The truth about cystic fibrosis

When Five Feet Apart, a movie about love between two patients living with cystic fibrosis, was released this spring, team members from the Cystic Fibrosis Center at Advocate Children’s Hospital headed to the theater to see how the film would portray the disease.

“I think the film did a very good job portraying the struggles our patients go through in their day-to-day lives such as the treatment burden and, especially, the struggle between living for today versus the future,” says Margaret Weides, a nurse clinic coordinator for the Cystic Fibrosis Center at Advocate Children’s Hospital and Advocate Lutheran General Hospital’s Adult CF program.

Weides says it’s accurate that people with CF must remain six feet apart from each other to avoid coming in contact with cough secretions that can travel and cause infections. And she says that many of the actual terms regarding different treatments and lab results were used correctly.

However, she says how patients spend their time in the hospital in the film was less realistic. She says in past years, patients with CF did become close friends due to long hospitalizations like the movie portrayed, but this is not as much the case today due to new infection control policies that prevent interactions even in open settings such as a hospital lobby.

“With patient safety measures in place, it’s also highly unlikely that patients would be able to leave their rooms to spend time in other areas of the hospital and not get caught,” says Weides in reference to a few scenes from the movie.

Still, Weides and others hope the film shed some light on a rare and serious disease. CF is a genetic disease that causes frequent lung infections and leads to a decline in one’s ability to breathe. The average age of survival is 37.5 years old. Roughly 30,000 people in the U.S. are living with CF, and Weides says many more people carry the CFTR defective mutation that causes this genetic condition without the presence of the disease or symptoms.

Weides says symptoms can vary among patients and may not be visible in the very young. In all states, newborns are screened for CF in order to detect the disease early on and begin treatment soon after birth.

“In infants, symptoms generally include an inability to absorb their food, which leads to failure to thrive prior to the newborn screening,” says Weides.

As the disease progresses, symptoms include very salty tasting skin, persistent cough (at times with phlegm), frequent lung infections, pneumonia or bronchitis, wheezing and shortness of breath, poor growth or weight gain despite a good appetite, and frequent greasy, bulky stools or difficulty with bowel movements. While there is no cure for CF, for the first time ever, there are genetic modulators that combat the basic defect of the disease.