What Pop Culture Gets Wrong (and Right) about Cystic Fibrosis

Anytime an illness is fictionally represented in the media, there are bigger conversations that need to be had. So, it was not surprising that the release of "Five Feet Apart," a love story centering on two young people living with cystic fibrosis, caused a quite a stir.

Cystic fibrosis is an illness that is not often portrayed in television or film. This genetic disease causes thicker than normal mucus to form in the lungs, pancreas and other organs. People with cystic fibrosis have mucus that is thick and sticky, so it can block airways, making it hard to breathe and increasing the possibility of serious infection. More than 70,000 people worldwide are living with the disease, according to the Cystic Fibrosis Foundation Patient Registry, with approximately 1,000 new cases being diagnosed each year.

"CF may be thought of as mostly a disease that impacts the lungs, but it also affects many systems in the body. Typical management of CF involves (at a minimum): at least four visits a year to the CF Care Center, daily airway clearance, daily oral, inhaled, and/or nebulized medications, and close management of diet and nutrition. Daily care is important to keep lungs and other body systems as healthy as possible," explained licensed clinical social worker and cystic fibrosis care team member, Anna Saulitis. Saulitis provides chronic disease support and care for patients with CF and their families at the Rush University Medical Center’s Pediatric and Adult Cystic Fibrosis Centers.

It is one specific rule that patients with CF must live by that is the center of the movie’s plot and controversy. The romantic teen drama focuses on two patients with CF, Stella (played by Haley Lu Richardson) and Will (played by Cole Sprouse), who meet and fall in love while being treated in a hospital. Though they are ordered to stay at least six feet apart to prevent infection, they decide to defy the rule and "take back a foot."

Why Six Feet?

In people with CF, the abundance of thick mucus in the lungs can trap dangerous bacteria that people with otherwise healthy immune systems may be able to fight off. These infections, in turn, can lead to worsening lung disease, more rapid decline in lung function, and even death. Two infections that are particularly threatening for cystic fibrosis patients can be resistant to antibiotics— Pseudomonas aeruginosa and Burkholderia cepacia complex. The film refers to B. cepacia in particular, with Stella telling her YouTube followers that this bacterium is "so adaptive it literally feeds on penicillin."

These bacteria are normally transmitted only between people with CF or compromised immune systems. This is why doctors insist that patients with CF stay six feet or more apart from anyone who is sick, but specifically other patients with CF whose unique bacteria could put them in increased danger. "This distance is specific for a reason—it is the distance germs can spread when a person coughs or sneezes," said Saulitis. "There are some germs and bacteria that are considered especially dangerous for people with CF to spread to one another—maintaining a six-foot distance can help to reduce that risk."

In addition to maintaining a six-foot distance and avoiding close proximity to other people with CF and people who are sick, patients with CF are also discouraged from sharing belongings with one another. Any way that an infection can be spread is a risk. That is why patients with CF have to be diligent about good hygiene, especially after being in and around the CF center or medical center. Cleaning and disinfecting nebulizer equipment appropriately and getting vaccinated are a few other ways for patients to protect themselves. "Wearing a mask can help people with CF reduce their risk of breathing in germs or spreading germs. Not all masks are the same though, so it is important for people with CF to talk to their care team about which masks are recommended. According to the Cystic Fibrosis Foundation’s Guidelines for Infection Prevention and Control, people with CF should wear a surgical or isolation mask in common areas of the hospital and clinic," Saulitis said.

The director Justin Baldoni and the movie’s leads defended the depiction by claiming they consulted cystic fibrosis activist and YouTube star Claire Wineland, who passed away in September 2018. However, many people with CF agree that putting yourself in danger for any reason is ridiculous. One particularly vocal patient, Luisa Parazola, told The Cut there was "absolutely no way in hell I would break that rule!"

Others were more concerned about the portrayal as a whole, showing patients with cystic fibrosis as weak and constantly sick. Yes, cystic fibrosis is a life-threatening condition, but over the last 50+ years, research into cystic fibrosis has advanced treatments, medicines and care standards which in turn have shifted life expectancy from childhood to adulthood. According to the CF Foundation, half of babies with CF born in 2017 are predicted to live to be 46 years or older, something that would have been unthinkable in the 1970s.

"Not everyone with CF has the same severity of disease, and the disease may impact their body in different ways," Saulitis clarified. "People with CF are going to college, having careers, starting families, and planning for the future in many ways. Which is why mainstream media, when planning to portray any component of life with CF, should consult the people and their families and support systems who are living every day with this disease."

What the Movie Gets Right

Though people are now living longer, fuller lives, when your survival depends upon staying away from others, it can be particularly isolating. Being unable to do certain things, as well as consistently being hospitalized for infections can be particularly stressful. "CF’s effects go beyond an individual’s physical health. People with chronic disease are at greater risk for developing depression and experiencing anxiety than the general population," said Saulitis. "And their family members and caregivers are coping with a loved one who is living with this disease and managing their own challenges with mental wellness and grief."

That is why people with CF have taken to the internet and there is now a plethora of online support groups that allow patients to form connections without putting themselves in physical danger. CF Peer Connect, CF virtual events and local CF chapters are all great resources. In addition, the American Lung Association’s Living with Lung Disease online support community and Lung HelpLine are also available to support patients with CF and their families.

Regardless of the arguments, most people can agree that "Five Feet Apart" was an important step toward shedding some light on an underrepresented disease. Other depictions including Foreverland, The Book of Stars and Red Band Society, only touch on a subject that, many who suffer from the disease agree, needs to be talked about. "CF is a disease that has no cure, and people with CF are still suffering from a host of medical issues, many of which are severe and debilitating," said Saulitis. We need to raise awareness if we hope to continue to work toward giving patients with CF even better outcomes.