A Strong Voice in the Fight Against Cystic Fibrosis

A U-M patient with cystic fibrosis is making an impact, raising awareness and money to improve the lives of people who have the inherited, chronic disease.

10:30 AM

Author | Kelly Malcom

Emily Schaller

In more ways than one, Emily Schaller is a rock star.

The vivacious, 37-year-old Detroit native played drums in a local band when she was younger.

LISTEN UP: Add the new Michigan Medicine News Break to your Alexa-enabled device, or subscribe to our daily audio updates on iTunes, Google Play and Stitcher.

She remains larger than life today, a star whom countless others look up to, as she works tirelessly to spread awareness about cystic fibrosis, the genetic disease she was born with.

Spreading awareness

About 30,000 Americans have this rare disease, which affects many organs but particularly the lungs and digestive system. It is caused by mutations in the gene that makes the CFTR protein.

People with CF experience frequent lung infections and persistent lung damage because they are unable to clear thickened mucus in their lungs. Over time, this damage makes it hard to breathe. Patients also have trouble gaining weight, because the disease prevents the pancreas from supplying the digestive enzymes that are needed to break down food.

Schaller has spent her life educating her peers about her condition.

"I grew up talking about CF — I'd bring my vest and nebulizers to school and teach kids about CF," she says of her treatment equipment.

As an adult, she eagerly sought to dispel some of the many myths surrounding the disease, including the myth that people with CF only live until their 20s.

"It's still thought of as a childhood disease," Schaller says.

MORE FROM MICHIGAN: Sign up for our weekly newsletter

In 2004, she and her brother started playing concerts around Detroit to raise money and teach people about CF.

She founded the nonprofit RockCF to raise awareness of the disease through music.

Things began to crystallize for Schaller's nonprofit as she searched for ways to fend off her increasingly worrisome symptoms.

"As I started to get sicker and sicker in my early 20s, I said to myself, 'What have I not been doing that might make me feel better?'"

The missing piece

Spurred by the increasing popularity of running and wellness, Schaller decided the missing piece to feeling better might be exercise, something her parents had encouraged her to do as a young child.

"After months and months of training, I worked up to being able to run 1 mile, then 2, then 3. I did my pulmonary function tests at U of M and found my lung function had gone up from around 50 percent to 74 percent.

SEE ALSO: Emily Schaller is raising awareness and money to help others

"Instead of being in the hospital three to four times a year, I was going only once a year," she says.

Since then, Schaller has run 18 half-marathons and two full marathons.

Her personal running journey has morphed into an annual half-marathon held by RockCF in Grosse Ile, Michigan. The University of Michigan co-sponsors the race.

"We've had about 18,000 people run this race since we started in 2011, people who come to run an awesome race, learn about CF and become a champion for our cause," Schaller says.

Treating CF

Schaller sought treatment from the adult cystic fibrosis team at Michigan Medicine, where she's been receiving care since 2007.

"Beyond adherence to a complex treatment regimen, daily exercise is huge for maintaining function with CF," says Shijing Jia, M.D., assistant professor in pulmonary and critical care medicine at U-M.

Treatment for CF can be burdensome, requiring patients to spend up to two hours a day on therapy. Typically it involves inhaled drugs, including dornase alfa, a drug that cuts up leftover DNA in thickened mucus to help patients clear their lungs, and hypertonic saline to improve lung function.

Patients also use a vibrating vest to help loosen mucus in their lungs, inhaled antibiotics for lung infections and several enzymes to help digest meals.

Bacteria in Healthy Lungs Linked to Immunity

Because of the intensity of treatment, people living with CF develop close relationships with their physicians starting at a young age.

Since 2007, virtually every newborn in Michigan has been screened for CF, allowing for early treatment for those who are diagnosed.

Hearing that a seemingly healthy baby with no symptoms has cystic fibrosis can be stressful, says Samya Nasr, M.B., B.Ch., director of the Cystic Fibrosis Center and professor of pediatrics at U-M's C.S. Mott Children's Hospital.

One of the first things her team does once a baby is found to have CF is route the parents to accurate sources of information, such as the Cystic Fibrosis Foundation website.

"There are a lot of blogs and websites with wrong information or a very dark view of CF," Nasr says.

One of the hallmarks of U-M's CF program — the largest in the state — is the close collaboration between the pediatric clinic and adult clinic, where Schaller receives care.

Bacteria in Your Lungs? New Microbiome Study Shows How They Get There

Around age 18 to 21, patients transfer from the pediatric team to the adult team. Nasr and Richard Simon, M.D., medical director of the Adult Cystic Fibrosis Center and professor in pulmonary and critical care medicine, have worked hard to ensure this process runs smoothly for patients and their families.

