Mucus plugs keep air from getting into or out of some alveoli air sacs. Oxygen cannot get into the blood and carbon dioxide cannot get out. The thick, sticky mucus in the small airways:. If not cleared from the lungs, mucus plugs can lead to lung infections and lung damage.
Lung infections cause serious problems for people with CF because:. Infections cause inflammation, which can damage the cilia and airways, making them even less able to clear mucus, particles and germs from the lungs.
Infections often cause more mucus to be made. This can lead to more mucus plugs and worse infections. This infection-inflammation cycle causes lung damage. Mucus plugs airways, allowing germs to grow and cause infection.
When the body's defense system -- white blood cells -- attack the germs, the lungs become inflamed. This inflammation spurs the creation of more mucus, which then blocks the airways and allows more germs to grow.
As you can see, it's a vicious cycle. Monitoring and minimizing the buildup of germs, like bacteria in your lungs, is one of the main ways your CF care team can help you protect your lungs. Starting at age 5 or older, your CF care team will ask you to take a type of breathing test, known as a pulmonary function test PFT , during one of your quarterly visits. You will also be asked about your breathing, coughing, and the amount of sputum mucus or phlegm from the lungs you are producing.
Your care team will ask for a sample of your sputum which is tested to see what kinds of bacteria are growing in your lungs and determine which antibiotics might fight the bacteria. Your PFT results will probably look like a sea of numbers and maybe a line graph.
Children with CF may have lung infections. This is because bacteria that are normally cleared from the lungs stay in the thickened mucus. Many of these lung infections are long-term chronic. Children with CF also can have problems in their upper respiratory tract.
They can have nasal polyps. These are small growths of tissue from the lining of the nose. They go into the air-filled space above and behind the nose nasal cavity. Sometimes these polyps must be taken out by a healthcare provider. Gastroesophageal reflux disease GERD. With this disease, stomach contents rise up into the esophagus and can cause serious damage. Cystic fibrosis CF is caused by an inherited gene mutation change.
Testing for the CF gene is recommended for anyone who has a family member with the disease, or whose partner is a known carrier of CF or affected with CF. Testing for the CF gene can be done from a small blood sample or from a cheek swab. This is a brush rubbed against the inside of your cheek to get cells for testing. Labs generally test for the most common CF gene mutations.
There are many people with CF whose mutations have not been identified. In other words, all of the genetic errors that cause CF have not been discovered. Because not all mutations are known, a person can still be a CF carrier even if no mutations were found by testing.
There are limitations of CF testing. If both parents have mutations in the CF gene, there is a 1 in 4 chance that a child with have CF. This risk is the same with each pregnancy. Options to discuss with your doctor include:.
In addition to routine immunizations, regular flu vaccines, the pneumonia vaccine, and other appropriate vaccines are important to help reduce the chance of infection. Long-term suppressive inhaled antibiotics may be recommended to prevent lung infections. It causes changes in the electrolyte transport system which causes cells to absorb too much sodium and water. This is how most cases are diagnosed. CF causes thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. People with CF will work closely with a medical team to manage symptoms and stay as healthy as possible.
Health Home Conditions and Diseases. What is cystic fibrosis? CF affects various organ systems in children and young adults, including the following: Respiratory system Digestive system Reproductive system Some people can carry the CF gene without being affected by the disease. How does CF affect the respiratory system? How does CF affect the gastrointestinal GI system? The symptoms of CF that may be due to involvement with the GI tract include: Bulky, greasy stools Rectal prolapse a condition in which the lower end of the bowel comes out of the anus Delayed puberty Fat in the stools Stomach pain Bloody diarrhea The liver may also be affected.
Symptoms of liver disease include: Enlarged liver Swollen belly Yellow color to the skin jaundice Vomiting of blood How does CF affect the reproductive system? What causes cystic fibrosis? Cystic fibrosis CF is a genetic disease. This means that CF is inherited. Who is at risk for cystic fibrosis?
What are the symptoms of cystic fibrosis? Symptoms may include: Thick mucus that clogs certain organs, such as the lungs, pancreas, and intestines. Many other medical problems can point to cystic fibrosis, as well. These include: Sinusitis Nasal polyps Clubbing of fingers and toes. Collapse of the lung often due to intense coughing Coughing up blood Enlargement of the right side of the heart due to increased pressure in the lungs Cor pulmonale Abdominal pain Excess gas in the intestines Rectal prolapse.
In this condition, the lower end of the bowel comes out of the anus. Liver disease Diabetes Pancreatitis, or inflammation of the pancreas that causes severe pain in the belly Gallstones Congenital bilateral absence of the vas deferens CBAVD in males. The following signs are suspicious of CF, and infants having these signs may be further tested for CF: Diarrhea that does not go away Foul-smelling stools Greasy stools Frequent wheezing Frequent pneumonia or other lung infections Persistent cough Skin that tastes like salt Poor growth despite having a good appetite The symptoms of CF may resemble other conditions or medical problems.
How is cystic fibrosis diagnosed? Other tests depend on which body system is affected and may include: Chest X-rays, ultrasound, and CT scans Blood tests Lung function tests Sputum cultures Stool tests For babies, who do not produce enough sweat, blood tests may be used. How is cystic fibrosis treated? Treatment generally focuses on the following 2 areas: Management of lung problems This may involve: Physical therapy Regular exercises to loosen mucus, stimulate coughing, and improve overall physical condition Medicines to thin mucus and help breathing Antibiotics to treat infections Anti-inflammatory medicines Management of digestive problems This may involve: A healthy diet that's high in calories Pancreatic enzymes to aid digestion Vitamin supplements Treatments for intestinal blockages Two new therapies have recently been approved specifically for the treatment of lung disease in CF.
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