Over the past 12 months, 19-year-old Marissa Bell has been admitted to Ascension Via Christi St. Francis 13 times, spending 168 days in the hospital’s Grant and Norma Davis ChildLife Center.
Marissa, who lives with her mother and step-father in Preston, Kansas, has cystic fibrosis, a condition that three years ago took the life of her older brother, Jonathon. He wasn’t diagnosed until age 3, leading Marissa to be tested and diagnosed at two months.
Their now 18-year-old sister Erica is a carrier of the gene, but does not have cystic fibrosis.
“Jon was so much more than a brother to me. He was like a life partner who'd be there for me ‘til the end,” says Marissa, who stands 4-foot, 10 inches and weighs just 90 pounds. “And he was, for the most part.”
An isolating illness
Cystic fibrosis is a progressive, genetic disease.
In people with CF, mutations in the cystic fibrosis transmembrane conductance regulator gene renders the CFTR protein unable to help move chloride to the cell surface. This causes the mucus in various organs to become thick, sticky and unable to perform their functions.
In the lungs, the mucus clogs the airways and traps bacteria and other germs, leading to infection, inflammation and other complications. In the pancreas, the mucus buildup can keep digestive enzymes that help the body absorb food and key nutrients from doing their job, resulting in malnutrition and poor growth. In the liver, it can block the bile duct, causing the liver to become diseased.
While Jonathon was constantly sick and in and out of the hospital all of his life, Marissa’s disease didn’t progress much until she developed pneumonia at age 12. Then the disease that had made it difficult for Marissa to keep weight on suddenly began making it difficult for her to get the oxygen she needed as well.
“It literally scarred me,” she says, causing her scoring on pulmonary function tests to go from a robust 120 to a constant struggle to hit 60.
Having cystic fibrosis can be extremely isolating because of the risk that infection poses to patients’ lowered immune systems.
“Nearly everyone has to turn into a germaphobe to keep themselves from getting sick from anything viral,” Marissa says. “Wearing a mask everywhere you go, staying secluded for the whole flu season, cleaning your hands with sanitizer at every opportunity.”
Marissa was home-schooled through the 8th grade, then simply “gave up” on school. Instead, she devotes her time to creating anime-style art on her lap-top computer, playing video games and the piano, and listening to music.
“I could easily spend an hour relaxing with my song list playing on shuffle,” she says. When at home, she enjoys playing with her cat and keeping up with her sister, who now lives in Indiana, through texts and emailed photos.
When in the hospital, Marissa often becomes bored and wishes that she could go to the teen room to play the games there, but she can’t because the risk of infection is too great.
Instead, she passes the time drawing, texting and playing board games with Alex Ambuehl, RN, a palliative care nurse she’s gotten to know during her frequent stays at St. Francis.
“He’s good company and he takes the time to play with me,” Marissa says.
For Ambuehl, it’s his pleasure and privilege.
“I get as much out of spending time with Marissa as she does with me,” he says. “She’s taught me so much about living with cystic fibrosis and about the value of each day that we’re given.”
To help fund activities and support services for hospitalized children and young adults like Marissa, go to viachristi/cmnh. To learn more about Ascension Via Christi’s nationally accredited cystic fibrosis outpatient clinic, go to viachristi.org/cystic-fibrosis.