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Primary Ciliary Dyskinesia in Children

WHAT YOU NEED TO KNOW:

Primary ciliary dyskinesia (PCD) is a disorder that affects the cilia in your child's body. Cilia are hair-like structures around cells that normally move like waves. Cilia in the lungs, ears, and sinuses normally carry mucus out. When mucus cannot be carried out, it sits in your child's airway. Bacteria in the mucus increase, leading to lung infections or respiratory failure. Your child may have fertility problems (trouble having children) later in life. PCD can keep certain parts of the reproductive system from working correctly. PCD is a genetic disorder (passed from both parents to the child).

WHILE YOU ARE HERE:

Informed consent

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.

Medicines:

  • Antibiotics help fight or prevent an infection caused by bacteria.
  • Mucolytics are breathed in to help thin lung mucus so your child can cough it up more easily.
  • Bronchodilators help open the air passages in your child's lungs, and help him or her breathe more easily.

Tests:

  • Pulmonary function tests will show how much oxygen your child's body is getting. Your child breathes into a mouthpiece connected to a machine. The machine measures how much air your child breathes in and out over a certain amount of time.
  • Pulse oximetry will show the decrease in blood oxygen without having to draw blood.
  • Nasal nitric oxide (NO) measurement is a test to find the amount in your child's body. PCD can cause the NO level to be lower than normal.
  • Transmission electron microscopy is sometimes used to find any problems with the cilia that can prevent them from moving correctly. Cells that have cilia are taken from inside your child's nose or airway.
  • CT scan or x-ray pictures may show a lung infection or other lung problem.
  • Genetic testing may be used to check for genes that cause PCD.

Treatment:

  • An incentive spirometer helps your older child take slow, deep breaths to expand and fill the lungs. For younger children, jumping and blowing games can help with deep breathing.
  • Chest physiotherapy (CPT) helps loosen mucus. During CPT, a healthcare provider lightly claps on your child's back and chest with his or her hands. This brings up mucus from the lungs and makes it easier to cough up.
  • Suction may be used to help remove mucus that your child is not able to cough up.
  • Oxygen may be given to help your child breathe easier. Oxygen can also decrease the strain on your child's heart and help prevent more problems.
  • Sinus surgery may be used to drain your older child's sinuses.

RISKS:

Your child may develop respiratory failure from lung infections. Respiratory failure is a condition that happens when your child's lungs cannot get enough oxygen into his or her blood. It can also happen when your child's lungs cannot get the carbon dioxide out of the blood. A buildup of carbon dioxide can cause damage to your child's organs. He or she may also develop an abnormal heart rhythm or heart failure.

CARE AGREEMENT:

You 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.

© Copyright IBM Corporation 2021 Information is for End User's use only and may not be sold, redistributed or otherwise used for commercial purposes. All illustrations and images included in CareNotes® are the copyrighted property of A.D.A.M., Inc. or IBM Watson Health

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.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.