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You are here: Home » eGM Resources » Health Information & Resources Portal|Home » Cystic Fibrosis

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Cystic Fibrosis

What Is Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited disease of your mucus and sweat glands. It affects mostly your lungs, pancreas, liver, intestines, sinuses, and sex organs.

girl undergoes a sweat test

A young cystic fibrosis patient undergoes a sweat test, which is an important diagnostic and therapy-monitoring tool. Photo courtesy of Singeli Agnew for the Cystic Fibrosis Foundation and the National Center for Research Resources.

Normally, mucus is watery. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. But in CF, an abnormal gene causes mucus to become thick and sticky. The mucus builds up in your lungs and blocks the airways. This makes it easy for bacteria to grow and leads to repeated serious lung infections. Over time, these infections can cause serious damage to your lungs. The thick, sticky mucus can also block tubes, or ducts, in your pancreas. As a result, digestive enzymes that are produced by your pancreas cannot reach your small intestine. These enzymes help break down the food that you eat. Without them, your intestines cannot absorb fats and proteins fully. As a result:

  • Nutrients leave your body unused, and you can become malnourished.
  • Your stools become bulky.
  • You may not get enough vitamins A, D, E, and K.
  • You may have intestinal gas, a swollen belly, and pain or discomfort.

The abnormal gene also causes your sweat to become extremely salty. As a result, when you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood. The imbalance may cause you to have a heat emergency.

CF can also cause infertility (mostly in men). The symptoms and severity of CF vary from person to person. Some people with CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they are adolescents or young adults. Respiratory failure is the most common cause of death in people with CF. Until the 1980s, most deaths from CF occurred in children and teenagers. Today, with improved treatments, people with CF live, on average, to be more than 35 years old.

Causes

Cystic fibrosis (CF) is caused by a defect in a gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene. This gene makes a protein that controls the movement of salt and water in and out of the cells in your body. In people with CF, the gene does not work effectively. This causes the thick, sticky mucus and very salty sweat that are the main features of CF.

proposed structure for CFTR

Image courtesy of Celia Hooper, Journal of NIH Research, Nov-Dec. 1989.

Each of us inherits two CFTR genes, one from each parent.

  • Children who inherit an abnormal CFTR gene from each parent will have CF.
  • Children who inherit an abnormal CFTR gene from one parent and a normal CFTR gene from the other parent will not have CF. They will be CF carriers.

CF carriers:

  • Usually have no symptoms of CF
  • Live normal lives
  • Can pass the abnormal CFTR gene on to their children

When two CF carriers have a baby, the baby has a:

  • One in four chance of inheriting two abnormal CFTR genes and having CF.
  • One in four chance of inheriting two normal CFTR genes and not having CF or being a carrier.
  • Two in four chance of inheriting one normal CFTR gene and one abnormal CFTR gene. The baby will not have CF but will be a CF carrier like its parents.

Who is at Risk

About 30,000 people in the United States have cystic fibrosis (CF).

  • It affects both males and females.
  • It affects people from all racial and ethnic groups but is most common among Caucasians whose ancestors came from northern Europe.

CF is one of the most common inherited diseases among Caucasians. About 1 in every 3,000 babies born in the United States has CF. CF is also common in Latinos and Native Americans, especially the Pueblo and Zuni. CF is much less common among African Americans and Asian Americans.

About 12 million Americans are carriers of an abnormal CF gene. Many of them do not know that they are CF carriers.

inheritance of CF

Image courtesy of NHLBI.

Signs and Symptoms

Most of the symptoms of cystic fibrosis (CF) are caused by the thick, sticky mucus. The most common symptoms include:

  • Frequent coughing that brings up thick sputum, or phlegm (flem).
  • Frequent bouts of bronchitis and pneumonia. They can lead to inflammation and permanent lung damage.
  • Salty-tasting skin.
  • Dehydration.
  • Infertility (mostly in men).
  • Ongoing diarrhea or bulky, foul-smelling, and greasy stools.
  • Huge appetite but poor weight gain and growth. This is called "failure to thrive." It is a result of chronic malnutrition because you do not get enough nutrients from your food.
  • Stomach pain and discomfort caused by too much gas in your intestines.

