How Is Cystic Fibrosis Inherited?

By: Cheryl Bowman

How is Cystic Fibrosis inherited? Cystic Fibrosis is caused by a defect in the CFTR gene. The CFTR gene produces a certain protein that controls salt and water movement in your cells. In people who do not have cystic fibrosis, mucous is watery, but people who have cystic fibrosis have thick, sticky mucous and extremely salty sweat.

Symptoms of Cystic Fibrosis
There are more than 1,000 different defects that can affect the CFTR gene. The defect affecting the gene determines how severe an individual case of Cystic Fibrosis is. This also determines which symptoms are displayed and when those symptoms are displayed. Symptoms may come and go for each person. A person may also exhibit a set of symptoms, then a few months later, exhibit different symptoms.

In children with Cystic Fibrosis, lung function starts to decline early in childhood. The damage is often permanent and causes severe breathing problems over time. The most common cause of death in people who have Cystic Fibrosis is respiratory failure. Cystic Fibrosis management generally focuses on slowing damage to the lungs or preserving lung function. It can include antibiotics, drainage of the airways, bronchiodialator medicines that open lung passages and lung transplants.

Genetics and Cystic Fibrosis
Each child born inherits a CFTR gene from each parent. If the child inherits a faulty CFTR gene from both parents, the child will have Cystic Fibrosis. If the child inherits a faulty CFTR from one parent, the child will be a carrier of Cystic Fibrosis. If this child marries another carrier, the combination of the two faulty genes will result in a child with cystic fibrosis.

The math, however, isn't quite as simple as it seems, since there is only a chance that any given child born to parents with a faulty CFTR gene will inherit the condition. Unless both parents have Cystic Fibrosis, they have one normal CFTR gene and one faulty gene. Since a child inherits genes from both parents, there is a 50% chance that the child will simply have CFTR trait, or one good gene and one faulty gene. There is a 25% chance the child will have two normal genes, and a 25% chance the child will develop Cystic Fibrosis.

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