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.
About Colorectal Cancer
The risk for colorectal cancer in adults with cystic fibrosis is 5-10 times greater compared to the general population, and even higher for people with CF who receive a lung or other solid organ transplant. Find out how early screening can help reduce your risk.
Colorectal cancer is the kind that begins in the colon or rectum. These cancers are also called colon cancer or rectal cancer, depending on where the cancer starts. Although many colorectal cancers can be prevented with regular colonoscopy screenings,
it remains the third leading cause of cancer deaths in the U.S. among the general population.1
The average lifetime risk for colorectal cancer is approximately 5 percent, but for adults with cystic fibrosis, this risk is 5-10 times higher and warrants screening 10 years earlier, at 40 years of age.
Meanwhile, those who receive a solid organ transplant are 20 times more likely to develop colorectal cancer and often need to begin screening even earlier -- at the age of 30, depending on when the transplant
Watch the animation above to learn more about colorectal cancer in CF and how early screening can help reduce your risk.
Most colorectal cancers start as a polyp -- a small, abnormal growth of tissue that can develop on the inner lining of the colon or rectum. Most polyps are harmless (benign), but a certain kind of polyp, known as an adenoma, can become cancerous over
There are two general types of colon polyps:
It can take many years for a polyp to develop into cancer, but once the cancer forms, it can grow into the wall of the colon or rectum. When cancer cells are in the wall, they can then spread to surrounding tissues and, eventually, other parts of the
body. The further the cancer spreads, the more difficult it is to treat.
In general, people with CF will experience many of the same symptoms of colorectal cancer as the general population, including:
At the same time, however, many people report experiencing no symptoms before receiving a colorectal cancer diagnosis, which is why it is sometimes called the “silent cancer.” That is why screening is incredibly important.
Although the Cystic Fibrosis Foundation’s Colorectal Cancer Screening Clinical Care Guidelines recommend that people with CF begin screening at age 40, symptoms can be an indicator to begin screening even earlier. If you think you are experiencing colorectal cancer symptoms, talk to a member of your CF care team.
“Before I was diagnosed, I found blood in my stool one time and then, months later, had two days of stomachaches despite regularly taking my enzymes. At the time, however, I didn’t know about my increased risk for colorectal cancer and attributed it to
CF-related digestion issues. I wish I had said something to my CF doctor about these small symptoms earlier when the cancer was still in its most curable stages.” -- Christine Borland, who had CF and colon cancer
The risk for colorectal cancer in adults with cystic fibrosis is 5-10 times greater compared to the general population. In addition, polyps are thought to develop earlier and progress into cancer more quickly in people with cystic fibrosis.
This risk is even higher (about 20 times) for people with CF who've undergone a solid organ transplantation like a lung transplant. People who have had a transplant are at an increased risk for cancer in general because the medications they must take to prevent rejection work by weakening the immune system.
We don't know for certain why people with CF have an increased risk of developing colorectal cancer, but it is thought to be associated with the cystic fibrosis transmembrane conductance regulator (CFTR) gene mutation. Additional research is being conducted to understand how mechanisms in the bodies of people with CF put them at an increased risk for developing colorectal cancer and, in turn, how to better treat it. Researchers recognize that there is still more to learn and have identified some of those questions in the section titled “Unanswered Questions” in the Colorectal Cancer Screening Guidelines.
An effective way to prevent and treat colorectal cancer is to have regular screenings called colonoscopies.
A colonoscopy allows the gastroenterologist or colonoscopist to identify and remove
polyps before they can develop into cancer. Colonoscopies can also help find the cancer in its earliest (and most treatable) stages.
A colonoscopy is a procedure that uses a long, flexible tube about the width of a finger. This tube is attached to a video camera to view your colon and rectum. If there is anything that looks abnormal, the gastroenterologist or colonoscopist can pass small surgical tools through the tube to take tissue samples for analysis and remove any polyps. The goal
of a colonoscopy is to identify and remove as many polyps as possible. Watch Mark B. Pochapin, MD, FACG, director of the Division of Gastroenterology at New York University Langone Medical Center, narrate a tour of a patient's colon during a colonoscopy where he discovers and removes a pre-cancerous polyp.
