Primary Ciliary Dyskinesia
Medically reviewed by Drugs.com. Last updated on Jan 5, 2023.
What is primary ciliary dyskinesia (PCD)?
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).
What are the signs and symptoms of PCD?
- Respiratory infections that start early in life and continue into adulthood
- Daily, year-round cough that produces mucus, and congestion in your sinuses
- Hearing problems from fluid in the ears and ear infections, nasal polyps, or abnormal sinuses
- Abdominal organs that are on the opposite side of the body than usual, more than 1 spleen, or no spleen
- Infertility in men and women, or ectopic pregnancy in women
- Congenital heart disease
- Rarely, too much fluid in the brain, or an eye condition such as retinitis pigmentosa
How is PCD diagnosed?
Your healthcare provider will ask about your signs and symptoms and when they began. He or she will listen to your heart and lungs, and listen to how you breathe. PCD might be diagnosed based on lifelong signs and symptoms. Examples are a constant cough or infections that antibiotics do not clear up. Tell your provider if you know you had respiratory distress as a newborn. Tests may be available to help confirm PCD:
- 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.
How is PCD treated?
PCD cannot be cured, but the following may help you control signs and symptoms:
- Medicine may be used to help fight or prevent an infection caused by bacterial, or to help thin mucus. Medicines to open your airway or lower inflammation may be given if you have asthma along with PCD. Do not use cough suppressants (medicine to keep you from coughing). It is important to cough out mucus and irritants to prevent respiratory infections.
- 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.
What can I do to 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.
- 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.
- 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.
- 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.
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.
When should I call my doctor?
- 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.
Care AgreementYou 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. 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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