Cystic Fibrosis In Children

What is cystic fibrosis?

Cystic fibrosis, also called CF, is a condition where your child's exocrine glands do not work normally. Exocrine glands make fluids such as sweat, mucus, tears, saliva (spit), and chemicals that break down food in the bowel. With CF, the fluids made are too thick and sticky, and can clog ducts inside your child's 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 child's breathing and includes the airways and lungs.

Your child may have trouble breathing, or problems with breaking down and absorbing food. He may not grow and gain weight as fast as other children his age would. He may lose a lot of chloride (salt) through his sweat, which may cause weakness. There is no cure for cystic fibrosis, and over time your child's symptoms may come and go and get worse. Some children have more severe (very bad) disease, while in others it is much milder. With proper management, children with CF now live longer, some well into their 30's and beyond.

What causes cystic fibrosis?

Cystic fibrosis is caused by an abnormal gene that affects functions of your child's body. Genes are little pieces of information that tell your child's body how to develop and what to do. If your child gets two abnormal genes for CF, he will have the disease. This happens when both parents have the abnormal gene for CF. Because it takes two genes to get the disease, if a person has only one gene for it, he will not have CF. Many people have only one of the CF genes. They usually have no symptoms and may not know that they have the CF gene. If you or a close relative have CF, there is a higher risk that your children may also have it.

What are the signs and symptoms of cystic fibrosis?

• The first sign of CF may be seen in some newborn babies with it. They may get a condition called meconium ileus. Meconium (me-KO-nee-um) is the dark green stool that babies pass before having formula or breast milk stools. An ileus (ILL-ee-us) is when the bowel gets obstructed and cannot pass stool. The meconium of babies with CF is very thick and sticky, and causes the ileus. This condition may need surgery to fix it. Ask your caregiver for more information about bowel obstruction.
  
  
• Signs and symptoms of CF may come and go, or may not be found until later on in your child's life. Your baby or child may have failure to thrive. This is where he does not grow or gain weight as fast as other children his age would. Ask your caregiver for more information about failure to thrive.
  
  
• Your child may have problems breathing and get frequent respiratory system infections, such as sinusitis or bronchitis. These may increase as he gets older. He may have repeated illness with pancreatitis (inflammation of the pancreas). Over time, your child's lungs, liver, or pancreas may become damaged. Your child may also have any of the following:
  
  
  • Clubbing of his fingers or toes. Clubbing is when the ends of the fingers or toes become round and thick. This happens when the body does not get enough oxygen over a very long time. Children with CF may have low oxygen levels while asleep or with exercise.
    
    
  • Frequent coughs, wheezing, and shortness of breath. These may get worse and may return many times.
    
    
  • Losing weight without meaning to, and even if your child still eats a lot.
    
    
  • Pain in his abdomen (stomach).
    
    
  • Skin that tastes salty.
    
    
  • Stools that are large, greasy, and smell very bad. Stools may be very loose or liquid.
    
    
  • Weakness and fatigue (getting tired very easily).

How is cystic fibrosis diagnosed?

Cystic fibrosis is usually diagnosed during childhood. In some cases, your child's symptoms may not appear until later on in his life. Your caregiver will ask about your child's medical history. He may also about the medical histories of you, the other parent, and close relatives. He will do a physical exam on your child. Your child may also have any of the following:

• Blood tests: A sample of your child's blood is sent to a lab for tests. It can be used to check for problems, such as infections or damage to his organs. His blood may also be tested to check his health condition and to look for the gene causing CF.
  
  
• Newborn screening: This procedure tests a sample of your child's blood a few days after his birth. This test can see if your child is carrying genes that cause CF.
  
  
• Sputum sample: Sputum (mucus from the lungs) is collected in a special cup when your child coughs. It may also be suctioned from his airways using a thin soft tube. It is sent to a lab for tests. The sputum may show what germ is causing his illness, if he has an infection. It can also help his caregiver choose what medicine is best for him.
  
  
• Sweat chloride test: This test measures the amount of chloride in your child's sweat. With CF, too much chloride is in his sweat. He may need to have this test more than once.
  
  
• Chest x-ray: This is a picture of your child's lungs and heart. A chest x-ray may be used to check your child's heart, lungs, and chest wall. It can help caregivers diagnose your child's symptoms, or suggest or monitor treatment for medical conditions.

How is cystic fibrosis treated?

There is no cure for cystic fibrosis. Treatment depends on your child's health condition and which organs are affected. He may need treatment to help prevent respiratory or other infections. He may need treatment to help him better absorb food and nutrients such as vitamins. Your child may need to visit his caregiver more than once for further tests and treatment. He may have any of the following:

• Diet: A special diet high in calories may be advised and extra vitamins may be given to your child. These will help him gain weight and get proper nutrition, and give him energy and strength.
  
