What is pediatric bronchiectasis?
Bronchiectasis is a condition in which the small airways of the lungs become large and dilated. Bronchiectasis occurs when repeated injury to the airways makes the airway tissue irritated and inflamed. In some cases, this irritation causes the airway wall to become thick and the airway becomes larger. The abnormally large airways then collect secretions (mucus, phlegm, pus) that contribute to a chronic wet cough. Bacteria can get stuck in these airway secretions and cause recurrent infections and further contribute to the irritation and inflammation.
We often describe bronchiectasis as a cycle of airway injury: airway irritation, mucus collection and infection. When the cycle worsens, people experience increased coughing, known as an exacerbation.
What causes bronchiectasis?
Bronchiectasis may be caused by an inherited or genetic condition, such as cystic fibrosis, or repeated airway injury. People with cystic fibrosis have thick mucus that is difficult to clear from their airways. The mucus contributes to inflammation and infection that cause repeated airway injury resulting in bronchiectasis.
The amount of inflammation and irritation created by airway injury is related to the development of bronchiectasis. Some children with repeated airway injury develop bronchiectasis and some children who seem to have the same amount of injury may not develop bronchiectasis.
- Airway injury may be caused by:
- Repeated pneumonia or other lung infections
- Trouble fighting infections (immunodeficiency)
- Severe asthma
- A very severe lung infection (called bronchiolitis obliterans)
- Repeated aspiration (food, liquid or saliva being inhaled into the lungs)
- Aspiration of a foreign object that becomes stuck and causes local airway injury
- Tracheoesophageal fistula (abnormal connection between the esophagus and trachea)
- Abnormal inflammation may be caused by:
- Autoimmune or rheumatologic diseases
- Abnormal mucus collection in the airways may be caused by:
- Abnormal airway anatomy like tracheomalacia, airway stenosis and esophageal atresia that makes it hard for children to cough out mucus
- Trouble coughing to clear mucus, which can occur in those with muscle weakness
- Primary ciliary dyskinesia, a rare inherited disease where the cilia (hair cells) that line the airways do not move correctly to clear mucus
Some children who develop bronchiectasis may have more than one cause. It is important to determine the cause of bronchiectasis as it can affect treatment. Because it’s hard to predict who will develop bronchiectasis over time, our goal is to prevent bronchiectasis by preventing or treating airway injury and inflammation whenever possible.
Who gets bronchiectasis?
Bronchiectasis develops at different ages depending on the injury, but it may be worse in children who are older and have had repeated airway injury for a longer period. Bronchiectasis appears worse in certain ethnic groups including Aboriginal Australians and Alaska Natives. There is no clear difference in bronchiectasis by gender.
What are the signs and symptoms of bronchiectasis?
Bronchiectasis signs and symptoms can include:
- Daily wet cough
- Wet cough that may be worse at certain times (during exacerbation)
- Increased mucus production
- Poor growth or weight loss
- Chest pain
- Coughing up blood
- Trouble with exercise
- Bronchiectasis signs and symptoms that your child’s provider may observe during a physical exam include: Mucus moving around when we listen to your lungs (called crackles)
- Low oxygen levels in the blood
- Clubbing, which is changes in the areas under and around the nails
- Airway obstruction during pulmonary function testing
What tests are used to diagnose bronchiectasis?
- Chest computerized tomography (CT) scan: Bronchiectasis is diagnosed when the airways of the lungs are abnormally large. A chest CT can help determine if bronchiectasis is present in one or several areas of the lungs. One goal for doing a CT scan is to identify airway injury when it is subtle and reversible, before it progresses to bronchiectasis. Another goal is to find and treat the causes of bronchiectasis so that the disease does not progress. Chest CT scans can be used to monitor the size of the airways over time and determine if bronchiectasis is getting worse.
- Chest X-ray: Chest X-rays are commonly done when children have a chronic cough. Bronchiectasis is often too subtle to see on a typical chest X-ray, but more severe bronchiectasis can sometimes be seen on a chest X-ray.
