Home > Health A – Z > Cystic Fibrosis

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. 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 effects of physical exercise on cystic fibrosis: a review of the literature]

Bibliographic details: Schaar B, Platen P.  [The effects of physical exercise on cystic fibrosis: a review of the literature]. [Literaturanalyse: sportliche Activitaten bei zystischer Fibrose.] Atemwegs und Lungenkrankheiten 2006; 32(9): 361-365

Enteral tube feeding for cystic fibrosis

Bibliographic details: Malt G.  Enteral tube feeding for cystic fibrosis. International Journal of Child and Adolescent Health 2011; 4(1): 111-112 Available from: https://www.novapublishers.com/catalog/product_info.php?products_id=21072

Nutritional intervention in patients with cystic fibrosis: a systematic review

BACKGROUND: To systematically assess the literature published after 1997 describing the effectiveness of nutritional interventions in Cystic Fibrosis patients. METHODS: An online search in PUBMED, EMBASE and COCHRANE databases was conducted. Original studies with 4 patients or more, describing a nutritional intervention and giving at least weight as an outcome parameter were included. RESULTS: The inclusion criteria were met by 17 articles, focusing on respectively behavioural interventions (n=6), oral supplementation (n=4) or enteral tube feeding (n=7). This latter intervention was universally successful to induce weight gain. One behavioural study and 2 oral supplementation studies also reported significant weight gain. CONCLUSION: Enteral tube feeding is effective to improve nutritional status, while the described effects of behavioural intervention and oral supplementation are not consistent at present.

See all (273)

Summaries for consumers

Human Growth Hormone for Children With Cystic Fibrosis: A Review of the Research for Parents and Caregivers

This guide is for anyone who takes care of a child or teen with cystic fibrosis (CF) and helps make decisions about treatment. You may have heard about using human growth hormone (HGH) for children with CF and want to know more about the research.

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.

Elective (regular) versus symptomatic intravenous antibiotic therapy for cystic fibrosis

Chronic infection of the airways by Pseudomonas aeruginosa in people with cystic fibrosis is associated with deterioration in respiratory function. Intravenous antibiotics are the standard therapy for pulmonary exacerbations caused by this micro‐organism. Many centres advocate the use of elective (regular) three‐monthly antibiotics to reduce the frequency of exacerbations and therefore slow the deterioration of lung function. Alternatively, intravenous antibiotics are only prescribed when symptoms indicate. Elective therapy may encourage multi‐resistance to antibiotics. This review aimed to identify randomised and quasi‐randomised controlled trials that evaluated the results of the two different approaches. No clear conclusions were identified.

See all (151)

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.
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.
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

Photo of a child

Also called: Mucoviscidosis, Fibrocystic disease

Other terms to know: See all 9
Body Fiber, Exocrine Glands, Fibrosis

Keep up with systematic reviews on Cystic Fibrosis:

RSS

PubMed Health Blog...

read all...