How much do you know about cystic fibrosis (CF) progression?
Put your knowledge to the test with CF Fact or Fiction.
If someone has a mutation in one of their CFTR genes, it means they’ll have CF.
Here's the truth: In order for someone to have CF, they must have a mutation in both of their CFTR genes.
CF is only a disease of the lungs.
Here's the truth: CF affects many different systems of the body.
Having high lung function means that CF is not progressing.
Here's the truth: Lung function tests don't always detect early lung damage, so lung damage may be present even when lung function is high.
Permanent lung damage in CF begins in early adulthood.
Here's the truth: Permanent lung damage can also happen in children with CF.
Scarring causes CF to progress throughout the body.
Scarring in different organs causes them to lose function over time.
CFRD and cirrhosis are serious, but not life-threatening complications of CF.
Here's the truth: CFRD and cirrhosis can be life-threatening, so people with CF are tested regularly for these conditions.
Some effects of CF may have no symptoms.
Many effects of CF are silent at first. For example, lung damage may be occurring before it can be detected by some tests, and many conditions in the digestive system do not have noticeable symptoms early on.
Lung function decline happens to everyone, even people who don’t have CF.
Losing lung function is a natural part of aging for everyone. But it happens more quickly in someone with CF.
CF Video Source
Check out the videos below to take a deeper look at CF.
Frequently Asked Questions
What is a genetic disease?
A genetic disease is a disease you are born with, like CF. It's inherited from your parents through your genes.
What causes CF?
CF is the result of 2 CFTR gene mutations that produce defective CFTR proteins. These proteins are not built correctly, so they don’t work the way they should. This causes a buildup of thick, sticky mucus throughout different parts of the body that can cause lasting damage over time.
What is a CFTR protein?
CFTR proteins are found on the surface of certain cells in different parts of the body. They help the body to maintain a healthy balance of salt and water that keeps mucus thin and watery. Thin and watery mucus is important for normal functioning of organs such as the lungs, pancreas, liver, and intestines.
Who gets CF?
CF is a genetic disease, which means you inherit it from your parents. It is most common in Caucasian populations.
How many people have CF?
There are approximately 30,000 people living with CF in the United States.
What are the symptoms of CF?
People with CF experience a wide variety of symptoms, including:
- Wheezing, shortness of breath, and persistent coughing
- Lung infections and lung disease
- Difficulty digesting food
- Poor growth, delayed puberty, and slow weight gain
- Salty sweat
How does CF affect the body over time?
CF affects different parts of the body in different ways over the course of a lifetime. This includes the lungs, digestive system, liver, and bones. Over time, the symptoms of CF can also cause other conditions.
What does disease progression mean for CF?
Progression can occur when the thick, sticky mucus building up throughout the body causes inflammation and scarring in many different organs, leading to permanent damage.
You may find this glossary to be a useful reference as you learn more about CF.
Progression of CF is the buildup of permanent damage throughout certain organs in the body that can lead to a loss of function.
The Cystic Fibrosis Questionnaire–Revised (CFQ-R) is a questionnaire used to measure the impact of a treatment on a person’s overall health, daily life, well-being, and symptoms.
Cystic fibrosis transmembrane conductance regulator (CFTR) gene
The CFTR gene tells the cell how to produce CFTR proteins, which are channels that control the movement of salt and water into and out of certain cells in a number of organs, including the lungs.
This stands for forced expiratory volume in 1 second, which measures the amount of air a person can exhale in 1 second. It is used to assess how well a person’s lungs are functioning.
Sometimes changes or mistakes occur in genes. These are called mutations. Some mutations may cause the gene to behave differently than normal. This can result in a person having certain diseases like cystic fibrosis.
Genes are like an instruction manual for the body. They carry the code that determines our physical characteristics (eg, height, eye, hair color, and other characteristics). People have 2 copies of most genes, 1 copy from their father and 1 from their mother.
Part of the digestive system found just behind your stomach. It produces enzymes (which help break down food) and insulin (which helps to keep the amount of glucose in the blood stable).
Substances produced by the pancreas, which help break food into very small fragments that can be absorbed into the bloodstream.
Periods of worsening respiratory symptoms in people with CF, which may require antibiotic treatment and/or a stay in the hospital.
These resources may help you learn more about CF and the latest research or manage your treatment routines.
CF & Progression: Understanding the Underlying Impact
This guide can help you understand the progression of CF and prepare to discuss it at the next care center visit.
Help for Your Disease Management Routine
CFDaily is a free iPhone® app for people with CF. This app is designed especially for people with CF and their caregivers to help manage their treatment routines.
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