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Primary Ciliary Dyskinesia

WHAT YOU NEED TO KNOW:

Primary ciliary dyskinesia (PCD) is a disorder that affects the cilia in your body. Cilia are hair-like structures around certain cells that normally move like waves. In the respiratory system, PCD can prevent lung cilia from clearing mucus and irritants out of the lungs, nose, and sinuses. Bacteria sit in the airway and increase, leading to respiratory infections. In a man's reproductive system, PCD can prevent sperm from moving toward an egg to fertilize. In a woman's system, PCD can prevent the fallopian tubes from moving an egg toward the uterus. These problems can cause infertility. 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 you 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 medical care in words you know. Before you sign the consent form, understand the risks and benefits of what will be done. Make sure all your questions are answered.

Medicines:

  • Antibiotics help fight or prevent an infection caused by bacteria.
  • Mucus thinning medicine is breathed in to help thin lung mucus so you can cough it up more easily.
  • Bronchodilators help open the air passages in your lungs, and helps you breathe more easily.

Tests:

  • Pulmonary function tests will show how much oxygen your body is getting. You breathe into a mouthpiece connected to a machine. The machine measures how much air you breathe 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 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 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 you take slow, deep breaths to expand and fill your lungs with air.
  • Chest physiotherapy (CPT) helps loosen mucus. During CPT, a healthcare provider lightly claps on your back and chest with his or her hands. This brings up the mucus from your lungs and makes it easier to cough it up.
  • Suction may be used to help remove secretions that you are not able to cough up.
  • Oxygen may be given to help you breathe easier. Oxygen can also decrease the strain on your heart and can help prevent more problems.
  • Sinus surgery may be used to drain your sinuses.

RISKS:

You may develop respiratory failure from lung infections. Respiratory failure is a condition that happens when your lungs cannot get enough oxygen into your blood. It can also happen when your lungs cannot get the carbon dioxide out of your blood. A buildup of carbon dioxide in your blood can cause damage to your organs. You may also develop an abnormal heart rhythm or heart failure. If you are a woman, your risk for ectopic pregnancy may be higher if PCD prevents movement in the fallopian tubes. Ectopic pregnancy means a fertilized egg implanted in the fallopian tube. This can be life-threatening.

CARE AGREEMENT:

You have the right to help plan your care. Learn about your health condition and how it may be treated. Discuss treatment options with your healthcare providers to decide what care you want to receive. You always have the right to refuse treatment.

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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.

Further information

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