Primary ciliary dyskinesia, PCD, treatment focuses on the specific symptoms and complications a person has. Overcoming challenges caused by PCD is difficult. Unfortunately, no treatment is available yet to fix faulty airway cilia.
The main goals of treating PCD are to:
- Manage and treat lung, sinus, and ear infections
- Removal of trapped mucus from the lungs and airways
Specialists Treat People Having PCD
A team of doctors is needed for someone who has PCD. For example, a neonatologist may suspect PCD if a newborn has breathing problems at birth. A neonatologist is a doctor who specializes in treating newborns.
A pediatrician may suspect PCD if a child has chronic sinus, ear, and/or lung infections.
An ear, nose and throat specialist, ENT, ( otolaryngologist), also 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 specialized in diagnosing and treating lung diseases and problems related to PCD. Most people who have PCD have lung problems at some point in their lives.
Heather and Logan both see a pulmonologist every three months. They have a pulmonary function test, PFT, which determines how their lungs are functioning.
A pathologist is a doctor who specializes in identifying diseases by studying cells and tissues under a microscope. This type of doctor may help diagnose PCD by looking at cilia under a microscope. This method was performed when Heather and Logan were children, however, no definite answers were found at that time.
A pathologist also may look at mucus samples to see what types of bacteria are causing infections. Heather and Logan have this done when they have a severe cold. This information can help your doctor decide which treatments to prescribe.
Treatment for Primary Ciliary Dyskinesia: Breathing and Lung Problems
Standard treatments for breathing and lung problems in people who have PCD are; chest physical therapy (CPT), exercise, and medicines. One of the main goals of these treatments is to produce a cough. Coughing helps clear mucus from the airways, important for people who have PCD. Medicines that suppress coughing are often avoided.
Chest Physical Therapy
CPT also is 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. Whenever Heather and Logan were hospitalized for surgery or pneumonia, this method was used several times a day by a pulmonary physical therapist.
You might sit down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs.
CPT Devices for Treatment of Primary Ciliary Dyskinesia PCD
Heather and Logan find CPT 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. The airwaves force the mucus that’s deep in your lungs toward your upper airways so you can cough it up.
A small hand-held device that you breathe out through. The device causes vibrations that 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.
Heather and Logan’s Treatments
Heather and Logan use a nebulizer for their lung therapy. A nebulizer changes medication from a liquid to a mist so that it can be more easily inhaled into the lungs. Heather does this therapy two times every day, each session taking an hour. Sometimes she gets discouraged at the amount of time and energy involved, however, she knows that overcoming challenges caused by excess mucus can only be achieved when she perseveres.
Part of the therapy involves breathing out through a bronchodilator; a device that helps keep the airways open so mucus can move up more easily, followed by a lot of coughing. Coughing is a lot of work! However, Heather has learned that she coughs less in general, and breathes more easily when she does her therapy regularly.
Logan does this therapy only when he has a severe cold to prevent getting pneumonia.
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.
Because Heather and Logan have Miller syndrome, which causes physical disabilities with all their joints, legs and arms, and chronic pain in the back and hips, they aren’t able to exercise for their pulmonary therapy.
Medicines
If you have PCD, your doctor may prescribe antibiotics, anti-inflammatory medicines, or bronchodilators. These medicines help treat lung infections, reduce swelling, and open up the airways.
Antibiotics are the main treatment to prevent or treat lung infections. As a result, a doctor might prescribe both oral or intravenous (IV) antibiotics.
Oral antibiotics are used to treat mild lung infections. For severe or hard-to-treat infections, IV antibiotics given through a tube inserted into a vein are used. Sending mucus samples to a pathologist to analyze will help to find out which bacteria are causing the infection.
Anti-inflammatory medicines can help reduce swelling in your airways caused by ongoing infections and may be inhaled or oral.
Bronchodilators help open the airways by relaxing the muscles around them. You inhale these medicines. Patients often, use a bronchodilator just before CPT to help clear mucus from their lungs. You also may take bronchodilators before inhaling be other medicines into your lungs.
Treatments for Sinus and Ear Infections
To treat infections, your doctor may recommend saline nasal washes and anti-inflammatory nasal spray. If these treatments aren’t enough, you may need medicines, such as antibiotics.
Doctors insert small tubes into the eardrums to help drain mucus from the ears in a procedure called Tympanostomy (tim-pan-OS-toe-me). Children who have hearing problems caused by PCD may hear better after this procedure. Doctors performed this procedure on Logan’s ears when he was a baby.
Treatments for Advanced Lung Disease
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. Heather and Logan both have bronchiectasis.
Doctors may recommend surgery to remove part of the lung when severely affected.
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