Cystic fibrosis, lung transplant — she was never supposed to be - CBS News 8 - San Diego, CA News Station - KFMB Channel 8

Cystic fibrosis, lung transplant — she was never supposed to be a ‘mama.’ But she is.

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Vivian Gustin was a whirlwind in a floral tunic.

The 10-month-old had a wide, bright smile, often paired with a high-pitched cackle that revealed all nine of her recently cut teeth. Her mother, Andrea Heichelbech, gazed starry-eyed at the infant, blowing raspberries on her belly and pretending to gobble up her fingers.

"I love being a mama," she said.

Because she has cystic fibrosis, Heichelbech, 31, was told by doctors that she would not be able to become pregnant. She was diagnosed with the genetic disease as a child, when a nurse at Riley Hospital for Children kissed her on the forehead and noticed her skin tasted excessively salty — a sign her body wasn't absorbing the nutrient.

CF causes excess mucus to form on and in a person's lungs, pancreas and other organs. Some women have cervical mucus so thick, sperm can't travel through it. Coupled with dietary issues — many with CF struggle to absorb vital nutrients — conceiving can be difficult.

As she grew older, Heichelbech began having lung infections, which led to prolonged hospital stays. By the time she was 26, her lung capacity had dropped to nearly 20 percent.

She was on the transplant wait list for 11 months, and went through three "devastating" dry runs — prepping for surgeries that fell through for different reasons — before receiving a new set of lungs on Dec. 13, 2014. After nearly 24 hours under sedation at IU Health Methodist Hospital, Heichelbech remembered the relief of taking that first, albeit shallow, breath.

By this time, Heichelbech had long believed she couldn't have a baby. After her lung transplant, doctors urged her that she shouldn't. A pregnancy could cause her body to attack, or reject, the new lungs.

So Heichelbech was in shock when, in 2016, she found out she was pregnant. She and Vivian's father, 35-year-old James Gustin, had never worried about contraception.

Heichelbech's left vocal cord had been paralyzed during her lung transplant surgery, and she was scheduled to get an implant in November 2016. Before the surgery, she underwent pre-anesthesia testing. A nurse came to talk to her about the results.

"She asked me if I was pregnant and I said no," Heichelbech said. "She was like, 'Are you sure?' I was like, 'Yeah, I'm sure.' ... She was like, 'Well, you're pregnant.'"

A complicated choice

Pregnancy after a lung transplant is rare, but it's not unheard of.

The Transplant Pregnancy Registry International began collecting self-reported cases of post-transplant pregnancies in 1991. Between then and 2016, the last year data was available, 41 cases of pregnancy after a lung transplant had been reported.

These pregnancies are considered "extremely high-risk," according to TPR. They often result in high incidences of premature births, low birth weights and miscarriages. Thirty percent of those 41 pregnancies ended in miscarriage, according to TPR. It also comes at a significant risk to the mother's life.

Dr. David Roe, IU Health lung transplant pulmonologist, and his colleagues did extensive research before discussing options with Heichelbech.

Her baby could be born with cystic fibrosis, which affects more than 30,000 Americans.

The medicines she took could affect the fetus' health. She and her doctors discussed abortion.

Or she could carry the baby. But it was risky. Cases like Heichelbech's are so rare, Roe could only say there was a "significant" chance that her body might reject the lungs. In the IU Health lung transplant program's 30-year history, there's never been another case like Heichelbech's, Roe said.

But Heichelbech, who was about six weeks along, had already made up her mind. Genetic testing had determined the fetus wouldn't have CF, and Heichelbech's heart was set on keeping the baby.

"Once Andrea discovered that she was pregnant and she decided to keep the baby, then we are in 100 percent support of that, and our goal is to make sure that she and the baby survive," Roe said. "Period."

More than a dozen IU Health providers created a plan for Heichelbech's treatment during pregnancy. Among the first steps: Adjusting the doses of some of her 15 to 20 daily medications. And quickly.

