Cystic Fibrosis (cont.)
In this Article
- Cystic fibrosis facts*
- What is cystic fibrosis?
- What are other names for cystic fibrosis?
- What causes cystic fibrosis?
- Is cystic fibrosis inherited?
- Who is at risk for cystic fibrosis?
- What are the signs and symptoms of cystic fibrosis?
- How is cystic fibrosis diagnosed?
- How is cystic fibrosis treated?
- Living with cystic fibrosis
- What is the outlook for cystic fibrosis?
Who is at risk for cystic fibrosis?
Cystic fibrosis (CF) affects both males and females and people from all racial and ethnic groups. However, the disease is most common among Caucasians of Northern European descent.
CF also is common among Latinos and American Indians, especially the Pueblo and Zuni. The disease is less common among African Americans and Asian Americans.
More than 10 million Americans are carriers of a faulty CF gene. Many of them don't know that they're CF carriers.
What are the signs and symptoms of cystic fibrosis?
The signs and symptoms of cystic fibrosis (CF) vary from person to person and over time. Sometimes you'll have few symptoms. Other times, your symptoms may become more severe.
One of the first signs of CF that parents may notice is that their baby's skin tastes salty when kissed, or the baby doesn't pass stool when first born.
Most of the other signs and symptoms of CF happen later. They're related to how CF affects the respiratory, digestive, or reproductive systems of the body.
Figure A shows the organs that cystic fibrosis can affect. Figure B shows a cross-section of a normal airway. Figure C shows an airway with cystic fibrosis. The widened airway is blocked by thick, sticky mucus that contains blood and bacteria.
Respiratory System Signs and Symptoms
People who have CF have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that's sometimes bloody.
People who have CF tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections caused by bacteria called mucoid Pseudomonas are much more common in people who have CF than in those who don't. An infection caused by these bacteria may be a sign of CF.
People who have CF have frequent bouts of sinusitis (si-nu-SI-tis), an infection of the sinuses. The sinuses are hollow air spaces around the eyes, nose, and forehead. Frequent bouts of bronchitis (bron-KI-tis) and pneumonia (nu-MO-ne-ah) also can occur. These infections can cause long-term lung damage.
As CF gets worse, you may have more serious problems, such as pneumothorax (noo-mo-THOR-aks) or bronchiectasis (brong-ke-EK-ta-sis).
Some people who have CF also develop nasal polyps (growths in the nose) that may require surgery.
Digestive System Signs and Symptoms
In CF, mucus can block tubes, or ducts, in your pancreas (an organ in your abdomen). These blockages prevent enzymes from reaching your intestines.
As a result, your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockages also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort.
A hallmark of CF in children is poor weight gain and growth. These children are unable to get enough nutrients from their food because of the lack of enzymes to help absorb fats and proteins.
As CF gets worse, other problems may occur, such as:
- Pancreatitis (PAN-kre-ah-TI-tis). This is a condition in which the pancreas become inflamed, which causes pain.
- 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 inflamed or blocked bile ducts.
Reproductive System Signs and Symptoms
Men who have CF are infertile because they're born without a vas deferens. The vas deferens is a tube that delivers sperm from the testes to the penis.
Women who have CF may have a hard time getting pregnant because of mucus blocking the cervix or other CF complications.
Other Signs, Symptoms, and Complications
Other signs and symptoms of CF are related to an upset of the balance of minerals in your blood.
CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. This can cause dehydration (a lack of fluid in your body), increased heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke, and, rarely, death.
CF also can cause clubbing and low bone density. Clubbing is the widening and rounding of the tips of your fingers and toes. This sign develops late in CF because your lungs aren't moving enough oxygen into your bloodstream.
Low bone density also tends to occur late in CF. It can lead to a bone-thinning disorder called osteoporosis (OS-te-o-po-RO-sis).
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