You receive services from doctors with specialty training in lung disease. We are one of approximately 130 centers in the U.S. accredited by the Cystic Fibrosis Foundation.
Our cystic fibrosis care teams teach other health care professionals about cystic fibrosis and take part in research to provide better diagnosis and treatments.
When you seek care at the UW Health Cystic Fibrosis Center, we work with you to:
Educate your family about providing the care and help you need
Promote your best health
Prevent your condition from getting worse
Help you thrive in the life you want to live
The National Institutes of Health recognizes our center as a model of effective and efficient health care for chronic disease.
Our patients enjoy healthy outcomes for lung function that are higher than the national average.
Improving lung and overall health through research
The cystic fibrosis doctors and scientists at UW Health lead and participate in many research projects and clinical trials. We want to provide better treatments and improve the care you receive.
Cystic Fibrosis Center outcomes data
The UW Health Cystic Fibrosis Center is one of approximately 130 care centers across the country accredited by the Cystic Fibrosis Foundation. We work closely with the foundation to continue to improve the care and quality of life of our patients and publicly report our center-specific data to give patients, families and ourselves an opportunity to look for areas of improvement.
FEV1 6-12 years
Pulmonary function outcomes are important indicators of the health of cystic fibrosis patients. The forced expiratory volume in 1 second (FEV1), the volume of air a person can forcefully blow out in a second is considered a good indicator of lung function.
This chart shows lung function for patients age 6–12 that were treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States from 2014–2023.
FEV1 13-17 years
Pulmonary function outcomes are important indicators of the health of cystic fibrosis patients. The forced expiratory volume in 1 second (FEV1), the volume of air a person can forcefully blow out in a second is considered a good indicator of lung function.
This chart shows lung function for patients age 13-17 that were treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States from 2014–2023.
FEV1 18+ years
Pulmonary function outcomes are important indicators of the health of cystic fibrosis patients. The forced expiratory volume in 1 second (FEV1), the volume of air a person can forcefully blow out in a second is considered a good indicator of lung function.
This chart shows lung function for patients age 18 and older that were treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States from 2014–2023.
BMI 2-19 years
The Body Mass Index (BMI, a ratio of body weight to stature) is considered a good indicator of nutritional status.
This chart shows BMI for patients treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States from 2014–2023.
Care provision performance
Current guidelines of care recommend that patients with cystic fibrosis be evaluated at an accredited CF Center at least quarterly. In addition, patients should undergo pulmonary function testing at least two times a year and have a culture of their respiratory secretions performed at least once a year. The recommendation is also to increase the frequency of follow up for those patients that have more severe disease.
We believe that more frequent clinic visits and more frequent monitoring of pulmonary function and sputum cultures can lead to better outcomes. Thus, we strive to exceed the Cystic Fibrosis Foundation's recommended testing and obtain pulmonary function test and a sputum culture (or throat swab culture for patients who cannot cough out sputum) at every clinic visit.
This chart shows the percent of children over 7 years old who had at least 1 visit, 1 culture and 1 PFT at the UW Health Cystic Fibrosis Center, compared to the average for all centers in the United States from 2014–2023.
More adults with less common symptoms are being diagnosed later in life. You might live with chronic coughing and sinus infections and not be able to gain weight.
We use a simple sweat test to make a diagnosis. We place two electrodes on your forearm. This causes your sweat glands to make sweat. We check your sweat for high levels of chloride. Your doctor may order additional tests such as blood work and X-rays to confirm the diagnosis.
Chronic or persistent cough
Frequent lung infections
Poor weight gain
Frequent loose stools
Frequent lung infections
Salty-tasting sweat
Arthritis
Infertility
Pancreatitis
Sinusitis
Complications as you age
You might experience complications related to your cystic fibrosis as you get older. These conditions can include:
Cystic fibrosis-related diabetes
Intestinal blockage
Liver disease
Nasal polyps
Osteoporosis
You need daily treatments and regular care to stay healthy and live well.
