Cystic Fibrosis In Children

WHAT YOU SHOULD KNOW:

• 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 up ducts inside body organs. It is caused by an abnormal gene that affects the normal functions of the body. Your child will have received one of these abnormal genes from each of his parents. Signs and symptoms of CF include frequent cough, wheezing, and shortness of breath that gets worse over time. He may get frequent respiratory infections. Your child may not gain weight or may lose weight, feel very weak, and have problems with absorbing food. He may lose a lot of chloride (salt) through sweating, which may cause weakness.
  
  
• To diagnose CF, your child may need newborn screening or other blood tests, chest x-ray, and a sweat chloride test. He may be given medicines to help him breathe easier and kill germs causing any infection. He may need breathing exercises and special devices to help remove mucus and let him breathe easier. Your child may need medicines to help break down food and absorb nutrients properly. He may be given a special diet and vitamins to help him gain weight and energy. Your child may need surgery to repair or completely replace damaged organs, such as his lungs or liver. Diagnosing and treating your child's CF as soon as possible may help relieve problems with digestion and trouble breathing.

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.

RISKS:

• Treatment of your child's cystic fibrosis may cause side effects. He may get an allergy with his medicines and have itching, swelling, and redness of his skin. Your child may have an infection or bleeding with surgery. Even after successful treatment, his symptoms may not disappear at once, may still continue, or may come back later on. Even with treatment, your child may still get chronic lung problems that can damage his lungs over time. A chronic condition is one that may slowly get worse, come and go, and last a long time. With proper management, children with CF now live longer, some well into their 30's and beyond. As an adult, your male child may have trouble getting his female partner pregnant.
  
  
• If left untreated, your child's organs may continue to get damaged and lose their function over time. Your child may not get enough nutrients, and he may lose weight and get very weak. He may not grow and gain weight as fast as other children his age would. His bones may get very weak and he may have an increased risk for fractures (broken bones). Your child may get pancreatitis or have diabetes if his pancreas gets badly damaged. His liver can have cirrhosis (scarring) and not work normally. He may get chronic lung damage. He may have trouble breathing, or get bleeding, collapse of a lung, or widening of his airways. Infections in your child's lungs can happen often and cause more trouble breathing or even death. Call your caregiver if you have questions about your child's condition, medicines, or care.

WHILE YOU ARE HERE:

Informed consent:

A consent form is a legal document that explains the tests, treatments, or procedures that your child may need. Informed consent means you understand what will be done and can make decisions about what you want. You give your permission when you sign the consent form. You can have someone sign this form for you if you are not able to sign it. You have the right to understand your child's medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done to your child. Make sure all of your questions are answered.

Diet:

Your child may need a special diet to help him gain weight and have good nutrition. A caregiver called a dietician will help you and your child choose the right foods to help him get back his energy and strength. Your child may be given extra nutrients such as vitamins A, D, E, and K, or calcium.

Oxygen:

Your child may need oxygen if his blood oxygen level is lower than it should be. Oxygen will help your child breathe easier. Your child may get oxygen through small tubes placed in his nostrils, or through a mask. He may instead be placed in an oxygen tent. Never take off your child's oxygen tubes or mask or remove him from the tent without asking his caregiver first.

Medicines:

Your caregiver may give your child the following kinds of medicines:

• Antibiotics: This medicine is given to help prevent or treat an infection caused by bacteria.
  
  
• Anti-inflammatory medicines: You child may need to take ibuprofen or steroid medicine for a short time to decrease inflammation (swelling), and help him breather better. Give your child these medicines exactly as ordered by his caregiver. Ask for more information about these medicines.
  
  
• Bronchodilators: Bronchodilators may be given to help open the air passages in your child's lungs to help him breathe easier.
  
  
• Mucus thinning medicine: This is a medicine your child inhales (breathe in) to help thin the mucus in his respiratory system.
  
  
• Pancreatic enzymes: These chemicals help your child's digestive system break down food and absorb nutrients properly.

Tests:

• 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 examine the gene that causes CF.
  
  
• Chest CT scan: This is also called a CAT scan. A special x-ray machine uses a computer to take pictures of your child's lungs and airway structures. Caregivers will look to see if he has bronchiectasis (widening and damage in large airways) caused by mucus blockage.
  
  
• Fecal fat test: Caregivers may ask for a sample of your child's stool to test its fat content. With CF, your child's body may not be able to absorb fat from the foods he eats.
  
  
• Pulmonary function tests: Pulmonary function tests, also called PFTs, help caregivers learn how well your child's lungs work. PFTs may also help your caregivers decide on the best treatment for your child. During the tests, your child will breathe into a mouthpiece connected to a machine. The machine measures how much air he breathes in and out over a certain amount of time. This helps caregivers to see how well his lungs are moving and working.
  
  
• 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. It can also help his caregiver choose which medicine is best for him.
  
  
• 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.

Treatment options:

Your treatment may change if your health problem is not being controlled. This is often decided after you have tests. You may have some of the following treatments alone or together.

• Airway clearance techniques: 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. Your child may have one or more of the following:
  
  
  • Active cycle breathing techniques: These involve controlled breathing, forced breathing, and chest exercises.
    
    
  • Autogenic drainage: This is a breathing technique used to bring up mucus from lower parts of the airways or lungs.
    
    
  • High frequency chest wall oscillation: Your child will need to wear a special vest during this treatment. The vest vibrates to loosen up mucus in his airways.
    
    
  • Oscillating positive end pressure (PEP) therapy: This uses a pocket sized device that controls your child's breath as he breathes out through it. The device may seem to vibrate, or flutter as the air goes out in a controlled way. This helps remove mucus and help your child breathe easier.
    
    
  • Percussion: This involves forcefully patting on your child's back with a cupped hand or soft plastic cup. This is used to loosen mucus stuck in his airways. This may look uncomfortable, but really is not. Percussion may also be done with an electric percussor.
    
    
  • Positive expiratory pressure (PEP) therapy: This is used to decrease the amount of air your child breathes out. This leaves some air in your child's small airways, so that they do not close up when he breathes out. A mask attached to a machine is put over your child's face to help control his breathing. A caregiver sets the machine and watches your child during this procedure.
    
    
  • Postural drainage (PD): This treatment uses body position and gravity to help bring up sputum (mucus) from your child's lungs. A caregiver will place your child in different positions to help the sputum drain to larger air passages. Then your child can cough it out more easily. During PD, a caregiver may also do percussion or use a small machine that vibrates on your child's chest. This breaks up the sputum in his lungs, making it easier to cough up. PD may make it easier for your child to breathe, decrease the chance of infection, and help him heal faster.
  
  
• Surgery: Organs damaged by CF can be repaired through surgery. If your child's lungs or liver get badly damaged by CF, he may need a lung or liver transplant.
  
  
• Ventilator: If your child gets a very bad lung condition, he may need to be put on a ventilator for awhile. A ventilator is a special machine that breathes for your child if he cannot breathe well on his own. He may have an endotracheal tube (ET tube) in his mouth or nose. A tube called a trach may go into an incision (cut) in the front of his neck. The ET tube or trach is hooked to the ventilator. The ventilator can also give oxygen to 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.

Learn more about Cystic Fibrosis In Children (Inpatient Care)