each pound he lost.

The contest was on.

Samantha gained 15 pounds. But to his dismay, Dr. Murphy gained three. So he anted up $180. In the end, she gained sufficient weight to reach about 80 pounds, which qualified her for the transplant.

"I just wanted a normal life again, so I decided to do it," Samantha said.

The wait was on for lungs to become available. The call from Dr. Geoffrey Kurland, Children's Hospital medical director of lung transplantation, came March 20: "We got lungs," he told the Moschettas. "Come on in."

A panicky Samantha threw up. But at 3:30 a.m., the surgery began and was completed by 10 a.m. Once off the ventilator, Samantha finally could breathe. Her new lungs will never be affected by CF.

"It was a shock," she said. "It was different. I could take a deep breath and not cough. I was glad."

She must undergo physical therapy three times a week to build up leg strength and overcome two years of virtual inactivity.

Her parents look forward for the first time in many years of not having to awaken before dawn to give her percussive therapy to help her breathe.

"I've been telling everyone that I'm ecstatic and excited for Samantha, for her health and her future," said Mr. Moschetta, who operates J&M Printing in Penn Hills. "Her future is brighter now.

"I'm looking forward to her doing what typical kids do -- go shopping and go to the movies -- and doing it without her parents attached to her hip," he said. "It's also wonderful for selfish reasons."

Yes, he can sleep in longer.

Dr. Kurland said a lung transplant was a last resort, and in Samantha's case, a necessary risk. Such transplants typically don't last as long as other transplants, so she faces future challenges.

But her prognosis looks strong.

"Her quality of life was pretty abysmal," he said. "She couldn't walk or go to school. No one wants to undergo this, and you have to think carefully beforehand to do it, and she thought about it."

"It was a good feeling to see her walk out of the hospital and not be on oxygen," said Dr. Peter D. Wearden, the cardiothoracic surgeon at Children's who helped perform the transplant.

Samantha's once pale cheeks now are pink. Her fingertips, once blue from lack of oxygen, now have lively color. She has her old spirit and determination, but now with new lungs to power them.

Samantha is back in action.

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