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.
Taking the right medications not only helps you meet your nutrition goals but also it prevents you from harming your health.
Most people with cystic fibrosis need to take pancreatic enzyme capsules with every meal and snack. The enzyme dosing guidelines are based on consensus rather than research. Doses vary greatly between individuals with CF. Avoid making dosing changes on your own. Always talk to your health care provider first.
The stomach makes acid. This acid can leak back from the stomach into the esophagus. This is called heartburn, acid reflux or gastroesophageal reflux disease (GERD in medical talk).
Coughing can make this worse, so people with cystic fibrosis may be more likely to have GERD. The acid reflux can cause injury to the esophagus. It also can trigger cough or asthma.
Your CF care team may prescribe medicine for this problem. These medications include:
Proton pump inhibitors
Old-fashioned, over-the-counter antacids, such as simethicone (Maalox® or Mylanta®), may be used along with -- not instead of -- proton pump inhibitors.
In addition to treating GERD, H2 blockers and proton pump inhibitors also can help reduce acid in the intestines to help the enzymes work better.
Other ways to treat GERD include:
There also are some surgical procedures that tighten the muscle where the esophagus meets the stomach. Your physician may want to refer you to a stomach specialist (gastroenterologist) if GERD becomes an ongoing problem. You can use the American Gastroenterological Association's search tool to find a doctor who is in your area.
In addition to making the digestive juices, the pancreas normally squirts 1 to 2 quarts of fluid into the intestines every day. The production of this intestinal fluid is often limited in people with CF, even if they make a normal amount of pancreatic enzymes.
As in the lungs, the intestines in CF can make thick mucus. If you have this thick mucus but not enough fluid in the intestines, you can get constipated or even develop a bowel obstruction. This can be a serious problem and may lead to surgery if it becomes very stubborn.
Keep these problems in mind if you are having abdominal pain and a hard time moving your bowels. Drinking plenty of fluids may help. The over-the-counter medication polyethylene glycol is very effective in reversing hard stools and constipation. This is available at most pharmacies and grocery stores. Brand names of polyethylene glycol 3350 include MiraLAX®, GoLYTELY® and NuLYTELY®, but generics are also available.
Two CF conditions make it hard to move your bowels:
The webcast below highlights the following information:
Mild cases of DIOS may be treated at home with polyethylene glycol electrolyte solutions (MiraLAX® or GoLYTELY®). Severe DIOS needs to be treated in the hospital. You should call a doctor if you have:
Treatments can include a nasogastric tube (NG), which goes through your nose and into your stomach to drain your intestine. Sometimes special enemas are used. Other treatments could include large-volume medication, such as polyethylene glycol electrolyte solutions (GoLYTELY®, MiraLAX® or NuLYTELY®), which can either be drunk or given through an NG tube.
These medicines draw water into the intestines and wash through the intestines to move blockages. If you have ever had DIOS, you probably need to be on a regular medicine every day to help your intestines move along.
You can learn more about these and other gastrointestinal medications at DailyMed, a service from the National Library of Medicine that provides information about drugs, including possible side effects. You can learn more about specific gastrointestinal medications by searching for the medication from the main DailyMed page. Please note that information about dosages apply to the general public and not necessarily to people with CF. For specific dosing information, talk to your CF care team.
Everyone's intestines have bacteria. We normally live in harmony with the bacteria. But CF can change that balance.
With CF, instead of having a wide variety of healthy bacteria, the intestines sometimes have only one or two strains. Also, instead of staying in the large intestine, bacteria may move up into the small intestine, where they can compete with our bodies for food. The bacteria can create gas and cause inflammation of the lining of the intestine, leading to pain, weight loss or diarrhea.
Your CF doctor may want to do a special test to look for bacterial overgrowth in the small intestine or may want to treat you with a special antibiotic to fix this problem (which can come and go).
Antibiotics kill both “good” and “bad” bacteria, so you may also want to consider daily use of over-the-counter products called probiotics, which are live microorganisms. These are “good” bacteria that you can take in pill form or by eating foods such as yogurt or milk with L. acidophilus. There are supplements that offer a source of probiotics in combination with calories and protein. Food sources of probiotics can be more convenient and enjoyable than taking a probiotic in pill form. Some of these foods include:
Discuss with your CF dietitian and your CF doctor whether eating these foods is safe for you.
For more information on probiotics, visit the National Center for Complementary and Integrative Health.
Things you can do to keep your GI tract as healthy as possible:
Reference to any specific product, process or service does not necessarily constitute or imply its endorsement, recommendation or favoring by the Cystic Fibrosis Foundation. The appearance of external hyperlinks does not constitute endorsement by the Cystic Fibrosis Foundation of the linked websites, or the information, products or services contained therein.
Information contained on this site does not cover all possible uses, actions, precautions, side effects or interactions. This site is not intended as a substitute for treatment advice from a medical professional. Consult your doctor before making any changes to your treatment.
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