"Because I trust the adult team, I can make sure the patients are not apprehensive," Nasr says. "The adult clinic is very involved early on, and that's important for patients."

Newer therapies and new hope

Beyond her healthy lifestyle, Schaller has benefited from participating in clinical trials sponsored by U-M on a new class of drugs called modulators, designed to help the mutated CF protein work the way it's supposed to.

"These drugs are small molecules that bind to the abnormal protein and make it perform more normally," Simon explains. Because of Schaller's particular CF gene mutation, she has responded well to the first version of the drug, called ivacaftor.

However, a newer version of the treatment that combines three modulating drugs holds enormous promise for the majority of people with CF. This treatment is in late clinical trials.

"Early results from the trials show that the triple combination is very effective. If the studies show that the treatment is also safe, leading to approval by the U.S. Food and Drug Administration, the triple-combination drug will help 90 percent of people with CF," Simon says.

All of this research and preventive care has resulted in a better quality of life and extended life spans for people with CF. "Since 2002, the average life span of a person with CF has improved from 33 to 44 years old. We have patients in their 60s and 70s, which would have been unheard of decades ago," Simon says.

Schaller echoes his enthusiasm.

"Kids treated today could potentially live a semi-normal life with even fewer treatments than I'm taking today," she says. "I think CF will be the best story in history — in the next decade, we'll be talking about CF as something in the past."

'Five Feet Apart'

In mid-March, a story of two lovestruck patients with cystic fibrosis hits the big screen in the movie "Five Feet Apart." In the film, the two patients who have been admonished to keep their distance, at least 6 feet apart to avoid potential serious health complications, explore the notion of becoming closer.

"Because of the inability to clear their mucus, a lot of CF patients will always have some bacteria in their lungs," says Shijing Jia, M.D., a pulmonologist at the University of Michigan. "Some of the bacteria have been shown to be worse than others, because they decrease lung function over time more quickly if you have it than if you don't."

People with CF are recommended to keep their distance because of the risk of picking up these bad bacteria. Some of the earliest studies demonstrating that people with CF can spread bacteria to each other were done at U-M by pediatrics professor John LiPuma, M.D.

"We never know exactly what bacteria patients are carrying, and we look for it, but we recommend they not be close together to prevent this spread," Jia says.

More Articles About: Health Management Cystic Fibrosis CS Mott Children's Hospital Lungs and Breathing
Health Lab word mark overlaying blue cells
Health Lab

Explore a variety of healthcare news & stories by visiting the Health Lab home page for more articles.

Media Contact Public Relations

Department of Communication at Michigan Medicine

[email protected]


Stay Informed

Want top health & research news weekly? Sign up for Health Lab’s newsletters today!

Featured News & Stories colorful illustration with human figure and highlighted lungs
Health Lab
Multimodal AI model may guide personalized treatments for tuberculosis
AI approach helps researchers interpret large biomedical data sets to accurately predict tuberculosis treatment prognosis
Breaking Down Mental Health on blue background and text inside a yellow head graphic
Breaking Down Mental Health
Depression and Sleep
In this episode, learn to understand the interplay between depression and how cognitive behavioral therapy can improve sleep.
Provider takes a pulse oximetry reading from a patient's finger
Health Lab
Inaccurate pulse oximeter readings could limit transplants, heart pumps for Black patients with heart failure
Racially biased readings of oxygen levels in the blood using pulse oximeters may further limit opportunities for Black patients with heart failure to receive potentially lifesaving treatments, such as heart pumps and transplants
An unconscious woman lies on a hospital table while a clinician's glove is seen placing an oxygen mask over her nose and mouth
Health Lab
Hispanic patients with respiratory failure much more likely to be oversedated
Hispanic individuals who are hospitalized with respiratory failure are five times more likely than non-Hispanic patients to receive deep sedation while on a ventilator, according to a new study published in the Annals of the American Thoracic Society.
sketched out bacteria in a dish yellow and blue colors of U-M
Health Lab
Bacteria in the mouth linked to pulmonary fibrosis survival
Bacteria in the mouth may play a role in survival from idiopathic pulmonary fibrosis (IPF).
Closeup of medical ventilator breathing tube
Health Lab
Patients on respiratory support in rural intermediate care units have higher death rates
Patients receiving ventilator life support in intermediate care units of rural hospitals had significantly higher death rates than patients in the same type of unit at urban hospitals, according to new study.