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 cystic fibrosis patients and families face complicated issues related to getting the care they need. But CF Foundation Compass makes sure that no one has to do it alone.
For many people with cystic fibrosis, dealing with insurance is as much a part of living with the disease as nebulizers and vests. Many people with CF and their families face issues related to getting the care they need, but no one has to do it alone.
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.
Due to the thick, sticky mucus associated with cystic fibrosis, adults with CF need to take additional steps for a successful colonoscopy. Learn more about why it's important to properly prepare for a colonoscopy and the steps you can take during your preparation.
In people with cystic fibrosis, the defective cystic fibrosis transmembrane conductance regulator (CFTR) gene causes a thick, sticky buildup of mucus in the lungs, pancreas, and other organs -- including the colon. This, combined with the fact that people with CF often have a thicker and stickier stool, makes it more difficult to empty the contents of the colon when preparing for a colonoscopy.
Because of these unique challenges, adults with CF will need to undergo a more intensive bowel preparation than people without CF.
“The CF digestive system is very different than the average person's. Our systems are slower and full of mucus, so it takes much more time, and greater quantities of prep solution, to clean us out sufficiently. When I have a colonoscopy, I spend three or four days prepping for the exam.” -- Amy Braid, an adult with CF, from the CF Community Blog
Currently, colonoscopy is the only method that has been studied as an effective colorectal cancer screening test for people with CF. Colonoscopies allow the gastroenterologist or colonoscopist to both identify and remove as many polyps as possible before the polyps develop into cancer.
When the colon is not properly cleaned out, the gastroenterologist or colonoscopist may not be able to find polyps and may even require you to reschedule or repeat the exam.
Watch the animation above to see why it's important to properly prepare for a colonoscopy when you have CF and the steps you can take for a successful exam.
Although the specific details of your bowel preparation regimen will depend on your individual health status and insurance coverage, there are common steps that people with CF should take to prepare for an effective exam:
Use this handout to guide your discussion with your endoscopist and CF care team to prepare for a successful colonoscopy.
If you have cystic fibrosis-related diabetes (CFRD), check with the doctor who manages your CFRD for specific diet and medication instructions. For example, you may need to schedule an early-morning appointment, adjust your insulin dose as part of your preparation, or discontinue certain medications in the few days leading up to your exam.
“The most important part of preparing for a colonoscopy when you have CF is splitting up the bowel cleansing solution into three or four separate sets of 64 ounces. It works great! By drinking the bowel cleansing solution in multiple, smaller washes, your colon gets cleaned out much better.” -- Amy Braid, an adult with CF, from the CF Community Blog
While preparing for your colonoscopy, you may experience issues such as weight loss, dehydration, and nausea.
Since people with CF have a more demanding bowel preparation regimen and are already at an increased risk for many of these problems, it is important to monitor your health and call your CF care team if you experience:
You may also lose weight while preparing for your colonoscopy since you can get dehydrated and will need to go on a special low-fiber diet as a part of the preparation process. If these or other issues last longer than 48-72 hours after the colonoscopy, contact your CF care team.
Prior to your colonoscopy, you will need to arrange for someone to drive you home once the colonoscopy is complete. Even though you will be awake when you leave, the medications administered during the procedure can impair your judgment and reflexes for the rest of the day.
The physician who will perform your colonoscopy will differ depending on your center and will typically not be a member of your CF care team. For this reason, it is important to know what to ask your gastroenterologist or colonoscopist for a successful, high-quality exam. Questions to ask include:
Although effectively cleaning out your bowels is one of the most important things you can do to prepare for a successful colonoscopy, there may be medical reasons why a colonoscopy cannot be completed as planned. For example, the physician performing the exam may stop the procedure may if he or she discovers something about your colon that increases your risk for injury -- such as large lesions -- or determines you need a different type of sedation.
Although most insurance plans will cover colonoscopy screenings, you may still be charged for some services. Since screening for people with CF is recommended at a younger age than the general population, your insurance company may ask for additional information, such as a letter of medical necessity from your CF care team. For this reason, it is important to understand exactly what your insurance will cover and review your plan for specific details, including if the colonoscopist and any other care providers are in-network.
Cystic Fibrosis Foundation Compass case managers can help by explaining what your insurance plan covers to help you maximize your benefits and coordinate communication with your insurance company, gastroenterology practice, or any other providers on your behalf.
To learn more about colorectal cancer screening coverage, call CF Foundation Compass at 844-COMPASS (844-266-7277) Monday through Friday, 9 a.m. until 7 p.m. ET, or email email@example.com.
Am J Gastroenterol 2017; 112:1016-1030. Table 3.
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