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Cystic Fibrosis in Children
WHAT YOU NEED TO KNOW:
What is cystic fibrosis?
Cystic fibrosis (CF) is a lifelong condition that affects your child's lungs, digestive system, and other organs. His mucus, tears, sweat, and saliva become so thick and sticky that they clog his lungs and digestive system. CF typically causes problems with breathing and with breaking down and absorbing food. Cystic fibrosis is a genetic disorder. If you or close relatives have CF, there is a higher risk that your children may also have it.
What are the signs and symptoms of CF?
The first sign of CF in your newborn is that he is unable to have a bowel movement. Your child may have frequent respiratory infections, such as sinusitis, bronchitis, or pneumonia. He may also have any of the following:
- Skin that tastes salty when you kiss him
- Frequent cough, wheezing, and shortness of breath
- Weight loss or failure to gain weight
- Clubbing of fingers or toes (become large, blunt, and rounded)
- Abdominal pain
How is CF diagnosed?
Your child's healthcare provider will ask if anyone in your family has CF. He will also ask about your child's symptoms. Your child may need any of the following tests:
- A sweat chloride test shows the amount of chloride in your child's sweat. The amount of chloride will be high if he has CF.
- Blood tests may be used to find signs of infection and to check kidney function. They may also show the gene that caused your child's CF.
- An x-ray will show inflammation and enlargement of your child's lungs. It will also show plugged airways and any fluid buildup.
- A bronchoscopy is a procedure to look inside your child's lungs to check for damage. A bronchoscope (thin tube with a light) is inserted into his mouth and moved down his throat to his lungs. Tissue and fluid may be collected from your child's lungs to be tested.
How is CF treated?
- Antibiotics help fight or prevent an infection caused by bacteria.
- Mucus thinning medicine is breathed in to help thin lung mucus so he can cough it up more easily.
- NSAIDs , such as ibuprofen, help decrease swelling, pain, and fever. This medicine is available with or without a doctor's order. NSAIDs can cause stomach bleeding or kidney problems in certain people. If your child takes blood thinner medicine, always ask if NSAIDs are safe for him or her. Always read the medicine label and follow directions. Do not give these medicines to children under 6 months of age without direction from your child's healthcare provider.
- Steroid medicine helps decrease inflammation.
- Bronchodilators help open the air passages in your child's lungs, and helps him breathe more easily.
- Pancreatic enzymes help your child's digestive system break down food and absorb nutrients properly.
- Extra oxygen may be needed if your child's blood oxygen level is lower than it should be. He may get oxygen through a mask placed over his nose and mouth or through small tubes placed in his nostrils.
- Surgery may be needed if your child has severe damage to organs, such as his liver or lungs. Ask your child's healthcare provider for more information about surgery.
What can I do to help my child breathe more easily?
- Airway clearance techniques are exercises to help remove mucus so your child can breathe more easily. Your child's healthcare provider will show him how to do the exercises. These exercises may be used along with machines or devices to help decrease your child's symptoms and risk for infection.
- Elevate your child's head when he sleeps. Your child may have trouble breathing when he lies flat. Use pillows or foam wedges to elevate his head. This may make it easier for him to breathe. Do not use pillows with a baby.
- Use a cool mist humidifier to increase air moisture in your home. This may make it easier for your child to breathe and to cough up mucus.
- Do not smoke around your child. If you smoke, it is never too late to quit. Smoke can make your child's coughing or breathing worse. Ask your healthcare provider for information if you need help quitting.
What can I do to help my child stay healthy?
- Take your child to get the flu vaccine each year. This will help prevent infection from the flu virus. Keep your child away from people who have a cold or the flu.
- Prevent the spread of germs. Cover your child's mouth when he coughs. Teach him to cough into a tissue or his shirt to prevent the spread of germs.
- Wash your hands and your child's hands often. Use soap and water. Carry germ-killing gel with you when there is no soap and water. Teach your child to avoid touching his eyes, nose, or mouth unless he has washed his hands first.
- Give your child a variety of healthy foods. Healthy foods include fruits, vegetables, whole-grain breads, low-fat dairy products, beans, lean meats, and fish. Your child may need to eat foods that have extra calories, fat, vitamins, or calcium. Ask your child's healthcare provider if your child needs to be on a special diet.
- Have your child exercise as directed. Ask your child's healthcare provider about the best exercise plan for your child. Physical activities can help loosen secretions in your child's airways and lungs, and help him breathe more easily.
When should I contact my child's healthcare provider?
- Your child has a fever.
- Your child has chills or feels weak or achy.
- Your child has trouble sleeping.
- Your child urinates less, has a dry mouth or cracked lips, or feels dizzy.
- You have questions or concerns about your child's condition or care.
When should I seek immediate care or call 911?
- Your child coughs up blood.
- Your child has trouble breathing.
- Your child's lips or fingernails turn blue or white.
- Your child has severe abdominal pain.
Care AgreementYou have the right to help plan your child's care. Learn about your child's health condition and how it may be treated. Discuss treatment options with your child's healthcare providers to decide what care you want for your child. 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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