Thriving in the face of cystic fibrosis

“The patient population of cystic fibrosis was originally mostly just in pediatrics.”

That statement from physician Samuel Goldfarb harbors an unspoken truth: Most CF patients didn’t make it very far into adulthood, if at all.

Today Goldfarb, a professor in the U of M Medical School and co-director of the M Health Fairview Minnesota Cystic Fibrosis Center, is changing the future for CF patients. Among them are Bryn and Vivian Fuechtmann, two young sisters looking forward to lives packed with the activities they love.

“Our team of doctors never says no to anything they want to do,” says their mother, Nicole Fuechtmann. “It’s always, ‘We’ll figure out a way.’”

Goldfarb agrees.

“If there’s a limitation, we haven’t done our job well enough,” he says.

An insidious foe

Cystic fibrosis results from a genetic mutation that thickens the mucus secreted by cells lining our respiratory system. In healthy people, mucus works its way up and is swallowed or expelled by coughing or sneezing. But the thick mucus in CF patients hardly flows upward at all. Instead, it clogs airways and becomes a breeding ground for bacteria or fungi that have been inhaled.

Similarly, the disease gums up the works in the pancreas, which secretes digestive enzymes into the small intestine. Pain, cramping, and diarrhea result. But that’s not all. 

“Thirty or 40 years ago, patients were dying of malnutrition,” Goldfarb says.

Bright colors

Today, however, the work of Goldfarb, his colleagues, and at least two U of M pioneers is painting the future of patients like Bryn and Vivian in bright colors. 

Twice a day, the girls strap on special vests developed at the U of M  by pediatrician and CF expert Warren Warwick and senior scientist Leland Hansen. Licensed in 1988, the vests generate rapid vibrations that loosen mucus from the airway walls and help move it up to where the patient can expel it.

As for infections that might slip through, “There are antibiotics for that” and sometimes extra vest sessions, Goldfarb notes. Bryn and Vivian also take digestive enzymes before meals.

Though younger, “Vivian is the mother hen, always making sure her sister takes her medicine,” observes Nicole Fuechtmann.

The outlook is good for the girls to continue enjoying soccer, hockey, tubing, and waterskiing with older brother Kade and numerous cousins.

But a cure?

“It’s just a matter of time,” Goldfarb says.