Daily treatments may include:
Airway clearance therapies to loosen mucus and clear it from your airways, such as:
Active cycle breathing
Autogenic drainage
Forced expiration technique
Manual chest physiotherapy
Exercise
Nutrition therapy, including enzymes and vitamin supplements
Other available treatments include:
Routine vaccinations
Fertility treatments
For some individuals with advanced disease, lung transplantation might be the next step to help regain health.
Tips for maintaining your health and wellness
You can find a balance between your busy lifestyle and your care. We know that living with cystic fibrosis can be emotionally and physically challenging. Your care team stays by your side to help you feel your best.
Some tips for staying healthy with cystic fibrosis:
Avoid others with upper respiratory illness
Complete your physiotherapy exercises
Contact your clinic if there are changes in your health
Eat a cystic fibrosis-specific diet high in calories and fat
Keep yourself hydrated
Manage your mental health
Stay active
Take your prescribed medication
Talk to your care team about annual flu and pneumonia vaccines
Visit the Cystic Fibrosis Center regularly throughout the year
Wear a mask that covers your nose and mouth when in a health care setting
Watch videos from Cystic Fibrosis Family Education Day.
If a newborn screen test shows your baby might have cystic fibrosis (CF), a sweat test will make the final determination.
For patients having telemedicine/video appointments, we offer throat swab testing at a drive-through site.
For patients over 6 years of age who are having telemedicine/video appointments, learn how to set up and use a spirometer.
Parents can take several steps to keep their infants and young children with cystic fibrosis healthy:
Parents who smoke are encouraged to quit or at least not smoke in the home or car.
Each fall, a flu shot is recommended for the person with cystic fibrosis and other family members.
During hot weather or times of physical exertion, parents should add salt to the child's food.
Regular aerobic exercise is encouraged to increase the child's oxygen intake, strengthen lung muscles and increase their sense of well-being.
Parents are also encouraged to look beyond the disease to see their children as unique individuals who happen to have cystic fibrosis. Children should be encouraged to pursue their interests and personal goals. Children with cystic fibrosis participate in sports, dance, music, outdoor activities or scouts, as well as their regular school activities.
Printable passport for cystic fibrosis patients to inform care providers about their diagnosis and precautions that should be taken while treating them.
Vivek Balasubramaniam, MD
Pediatric Pulmonology and Sleep MedicineCarrie Barker, MD
Pediatric Pulmonology and Sleep MedicineChristina Barreda, MD
Pediatric Pulmonology and Sleep MedicineAndrew T. Braun, MD, MHS
Pulmonary and Critical Care MedicineLisa Burns, MD
Pediatric Pulmonology and Sleep MedicineJessica La Mar, NP
Pulmonary and Critical Care Medicine
University Hospital Adult Cystic Fibrosis Clinic
American Family Children's Hospital Pediatric Cystic Fibrosis Center
The newborn-screening test for cystic fibrosis involves two steps. First, blood obtained through routine newborn screening is examined for trypsinogen, a substance found to be higher in infants with cystic fibrosis. If the trypsinogen level is elevated, a second test is done on the blood sample to examine it for cystic fibrosis gene mutations.
There are several ways in which the cystic fibrosis screening test can be abnormal:
If the trypsinogen level is elevated and there is one cystic fibrosis gene mutation, there is a small chance that the child has cystic fibrosis. A single gene mutation is not sufficient for cystic fibrosis. The possibility exists that the child could have a second cystic fibrosis gene mutation not identified through the newborn screening test. A sweat test is needed to determine whether the child has cystic fibrosis. Genetic counseling for the parents is also recommended. If a child has one cystic fibrosis gene mutation, then one of the parents is also a carrier of the cystic fibrosis gene mutation. The carrier status of the parents can only be determined by further genetic testing.
If the trypsinogen level is elevated and there are two cystic fibrosis gene mutations, the child is presumed to have cystic fibrosis. A sweat test is needed to confirm the diagnosis. Genetic counseling for the parents is also recommended.