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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).

DISCHARGE INSTRUCTIONS:

Call your local emergency number (911 in the US) if:

  • You have sudden chest pain.
  • You have sudden or more severe trouble breathing.
  • You are confused or feel faint.

Call your doctor or pulmonologist if:

  • Your lips or fingernails turn gray or blue.
  • You cough up blood.
  • You have a fever.
  • You cough more than usual or wheeze.
  • Your medicines do not relieve your symptoms.
  • You have questions or concerns about your condition or care.

Medicines:

Do not use cough suppressants (medicine to keep you from coughing). It is important to cough out mucus and irritants to prevent respiratory infections. You may need any of the following:

  • Antibiotics help prevent or fight an infection caused by bacteria. Antibiotics are usually given to treat an active infection. Your healthcare provider will tell you if he or she thinks you should take antibiotics to prevent an infection.
  • Medicines to open your airway or lower inflammation may be given if you have asthma along with PCD.
  • Take your medicine as directed. Contact your healthcare provider if you think your medicine is not helping or if you have side effects. Tell him or her if you are allergic to any medicine. Keep a list of the medicines, vitamins, and herbs you take. Include the amounts, and when and why you take them. Bring the list or the pill bottles to follow-up visits. Carry your medicine list with you in case of an emergency.

Airway clearance techniques (ACTs)

make it easier for you to cough mucus or objects out of your airway:

  • Breathing exercises include controlled breathing to help force mucus out of your airway. These may be used with techniques to help you cough forcefully to force something out of your airway. Chest expansion exercises help you fill your lungs fully.
  • Drainage and percussion are techniques to help loosen and drain mucus from several parts of your lungs. For percussion, another person forcefully pats your back. For drainage, you will be shown how to move into several positions to get mucus to drain.
  • Equipment may be used to help loosen, drain, or force mucus out. Machines may be used to vibrate your chest or airway. You may be shown how to combine breathing techniques with the vibration. A device may be used to keep some air in your airway when you breathe out. This helps prevent mucus from getting trapped.
  • PEP (positive expiratory pressure) therapy helps open and move mucus out of your lungs. You breathe into a small handheld device that has a valve. You breathe out against a set amount of pressure that helps hold your airway open. Pressure also allows air to get behind and loosen mucus so you are able to cough it up. Some PEP therapy devices use vibrations as you breathe out to help loosen mucus.

Do not smoke, and avoid secondhand smoke:

Nicotine and other chemicals in cigarettes and cigars can cause lung damage and make breathing problems worse. Ask your healthcare provider for information if you currently smoke and need help to quit. E-cigarettes or smokeless tobacco still contain nicotine. Talk to your healthcare provider before you use these products.

Manage PCD:

  • Go to pulmonary rehabilitation (rehab) as directed. Pulmonary rehab is a program that can help you learn how to manage PCD and prevent breathing problems. Your program will include an aerobic exercise plan. Examples of aerobic exercises are walking quickly or jogging, swimming, and riding a bicycle. This kind of exercise helps strengthen your heart and lungs. Aerobic exercise is one of the best ways to manage PCD.
    Walking for Exercise
  • Keep your airway clear and open. Do airway clearance techniques (ACTs) as needed. Healthcare providers will show you how to do these in pulmonary rehab. Thin mucus by drinking more liquids or using a cool mist humidifier. You may be able to breathe more easily while you sleep if you keep your head higher than your body. If your bed is not adjustable, put extra pillows under your shoulders and neck.
  • Prevent infections. Tell your healthcare provider when you have signs of a respiratory infection. Early treatment may help control the infection so it does not become severe. Ask about vaccines you may need. Get a flu vaccine every year as soon as it becomes available. You may also need a vaccine to help prevent pneumonia. Stay away from people who are sick. Wash your hands often. Use soap and water, or a gel hand sanitizer if soap and water are not available.
    Handwashing
  • Talk to healthcare providers about your plans to have children. If you have infertility problems, procedures may be available to help you achieve a pregnancy. Your healthcare provider may be able to refer you to a fertility clinic if needed.

Use a nasal rinse as directed:

A nasal rinse is saline (a salt water solution) used to thin mucus and clear irritants and allergens from your sinuses. Saline is flushed into your sinuses through your nose. You can get a nasal rinse kit at a pharmacy. A nasal rinse kit provides all the supplies you need. You can also make salt water solution at home. Mix ½ teaspoon of uniodized salt with 8 ounces of lukewarm water. Use only distilled water, sterile water, or filtered water. A bulb syringe, sinus rinse bottle or irrigator device, nasal cup, or neti pot can be used for the flush.

Follow up with your doctor or specialist as directed:

You may need to come in 4 or more times each year for tests. Write down your questions so you remember to ask them during your visits.

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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

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