- Spirometry (pulmonary function testing): Our lung doctors commonly recommend pulmonary function testing. Bronchiectasis is one of the causes of “fixed airway obstruction,” or low lung function that does not improve after being given an inhaled medication called albuterol. If your child has fixed airway obstruction during pulmonary function testing, their doctor may ask if they have signs or symptoms of bronchiectasis.
- Sputum (mucus) collection or throat swab: Children with bronchiectasis can have bacteria present in their sputum. If children can cough up sputum, we will collect it to evaluate for infection. If a child is unable to cough up sputum, we can take a throat culture from the back of the throat using a swab to test for bacteria.
- Bronchoscopy with bronchoalveolar lavage: Flexible bronchoscopy is a surgical method to evaluate the anatomy and size of the largest airways (the trachea and mainstem airways). Flexible bronchoscopy does not show the smallest airways where bronchiectasis occurs, but the lung fluid sample that we collect during bronchoscopy, called the bronchoalveolar lavage, is often used to evaluate for infection and inflammation that can cause or worsen bronchiectasis.
- Swallowing assessment: Children with repeated aspiration are at increased risk of bronchiectasis. The swallowing team at Children’s Colorado will perform a video fluoroscopic swallowing study and fiberoptic endoscopic evaluation of swallowing, which uses a camera to diagnose aspiration.
- Other tests: Lab work may help determine if your child has a genetic disorder that predisposes them to the development of bronchiectasis or if they have abnormal inflammation, atypical infections or trouble fighting infections. Sweat tests are used to diagnose cystic fibrosis. Nasal ciliary biopsies can be used to diagnose primary ciliary dyskinesia.
What to expect from tests used to diagnose bronchiectasis
If there are concerns that your child has bronchiectasis, your child’s lung doctor will explain the next steps for testing. The type of tests we perform depend on the symptoms your child is experiencing. Your child’s doctor will discuss what to expect from each of the tests and what we can do to minimize stress, pain or other concerns during the tests.
Chest CT scans only take a few seconds, but young children often need anesthesia to get the best image quality. Older children who have difficulty holding still or have anxiety can discuss with our child life, radiology and anesthesia team members to determine the best way to obtain a chest CT scan.
How do we diagnose bronchiectasis?
- Evaluating chronic wet cough: Children with chronic wet cough may be evaluated to determine if they have bronchiectasis. A chest CT scan is the best way to evaluate the small airways and determine if they have been damaged. Sometimes children with chronic wet cough have airway damage but have not yet developed bronchiectasis. You can discuss with your lung doctor if your child would benefit from treatment and monitoring to try to prevent bronchiectasis.
- Monitoring for bronchiectasis: Children with cystic fibrosis, primary ciliary dyskinesia, chronic aspiration and others are at increased risk of developing bronchiectasis over time. Your lung doctor will help discuss ways to limit airway injury to prevent bronchiectasis from developing or getting worse. We may perform a chest CT scan, chest X-ray, lung function and sputum/throat swab cultures more than once to monitor for the development of bronchiectasis.
How is bronchiectasis treated?
Bronchiectasis is treated by trying to stop the cycle of airway injury, inflammation, mucus accumulation and infection. The treatments for bronchiectasis are aimed at stopping this cycle of injury.
- Airway clearance: Airway clearance is the most important treatment for bronchiectasis. Airway clearance, also known as chest percussion therapy (CPT), helps to move mucus from the smaller airways to the larger airways so it can be cleared more easily. CPT can be done using a hand percussor, a chest vest or handheld devices (acapella or aerobika). Most people with bronchiectasis perform airway clearance one or more times per day, and your doctor may recommend that you do extra airway clearance if you are experiencing an exacerbation. The type of airway clearance recommended will depend on several factors including the cause of the bronchiectasis, your child’s age and your child’s development. Exercise is also a great form of airway clearance, so your child’s lung doctor may recommend it, if appropriate, based on your child’s development.
- Coughing: Our goal is to decrease the mucus in your child’s airways so they don’t cough all day. However, directed coughing, known as huff coughs, during airway clearance treatments can help remove mucus from the lungs. If your child’s mucus is especially thick or sticky, some children require inhaled medications to help thin their mucus to make it easier to cough up. Children with poor muscle tone may require an insufflation/exsufflation device to make their cough more effective.
