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About Cystic Fibrosis

What is Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited chronic disease that affects the respiratory, digestive and reproductive systems. Mutations of a specific gene (the CFTR) affect the transfer of salts and chlorides into and out of cells that line the organs of these three systems. This defective gene causes the production of an unusually thick, sticky mucus.

For those without cystic fibrosis, mucus is watery. It protects the linings of organs and moves bacteria out of the body. However, for those with cystic fibrosis, thick and sticky mucus builds up and clogs the small airways and passageways of the respiratory, digestive, and reproductive systems. Rather than carry harmful bacteria out of the body, the mucus traps bacteria, which leads to serious infections.

Respiratory System

In the respiratory system of a person with cystic fibrosis, airways become obstructed. Mucus builds in the lungs, leading to severe breathing problems. Trapped bacteria create inflammation and lung infections. Eventually, the damaged lungs lose capacity and elasticity, which can result in permanent damage and lung function decline. Medications for lung infections and treatments to dislodge the mucus are part of a daily regimen for people with cystic fibrosis.

Sinuses are also covered with a mucus layer. In people with cystic fibrosis, the thick and sticky mucus traps bacteria, leading to chronic sinusitis for many people. Treatments such as nasal saline irrigations and medications such as antihistamines, corticosteroids, and antibiotics are frequently used to treat sinusitis.

Digestive/Endocrine System

Thick mucus can also block tubes or ducts, such as the pancreatic duct, in the digestive system, causing severe digestive problems. The pancreas produces insulin and makes enzymes that help digest food. When the pancreatic duct is blocked, these enzymes cannot reach the intestines.

Without these enzymes, the body cannot properly absorb nutrition from food, regardless of how much a person eats. The resulting malnutrition compromises the body’s immune system and can lead to diarrhea, poor growth and /or weight loss. Most people with cystic fibrosis take enzyme replacement before every meal (and with some snacks) to digest food properly. They also take supplemental vitamins and maintain a diet that maximizes healthy weight gain.

Some babies with cystic fibrosis are born with a blockage of the intestine, a condition known as meconium ileus. This requires immediate surgery, which can correct the problem. Other organs in the digestive system, such as the intestines and the liver, can also be compromised.

As they grow, people with cystic fibrosis are likely to develop cystic fibrosis–related diabetes (CFRD). While different from Type I and Type II diabetes, CFRD is treated with the same vigilance in monitoring the body’s blood sugar levels and administering insulin. Poorly managed CFRD is associated with increased infections and lung function decline.

Reproductive System

The reproductive system is also affected by cystic fibrosis, which can cause infertility, particularly in men. At least 97 percent of men with cystic fibrosis are infertile but are not sterile; they can have children with assisted reproductive techniques. These men make normal sperm but the vas deferens is blocked or absent. Some women also have fertility difficulties due to thickened cervical mucus or malnutrition.


For more information, enter cystic fibrosis in the epocrates.com box here and click "Go".

 

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