Cystic Fibrosis

WHAT YOU SHOULD KNOW:

• Cystic fibrosis, also called CF, is a condition where your exocrine glands do not work normally. Exocrine glands make fluids such as sweat, mucus, tears, and saliva (spit). With CF, the fluids made are too thick and sticky, and can clog-up ducts inside body organs. It is caused by problems with the gene that affects normal functions of your body. Your parents may have passed on genes that cause CF. Signs and symptoms include frequent cough, wheezing, and shortness of breath that gets worse through time. You may have frequent respiratory system infections or repeated illness with pancreatitis. You may also have problems breaking down and absorbing food, losing weight, and feeling very weak. You may have a hard time getting your female partner pregnant.
  
  
• To diagnose CF, you may need blood tests, genetic testing, chest x-ray, and a sweat chloride test. There is no cure for CF and the treatments given are to prevent you from getting worse. You may be given medicines to help you breathe easier and kill germs causing any infection. You may need breathing exercises and special devices to help remove mucus and let you breathe easier. You may need medicines to help break down and absorb nutrients in food properly. Your caregiver may put you on a special diet and give you extra vitamins. These diet changes should help you gain weight and have more energy. You may need surgery to repair, or completely replace damaged organs, such as you lungs or liver. Diagnosing and treating your CF as soon as possible may help relieve problems with breathing and digestion.

AFTER YOU LEAVE:

Ask for information about where and when to go for follow-up visits:

For continuing care, treatments, or home services, ask for more information.

• You need to attend your follow-up visits always and on time. Your caregiver will check your condition each time you visit and may give other therapies. It is also very important to follow his instructions regarding your medicines or breathing therapies.
  
  

Avoid the spread of germs:

• Wash your hands often with soap and water. Carry germ-killing gel with you. You can use the gel to clean your hands when there is no soap and water available.
  
  
• Do not touch your eyes, nose, or mouth unless you have washed your hands first.
  
  
• Always cover your mouth when you cough. Cough into a tissue or your shirtsleeve so you do not spread germs from your hands.
  
  
• Try to avoid people who have a cold or the flu. If you are sick, stay away from others as much as possible.

Diet:

• Having CF can affect how your body absorbs food and nutrients. You may need extra calories, vitamins, or calcium added to your diet. You may need to take pancreatic enzymes to help you better absorb your food. Ask your caregiver for more information about if any of these should be added to your diet.
  
  
• Eat a variety of healthy foods from all the food groups every day. Include whole grain bread, cereal, rice and pasta. Eat a variety of fruits and vegetables, including dark green and orange vegetables and legumes (dry beans). Include dairy products such as low-fat milk, yogurt and cheese. Choose protein sources such as lean meat and poultry (chicken), fish, beans, eggs and nuts. Ask your caregiver how many servings of fats, oils, and sweets you may have each day, and if you need to be on a special diet.

Exercises:

• Airway clearance techniques: The thick mucus in your lungs caused by CF can lead to infections and lung damage. Your caregiver may teach you special exercises to help remove mucus and let you breathe easier. These exercises may be used along with machines or special devices to help decrease your symptoms. Ask your caregivers for more information about these exercises.
  
  
• Physical exercise program: You and your caregiver can plan a suitable exercise program for you. Doing exercise and other physical activities can back your energy and improve your appearance. Exercising also makes the heart stronger, lowers blood pressure, and keeps you healthy. Physical activities can help loosen secretions in your airways and lungs, and help you breathe easier.

Medicines:

• Take your medicine as directed: Call your primary healthcare provider if you think your medicine is not working as expected. Tell him if you are allergic to any medicine. Keep a current list of the medicines, vitamins, and herbs you take. Include the amounts, and when, how, and why you take them. Take the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency. Throw away old medicine lists.
  
  
• Antibiotics: This medicine is given to fight or prevent an infection caused by bacteria. Always take your antibiotics exactly as ordered by your primary healthcare provider. Do not stop taking your medicine unless directed by your primary healthcare provider. Never save antibiotics or take leftover antibiotics that were given to you for another illness.
  
  
• Anti-inflammatory medicine: You may need to take ibuprofen or steroids for a short time to decrease inflammation (swelling) in your lungs, and help you breathe more easily. Ask caregivers for more information about these medicines.
  
  
• Respiratory medicines: You may need medicines to help you breathe more easily, such as bronchodilators. These are medicines that make the airways in your lungs wider. You may also need medicines to help thin the mucus in your lungs.

Oxygen:

You may need extra oxygen to help you breathe easier. It may be given through a plastic mask over your mouth and nose. It may be given through a nasal cannula, or prongs, instead of a mask. A nasal cannula is a pair of short, thin tubes that rest just inside your nose. Tell your caregiver if your nose gets dry or if you get redness or sores on your skin. Never smoke or let anyone else smoke in the same room while your oxygen is on. Doing so may cause a fire.

Tips to help you breathe easier:

• Prop pillows behind you when resting or sleeping to help you breathe.
  
  
• Do not smoke, or let anyone smoke around you. Smoke can make your coughing or breathing worse.
  
  
• Using a humidifier will moisten the air in your home. The moist air will make it easier to cough up your sputum. Wash the humidifier each day with soap and warm water to keep it germ free.

For support and more information:

Accepting that you have cystic fibrosis may be hard. You may feel very sad and helpless to do anything about your condition. Those close to you may feel scared, angry, or sad. These are normal feelings. You may talk to your caregiver, family, or friends about your feelings. You can join a CF support group with others who also have this condition. You may write or call the following:

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

• National Heart, Lung and Blood Institute
  Health Information Center
  P.O. Box 30105
  Bethesda , MD 20824-0105
  Phone: 1- 301 - 592-8573
  Web Address: http://www.nhlbi.nih.gov/health/infoctr/index.htm
  

CONTACT A CAREGIVER IF:

• You have a fever.
  
  
• Your skin is itchy, you have a rash, or you have other new signs and symptoms.
  
  
• You have questions about your medicines.
  
  
• You urinate less, have a dry mouth or cracked lips, or feel dizzy.
  
  
• You have chills, or feel weak or achy.
  
  
• You have trouble sleeping.
  
  
• You cannot make it to your next appointment.

SEEK CARE IMMEDIATELY IF:

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

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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.

Learn more about Cystic Fibrosis (Discharge Care)