“Knowing that people with CF have a higher risk of colon cancer can seem daunting. We already deal with so much and face enough challenges trying to balance daily life with our health. As someone who has had 10-12 colonoscopies in the last decade, they
still slightly stress me out. But, I know I need to have them done so that I can be sure I don't develop cancer. Plus, I'm finally equipped with the knowledge of what to expect and how to get the best results.” -- Amy Braid, an adult with CF, from
the CF Community Blog
Colonoscopy is the only method that has been studied as an effective screening test for people with cystic fibrosis. Unlike other screening procedures, a colonoscopy can detect and remove polyps, which is one of the main reasons why it is the recommended
screening procedure for other high-risk groups, such as those with Lynch syndrome.
“If your endoscopist finds any polyps, he or she will remove them during the procedure. This eliminates the need to return a second time.” -- Amy Braid, an adult with CF, from the CF Community Blog
Although less invasive, screening methods like fecal immunochemical testing (FIT) are not recommended for people with CF because there is a lack of CF-specific
evidence to support the method's effectiveness. If you are interested in stool-based testing, it is important that you discuss this with your CF care team before proceeding with the
Due to the increased risk of developing colorectal cancer, people with CF are generally recommended to begin screening at age 40, 10 years earlier than for the general population.
In general, adults with CF age 40 or older should be screened for colorectal cancer every five years unless they have other symptoms or have had a positive screen in the past.
If you've had a colonoscopy and your physician found adenomatous (pre-cancerous) polyps, you will likely need to come back to get screened every three years.
Because there is an increased risk of developing cancers post-transplant, organ transplant recipients -- such as people who have received a lung transplant -- should generally start screening for colorectal cancer at age 30.
If you are older than 30 when you receive your transplant, it is recommended that you start screening within two years of transplantation (unless you've had a negative colonoscopy within the past five years -- which may be part of the workup depending on where you received your transplant).
A colonoscopy is a medical procedure, which means there are important aspects to consider when you have cystic fibrosis. For example, CF-specific factors, such as lung function, level of cough, and mucus production, can all impact your screening decisions -- particularly when it comes to the type of level of pre-exam sedation you receive.
For this reason, it is important to discuss the timing and appropriateness of screening with your CF care team. Together, you can assess your individual cancer risk and how other medical needs may impact your screening decisions.
Some questions you can ask your care team before a colonoscopy include:
Use this handout to guide your discussion with your endoscopist and CF care team to prepare for a successful colonoscopy.
Although the colonoscopy itself will be performed by a gastroenterologist or colonoscopist who is a clinical specialist outside of your CF care team, let your CF team know if you are planning to have a colonoscopy and encourage open communication with the physician performing your colonoscopy. Working closely with your care team and the specialist will help you coordinate your care by addressing your individual CF-specific needs.
Unlike the typical preparation for a colonoscopy, the bowel preparation for people with CF is more intensive than for the general population. People with CF tend to have thick, sticky intestinal mucus that makes it more difficult to clean out their colons. That's why it's important to carefully review the steps of a CF-specific bowel preparation with your CF care team so that your colonoscopy can be effectively performed the first time it is attempted.
Colonoscopy is generally a low-risk procedure, but there is always a small chance of bleeding and injury, such as colonic perforation, infection, and missed lesions.
“I typically say to my patients, 'There is no perfect test for colon cancer, and even with good preparation, some lesions can be missed. Colonoscopy is the best test currently available to look for and remove colonic polyps, and to identify potential cancers.'” -- James Abraham, MD, a CF gastroenterologist, University of Minnesota
Although the benefits of colorectal cancer screening typically outweigh the risks, it is important to discuss colonoscopy screening with your CF care team and colonoscopist to ensure that colonoscopy is the right screening choice for you.
The CF Foundation supports research focused on discovering why people with CF face increased colorectal cancer risk as well as ways to provide caregivers and people with CF more information about colorectal cancer.
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