Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is a lifelong condition that affects your lungs, digestive system, and other organs. When you have CF, your mucus, tears, sweat, and saliva are too thick and sticky. These thick fluids clog your lungs and digestive system. CF typically causes trouble breathing and problems breaking down and absorbing food.

What causes cystic fibrosis?

Cystic fibrosis is caused by an abnormal gene. If your parents or close relatives have CF, there is a higher risk that you may have it.

What are the signs and symptoms of cystic fibrosis?

You may have frequent respiratory infections, such as sinusitis, bronchitis, or pneumonia. You may have repeated episodes of pancreatitis (inflammation of the pancreas). You may have any of the following:

• Clubbing of your fingers or toes
  
  
• Frequent coughs, wheezing, and shortness of breath
  
  
• Pain in your abdomen
  
  
• Infertility in men
  
  
• Bowel movements that are large, loose, greasy, and smell very bad
  
  
• Weakness and fatigue
  
  
• Weight loss without trying

How is cystic fibrosis diagnosed?

Cystic fibrosis is usually diagnosed during childhood. In some cases, symptoms may not appear until young adulthood. Your caregiver will ask you about your medical history and examine you. You may need the following tests:

• Blood tests: These tests can be used to check for problems, such as infections or damage to your organs. Your blood may also be tested to look for the gene that causes CF.
  
  
• Sweat chloride test: This test measures the amount of chloride in your sweat. With CF, too much chloride is in your sweat. You may need to have this test more than once.

How is cystic fibrosis treated?

There is no cure for cystic fibrosis. Treatment may help to prevent respiratory or intestinal infections. It may also help you better absorb food and nutrients such as vitamins.

• Nutritional therapy: A special diet high in calories may be advised and extra vitamins may be given. These will help you gain weight and get proper nutrition.
  
  
• Medicines:
  
  
  • Antibiotics: This medicine is given to help treat or prevent an infection caused by bacteria.
    
    
  • Bronchodilators: You may need bronchodilators to help open the air passages in your lungs, and help you breathe more easily.
    
    
  • Mucus thinning medicine: This is a medicine you breathe in to help thin the mucus in your respiratory system.
    
    
  • Anti-inflammatory medicine: Ibuprofen and steroids may help decrease inflammation in your lungs. Steroids may help you breathe more easily.
    
    
  • Pancreatic enzymes: These medicines help your digestive system break down food and absorb nutrients properly.
  
  
• Oxygen: You may need extra oxygen if you have difficulty breathing or low oxygen levels in your blood. You breathe the oxygen through a face mask or through short, thin tubes that rest just inside your nose.
  
  
• Surgery: If you have severe damage to organs such as your liver or lungs, you may need surgery to correct problems, or a liver or lung transplant.

How can I manage my symptoms?

Airway clearance techniques (ACTs) are techniques to help loosen mucus so you can more easily cough it up. ACTs may be used to help prevent infections caused by mucus blocked in your lungs. ACTs may consist of techniques, machines, or devices. Doing these therapies on a set schedule can help decrease your symptoms and help you stay healthy.

• Active cycle of breathing techniques: You may be taught controlled breathing, chest expansion, or forced expiration techniques. With these techniques, you learn ways to control your breathing and force mucus out of your lungs. You may be taught huffing, which is less forceful than coughing. It is a controlled way to use your diaphragm and chest muscles to cough up mucus.
  
  
• Autogenic drainage: In this exercise you sit up straight and slowly breathe in through your nose to fill your lungs. You then hold your breath for 3 to 4 seconds and breathe out as fast as you can through your nose. Repeat this exercise several times to push out the mucus from the lower parts of your lungs.
  
  
• Chest physical therapy: Physical therapy may involve percussion or postural drainage. With percussion, a person forcefully pats on your back with a cupped hand or soft cup. It is done to loosen mucus stuck in your airways. Percussion may also be done with an electric percussor. With postural therapy, a caregiver will show you positions to help drain mucus from different sections of your lung.
  
  
• High frequency chest wall oscillation: This therapy uses a vest that attaches to a machine and causes the vest to vibrate your chest.
  
  
• Oscillating positive end pressure therapy: Oscillating PEP uses a small, pocket-sized device that works by vibrating your airways as you breathe out through it.
  
  
• Positive expiratory pressure therapy: PEP therapy uses a device that keeps some air in your airways when you breathe out. This helps prevent trapping of mucus in your small airways so you can more easily cough it up.

What are the risks of cystic fibrosis?

• Treatment of CF may cause side effects. You may have an infection or bleeding with surgery. Even after successful treatment, your symptoms may not disappear at once, may continue, or may come back.
  
  
• If left untreated, you may get damage to your lungs, liver, or pancreas. You may not get enough nutrients, and you may lose weight and get very weak. You may have an increased risk for broken bones. CF may lead to pancreatitis or diabetes. Your liver can have cirrhosis (scarring) and not work normally. You may have ongoing damage to your lungs. You may have trouble breathing, bleeding problems, collapsed lung, or other lung problems. You may be at higher risk of lung infections, which may lead to more trouble breathing, or even death.

Where can I find support and more information?

• Cystic Fibrosis Foundation
  6931 Arlington Road, Suite 2000
  Bethesda , MD 20814
  Phone: 1- 800 - 344-4823
  Web Address: http://www.cff.org
  

When should I contact my caregiver?

Contact your caregiver if:

• You have a fever.
  
  
• You have a rash, itchy skin, or new signs and symptoms.
  
  
• You have chills or feel weak or achy.
  
  
• You have trouble sleeping.
  
  
• You urinate less, have a dry mouth or cracked lips, or feel dizzy.
  
  
• You have questions or concerns about your condition or care.

When should I seek immediate care?

Seek care immediately or call 911 if:

• You cough up blood.
  
  
• You have pain in your chest or trouble breathing.
  
  
• Your lips or fingernails turn blue or white.

Care Agreement

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your caregivers to decide what care you want to receive. You always have the right to refuse treatment.

Learn more about Cystic Fibrosis