Cystic Fibrosis

GENERAL INFORMATION:

What is cystic fibrosis? 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 ducts inside body organs. The digestive and respiratory systems are most commonly affected with this condition. The digestive system gets nutrients from food, and includes the esophagus, stomach, and intestines (bowels). The respiratory system controls your breathing and includes the airways and lungs. You may have trouble breathing, or problems with breaking down and absorbing food. You may lose a lot of chloride (salt) through your sweat, which may cause weakness. There is no cure for cystic fibrosis, and over time your symptoms may come and go and get worse.

What causes cystic fibrosis? Cystic fibrosis is caused by an abnormal gene that affects the normal functions of your body. Genes are little pieces of information that tell your body how to develop and what to do. Your parents may have passed on to you the genes that cause CF. If you or any close relatives have CF, there is a higher risk that your child may also have it.

What are the signs and symptoms of cystic fibrosis? Signs and symptoms of CF may come and go, or may not be found until later in life. You may have frequent respiratory system infections, such as sinusitis, bronchitis, or pneumonia. You may have repeated illness with pancreatitis (inflammation of the pancreas) where a cause is not found. Over time, you may get damage to your lungs, liver, or pancreas. You may have any of the following:

• Clubbing of your fingers or toes. Clubbing is when the ends of your fingers or toes become round and thick. This happens when your body does not get enough oxygen over a very long time.
  
  
• Frequent coughs, wheezing, and shortness of breath. These may get worse and may return many times.
  
  
• Pain in your abdomen (stomach).
  
  
• Problem getting your female partner pregnant.
  
  
• Stools that are large, greasy, and smell very bad. Stools may also be very loose or liquid.
  
  
• Weakness and fatigue (getting tired very easily).
  
  
• Weight loss without really trying and even if you still eat a lot.

How is cystic fibrosis diagnosed? Cystic fibrosis is usually diagnosed during childhood. In some cases, symptoms may not appear until later on in your late teens. Your caregiver will ask you about your medical history and do a physical exam. He will also ask about the health condition of your family members. You may also have any of the following:

• Blood tests: A sample of your blood is sent to a lab for tests. It can be used to check for problems, such as infections or damage to your organs. Your blood may also be tested to check your health condition and to look for the gene causing CF.
  
  
• Sputum sample: Sputum (mucus from your lungs) is collected in a special cup when you cough. It is sent to a lab for tests. The sputum may show what germ is causing your illness. It can also help your caregiver choose what medicine is best for you.
  
  
• 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.
  
  
• Chest x-ray: This is a picture of your lungs and heart. Caregivers use it to see how your lungs and heart are doing. Caregivers may use the x-ray to look for signs of infection like pneumonia, or to look for collapsed lungs. Chest x-rays may show tumors, broken ribs, or fluid around the heart and lungs.

How is cystic fibrosis treated? There is no cure for cystic fibrosis. Treatment depends on your health condition and which organs are affected. You may need treatment to help prevent respiratory or intestinal infections. You may need treatment to help you better absorb food and nutrients such as vitamins. You may need to visit your caregiver more than once for further tests and treatment. You may have any of the following:

• Diet: 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, and give you energy and strength.
  
  
• Medicine: You may need medicines to kill germs that cause infections. You may need medicines to help you breathe easier. You may receive medicine to make the mucus in your lungs thinner and easier to cough up. You may also be given medicine to help break down and absorb food properly.
  
  
• Supplemental oxygen: If your lungs become damaged from CF, 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 the mask or prongs bother you. Ask your caregiver before taking off your oxygen. Never smoke or let anyone else smoke in the same room while your oxygen is on. Doing so may cause a fire.
  
  
• Surgery: This may be done to correct problems in organs damaged by CF. If your lungs or liver get badly damaged by CF, you may need a lung or liver transplant.

What airway clearance techniques can I use to stay healthy and breathe easier? The thick respiratory secretions in your lungs caused by CF can block small airways and lead to infections. There are techniques you can learn to help loosen mucus in your respiratory system so you can more easily cough it up. There are many different types of airway clearance techniques (ACTs) that you can use. You and your caregivers will want to work together to find the techniques that work best for you. Your program of ACTs may also use special machines or devices. These therapies may take some time and effort on your part. Doing these therapies on a set schedule can help decrease your symptoms quicker and help you stay healthy.

• ACT exercises:
  
  
  • Active cycle of breathing techniques:
    
    
    • Controlled breathing: Controlled breathing involves paying attention to each breath while breathing gently, slowly, and deeply. Sometimes, you may need to breathe out air as fast as you can to force out mucus in your airways.
      
      
    • Chest expansion exercises: Chest exercises help you completely fill your lungs with air by letting your abdomen swell as you breathe in. Minding how your abdomen moves can increase the amount of air you can take in.
      
      
    • Forced expiration technique: Coughing is a reflex to mucus or other irritation in your airway. It can be very forceful. Huffing is a technique you can learn that is less forceful and less tiring to do. It is a controlled, moderately forceful pushing out of breath with your diaphragm and chest muscles. The diaphragm is the large muscle at the bottom of your chest that helps you breathe.
    
    
  • Autogenic drainage: This is a breathing technique used to bring up mucus from lower parts of your airways or lungs. 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. You may need to repeat this exercise several times to push out the mucus.
    
    
  • Chest physical therapy:
    
    
    • Percussion: This involves having another person forcefully pat on your back with a cupped hand or soft plastic cup. It is done to loosen mucus stuck in your airways. This may look to be uncomfortable, but really is not. Percussion may also be done with an electric percussor.
      
      
    • Postural drainage: This therapy is designed to help drain mucus from different sections of your lung. Your caregiver will show you the different positions you need to use for this therapy.
  
  

• ACT with equipment:
  
  
  • High frequency chest wall oscillation: This therapy uses a special vest that you wear. It attaches to a machine that 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. You will need to breathe in and fill your lungs, and hold your breath for 2 to 3 seconds. You then put the device tightly to your mouth and breathe out normally 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.
  
  

When should I call my caregiver? Call your caregiver if:

• You have a fever.
  
  
• Your skin is itchy, or you have a rash.
  
  
• 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 any questions or concerns about your condition, treatment, or care.

When should I seek immediate help? Call 911 or get to the nearest emergency room if:

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

Where can I find 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
  

CARE AGREEMENT:

You have the right to help plan your care. To help with this plan, you must learn about your health condition and how it may be treated. You can then discuss treatment options with your caregivers. Work with them to decide what care may be used to treat you. You always have the right to refuse treatment.

Copyright © 2008 Thomson Healthcare Inc. All rights reserved. Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes.

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.

Link to Page

Print Page

Email Page