By KATY BISHOP | Naples News
After 28 years of living with cystic fibrosis, Jennifer Likness’s lungs stopped working.
For three days, a machine pumped oxygenated blood through her body, keeping her alive. Late in the evening on the third day, when doctors say risk of complications start to shoot up, she finally received a set of donated lungs. Without them, she would have died.
“She’s a miracle,” says her mother, Heather Knight. “Dec. 2 she got her transplant. ... You ask any doctor or any nurse or ask me, she is a miracle. It’s amazing what she’s gone through. She took the hardest road ever to get her transplant.”
Knight speaks over the phone from the hospital in Gainesville, and her voice quivers with emotion. Then she repeats it again: “She’s a miracle.”
Jen can’t speak yet because she still has a tube in her throat to aid her breathing, but she’s communicating with her family by mouthing words so they can read her lips. When asked what she looks forward to most, after her recovery, she wrote in an email: “Just being able to do day to day things like everyone else.”
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Throughout her life, Jen has been in and out of hospitals. When she was younger, it was only a time or two a year for a week or so each time. In recent years, she’d have to go in about every two months. She’d go in feeling sick, but after days of therapies including antibiotics and physical therapy to loosen mucus in her lungs, she’d leave feeling some relief.
During her last stay at Lee Memorial Children’s Hospital in November, something had changed. Her lungs just didn’t respond to treatment. The Daily News published a story about Jen during this time, focusing on the fund she started at the hospital to raise money to rennovate the general pediatrics unit.
Cystic fibrosis is a genetic disease that affects the lungs and digestive system. It’s caused by a defective gene that causes your body to make a protein product, which then makes the body produce an unusually thick mucus.
About 30,000 children and adults in the United States have it, according to the Cystic Fibrosis Foundation. In the 1950s, most cystic fibrosis victims died before they reached elementary-school age, but today the predicted median age of survival is mid-30s.
At age 28, Jen’s disease had progressed so far that her doctors at Lee Memorial couldn’t help her anymore. She needed a lung transplant.
The doctors put her on a ventilator and transported her by jet to Shands Hospital in Gainesville, where she would be in the hospital until lungs became available.
When Jen arrived in Gainesville on Nov. 22 she was placed on a breathing machine, and for the first few days she was doing OK, says Dr. Maher Baz, her pulmonologist. But then her lungs began to struggle to expel carbon dioxide and the doctors had to put her on a heart-lung bypass machine.
That happened on Monday, Nov. 29. On Wednesday, donor lungs were available, but they were infected with pneumonia. Jen didn’t have much time, because after about three days on the heart-lung bypass complications start. Still, the doctors wanted a better pair of lungs.
But they had to find them fast, because Jen’s time was running out.
“Everything leading up to Jennifer’s transplant was the worst nightmare that does not happen to most people,” her mother says.
Jen was the 498th lung transplant at Shands, Dr. Baz says. But she was the first person in the center’s 16-year history who had to be placed on the heart-lung bypass machine before her transplant.
Late Wednesday night, lungs finally became available. Jen was in surgery overnight, making the official date of her transplant Thursday, Dec. 2.
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“Please remind everyone the importance of becoming an organ donor,” Jen wote in an email the weekend before Christmas. “I am so grateful to my donor family — without their generosity I wouldn’t be here today.”
Dr. Baz can’t reveal where Jen’s donor lungs came from, but they were a good match, he says. Lungs are harder to find than other organs, because there are more things that can happen to them. If they come from a donor who died in a car accident there can be trauma to the chest, or the donor could be a smoker, or the donor could inhale fluids into the lungs.
Only about 25 out of 100 organ donors can donate their lungs, in comparison to about 80 or 90 out of 100 for livers or kidneys, Dr. Baz says.
Sex doesn’t matter for lung transplants, and age doesn’t matter until you hit 60 or 65 years, he adds. Size is another consideration: The donor lungs must fit in the recipient’s chest. Smoking history is also important: 10 to 15 years smoking history is OK, but not much more.
The day after surgery Jen got out of bed, sat in a chair and ate and drank, Dr. Baz says. Two or three days later, she was up and walking short distances, and about a week after, she was off the breathing machine.
Now, Jen and her mom are staying in an apartment across the street from the hospital, and they go there every day. They’ll spend about a month in Gainesville to be near the hospital for follow up appointments and therapy. About four weeks after her surgery they started weaning her off oxygen — a huge step, since she’s been on it for about three years.
During her time in Gainesville, Jen will learn to monitor her health, so she can pick up any signs of infection or rejection of her new lungs.
“The first year is the window for infection, years two to four are the window for rejection,” Dr. Baz explains. “Those who come out of those windows with good lung function have a good chance at the next five years because those risks go down.” Nationally, survival rates are about 80 percent for the first year post-transplant, and 50 percent for five years, he says. Shands’ rates are a little higher, with survival rates from transplant to one year at 87 percent and from transplant to five years more than 50 percent.
Rejection is more common in lung transplants than other organ transplants, probably because the immune cells in the lungs are more active than organs like the liver or kidneys, he explains. Rejection happens when the immune system recognizes that the donor lung is not part of it and attacks it.
“You sometimes can quiet it down and sometimes you can’t,” he says. Triggers can be something as simple as a cold or acid reflux, or it can happen seemingly without cause.
But there are also people who received their lung transplants in the mid-90s at Shand’s who are still alive and healthy, Dr. Baz says.
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Since her transplant, Jen’s days are filled with rehabilitation, procedures and therapy. Her mother stays with her at the hospital in Gainesville. Her husband Danny stayed for a time, but had to go back home to Cape Coral to go back to work. It’s hard on them to be apart.
“It’s real interesting watching Jennifer,” Knight says. “She has spent her whole life, especially these last couple of years so struggling to breathe that it’s really hard for her brain, even unconsciously to be able to understand that she can breathe now.”
One day, shortly after her surgery, Jen looked down at her hands and got really excited, her mother recalls. She had noticed a tiny physical change. Her fingernails had always been blue — the sign of poorly oxygenated blood underneath.
Now they were pink.
To become an organ donor sign-up at Donate Life Florida