- Antibiotics: Most children with bronchiectasis need antibiotics to treat a bronchiectasis exacerbation (when the child is having more cough and mucus production than normal). Some children with bronchiectasis may require daily or weekly antibiotics to treat infection or inflammation in their airway. Some children may also require inhaled antibiotics if they grow certain bacteria in their mucus.
- Anti-inflammatory medication: The best way to decrease inflammation is to stop or limit the injury to the airway. Some children with bronchiectasis benefit from inhaled steroid medication daily or inhaled or oral steroids during exacerbations. Other specific anti-inflammatory medications may be indicated based on the specific cause of bronchiectasis.
Treating the cause of airway injury is the best way to prevent bronchiectasis from developing or getting worse. Children with cystic fibrosis or primary ciliary dyskinesia are evaluated for bronchiectasis in their regular visits at our pulmonary clinic.
Why choose us for treatment of bronchiectasis?
The Breathing Institute at Children’s Colorado is nationally ranked by U.S. News & World Report as one of the best pulmonology programs in the country. Our researchers are continuously working to improve the way we measure airways and diagnose bronchiectasis. Our multidisciplinary approach to the treatment of bronchiectasis enables us to provide high-quality, specialized care for your child. We have access to a variety of pediatric subspecialists, which enables us to provide a full spectrum of care. Our specialty care includes:
- The Primary Ciliary Dyskinesia Clinic is part of a national consortium of PCD clinics.
- The multidisciplinary Aerodigestive Program is a group of experts in different disciplines who can help determine and treat the cause of chronic wet cough and bronchiectasis. They can also evaluate children with chronic aspiration and airway abnormalities including tracheomalacia, airway stenosis and esophageal atresia (with or without tracheoesophageal fistula). These pediatric experts are nationally recognized for treatment of airway problems and esophageal atresia.
- Experts from the Neuromuscular Program in the Neuroscience Institute can evaluate children with impaired cough due to low muscle tone. This program is nationally recognized for their treatment of children with low muscle tone.
- We collaborate with pediatric radiologists in our Imaging Services Department at Children’s Colorado to diagnose bronchiectasis earlier and improve chest CT scans and other imaging technology while limiting radiation exposure.
- Researchers in The Mike McMorris Cystic Fibrosis Research and Care Center are part of national clinical trials to improve the treatment of bronchiectasis in children with cystic fibrosis and they have been involved in ways to diagnose bronchiectasis in all patients earlier.
- The American Thoracic Society is a nonprofit organization that works to improve health worldwide by advancing research, clinical care and public health in respiratory disease, critical illness, and sleep disorders. They provide additional patient education information on bronchiectasis.
- The National Institutes of Health, National Heart, Lung, and Blood Institute provides global leadership for a research, training and education program to promote the prevention and treatment of heart, lung and blood disorders and enhance the health of all individuals.
- The American Lung Association leads the way in helping all Americans breathe freely through their pioneering model of public health that includes health education and advocacy, which is grounded in lifesaving research.
- The Cystic Fibrosis Foundation funds research and drug development, partners with the CF community and advances high-quality, specialized care.
- The PCD Foundation is a nonprofit patient advocacy foundation for individuals with inherited ciliary disorders and their caregivers.
What happens to children with bronchiectasis over time?
Some forms of bronchiectasis continue to worsen over time. Some forms of bronchiectasis are more localized and may not progress over time.
What if a cause of bronchiectasis cannot be found?
Sometimes your lung doctor will order tests to determine the cause of airway injury and bronchiectasis, but no clear cause can be found. Ongoing discussions with your lung doctor will be important as the medical community continues to learn more about the causes of bronchiectasis and the ways to diagnose these disorders. Treating the cycle of airway injury, inflammation and infection and limiting ongoing airway injury are important to lung health. Avoiding smoking, vaping or secondhand smoke exposure and getting regular vaccinations including flu vaccines, are other important ways to improve and maintain your child’s lung health.
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