Cystic fibrosis (CF) is a progressive, genetically-inherited disease impacting the cells that produce mucus, sweat, and digestive juices. Rather than being thin and slippery lubricants, these secreted fluids become sticky and thick because of a defective CF gene. This causes the secretions to obstruct tubes, ducts, and passageways, especially in the lungs and pancreas.
In the lungs, it enables bacteria to stick and multiply more easily, increasing the risk of infection. The lungs become obstructed, making breathing more difficult. In the pancreas, scarring from blocked ducts reduces the absorption of fats and vitamins. Furthermore, blockages in the liver can result in liver disease, and increased mucus in the reproductive tract can cause decreased fertility or infertility in men and women.
CF is most common in Caucasian patients, but it is growing more diverse every year. In 2022, more than 15% of people with CF were identified as a non-Caucasian race. It is anticipated that this trend towards increased diversity will continue.
In the United States alone, there are about 30,000 people with cystic fibrosis, and there are an estimated 70,000 people with CF worldwide. Approximately 1 out of every 30 Americans is a CF carrier. Because of this, every state in the U.S. routinely screens newborns for CF, to allow for early diagnosis and treatment.
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1. “Cystic Fibrosis.” Mayo Clinic, Mayo Foundation for Medical Education and Research, 5 July 2023. www.mayoclinic.org/diseases- conditions/cystic-fibrosis/symptoms-causes/syc-20353700.
2. “Cystic Fibrosis (CF).” American Lung Association, 5 July 2023. www.lung.org/lung-health-and-diseases/lung-disease-lookup/cystic- fibrosis/learn-about-cystic-fibrosis.
3. 2022 Cystic Fibrosis Foundation Patient Registry Highlights. Bethesda, Maryland. ©2023 Cystic Fibrosis Foundation. Accessed July 5, 2023.
What are the symptoms of cystic fibrosis? 1
- Symptoms can vary in severity and based on age:
- Persistent coughing, at times with phlegm
- Salty-tasting skin
- Frequent respiratory and/or sinus infections
- Wheezing/shortness of breath
- Poor growth or limited weight gain, in spite of a good appetite
- Frequent greasy stools, or bowel movements
- Nasal polyps
- Clubbed fingers
- Male infertility
How is it diagnosed?1
All newborns are screened for cystic fibrosis (CF) in the United States. This is done using a heel prick and testing for Immunoreactive Trypsinogen (IRT). IRT levels can be elevated due to CF, prematurity, stressful birth, and other reasons, so additional testing is performed to confirm CF. One confirmatory test is known as a sweat test, where the amount of salt in the baby’s sweat is measured.
In addition to newborn screening, prenatal genetic testing may be available prior to birth.
1 “Newborn Screening for CF.” CF Foundation, Cystic Fibrosis Foundation, 5 July 2023. www.cff.org/intro-cf/newborn-screening-cf.
Cystic FibrosisThe following specialty medications are available at Accredo, a specialty pharmacy for cystic fibrosis.
Bethkis® (tobramycin inhalation solution)
Financing Your Care
Financial assistance may be available to help with your medication costs, including manufacturer and community programs. Accredo specialists are available to help find a program that may work for you.
Community Financial Resources
- Cayston® Access Program | 877-550-1705
- PodCare+® Patient Support Program | 877-999-8624
- Kitabis® Pak Co-Pay Assistance Program | 855-334-6730
- Pulmozyme® Co-pay Program | 866-422-2377
- Chiesi CareDirect® | 866-422-2377
- Vertex GPS™ (Kalydeco, Orkambi, Symdeko, and Trikafta Patient Support & Guidance) | 877-752-5933
Life-saving specialty medication can be expensive. Learn how the Accredo teams help individuals find ways to afford the medication they need to survive in this video.
Cystic Fibrosis Organizations
There are many organizations that support research and advocacy for cystic fibrosis. See below for a few of those organizations.
Meet the Team
Accredo’s Cystic Fibrosis care team is dedicated to serving you and we understand the complexity of your condition. Our expert clinicians are available 24 hours a day, seven days a week to answer any questions.
Why We Do It
Accredo supports patients with chronic and complex conditions and helps them live their best life. Watch our video to learn why we do what we do for our patients.