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Cystic Fibrosis (CF)

A common hereditary disease in which exocrine (secretory) glands produce abnormally thick mucus. This mucus can cause problems in digestion, breathing, and body cooling.

PubMed Health Glossary
(Source: NIH - National Cancer Institute)

Cystic Fibrosis

Cystic fibrosis (SIS-tik fi-BRO-sis), or CF, is an inherited disease of the secretory (see-KREH-tor-ee) glands. Secretory glands include glands that make mucus and sweat.

"Inherited" means the disease is passed from parents to children through genes. People who have CF inherit two faulty genes for the disease - one from each parent. The parents likely don't have the disease themselves.

CF mainly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.

Overview

Mucus is a substance made by tissues that line some organs and body cavities, such as the lungs and nose. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected.

If you have CF, your mucus becomes thick and sticky. It builds up in your lungs and blocks your airways. (Airways are tubes that carry air in and out of your lungs.)

The... Read more about Cystic Fibrosis

What works? Research summarized

Evidence reviews

The use of regular vitamin A preparations for children and adults with cystic fibrosis

Cystic fibrosis can lead to certain vitamins, such as vitamin A, not being properly absorbed by the body. This can result in problems caused by vitamin deficiency. A lack of vitamin A (vitamin A deficiency) can cause specific problems such as eye and skin problems. It can also be associated with poorer general and respiratory health. Therefore people with cystic fibrosis are usually given regular vitamin A preparations from a very young age. However, too much vitamin A can also cause respiratory and bone problems. The review aimed to show whether giving vitamin A regularly to people with cystic fibrosis is beneficial or not. However, the authors did not find any relevant trials to include in the review. They are therefore unable to draw any conclusions regarding the routine administration of vitamin A supplements and recommend that until further evidence is available, local guidelines are followed regarding this practice.

Pneumococcal vaccines for children and adults with cystic fibrosis

We reviewed the evidence about the effect of vaccinating people with cystic fibrosis against infection with pneumococcal bacteria.

The training of muscles that cause the chest to expand in order to take air into the lungs for people with cystic fibrosis

Cystic fibrosis is the most common life‐limiting genetic condition in Caucasians. The life‐expectancy of newly diagnosed patients is increasing. Inspiratory muscle training may improve quality of life, lung function and exercise tolerance in people with cystic fibrosis, so that these are closer to the levels found in people who do not have cystic fibrosis. It may also boost the clearance of mucus. Inspiratory muscle training can be performed without the help of a carer and wherever the individual feels appropriate. We searched for randomised or quasi‐randomised controlled clinical trials to determine the effects of inspiratory muscle training in the treatment of people with cystic fibrosis. We included eight studies with 180 participants in the review, but we could not combine results from these studies to answer our questions, because the studies either did not publish enough details or did not use the same standard measurements. Given this, we cannot recommend the use, or not, of this intervention. We do recommend that future studies make more use of health‐related quality of life and exercise tolerance measures. We also suggest there should be agreement upon standard measurements used.

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Summaries for consumers

The use of regular vitamin A preparations for children and adults with cystic fibrosis

Cystic fibrosis can lead to certain vitamins, such as vitamin A, not being properly absorbed by the body. This can result in problems caused by vitamin deficiency. A lack of vitamin A (vitamin A deficiency) can cause specific problems such as eye and skin problems. It can also be associated with poorer general and respiratory health. Therefore people with cystic fibrosis are usually given regular vitamin A preparations from a very young age. However, too much vitamin A can also cause respiratory and bone problems. The review aimed to show whether giving vitamin A regularly to people with cystic fibrosis is beneficial or not. However, the authors did not find any relevant trials to include in the review. They are therefore unable to draw any conclusions regarding the routine administration of vitamin A supplements and recommend that until further evidence is available, local guidelines are followed regarding this practice.

Pneumococcal vaccines for children and adults with cystic fibrosis

We reviewed the evidence about the effect of vaccinating people with cystic fibrosis against infection with pneumococcal bacteria.

The training of muscles that cause the chest to expand in order to take air into the lungs for people with cystic fibrosis

Cystic fibrosis is the most common life‐limiting genetic condition in Caucasians. The life‐expectancy of newly diagnosed patients is increasing. Inspiratory muscle training may improve quality of life, lung function and exercise tolerance in people with cystic fibrosis, so that these are closer to the levels found in people who do not have cystic fibrosis. It may also boost the clearance of mucus. Inspiratory muscle training can be performed without the help of a carer and wherever the individual feels appropriate. We searched for randomised or quasi‐randomised controlled clinical trials to determine the effects of inspiratory muscle training in the treatment of people with cystic fibrosis. We included eight studies with 180 participants in the review, but we could not combine results from these studies to answer our questions, because the studies either did not publish enough details or did not use the same standard measurements. Given this, we cannot recommend the use, or not, of this intervention. We do recommend that future studies make more use of health‐related quality of life and exercise tolerance measures. We also suggest there should be agreement upon standard measurements used.

See all (175)

Terms to know

Body Fiber
In the body, fiber refers to tissue made of long threadlike cells, such as muscle fiber or nerve fiber.
Exocrine Glands
An organ that makes one or more substances, such as sweat, tears, saliva, or milk. Exocrine glands release the substances into a duct or opening to the inside or outside of the body.
Fibrosis
Fibrosis is the formation of excess fibrous connective tissue in an organ or tissue. Fibrosis is similar to the process of scarring.
Genetic
Inherited; having to do with information that is passed from parents to offspring through genes in sperm and egg cells.
Glands
A group of cells that secrete substances. Endocrine glands secrete hormones. Exocrine glands secrete salt, enzymes, and water.
Mucus
A thick, slippery fluid made by the membranes that line certain organs of the body, including the nose, mouth, throat, and vagina.
Phlegm
A more than normal amount of thick mucus made by the cells lining the upper airways and lungs. A buildup of phlegm may be caused by infection, irritation, or chronic lung disease, and can cause discomfort in the chest and coughing.
Reproductive System
The organs involved in producing offspring. In women, this system includes the ovaries, the fallopian tubes, the uterus, the cervix, and the vagina. In men, it includes the prostate, the testes, and the penis.
Respiratory System
The organs that are involved in breathing. These include the nose, throat, larynx, trachea, bronchi, and lungs. Also called respiratory tract.
Secretion
To form and release a substance. In the body, cells secrete substances, such as sweat that cools the body or hormones that act in other parts of the body.
Sputum
Mucus and other matter brought up from the lungs by coughing.
Sweat
Fluid that exits the body through pores in the skin usually due to physical stress and/or high temperature for the purpose of regulating body temperature and removing certain compounds from the circulation.

More about Cystic Fibrosis

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Also called: Mucoviscidosis, Fibrocystic disease

Other terms to know: See all 12
Body Fiber, Exocrine Glands, Fibrosis

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