"There's concern about loss of pregnancy, birth defects, congenital abnormalities," Roe said. "All those types of things that you worry about with any pregnancy, let alone the fact that you're immunosuppressed and these medicines cross into the fetus' circulation and can cause problems."

With the medical issues came an ethical dilemma. Patients have a certain level of responsibility to their donor's family, Roe said. Someone gave Heichelbech those lungs, so she needed to safeguard them.

"The chance that I may pass away because of the pregnancy ... I didn't want that to be a wasted life," Heichelbech said. "I don't want that family to feel like because I had Vivian or because I got pregnant that I wasted that donor's lungs."

Roe said he often thinks of Heichelbech's unknown donor and his or her legacy.

"The donor gave Andrea life, because Andrea was dying when she got her transplant," Roe said. "And now that donor has enabled Andrea to give life."

'She was just so tiny'

Heichelbech drove from Lafayette to Indianapolis every two to three weeks for doctor visits throughout her pregnancy. Her lungs were fine, she said, but she began having contractions about six months into her pregnancy.

Doctors had tried to stave off active labor by giving her injections that would slow the progress and help the baby develop. But when contractions woke her in the early morning hours of June 15, 2017, she knew this time was different.

"I was searching what it felt like when your water breaks," she said, "and then as I was reading, my water broke."

Gustin rushed his girlfriend to Methodist Hospital.

"I think I was probably going 85, hoping that she wouldn't go into labor in the car," he said.

She was 5 centimeters dilated by the time she got to the hospital, and they discovered Vivian was breech.

"I'm like, 'Oh, she's coming out feet-first. I can feel it. I feel her feet coming out,'" Heichelbech recalled.

Heichelbech refused to push, saying she didn't want to risk Vivian's health by going through with a breech delivery. She asked for a cesarean, but doctors didn't want to sedate her.

She remembers being told: "We can't put you under, because there's a 20 percent chance you won't wake up."

Heichelbech convinced the anesthesiologist that she had taken the drugs before and she could — and would — do it again.

Vivian was born via C-section at 8:41 a.m., weighing 4 pounds and 14 ounces.

Vivian stayed in the neonatal intensive care unit at Methodist for three weeks. Heichelbech slept most nights on a cot in Vivian's room.

"She was just so tiny," Heichelbech said. "She had a little 'V' on her head, and I was like, 'How perfect.'"

A good fight

As complicated as Heichelbech's pregnancy was, she now faces another hurdle: Rejection of her new lungs.

The immune system protects the body against foreign objects, and as a result of her pregnancy, Heichelbech's body has created antibodies that are attacking the new lungs.

She receives intravenous immune globulin (IVIG) once a month, helping her fight those bad antibodies. She's still taking at least 20 medications.

Heichelbech said a recent biopsy indicated the IVIG is doing what it's supposed to do — the rejection is limited to the antibodies, meaning her graft is still healthy.

"My lungs themselves are in good condition," she said. "They're fighting a good fight."

But Roe said he hopes to never see another case like Heichelbech's. If not managed correctly, antibody rejection can cause graft dysfunction and chronic rejection, the leading cause of death among transplant patients.

"This is a story I wish had never happened," Roe said, "because you are really putting yourself at risk."

Even though Vivian is healthy and happy, Gustin doesn't deny the reality that her birth comes with major risks. Gustin said he tries not to dwell on the possibility of losing Heichelbech, but there are still things he does to prepare Vivian.

"We try to get a lot of pictures and videos, or I do, of her and her, just in case something were to ever come up," Gustin said. "I can put it all together and show her how cool her mom was."

As he spoke, Heichelbech looked down, her eyes seemingly fixed on a spot on the tan carpet in her living room. Slowly, she shifted her gaze upward.

"I think about it a lot," she said, her eyes now misty. "I try (to be present), but there's times that the rejection really just makes me feel ill, like I'll get really super tired, and I just can't — I just can't function. But I do my best. I try to do my best and be present for her, that way she knows who I am."

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