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Article disclaimer

Edited by Lahle Wolfe


NHLBI/National Institute of Health Publication edited for content, used with permission.

Additional resources used:

Cystic Fibrosis  (MedlinePlus)
Cystic Fibrosis Foundation

Fast Links to Disorders Often Associated with

Addison's Disease
Asthma and Allergies
Celiac Disease (Sprue)
Cyclic Vomiting Syndrome
Cystic Fibrosis 
Eating Disorders
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Frozen Shoulder
Hashimoto's Thyroiditis
  (Iron Overload)
Irritable Bowel Syndrome
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Alex, The Life of a Child
By Frank Deford

Alexandra Deford, a precious and precocious girl, was just eight years old when she died in 1980 following a battle against the debilitating effects of cystic fibrosis, the number-one genetic killer of children. Her poignant and uplifting story touched the hearts of millions when it was first published and then made into a memorable television movie. A new introduction contains information on the latest cystic fibrosis research, and a touching postcript reveals how the Deford family came to terms with the loss of Alex.

Resource Links to
Cystic Fibrosis Sites

Cystic Fibrosis.Com
Cystic Fibrosis Foundation
CF Chapter & Care Centers
Books On Cystic Fibrosis

Key Points From NIH

"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. 

  • "In CF, an abnormal gene called the cystic fibrosis transmembrane conductance regulator (CFTR) gene causes mucus to become thick and sticky. The mucus builds up in the lungs and blocks the airways, creating an environment that makes it easy for bacteria to grow. This leads to repeated serious lung infections that can damage your lungs. 

  • "The mucus can also block tubes, or ducts, in your pancreas so that the digestive enzymes it produces cannot reach the intestines where they are needed to break down food. 

  • "You have extremely salty sweat. When you perspire, your body loses large amounts of salt. This can upset the balance of minerals in your blood, which can cause a heat emergency. 

  • "The most common symptoms of CF are frequent coughing with phlegm, frequent bouts of bronchitis and pneumonia, salty-tasting skin, dehydration, poor growth, and infertility, mostly in men. 

  • "The sweat test is the most common diagnostic test for CF. It measures the amount of salt in your sweat. 

    "Other tests that can be used to help diagnose CF include a chest x ray, sinus x ray, lung function tests, analysis of sputum cultures and/or stool samples, and genetic testing of a blood sample. 

  • "Prenatal genetic testing can help you find out if your baby is likely to have CF. 

  • "Antibiotics are the primary treatment for lung problems in CF. They treat airway infections. Other treatments include chest physical therapy, exercise, mucus-thinning drugs, and other medications to reduce inflammation in your airways and help open them up. 

  • "Lung transplantation is an option for some people with CF. 

  • "The digestive problems in people with CF can be managed with nutritional therapy, enemas, mucus-thinning drugs, and medications to reduce stomach acid. 

  • "Ongoing medical care from a team of health care providers who specialize in CF is important.

    "Good self-management includes eating a healthy diet, avoiding tobacco smoke, exercising frequently, doing chest physical therapy every day, drinking lots of fluids, and washing your hands often to reduce your chances of infection."

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    Cystic Fibrosis - Part 1 of 2
    (also known as CF,
    Cystic fibrosis of pancreas,
    Fibrocystic Disease of Pancreas, and Mucoviscidosis)

    Causes, Symptoms, Diagnosis, Treatment           Join IOH Cystic Fibrosis Support Group

    Mini Site Index
    What is Cystic Fibrosis (CF)?
    What Causes CF?
    What are Symptoms of CF?
    Tests and Diagnosis of CF
    CF Carrier Testing

    Section 2
    Treatment for CF
    Management of Digestive Problems
    Living With CF  


    What is Cystic Fibrosis (CF)?

    CF is a chronic, genetic (hereditary), debilitating disease where the body over produces mucous.  Excess mucous can clogged the pancreas contributing to the secondary onset of type 2 diabetes.  CF affects approximately 30,000 children and adults in the U.S.  It can lead to serous life-threatening lung infections because the thick mucous clogs the lungs. This mucous can also block ducts in the pancreas causing permanent damage that can lead to the onset of type 1 diabetes.

    Cystic fibrosis related diabetes (CFRD) shares features of types 1 and 2 diabetes, but it is a unique and distinct form of diabetes that requires a special management approach.

    Most persons with CF are diagnosed prior to age three, with approximately 1,000 new cases diagnosed each year.

    Other names for cystic fibrosis are:

    • CF
    • Cystic fibrosis of the pancreas
    • Fibrocystic disease of the pancreas
    • Mucoviscidosis
    • Mucoviscidosis of the pancreas
    • Pancreas fibrocystic disease
    • Pancreatic cystic fibrosis

    What Causes Cystic Fibrosis?
    Courtesy of NHBLI

    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.

    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, but 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.

    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.

    What Are Symptoms of Cystic Fibrosis?

    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, or 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.
    • Diabetes.
    • Gallstones.
    • Low bone density because you do not get enough Vitamin D.

    Tests and Diagnosis of CF

    The sweat test is the most useful test for diagnosing cystic fibrosis (CF). It measures the amount of salt in your sweat. For this test, doctors rub a small amount of a chemical called pilocarpine (pi-lo-KAR-pen) on your arm or leg. They then attach an electrode to this spot. The electrode provides a mild electric current that produces sweat. This may cause tingling or a feeling of warmth. They then cover the area with a gauze pad or filter paper and wrap in plastic. After 30 to 40 minutes, they remove the plastic so the sweat that collected on the pad or paper can be analyzed. The test is usually done twice. High salt levels mean CF.

    Your doctor may also do the following tests to understand more about your condition and how to treat it:

    • Blood tests to look for an abnormal CF gene or other things that indicate CF.
    • Chest x ray.A chest x ray takes a picture of your lungs. It can show scarring from inflammation in your lungs.
    • Sinus x ray.This test may show signs of sinusitis.
    • Lung function test scan measure:
      • How much air your lungs can hold
      • How quickly you can breathe air out of your lungs
      • How well your lungs add oxygen to and remove carbon dioxide from your blood
    • Sputum (phlegm) cultures. Doctors take a sample of your sputum to see what bacteria are growing in it.

    Some States are now testing the blood of all newborns for CF.

    CF Carrier Testing

    You may want to check whether you are a CF carrier, if:

    • You have a family history of CF.
    • You are a partner of someone with CF.
    • You are a couple planning a pregnancy.

    A genetics counselor at your local hospital can take a blood or saliva sample to see if it contains the abnormal CFTR gene that causes CF. It will detect 9 out of 10 cases of CF. Some insurance plans cover genetic testing.


    On to next section  (treatment, management of digestive disorders, and living with CF)


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    Page Updated 03/17/2006