CF can also lead to other medical problems, including:

  • Sinusitis » The sinuses are air-filled spaces behind your eyes, nose, and forehead. They produce mucus and help keep the lining of your nose moist. When the sinuses become swollen, they get blocked with mucus and can become infected. Most people with CF develop sinusitis.
  • Bronchiectasis » Bronchiectasis is a lung disease in which the bronchial tubes, or large airways in your lungs, become stretched out and flabby over time and form pockets where mucus collects. The mucus provides a breeding ground for bacteria. This leads to repeated lung infections. Each infection does more damage to the bronchial tubes. If not treated, bronchiectasis can lead to serious illness, including respiratory failure.
  • Pancreatitis » Pancreatitis is inflammation in the pancreas that causes pain.
  • Episodes of intestinal blockage, especially in newborns.
  • Nasal polyps » Growths in your nose that may require surgery.
  • Clubbing » Clubbing is the widening and rounding of the tips of your fingers and toes. It develops because your lungs are not moving enough oxygen into your blood stream.
  • Collapsed lung » This is also called pneumothorax.
  • Rectal prolapse » Frequent coughing or problems passing stools may cause rectal tissue from inside you to move out of your rectum.
  • Liver disease » Due to inflammation or blocked bile ducts.
  • Low bone density » Because you do not get enough Vitamin D.
  • Diabetes
  • Gallstones

Treatment

There still is no cure for cystic fibrosis (CF), but treatments have improved greatly in recent years. The goals of CF treatment are to:

  • Prevent and control infections in your lungs.
  • Loosen and remove the thick, sticky mucus from your lungs.
  • Prevent blockages in your intestines.
  • Provide adequate nutrition.

Treatment for Lung Problems

The main treatments for lung problems in people with CF are:

  • Antibiotics » Most people with CF have ongoing, low-grade lung infections. Sometimes, these infections become so serious that you may need to be hospitalized. Antibiotics are the primary treatment.
  • Chest Physical Therapy » Also called chest clapping or percussion. It involves pounding your chest and back over and over again to dislodge the mucus from your lungs so that you can cough up the mucus. CPT for cystic fibrosis should be done three to four times each day. CPT is also often referred to as postural drainage. This involves your sitting or lying on your stomach with your head down while you do CPT. This allows gravity to help drain the mucus from your lungs.
  • Exercise » Aerobic exercise helps loosen the mucus, encourage coughing to clear the mucus, and improve your overall physical condition. If you exercise regularly, you may be able to cut back on your chest therapy.
  • Other Medications » Anti-inflammatory medications may help reduce the inflammation in your lungs that is caused by ongoing infections. Mucus-thinning drugs reduce the stickiness of mucus in your airways.
  • Oxygen Therapy » If the level of oxygen in your blood is too low, you may need oxygen therapy. Oxygen is usually given through nasal prongs or a mask.
  • Lung Transplantation » Surgery to replace one or both of your lungs with healthy lungs from a human donor may help you.

Treatment for Digestive Problems

Nutritional therapy can improve your growth and development, strength, and exercise tolerance. It may also make you strong enough to resist some lung infections. Nutritional therapy includes a well-balanced, high-calorie diet that is low in fat and high in protein.

Living With Cystic Fibrosis

If you have cystic fibrosis (CF), you should learn as much as you can about the disease and work closely with your doctors to learn how to manage it. Ongoing medical care is important. You should seek treatment from a team of doctors, nurses, and respiratory therapists who specialize in CF. These specialists are often located at CF Foundation Centers in major medical centers.

Good self-care includes:

  • Eating a healthy diet
  • Avoiding tobacco smoke
  • Washing your hands often to reduce your chances of infection
  • Exercising frequently
  • Drinking lots of fluids
  • Doing chest physical therapy every day
  • Having annual flu and other appropriate vaccinations
  • Taking your medicines as prescribed

You can expect to have a normal sex life. Most men with CF are infertile, but they may be helped with modern reproductive techniques. Although most women with CF may be less fertile than women who don’t have CF, they usually can have children. Talk to your doctor before becoming pregnant. Having a positive attitude is also helpful. If you are a parent of someone with CF, do not feel guilty about passing it on to your child. And do not be overprotective; encourage your child to be active and self-reliant.

Source: National Heart, Lung, and Blood Institute (NHLBI)


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