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Diseases

Genetic and Rare Diseases Information Center (GARD)

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Cystic fibrosis


Other Names for this Disease
  • CF
  • Mucoviscidosis
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Your Question

Could you please send any and all information on Cystic Fibrosis.  One of the staff members has a child with CF and stated the child is not suppose to be in the same classroom with another child with CF.  I would like to know why that is? I assume it is related to the altered immunity state. How does this work?   What do they do to address this problem?

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

What is cystic fibrosis?

Cystic fibrosis (CF) is an inherited condition that causes mucus to build up and clog some of the organs in the body, particularly in the lungs and pancreas. When mucus clogs the lungs, it can make breathing very difficult. The thick mucus also causes bacteria to get stuck in the airways, which causes inflammation and infections. These infections can cause chronic coughing, and wheezing. Over time, mucus buildup and infections can lead to permanent lung damage, including the formation of scar tissue (fibrosis) and cysts in the lungs. Mucus can also block the digestive tract and pancreas, leading to digestive problems. CF is caused by mutations in the CFTR gene and is inherited in an autosomal recessive pattern.[1]
Last updated: 4/4/2013

What causes cystic fibrosis?

Mutations in a single gene, namely the Cystic Fibrosis Transmembrane Regulator (CFTR) gene, cause CF. More than 900 mutations in this gene have been identified. This gene provides the instructions for the CFTR protein. In normal cells, the CFTR protein acts as a channel that allows cells to release chloride and other ions. But in people with CF, this protein is defective and the cells do not release the chloride. The result is an improper salt balance in the cells which leads to thick, sticky mucus.[2]
Last updated: 8/23/2011

Should a child with cystic fibrosis be in the same classroom in school with another child with CF?

Ideally, children with CF should not be in the same classroom together. CF-specific germs, such as Pseudomonas aeruginosa (Pseudomonas) and Burkholderia cepacia complex, usually are not harmful to people who do not have CF. However, these germs can be harmful to others with CF. If there is more than one person with CF in the school, special attention should be paid to infection control guidelines. Affected individuals should not be in shared spaces at the same time (e.g., the library, gymnasium, or computer room). It is critical that the nurse and the nurse’s aide understand the higher risk of infection for an individual with CF and make sure to practice good hand hygiene. If a child does a respiratory treatment in the nurse’s office, the area should be disinfected before and after the treatment. Families of affected children should work with their CF care teams to educate their schools, day cares, and other non-health care settings about CF and how to avoid germs. The Cystic Fibrosis Foundation has educational material that can be used to educate schools in this situation. They can be contacted at info@cff.org or (800) FIGHT CF.[3]
Last updated: 6/19/2012

References