Effects of Cystic Fibrosis: How Organs Are Affected

The Effect of a Defective Gene on the Body

Doctors looking at xray of lungs
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Cystic fibrosis (CF) is a chronic, life-shortening disease that occurs as a result of a genetic defect. The defective gene affects the body’s ability to transfer water and salt to and from cells that produce sweat, mucus, and digestive enzymes. This causes secretions, which are normally thin and watery in healthy people, to become very thick and sticky. The thick secretions clog up organs and prevent them from working properly.

The Cystic Fibrosis Effect

In CF, many body organ and systems are affected, including:

  • respiratory system (specific organs such as the lungs and other structures in the body that allow you to breathe and circulate oxygenated blood throughout the body)
  • gastrointestinal system (relating to the stomach and the intestines)
  • musculoskeletal system (the muscles and skeleton)
  • genitourinary system (relating to the genital and urinary organs)
  • reproductive system (system of sex organs including fluids, hormones, and pheromones)

Particular Organs Affected by CF

Some of the major organs affected by CF include the lungs, pancreas, liver and gallbladder, intestines and the reproductive organs.

Lungs

There is abnormal mucus production along the respiratory tract due to the faulty transport of electrolytes across airway tissues. This mucus drys out easily and is hard to clear from the airway, which can cause blockage, damage the alveoli (tiny air sacs) in the lungs and allow for foreign particles to build up (causing chronic lung infections)

Cystic fibrosis causes problems in the lungs when the thick mucus builds up and gets stuck in the airways.

When this happens:

  • Breathing passageways become blocked and air can’t get through.

  • Bacteria grow in the mucus collections and causes infection in the lungs, nose, and sinuses.

  • Nasal polyps may develop in some CF patients.

    Pancreas

    The pancreas is part of the digestive system. Its job is to secrete enzymes that are needed to digest food, and a hormone called insulin that controls blood sugar. Cystic fibrosis also causes these secretions to become thick.

    When this happens:

    • The pancreatic ducts become clogged.

    • Enzymes can’t get past the obstruction.

    • Food is not digested properly and the body can’t absorb nutrients.

    • Eventually, the obstruction of the pancreas may cause scarring that damages the insulin-producing cells and prevents them from producing insulin. Insulin is not available to the cells, which causes blood sugar levels to rise. This condition is called insulin-dependent diabetes, which happens in about 15% of all CF patients.

    Liver and Gallbladder

    Although it is not common, thickened secretions can also clog up the bile ducts of the liver and gallbladder and prevent them from working properly.

    If blockage of ducts in the liver continues for long periods of time, the liver could become permanently damaged. If the ducts of the gallbladder become clogged, the gallbladder is usually removed.

    Intestines

    Sometimes, the intestines are the first organ to be affected by cystic fibrosis. In about 20% of all newborns with CF, thick secretions form in the intestines causing a life-threatening blockage of the intestines known as a meconium ileus.

    Reproductive Organs

    Cystic fibrosis affects the reproductive organs of men and women differently and in the following ways: 

    • Men are almost always infertile because the thick secretions in the sperm canal cause a blockage that prevents sperm from getting through.
    • Women are often able to conceive but may have decreased fertility because of thick cervical mucus blocking the entry of sperm.

    Source:
    Cystic Fibrosis. All Children's Hospital. Health Information.

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