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.
The Digestive Tract
If you have cystic fibrosis, you have to pay more attention than most people to what you eat because sticky mucus in the pancreas interferes with the proper digestion of your food and causes blockages in the intestines.
Your digestive tract, which may also be called the “gastrointestinal tract” or “GI tract,” is the route your food follows after you put it in your mouth.
The GI tract is where the food you eat is turned into the nutrients your body needs to do the things you want to do. It starts with your mouth, teeth and tongue.
Once you swallow, the food goes down your esophagus and into the stomach, where it is broken down into a pulp. From your stomach, the pulpy mixture moves into the small intestine, where the digestive enzymes and bicarbonate from the pancreas and bile from the liver help digest the food further so your body can use the nutrients
The last parts of the GI tract are the large intestine, rectum and anus -- this is where the unusable bits of food you ate, along with bacteria, move out of your body.
Once you chew and swallow, your food goes down the esophagus and into the stomach. The stomach makes acid that helps break down the food. Next, the food moves into the small intestine where the body starts taking the nutrients and vitamins out of the food.
To extract these nutrients, the body needs the help of the pancreas. This organ, shaped like a small fish, is attached to the small intestine behind your stomach. It is crucial for proper digestion.
The pancreas makes fluid -- from 1 to 2 quarts -- that mixes with the food. One part of that fluid is a product that neutralizes the acid from the stomach. It is called bicarbonate. The fluid also contains chemicals called pancreatic enzymes, which mix with the food to break
them into small bits the body can absorb.
Pancreatic enzymes are very important for getting nutrients, calories and vitamins into our bodies; they are the heart of proper digestion and absorption.
In addition to playing these important roles in the digestive process, the pancreas also makes important hormones, including insulin. Insulin works in your bloodstream, not in your intestines. It helps move glucose (sugar) from the blood into the cells so they can turn food into
For many people with CF, the pancreas doesn't produce enough insulin or the body doesn't let insulin do its job, leading to
CF-related diabetes (CFRD). CFRD is not caused by diet, and there is nothing you can do to prevent it.
One way to simplify this is to think of the pancreas as two separate organs: one is a digestive organ that makes pancreatic enzymes for the intestines, the other is a hormone organ that makes insulin for the body.
The healthy pancreas:
About 90 percent of people with CF have sticky mucus that blocks ducts in the pancreas and prevents enzymes from reaching the small intestine to digest food. Undigested food in the intestines can cause pain, cramping, gas and either loose, greasy, floating stools or constipation
Everyone with CF (including people who don't need enzyme supplements) has a pancreas that does not make enough bicarbonate to neutralize stomach acid. This can also contribute to pain, cramping, gas and constipation. Bloating and excessive gas also can be caused by small bowel
overgrowth, gastric paresis and gastroesophageal reflux disease.
The webcast below highlights the following information:
Read the Foundation's care guidelines on pancreatic enzyme replacement.
Among its many functions, the liver makes a fluid called bile that helps the body absorb fat. Bile travels through small tubes or ducts in the liver and is stored in the gallbladder, which empties the bile into the small intestine.
In some people with CF, bile gets thick and flows very slowly. It can even get so thick that it forms stones in the gallbladder, which sometimes need to be removed. You can still have normal digestion if your gallbladder has been removed.
As the mixture of food, pancreatic fluid, bile and pancreatic enzymes moves along the small intestine, the important nutrients make their way into the body by absorption through special cells in the walls of the small intestine.
Below the small intestine comes the large intestine. A few nutrients are taken in by the large intestine, but its more important function is to keep the balance of fluid just right in the mass of what remains of the digested food.
The very end of the large intestine is called the rectum. This is where the mass of fully digested food and bacteria leaves your body in the form of stool, or bowel movement. As you probably know by now, your care team will usually ask you about your bowel movements.
The CF Foundation supports research focusing on the treatment and research of GI issues in CF.
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