At first, Travis and Nicole Fuechtmann wanted to bubble wrap their young family.
Two of their three kids – Bryn, now 12, and Vivian, 9 – were diagnosed with cystic fibrosis, a genetic disease that causes serious breathing and sometimes digestive issues.
But, the Little Falls couple quickly realized that isolation and extreme caution is no way for a kid to live.
Besides, the team of doctors behind them, from the world-renowned M Health Fairview Minnesota Cystic Fibrosis Center, enthusiastically agreed.
Now, Bryn and Vivian are just as active as their 14-year-old brother Kade, playing soccer, hockey and even tubing and waterskiing with a pack of cousins.
Says Nicole: “Our team of doctors never says no to anything they want to do. It’s always, ‘We’ll figure out a way.’”
Adds the team’s Dr. Samuel Goldfarb, an M Health Fairview pediatric pulmonologist and co-director of the Minnesota Cystic Fibrosis Center: “If there’s a limitation, we haven’t done our job well enough.”
"When I first started, pediatric patients were either dying or getting a lung transplant."
Once the prognosis for kids diagnosed at birth with CF was grim.
In fact, in the early days, there were only pediatric centers for CF. Now, because people are living longer, there are adult ones, as well.
Even then, the medicine is so good, the doctor says, that wards that once housed four to five patients “are empty.”
With that much progress and more promising research on the way, will there ever be a cure?
“It’s just a matter of time,” Dr. Goldfarb says. “Not whether it’s possible, but when.”
Sometimes, Vivian says, it’s hard to breathe. Adds Bryn: “I just use my inhaler.”
CF is caused by a faulty gene that affects the mucus in our lungs and sometimes GI tract.
Instead of being slippery, that mucus gets thick and sticky and clogs the airways.
It can do the same in the pancreas, causing pain, cramping and diarrhea – “Thirty or 40 years ago, patients were dying of malnutrition,” Dr. Goldfarb says.
Twice a day, Bryn and Vivian strap on special vests, developed at the U of M by the late CF trailblazer Dr. Warren Warwick, to massage that mucus from their lungs.
They also take enzymes before meals to help them digest their food.
Colds, flus, and other infections – threats that once had Travis and Nicole considering bubble wrap – are certainly still out there.
“But there are antibiotics for that” – and, sometimes, additional vest sessions, says Dr. Goldfarb.
Adds Nicole: “We have all the doctors on speed dial.”
Nicole tears up thinking back at Bryn’s diagnosis
Travis and Nicole, like pretty much all of us, had no idea that they each carried the gene for CF.
It takes both parents to pass CF onto a child and, even then, it’s not certain, as it wasn’t for their first child.
A test, administered at birth to all babies, screens for the disease.
Travis and Nicole thought about adoption for their third child.
But with the medicine so good, and just a one in four chance of having another child with CF, they left it “up to God.”
If nothing else, the couple thought, the kids would have each other, which is exactly how it worked out.
“Vivian is the mother hen,” Nicole says – “always making sure her sister takes her medicine.”
They’re pretty strong-willed girls. Plus, they’ve got an older brother pushing them.
Twice a day, Bryn and Vivian gather in a special room at home and strap on their vests for 45-minute sessions.
When they need in-person care, Bryn and Vivian are typically seen at M Health Fairview Pediatric Specialty Clinic-Discovery.
Their team of doctors have them enrolled in a drug trial that may one day clear their lungs without the need for the devices.
But, in the meantime, the break times are just something the girls have always done and are learning to do for themselves.
Sometimes, they watch videos together. Other times, they read. If friends or cousins are visiting, everybody joins in.
“It’s just a time management thing,” Nicole says.
Says Dr. Goldfarb: “Credit goes to the kids and their families. We ask a lot of them.”
Adds Travis: “We know we probably have a lot of unknowns ahead. But we have that team behind us – and, in the meantime, we’re going to live life.”
PUTTING DISCOVERY INTO PRACTICE
M Physicians are an extension of the University of Minnesota Medical School. As Medical School faculty, they are always looking for new and better ways to treat patients, whether in the laboratory or the clinic. And through clinical trials, cutting-edge therapies are sometimes available to patients when the standard of care is no longer enough.
What is Academic Medicine?
Most medicine is practiced within what is called “the standard of care.” Simply put, “standard of care” is the treatment that is commonly accepted for treating illness. This is a good thing! It means that patients receive treatments that are known to be generally effective and reliable.
The goal of academic medicine is to treat patients while looking for better therapies. It takes the toughest problems from the clinic and looks for solutions in our research. Many of our physicians — leaders in their fields — are also scientists.
When a patient faces an illness that requires treatment that exceeds the standard of care, academic medicine can provide access to newer therapies.
Co-director, Minnesota Cystic Fibrosis Center
M Health Fairview (University of Minnesota Physicians)
