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Orthopedics
What Do You Know About Cystic Fibrosis?

Diagnosis

What Do You Know About Cystic Fibrosis?

Cystic fibrosis (CF) is an inherited disease. Nearly 40,000 people in the U.S. have CF. Most of them are descendants of people from northern Europe. Find out more about CF by taking this quiz.

1. CF mainly affects which body system?

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The sweat glands and the reproductive system usually are involved, as well. A person with CF makes too much thick, sticky mucus. This clogs the air passages in the lungs. It makes the person more likely to get an infection. The clogged passages also make it harder to breathe. The sweat of a person with CF often is more salty than normal. Thick secretions also clog the ducts of the pancreas. This keeps digestive enzymes and other chemicals made by the pancreas from helping with digestion. Most men with CF are infertile. Most people with CF have trouble getting pregnant.

A. Circulatory system
B. Respiratory system
C. Digestive system
D. Nervous system
E. B and C

2. Which symptom in a baby or young child might mean CF?

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Symptoms can vary from child to child. Most people with CF are diagnosed by age 2. Some children with CF may not be diagnosed until they are teens.

A. Stools that are pale, foul, or float
B. Frequent wheezing or pneumonia
C. Salty-tasting skin
D. Chronic cough
E. All of the above

3. What happens to the mucus glands in a child with CF?

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An abnormality in the glands that make mucus causes the mucus to be thick. The mucus builds up in the intestines and lungs. This causes malnutrition, poor growth, and breathing problems. The extra mucus becomes a place for bacteria to grow. This causes frequent respiratory infections. People with CF also lose too much salt when they sweat. A severe loss of salt can upset the heart's rhythm and may cause shock.

A. Too little mucus is made
B. The mucus made is too thin
C. The mucus made is too thick
D. The mucus builds up in the body
E. C and D

4. How is the digestive system affected by extra mucus in CF?

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The pancreas helps in digestion by making enzymes and bicarbonate to neutralize stomach acid. If the pancreas ducts are blocked by mucus, digestion is affected. Enzymes can't help digest food, which means the person won't get the nutrients they need. Bowel movements become bulky and foul-smelling. Malnutrition and slowed growth and development can also occur. If the cells in the pancreas that make insulin are damaged, the person can develop cystic fibrosis-related diabetes. If the bile ducts in the liver are affected, biliary cirrhosis may result. Other symptoms and problems linked with CF include sinusitis, nasal polyps, clubbed fingers and toes, ruptured lung tissue, belly (abdominal) pain, gassiness, gallstones, coughing blood, enlargement of the right side of the heart, and infertility.

A. The mucus can cause stomach ulcers
B. The mucus can damage the bile ducts in the liver
C. The mucus can clog the ducts in the pancreas
D. The mucus can damage the rectum
E. B and C

5. How is CF diagnosed?

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All newborns are screened for cystic fibrosis. The screening method may vary from state to state. The sweat test measures the amount of sodium chloride (salt) in a person's sweat. A person with higher than normal amounts of sodium and chloride may have CF. A blood test called an immunoreactive trypsinogen test measures the level of a protein called trypsinogen. Higher levels of this protein may mean CF. Testing for CF also can be done during pregnancy to find out if the developing fetus has the disease. Genetic testing also can be done to help figure out the diagnosis and for couples with a family history of CF who are considering having children.

A. Sweat test
B. Blood test
C. Lung volume test
D. Urine test
E. A and B

6. Currently, no effective medicine is available to cure CF. But healthcare providers can prescribe medicines that help slow the progression of the disease. Which of these types of medicine can do this?

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The first medicines just for CF were released in 2012. They are called cystic fibrosis transmembrane conductance regulator (CFTR) modulators. These are the first treatment that targets the actual source of the disease, not just the symptoms. Other medicines focus on the symptoms of the disease. Bronchodilators widen the bronchial passages in the lungs. Antibiotics kill the bacteria that cause lung infections. Decongestants ease the swelling of bronchial membranes. Mucolytics thin the mucus in the lungs. Vitamin supplements and enzymes are also often given.

A. Bronchodilators
B. Antibiotics
C. Decongestants
D. Mucolytics
E. All of the above

7. A defect in a gene that makes the protein cystic fibrosis transmembrane regulator is the cause of CF. According to recent research, this problem causes an imbalance of which key substance in the body?

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Fatty acids are long molecules that serve as the building blocks of the cell membrane. Certain fatty acids play a key role in controlling the body's inflammatory response. The hallmark of CF is chronic inflammation of the lung tissue. People with CF appear to have high levels of the fatty acid arachidonic acid (AA) and low levels of the fatty acid docosahexaenoic acid (DHA). This combination leads to tissue inflammation.

A. Hormones
B. Glucose
C. Carbohydrates
D. Fatty acids

8. CF is an inherited disease. How is CF passed down through families?

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To have CF, a person must inherit 2 defective CF genes, 1 from each parent who carries the abnormal CF gene. Each time 2 carriers conceive, they have a 1 in 4 chance that their child will have CF. They also have a 1 in 2 chance that the child will be a carrier of the CF gene. And they have a 1 in 4 chance that the child will not be a carrier.

A. One parent is a carrier of the CF gene
B. One grandparent is a carrier of the CF gene
C. Both parents are carriers of the CF gene
D. All of the above

Your score was:

Author: Sinovic, Dianna

Online Medical Reviewer: Fraser, Marianne, MSN, RN

Online Medical Reviewer: Gotwals, Jessica, RN, BSN, MPH

Online Medical Reviewer: Shriber, Andrew D., MD

Date Last Reviewed: 01/01/2023

© 2000-2026 The StayWell Company, LLC. All rights reserved. This information is not intended as a substitute for professional medical care. Always follow your healthcare professional's instructions.

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