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Cystic Fibrosis

What is cystic fibrosis?

Cystic fibrosis (CF) is a lifelong condition that affects your lungs, digestive system, and other organs. Your mucus, tears, sweat, and saliva become so thick and sticky that they clog your lungs and digestive system. CF usually causes problems with breathing and with breaking down and absorbing food. Cystic fibrosis is a genetic disorder. 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 CF?

You may have frequent respiratory infections, such as sinusitis, bronchitis, or pneumonia. You may also have any of the following:

  • Frequent cough, wheezing, and shortness of breath

  • Clubbing of fingers or toes (become large, blunt, and rounded)

  • Weakness and fatigue

  • Abdominal pain

How is CF diagnosed?

Your caregiver will ask if anyone in your family has CF. He will also ask about your symptoms. He may recommend genetic counseling to learn why you have CF. If you plan to have a baby, genetic counseling may show if your child is at risk for CF. You may need any of the following tests:

  • A sweat chloride test shows the amount of chloride in your sweat. The amount of chloride will be high if you have CF.

  • Blood tests may be used to find signs of infection and to test your kidney function. They may also show the gene that caused your CF.

  • An x-ray will show inflammation and enlargement of your lungs. It will also show plugged airways and any fluid buildup.

  • A bronchoscopy is a procedure to look inside your lungs to check for damage. A bronchoscope (thin tube with a light) is inserted into your mouth and moved down your throat to your lungs. Tissue and fluid may be collected from your airway or lungs to be tested.

How is CF treated?

There is no cure for CF. Treatment may help to prevent respiratory or intestinal infections. It may also help you absorb nutrients.

  • Medicines:

    • Antibiotics help fight or prevent an infection caused by bacteria.

    • Mucus thinning medicine is breathed in to help thin lung mucus so you can cough it up more easily.

    • NSAIDs help decrease swelling and pain. This medicine can be bought with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If you take blood thinner medicine, always ask your caregiver if NSAIDs are safe for you. Always read the medicine label and follow the directions on it before using this medicine.

    • Steroid medicine helps decrease inflammation.

    • Bronchodilators help open the air passages in your lungs, and help you breathe more easily.

    • Pancreatic enzymes help your digestive system break down food and absorb nutrients properly.

  • Extra oxygen may be needed if your blood oxygen level is lower than it should be. You may get oxygen through a mask placed over your nose and mouth or through small tubes placed in your nostrils.

  • Surgery may be needed if you have severe damage to organs, such as your liver or lungs. Ask your caregiver for more information about surgery.

What can I do to breathe more easily?

  • Airway clearance techniques are exercises to help remove mucus so you can breathe more easily. Your caregiver will show you how to do the exercises. These exercises may be used along with machines or devices to help decrease your symptoms and risk for infection.

  • Rest or sleep with your head elevated to help keep your airway open. Use pillows or foam wedges to elevate your head.

  • Use a cool mist humidifier to increase air moisture in your home. This may make it easier for you to breathe and to cough up mucus.

  • Do not smoke. If you smoke, it is never too late to quit. Smoke can make your coughing or breathing worse. Ask your caregiver for information if you need help quitting.

What can I do to stay healthy?

  • Get the flu vaccine each year. This will help prevent infection from the flu virus. Avoid people who have a cold or the flu.

  • Prevent the spread of germs. Cover your mouth when you cough. Cough into a tissue or your shirtsleeve so you do not spread germs from your hands. Wash your hands often. Use soap and water. Wash your hands after you use the bathroom, change a child's diapers, or sneeze. Wash before you prepare or eat food.

  • Eat a variety of healthy foods. Healthy foods include fruits, vegetables, whole-grain breads, low-fat dairy products, beans, lean meats, and fish. You may need to eat foods that have extra calories, fat, vitamins, or calcium. Ask your caregiver if you need to be on a special diet.

  • Exercise as directed. Ask your caregiver about the best exercise plan for you. Physical activities can help loosen secretions in your airways and lungs, and help you breathe more easily.

When should I contact my caregiver?

  • You have a fever.

  • 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 or call 911?

  • You cough up blood.

  • You have chest pain or trouble breathing.

  • Your lips or fingernails turn blue or white.

  • You have severe abdominal pain.

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. The above information is an educational aid only. It is not intended as medical advice for individual conditions or treatments. Talk to your doctor, nurse or pharmacist before following any medical regimen to see if it is safe and effective for you.

© 2014 Truven Health Analytics Inc. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or Truven Health Analytics.

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