The best news of Paige Montgomery’s life was that she was pregnant. Diagnosed with cystic fibrosis at age 22, doctors told her she’d never be healthy enough to conceive, strong enough to carry a pregnancy or live long enough to see her own child grow up.
The worst news of Paige Montgomery’s life came moments later: Either she or the baby likely wouldn’t make it. Due to the collapse of her upper right lung prior to her pregnancy, her doctor insisted she undergo surgery to repair it at six months. “He told me to choose my life or the baby’s life,” recalls Montgomery, who ended up getting a second opinion, avoiding the surgery and carrying her son, now a healthy 2-year-old, to 37 weeks.
[See: Lessons from Empowered Patients.]
“I get to look at every day as such a gift, and to have him in front of my eyes as a huge miracle,” says Montgomery, now a 28-year-old in Fort Worth, Texas, whose lung is still collapsed. Over the past year, she’s spent more than 140 nights in the hospital. “I continue to fight for my life,” she says.
Montgomery’s story illustrates just how complicated becoming a parent can be when you have cystic fibrosis — a genetic disease that progressively compromises lung function, among other life-threatening consequences, due to overactive mucus production. The condition can affect fertility (particularly among men), raise pregnancy and childbirth risks among women and exacerbate the challenges of parenthood. Add to that the toll of weighing the chances of passing down the disease, plus the reality that, on average, today’s adults with cystic fibrosis don’t live past their late 30s. “The thought of one’s own mortality plays a role” in when and if a person with cystic fibrosis becomes a parent, says Dr. Seth Walker, director of Emory University’s Adult Cystic Fibrosis Program.
What’s more, cystic fibrosis specialists were traditionally pediatric providers with little or no experience discussing reproductive health options with their patients who, 40 years ago, typically didn’t live past their teens. Twenty years ago, they more regularly lived to see 30. Today, while the increased life expectancy for cystic fibrosis patients is a sign of progress, the handoff between pediatric and adult care — if it happens at all — can still be rocky.
“We’ve gotten better at taking care of patients with CF; things that weren’t an issue are all of a sudden questions and issues [like,] ‘Our patients are now becoming parents, what does that look like?'” says Dr. Traci Kazmerski, a pediatric pulmonologist at Boston Children’s Hospital. “My ultimate hope is that [we’re asking,] ‘Our patients are becoming grandparents, what does that look like?'”
But for now, people with cystic fibrosis and their partners who are considering starting a family can heed these expert tips:
1. Speak up.
In a survey of nearly 200 15- to 24-year-old girls and women with cystic fibrosis, Kazmerski found that 78 percent of them wanted to become moms in the future. But in a series of interviews with similar patients, she also found that few had discussed reproductive health with their providers — despite seeing them at least several times a year — and were often misinformed about their own sexual health risks as a result. “That seemed really unfair,” Kazmerski says. To help right it, patients should bring up their parental hopes with the doctor who knows them best, she says. “Have that conversation … and really think about what are the steps to take so this can be as successful as it possibly can be.”
[See: 10 Questions Doctors Wish Their Patients Would Ask.]
2. Get the facts.
For women with the disease, fertility varies widely, though it can be affected by medications, low estrogen levels and excess cervical mucus, a byproduct of cystic fibrosis. “We can’t predict a woman’s fertility based on any other aspects of their disease,” Walker says. Pregnancy itself is considered high-risk among cystic fibrosis patients, who are at increased risk for diabetes and preterm delivery, according to the The American College of Obstetricians and Gynecologists, which recommends women with cystic fibrosis seek preconception counseling.
For men, on the other hand, cystic fibrosis almost always comes with infertility; up to 98 percent of male patients are infertile, since cystic fibrosis almost always causes a blockage or absence of the canal that allows sperm to travel to the semen, according to the Cystic Fibrosis Foundation. While the disease and its drug treatments can also affect sperm quality, men can still become biological fathers through assisted reproductive technology using a medical procedure that can extract the sperm from the testicle.
3. Be prepared to make hard decisions.
Deciding whether to pursue a pregnancy is just the first of many more complicated decisions people with cystic fibrosis and their partners must face. Others include how to proceed once results of genetic testing come back (couples in which only one partner has cystic fibrosis will either have a near-zero or 50-50 percent chance of having a child with the condition); whether to consider an alternative form of reproduction like a surrogate (if the woman has cystic fibrosis and doesn’t want to risk her health); and which cystic fibrosis medications are worth the often-unknown risks to the fetus.
Such decisions may require the guidance of multiple providers, including maternal-fetal medicine doctors, cystic fibrosis specialists, genetic counselors and, in cases where assisted reproductive technology is used, infertility specialists. Their work isn’t to push the latest medical advances, but rather consider “what is going to fit into their ethical beliefs, their personal beliefs, the cultural norms,” says Dr. Jeanne E. O’Brien, a reproductive endocrinology and infertility specialist at Shady Grove Fertility in Rockville, Maryland. “All of those things have to go into the decision-making.”
4. Seek support.
Nearly the entire time Katherine Proctor, 28, who also lives in Fort Worth, was pregnant with her now-toddler, she also played the role of caregiver to her husband, who has cystic fibrosis. At the time, he was undergoing a liver transplant and coping with the numerous complications of the procedure and disease. The pair, who underwent in vitro fertilization due to cystic fibrosis’s effect on his fertility, got through it by focusing on their shared goal of parenthood, remembering to laugh and leaning on family and friends who live nearby. “It’s a serious thing, it’s an intense thing, there’s a lot of money [involved,] it’s physically and emotionally taxing,” says Proctor, who now also has a 9-month-old son with her husband. “And in the end, it’s totally worth it.”
[See: What Only Your Partner Knows About Your Health.]
It’s been more than worth it for Montgomery, too. “I don’t think anything burdens me like other moms — to be able to take my kid to school is the greatest gift of all,” she says. “Being able to put him to bed, being able to wake up next to him is beyond my wildest dreams.”
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Becoming a Parent With Cystic Fibrosis originally appeared on usnews.com