  
• Medicine: Your child may need medicines to kill germs that cause infections. He may need medicines to help him breathe easier. He may receive medicine to make the mucus in his lungs thinner and easier to cough up. He may also be given medicine to help his body break down and absorb food properly. Your child may need to take many medicines. It is very important to give your child his medicines exactly as ordered by his caregiver. Ask caregivers for more information about the medicines that your child needs to take.
  
  
• Supplemental oxygen: If your child's lungs become damaged from CF, he may need extra oxygen to help him breathe easier. It may be given through a plastic mask over his 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 his nose. Tell your caregiver if his nose gets dry or if the mask or prongs bother him. Ask your caregiver before taking off his oxygen. Never smoke or let anyone else smoke in the same room while your child's oxygen is on. Doing so may cause a fire.
  
  
• Surgery: This may be done to correct problems in organs damaged by CF. If your child's lungs or liver get badly damaged by CF, he may need a lung or liver transplant.

What airway clearance techniques can be used to help my child stay healthy and breathe easier?

The thick respiratory secretions in your child's lungs caused by CF can block small airways and lead to infections. There are techniques you and your child can learn to help loosen mucus in his respiratory system so he can more easily cough it up. There are many different types of airway clearance techniques (ACTs) that he can use. You, your child, and his caregivers will want to work together to find the techniques that work best for him. Your child's program of ACTs may also use special machines or devices. These therapies may take some time and effort on your and your child's part. Doing these therapies on a set schedule can help decrease your child's symptoms quicker and help him 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, your child may need to breathe out air as fast as he can to force out mucus in his airways.
      
      
    • Chest expansion exercises: Chest exercises help your child completely fill his lungs with air by letting his abdomen swell as he breathes in. Minding how his abdomen moves can increase the amount of air he can take in.
      
      
    • Forced expiration technique: Coughing is a reflex to mucus or other irritation in your child's airway. It can be very forceful. Huffing is a technique your child can learn that is less forceful and less tiring to do. It is a controlled, moderately forceful pushing out of breath with his diaphragm and chest muscles. The diaphragm is the large muscle at the bottom of his chest that helps him breathe.
    
    
  • Autogenic drainage: This is a breathing technique used to bring up mucus from lower parts of your child's airways or lungs. In this exercise he sits up straight and slowly breathes in through his nose to fill his lungs. He then holds his breath for 3 to 4 seconds and breathes out as fast as he can through his nose. Your child may need to repeat this exercise several times to push out the mucus.
    
    
  • Chest physical therapy: These therapies can be done on children of any age, including babies:
    
    
    • Percussion: This involves forcefully patting your child's back with a cupped hand or soft plastic cup. It is done to loosen mucus stuck in his 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 his lungs. Your child's caregiver will show you the different positions your child needs to use for this therapy.
  
  

• ACT with equipment:
  
  
  • High frequency chest wall oscillation: This therapy uses a special vest that your child wears during the treatment. It attaches to a machine that causes the vest to vibrate his chest.
    
    
  • Oscillating positive end pressure (PEP) therapy: Oscillating PEP uses a small, pocket-sized device that works by vibrating your child's airways as he breathes out through it. Your child will need to breathe in and fill his lungs, and hold his breath for 2 to 3 seconds. He then puts the device tightly to his mouth and breathes out normally through it.
    
    
  • Positive expiratory pressure (PEP) therapy: PEP therapy uses a device that keeps some air in your child's airways when he breathes out. This helps prevent trapping of mucus in his small airways so he can more easily cough it up.
  
  

When should I call my child's caregiver?

Call your child's caregiver if:

• Your child has a fever.
  
  
• Your child's skin is itchy or has a rash.
  
  
• Your child has chills, or feels weak or achy.
  
  
• Your child has trouble sleeping.
  
  
• Your child urinate less, has a dry mouth or cracked lips, or feels dizzy.
  
  
• You have questions about the medicines that your child needs to take.
  
  
• You have any questions or concerns about your child's condition, treatment, or care.

When should I seek immediate help?

Call 911 or get to the nearest emergency room if:

• Your child coughs up blood.
  
  
• Your child has pain in his abdomen that does not go away.
  
  
• Your child has trouble breathing.
  
  
• Your child's lips or fingernails are turning blue or white.

Where can I find support and more information?

Accepting that your child has cystic fibrosis may be hard. You may feel guilty about giving him on the CF gene and very sad and helpless to do anything about his condition. Your child and those close to him may feel scared, angry, or sad. These are normal feelings. You and your child may talk to his caregiver, family, or friends about the feelings you each have. You and your child can join a CF support group with others who also have children with 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 child's care. Discuss treatment options with your child's caregivers to decide what care you want for your child.

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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.

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