Overview

How we can help

When you are living with cystic fibrosis, you need access to daily support and regular care. At the UW Health Cystic Fibrosis Center, we provide nationally recognized treatment for children and adults.

You receive services from doctors with specialty training in lung disease. We are one of 120 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.

Programs and research

Specialized care for lifelong health

When you come to the UW Health Cystic Fibrosis Center, we work with you to:

  • Educate your family about providing the care and help you need 

  • Promote your good health

  • Prevent your condition from getting worse 

  • Help you live the life you want 

The National Institutes of Health recognizes our center as a model of effective and efficient health care for a 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 more than 115 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.

Pulmonary Function and Nutritional Outcomes

Pulmonary function and nutrition are important indicators of the health of cystic fibrosis patients. The Forced Expiratory Volume in 1 second (FEV1, the volume of air a person is able to forcefully blow out in a second) is considered a good indicator of lung function.

The following is data from 2019 and displays lung function for patients treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States.

6-12 years old

  • 98.3% (UW Health)

  • 97.4% (U.S. average)

13-17 years old

  • 95.3% (UW Health)

  • 91.8% (U.S. average)

The Body Mass Index (BMI, a ratio of body weight to stature) is considered a good indicator of nutritional status. The following is data from 2013 and displays BMI for patients treated at the UW Health Cystic Fibrosis Center compared to the average score for all centers in the United States.

Median BMI percentile for patients 2-19 years old

  • 63.7% (UW Health)

  • 58.4% (U.S. average)

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.

The following information represents two groups of patients who had four or more visits, four or more cultures and two or more PFTs.

Children ≥7 with ≥4 visits, ≥1 culture, ≥2 PFTs

  • 58.7% (UW Health)

  • 60.0% (U.S. average)

Our data is shown compared to the average outcomes reported by the Cystic Fibrosis Foundation for all accredited CF Centers in the U.S. (2019 data).

Conditions and symptoms

How we diagnose cystic fibrosis

At UW Health, we diagnose patients with cystic fibrosis at birth and in adulthood. Most people with cystic fibrosis know they have the condition as children.

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.

Common symptoms for adults and children

If you have cystic fibrosis, your body makes thick, sticky mucus. This mucus can affect how your lungs, pancreas, sweat glands and reproductive system work. Cystic fibrosis is a genetic condition. Symptoms are different for everyone.

  • 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

Treatments

Living well with cystic fibrosis

You need daily treatments and regular care to stay healthy and live well.

Daily treatments 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

    • Positive expiratory pressure

  • Exercise

  • Medications inhaled into the lungs

  • Nutrition therapy, including enzymes and vitamin supplements

Other available treatments include:

  • Fertility treatments

  • Lung transplant

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. At UW Health, 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 the cystic fibrosis 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

Patient resources

Learn more

Events

The Cystic Fibrosis Foundation has "Cons" (virtual conventions) for patients and families. The remaining scheduled virtual event in 2021 is:

  • Sept. 24 and 25: CF BreatheCon. BreatheCon is a two-day event for adults with cystic fibrosis that will provide a virtual space to share, connect, and learn while supporting one another.

Register here

Other information

For patients having telemedicine/video appointments, we offer throat swab testing at a drive-thru site.

Learn more

For patients over 6 years of age who are having telemedicine/video appointments, learn how to set up and use a spirometer.

Learn more

Helpful links

Meet our team

Lung care experts by your side

The pulmonology team at UW Health includes experts in lung disease and cystic fibrosis nutrition.

Meet our team

Locations

How to find us

  • University Hospital - Adult Cystic Fibrosis Clinic
    • 600 Highland Ave. / Madison, WI
    • (608) 263-7203
    • Closed now
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  • American Family Children's Hospital - Pediatric Cystic Fibrosis Center
    • 1675 Highland Ave. / Madison, WI
    • (608) 263-6420
    • Closed now
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