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.
Cystic Fibrosis-Related Diabetes
Cystic fibrosis-related diabetes (CFRD) is a unique type of diabetes that is common in people with CF. With early diagnosis and proper treatment, CFRD can be managed successfully.
There are two types of diabetes in people without cystic fibrosis: type 1 and type 2. Type 1 diabetes is an autoimmune disease that occurs when the body's immune system attacks and destroys the cells in the pancreas that make insulin (beta cells), which helps the body absorb and use the energy from food. Type 1 diabetes is most commonly diagnosed in childhood and requires you to take insulin to stay alive.
Type 2 diabetes, which occurs more often in adults who are overweight or obese, is caused by the lack of a normal response to insulin in addition to the pancreas not making enough insulin.
Cystic fibrosis-related diabetes (CFRD) shares some features with both type 1 and type 2 diabetes. In people with CF, the thick, sticky mucus that is characteristic of the disease causes scarring of the pancreas. This scarring prevents the pancreas from producing normal amounts of insulin; so, like people with type 1 diabetes, they become insulin deficient. Their pancreas still makes some insulin, but not enough to stay healthy and maintain good nutrition.
Additionally, people with CFRD may not respond to insulin in the right way like people with type 2 diabetes do, especially when sick, taking steroid medication, or pregnant. This is referred to as being "insulin resistant."
People with CFRD may not experience any symptoms. Some diabetes symptoms are similar to other CF symptoms you may already have. Many people with CFRD do not know they have CFRD until they are tested for diabetes.
Some common symptoms, like increased thirst and urination, are caused by high blood sugar levels, known as hyperglycemia. Other symptoms of CFRD are excessive fatigue, weight loss, and unexplained decline in lung function.
The CF care guidelines for CFRD recommend that people with CF ages 10 and older be tested every year for CFRD with an oral glucose tolerance test (OGTT). The OGTT is the best way to diagnose CFRD and is usually done in the morning after an eight-hour fast. If you are diagnosed with CFRD, you will receive proper treatment in order to feel better, gain weight, and improve your lung function.
It is important to note that OGTT is different than hemoglobin A1c (HbA1c). HbA1c is not recommended as a screening test for CFRD since it is often low in people with CF. This marks another important difference between CFRD and other types of diabetes.
Listen to Andrea Kelly, M.D., talk about CFRD and what's changed since she's been in the field of CF.
The goal for treating CFRD is to keep blood sugar (glucose) at normal or near-normal levels. Doing so will help you gain weight, maintain muscle mass, feel better, and have more energy. Maintaining normal glucose levels also lowers the risk of problems
caused by diabetes.
CFRD can be well managed with insulin, along with monitoring your blood sugar levels, eating your usual high-calorie diet,
and staying active.
“Many people with CFRD are unsure about what to eat to manage their blood sugar levels. Fortunately, certain meal planning techniques can help.” -- Gretchen Garlow, MS, RD, LDN, a CF dietitian, from the CF Community Blog
There are many types of insulin, which are grouped by how fast they work and how long they last in the body. Insulin is injected into the body and helps your body cells absorb the energy (calories) from the food you eat. Calories in food come from carbohydrates,
protein, and fat. Insulin helps the body cells absorb these three nutrients so that you can achieve and maintain a healthy body weight and good nutritional status.
People with CFRD still need to eat the same high-calorie, high-protein,
high-fat, and high-salt diet to help achieve and maintain a healthy body weight. Since foods with carbohydrates turn into blood glucose when eaten, people with CFRD need to count the carbohydrates in the foods they eat so they can give themselves
the right amount of insulin. Your diabetes care team will help you determine the amount of insulin you need for your carbohydrates.
Physical activity like
exercise is good for lung function and can also improve your body's response to insulin. People with CFRD are encouraged to do at least 150 minutes of some type of moderate
aerobic exercise -- activities that require you to breathe in oxygen, like jogging or playing sports -- every week. Monitoring your blood sugar levels will allow you to remain active since exercise may cause the levels to drop when muscles use the
sugar for energy.
“If you have CFRD, be sure to check your blood sugar before and after exercise.” -- Lee Degiorgio, an adult with CF, from the CF Community Blog
If you are diagnosed with CFRD, your CF care team may expand to include an endocrinologist (a doctor with special training in the treatment of diabetes) and certified diabetes educators. Working with you, this team will design a treatment plan to help
you manage your diabetes while also maintaining your health with CF.
Download the manual, "Managing Cystic Fibrosis-Related Diabetes, An Instruction Guide for Patients and Families."
The manual is also available in Spanish:
"El Manejo de la Diabetes Relacionada con la Fibrosis Quística."
Learn how you can manage CFRD while traveling.
The CF Foundation has funded researchers who are investigating the effect that the cystic fibrosis transmembrane conductance regulator (CFTR) protein has on the development of CFRD to find ways to treat it.
In this video, a panel of research experts and community members discuss several research studies aimed at improving CFRD diagnosis and treatment.
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