Primary Ciliary Dyskinesia Treatment

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Unfortunately, no treatment is available yet to fix faulty airway cilia, which are the tiny, hairlike structures that line the airways. Treatments for primary ciliary dyskinesia (PCD) focus on which symptoms and complications you have.

The main goals of treating PCD are to:

• Control and treat lung, sinus, and ear infections
• Remove trapped mucus from the lungs and airways

You may have several providers caring for you besides your primary healthcare provider.

• An ear, nose, and throat (ENT) specialist may help diagnose and treat PCD. If a child has chronic sinus or ear infections, an ENT specialist may be involved in the child’s care.
• A pulmonologist may help diagnose or treat lung problems related to PCD. This type of doctor specializes in diagnosing and treating lung diseases and conditions. Most people who have PCD have lung problems at some point in their lives.

Standard treatments for breathing and lung problems are chest physical therapy (CPT), exercise, and medicines.

One of the main goals of these treatments is to get you to cough. Coughing clears mucus from the airways, which is important for people who have PCD. For this reason, your provider may also advise you to avoid medicines that suppress coughing.

Chest physical therapy

CPT is also called chest clapping or percussion. It involves pounding your chest and back over and over with your hands or a device to loosen the mucus from your lungs so that you can cough it up.

You might sit down or lie on your stomach with your head down while you do CPT. Gravity can help drain the mucus from your lungs.

Some people who perform CPT find it hard or uncomfortable to do. Several devices have been made to help with CPT, such as:

• An electric chest clapper, known as a mechanical percussor
• An inflatable therapy vest that uses high-frequency airwaves that forces the mucus deep in your lungs toward your upper airways so you can cough it up
• A small handheld breathing device that causes vibrations to dislodge the mucus
• A mask that creates vibrations to help break the mucus loose from your airway walls

Breathing techniques also may help dislodge mucus so you can cough it up. These techniques include forcing out a couple of short breaths or deeper breaths and then doing relaxed breathing. This may help loosen the mucus in your lungs and open your airways.

Exercise

Aerobic exercise that makes you breathe harder helps loosen the mucus in your airways so you can cough it up. Exercise also helps improve your overall physical condition.

Talk with your healthcare provider about what types and amounts of exercise are safe for you or your child.

Medicines

If you have PCD, your provider may prescribe antibiotics, bronchodilators, or anti-inflammatory medicines. These medicines help treat lung infections, open up the airways, and reduce swelling.

• Antibiotics are the main treatment to prevent or treat lung infections. Oral antibiotics are often used to treat mild lung infections. For serious or hard-to-treat infections, you may be given intravenous (IV) antibiotics through a tube inserted into a vein. To help decide which antibiotics you need, your provider may send mucus samples to a pathologist. The pathologist will try to find out which bacteria are causing the infection. Side effects of antibiotics depend on which antibiotic is used but may include diarrhea; problems with your hearing, balance, kidneys; or low white blood cell counts.
• Bronchodilators help open the airways by relaxing the muscles around them. You inhale these medicines. Often, they’re taken just before CPT to help clear mucus from your lungs. You may also take bronchodilators before inhaling other medicines into your lungs. Side effects can include trembling in the hands, headache, dizziness, and nausea.
• Anti-inflammatory medicines can help reduce swelling in your airways that’s caused by ongoing infections. These medicines may be inhaled or taken by mouth. Side effects can include mainly gastrointestinal symptoms, such as abdominal pain, nausea, vomiting, and diarrhea.

To treat infections, your healthcare provider may recommend saline nasal washes and anti-inflammatory nasal sprays. If these treatments aren’t enough, you may need medicines, such as antibiotics. If antibiotics don’t work, surgery may be an option.

Tympanostomy is a procedure in which small tubes are inserted into the eardrums to help drain mucus from the ears. This procedure may help children who have hearing problems caused by PCD.

Nasal or sinus surgery may help drain the sinuses and provide short-term relief of symptoms. However, the long-term benefits of this treatment are unclear.

People who have PCD may develop a serious lung condition called bronchiectasis. This condition often is treated with medicines, hydration (drinking plenty of fluids), and CPT. If you have serious lung damage from bronchiectasis, you may need surgery to remove part of the lung.

Rarely, and when other treatments haven’t worked, lung transplant may be an option for serious lung disease. A lung transplant is surgery to remove the damaged lung and replace it with a healthy lung from a deceased donor.