Learn about cystic fibrosis, a genetic disorder that affects the lungs, pancreas, and other organs, and how to treat and live with this chronic disease.
CF is a rare genetic disease found in about 30,000 people in the U.S. If you have CF or are considering testing for it, knowing about the role of genetics in CF can help you make informed decisions about your health care.
If you or your child has just been diagnosed with cystic fibrosis, or your doctor has recommended testing for CF, you may have many questions.
Diagnosing CF is a multistep process. A complete diagnostic evaluation should include a newborn screening, a sweat chloride test, a genetic or carrier test, and a clinical evaluation at a CF Foundation-accredited care center.
Raising a child with cystic fibrosis can bring up many questions because CF affects many aspects of your child’s life. Here you’ll find resources to help you manage your child’s daily needs and find the best possible CF care.
Living with cystic fibrosis comes with many challenges, including medical, social, and financial. By learning more about how you can manage your disease every day, you can ultimately help find a balance between your busy lifestyle and your CF care.
People with CF are living longer, healthier lives than ever before. As an adult with CF, you may reach key milestones you might not have considered. Planning for these life events requires careful thought as you make decisions that may impact your life.
People with cystic fibrosis are living longer and more fulfilling lives, thanks in part to specialized CF care and a range of treatment options.
Cystic Fibrosis Foundation-accredited care centers provide expert care and specialized disease management to people living with cystic fibrosis.
We provide funding for and accredit more than 120 care centers and 53 affiliate programs nationwide. The high quality of specialized care available throughout the care center network has led to the improved length and quality of life for people with CF.
The Cystic Fibrosis Foundation provides standard care guidelines based on the latest research, medical evidence, and consultation with experts on best practices.
As a clinician, you’re critical in helping people with CF maintain their quality of life. We’re committed to helping you partner with patients and their families by providing resources you can use to improve and continue to provide high-quality care.
As part of the Cystic Fibrosis Foundation's mission to help improve the lives of people living with cystic fibrosis, the PSDC initiative taps the CF community to inform key efforts to support the management of daily care.
Your cystic fibrosis care team includes a group of CF health care professionals who partner with you to provide specialized, comprehensive CF care.
Many people living with cystic fibrosis and their families face complicated issues related to getting the care they need. Cystic Fibrosis Foundation Compass makes sure that no one has to do it alone.
CF Foundation Compass is a service that helps people with CF and their families with navigating insurance options, connecting to legal information and experts, finding available financial resources, and tackling other life issues.
CF care team members are paramount in providing highly specialized care to people living with CF. CF Foundation Compass can help by serving as a strategic ally for care teams, so team members can focus on their patients’ care.
CF Foundation Compass can help you navigate insurance, financial, legal, and other issues you are facing. Use this online form to start your conversation with a Compass case manager today.
The Cystic Fibrosis Foundation is the world’s leader in the search for a cure for CF and supports a broad range of research initiatives to tackle the disease from all angles.
The CF Foundation offers a number of resources for learning about clinical trials and treatments that are being developed to improve the treatment of cystic fibrosis.
Our understanding of CF continues to evolve as scientists study what causes the disease and how it affects the body. These insights drive the development of new and better treatments and bring us one step closer to a cure.
Researchers, supported by the CF Foundation, have made tremendous advances to improve the health and quality of life of people with CF. We are committed to providing the tools and resources you need to continuously build upon this work.
Gastrointestinal Issues and Cystic Fibrosis-Related Diabetes
People with cystic fibrosis can experience complications in the pancreas, liver, and intestines that can lead to malnutrition, constipation, liver disease, CF-related diabetes, and other digestive issues -- including abdominal pain and poor appetite.
The pancreas has tissue that makes digestive enzymes. The enzymes move from the tissue through a duct (tube), mixing with a bicarbonate-rich fluid that is produced in the duct. The enzymes and the fluid flow into the small intestine where the bicarbonate neutralizes stomach acid and the enzymes digest food. The fluid also hydrates the intestines. In most people with cystic fibrosis, this process is affected in the following ways:
The Cystic Fibrosis Foundation has helped organize a group of gastrointestinal (GI) specialists to focus on the treatment and research of GI issues in CF. Called the Developing Innovative Gastroenterology Specialty Training program, or DIGEST, this group of GI doctors is developing best practices and gaining an improved understanding of abdominal symptoms to lead to better treatments.
