Cystic Fibrosis

Jessica Justmann, Fall 2014 J1 student

A common morning for teenagers consists of getting up for school just in time to get dressed, grabbing some breakfast and driving to school.

For junior Jessie Jilovec, the morning routine is different.

Jilovec said she performs an airway clearance every morning and every night to loosen up the thick, sticky mucus in her lungs caused by Cystic Fibrosis (CF).

She was diagnosed with the disease when she was 2 months old, so Jilovec said she has dealt with CF for almost her whole life.

According to the National Heart, Lung and Blood Institute, CF is a chronic illness caused by a defect in the CFTR protein, which everyone has. The CFTR protein is a protein that provides instructions for producing mucus, sweat, saliva, tears and digestive enzymes. More than 1,000 mutations of the CFTR protein have been identified in those who have CF.

According to the National Heart, Lung and Blood Institute, those who only have one copy of the defective CFTR protein are called “carriers.” They do not have CF, nor do they have any symptoms of it. Most carriers are not even aware they are a carrier.

If a female carrier and a male carrier have children, there is a one in four chance the child will have CF. Jilovec’s brother, a Blue Valley graduate, does not have CF even though he and Jilovec have the same biological parents.

Due to the defective protein, the amount of salt in the body fluctuates. The mucus in the lungs is also affected by the protein.

“The mucus in my lungs is thicker and stickier than normal, so it clogs the passageways in the lungs,” Jilovec said.

However, she said the disease is not painful, and she does not usually find it difficult to breathe.


The lungs are not the only organ affected by the disease. According to the National Heart, Lung and Blood Institute, many body systems are affected by the disease as well. This includes the digestive system, the reproductive system, and the endocrine system.

“My pancreas doesn’t make the enzymes that digest food, [and that] is why I take enzymes,” Jilovec said.

She said she has to take enzymes with every meal and snack she eats according to how much fat the food contains.

Without the enzymes, Jilovec’s digestive system would not be able to digest foods, she said. Otherwise, she said nutrients would just pass through her body unused, which could cause malnutrition and vitamin deficiencies.

According to the National Heart, Lung and Blood Institute, the life expectancy for those with CF is in the 30s and 40s, though it is now possible to live longer into the 50s and 60s because of medical advancements and treatments.

There is currently no cure for CF but there are some effective treatments, such as the airway clearance, also called the Vest.

“The Vest is where I just sit there for 30 minutes, and it shakes me to get the mucus loose so I can cough it up,” Jilovec said.

She said this system is the most effective treatment for her.

“I do a nebulizer during my airway clearance Vest, which is [a] medicine I breathe in,” Jilovec said. “One of them is basically salt water, which irritates the mucus, and the other one opens up airways.”

A friend of Jilovec, junior Maegan Rice, said she doesn’t notice Jilovec’s CF.

“I don’t notice her Cystic Fibrosis, and no one treats her any differently because of it,” Rice said.

With the combination of medicines Jilovec takes, she said she is able to stay healthy and active, just like others her age.

“Cystic Fibrosis doesn’t prevent me from doing anything [such as activities or sports],” Jilovec said. “I run almost every day, which helps keep me healthy.”

Jilovec said her life is no different from her peers. She played sports — soccer up until the end of sophomore year and softball and track until a few years ago — with little difficulty.

“It doesn’t really affect my life that much,” Jilovec said. “All I have to do is get up a little bit earlier in the morning and do all the treatments, and then at night I just have to do them again. Also, before I eat, I have to take enzymes.”

Even though Jilovec no longer plays soccer, softball or track, she said she still exercises.

“I don’t really ever find it hard to breathe,” Jilovec said.

Though Jilovec can easily do the same things her peers can do, Rice said Jilovec is still careful.

“If she didn’t have the disease, she wouldn’t be as cautious,” Rice said. “But she’s a clean freak — it’s just who she is.”

Jilovec said she doesn’t get sick very easily despite her CF.

“My mom is very conscious about germs, and she tells me not to touch my face,” Jilovec said.

CF puts her at risk for other diseases, Jilovec said.

“It is possible that I could get Cystic Fibrosis-related diabetes.”

CF-related diabetes is different than both Type 1 diabetes and Type 2 diabetes. It includes parts of both types.

According to the National Heart, Lung and Blood Institute, less than half of those living with Cystic Fibrosis have CF-related diabetes.

Jilovec doesn’t have a family history of CF — she said she is the only one in her family who has it.

“[Raising a child with CF means] I just have to remind her a lot to do her chest therapy because she doesn’t do it on her own,” Jessie’s mother, Sharon Jilovec said.

As mentioned before, Jessie does not get sick easily; however, she said her mom still tells her to be cautious of germs.

“I tell her to be cautious, to wash her hands and to stay away from sick people,” Jessie said.

Jessie said in addition to her mom’s suggestions, she also stores a bottle of hand sanitizer in her locker, which she uses between almost every class.

“My ultimate goal is to have a normal, long and happy life,” Jessie said. “I won’t let Cystic Fibrosis get the best of me.”