Additionally, the CF Foundation is supporting clinical trials to test the effects of oral glutathione, an antioxidant that is important to the normal functioning of the intestine and lungs. Glutathione levels have been shown to be lower in people with CF. Oral glutathione may improve growth and decrease digestive inflammation in children with CF.
In this video, Meghana N. Sathe, M.D., associate professor of pediatrics and co-director of the pediatric CF center at the University of Texas Southwestern Medical Center/Children's Health, explains why people with CF can have problems with getting proper nutrition and experience gastrointestinal (GI) issues. She also discusses the GI Wellness study (now called the GALAXY study), which is being conducted by DIGEST doctors to gauge which GI symptoms affect people with CF the most, so that researchers can prioritize them for further study.
To relieve the various symptoms associated with pancreatic insufficiency, people with CF can take various medications.
In people with CF, continual scarring in the pancreas can lead to the development of cystic fibrosis-related diabetes (CFRD), a condition in which the pancreas doesn't produce sufficient insulin. More than 20 percent of people with CF have CFRD, which typically occurs in adulthood.
Researchers, funded by the CF Foundation, are examining the effect that the CFTR protein has on the development of CFRD to find ways to treat it. They are looking specifically at whether CFRD occurs because the CFTR protein is not present in islet cells -- cells in the pancreas that make hormones, such as insulin -- or in other cells in the pancreas. The Foundation also has funded research to understand the risk factors associated with developing CFRD, to improve treatment approaches, and to assess the impact of CFTR modulators on this condition.
For more information, visit CFRD.
People with CF also can experience problems in the liver because bile, a fluid that helps the body absorb fat, becomes abnormal and thick, blocking small bile ducts in the liver. The ducts become inflamed, which leads to scarring. Consequently, approximately 10 percent of people with CF develop a condition called CF-associated liver disease (CFLD).
In advanced CFLD, scarring (cirrhosis) can make it difficult for blood to flow through the liver and can cause blood to back up into the portal vein, the main vein going to the liver. This backup leads to increased venous blood pressure -- a condition called portal hypertension -- and can lead to complications such as fluid accumulation in the abdomen or bleeding from enlarged veins in the esophagus or stomach.
Because CFLD is difficult to identify in its early stages, doctors need better methods to diagnose it in time to minimize liver damage. In an innovative partnership with the National Institutes of Health (NIH), the Foundation is investigating the best methods of screening for the presence of liver disease. Researchers in the PUSH study are using ultrasound to see if they can identify biomarkers that would help them detect cirrhosis. If they are successful, scientists would be able to study how cirrhosis develops in people with CF and identify potential ways to treat it.
People with CFLD do not have many treatment options, but a multidisciplinary approach that includes the CF care center team is recommended in clinical care guidelines. The team will determine the causes of specific liver abnormalities and treat them accordingly. This treatment can include nutritional therapy and a medication that thins the bile.
Colorectal cancer is a cancer that begins in the colon or rectum. Adults with CF have a 5-10 times greater risk of colorectal cancer than the general population and a 25-30 times greater risk after an organ transplant.
Although we do not know for certain why people with CF have a greater risk of developing colorectal cancer, it is thought to be associated with the CFTR gene mutation. In general, transplant recipients have an increased risk for all types of cancer because the medications they must take to prevent rejection weaken the immune system.
Additional research is being conducted to understand the mechanism -- or the underlying process -- that causes this higher risk. In the interim, the Foundation convened a multidisciplinary team of experts to develop colorectal cancer screening recommendations.
The Foundation also plans to conduct research to find ways to provide caregivers and people with CF more information to help inform their discussions about colorectal cancer screening.
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Drug Development Pipeline
We're attacking the disease from every angle. The Foundation is propelling every drug in our pipeline forward and will not stop until there is a cure.
About the Drug Development Pipeline
The Cystic Fibrosis Foundation facilitates the development of promising new drugs, and the drug development pipeline enables you to track the progress of these potential therapies.
How Drugs Get on the Pipeline
The Drug Development Pipeline features an extensive list of drugs that are in development or approved for treating cystic fibrosis. For a drug or program to be shown on the pipeline, it must meet certain conditions.
How an Experimental Drug Becomes an Approved Therapy
Ever wondered what it takes for a potential cystic fibrosis drug to become approved by the U.S. Food and Drug Administration? Here's an explanation of the four phases of clinical research.
Help us blaze a trail to better treatments and a cure for CF. Find a clinical trial that may be right for you.
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