Bronchiectasis

What is ? Bronchiectasis (pronounced bron-kee-ek'-tas- is) is a condition of the airways in the . These airways (bronchial tubes) are tube-like structures that branch from the into the right and left lungs. When a person has bronchiectasis the airways are permanently and abnormally widened (dilated) and inflamed. These damaged airways can no longer clear and from the well. This causes a , production and to occur more often.

Bronchiectasis is caused by one or more ) infectious or inflammatory insults to the lungs. Bacterial (Pseudomonas People with bronchiectasis are more likely to aeruginosa, Staphylococcus aureus, get lung infections. Each lung can Klebsiella) make the bronchiectasis worse. Therefore, Mycobacterial infections ( early diagnosis and treatment of avium complex) bronchiectasis is very important. Fungal infections (histoplasmosis)

How Can You Develop Bronchiectasis? Immune Diseases There are many causes of bronchiectasis — People with immune deficiencies such as some are acquired and others you may be deficiencies are more likely to have born with (congenital). The following is a list of repeated lung infections which can damage the the most important and/or common causes. airways and cause bronchiectasis.

Aspiration Infections Chronic occurs when a person Infections can damage the airways and cause inhales oral or stomach material into his/her lungs. If bronchiectasis. They may also be a severe or recurrent, aspiration can lead to consequence which in turn can lead to of the airways and causes worsening bronchiectaisis. bronchiectasis. The aspiration can occur from: Impaired ability to swallow (oropharyngeal Examples include: dysphagia) which may cause saliva or Viral infections (, adenovirus, food to enter the lung. Gastroesophageal reflux disease (GERD)

which occurs when the valve of smooth Chronic obstructive pulmonary disease muscle between the esophagus and the (COPD) and allergic bronchopulmonary stomach does not function properly. This are diseases that can cause allows contents (acid and non-acid) to flow obstruction of the airways. back up into the esophagus. The stomach contents may enter the lungs and irritate What Happens in the Lungs with the airways. Some of Bronchiectasis? GERD include: heartburn or sour in First, inflammation occurs in the walls of the airways mouth, but many (possibly up to 30 from a number of causes just discussed. This percent) of people with GERD may have inflammation causes injury to the airways. The no symptoms (“silent” GERD). resulting loss of the normal defenses in the lungs leads to impaired drainage of the airways. This Autoimmune Diseases makes the airways susceptible to infections. , , Sjogren’s syndrome and Repeated lung infections can worsen the damage to Wegener’s granulomatosis are examples of the airway walls. rheumatologic, autoimmune or connective tissue diseases that can cause bronchiectasis. What are the Symptoms? Symptoms of bronchiectasis include a cough. The Genetic Diseases cough may be productive of mucus. With infections causes impaired drainage of the mucus may be discolored, foul-smelling and may mucus and bacteria from the airways. This contain blood (). Shortness of breath, leads to recurrent lung infection and wheezing, weight loss and fatigue can also occur. bronchiectasis. Classic cystic fibrosis is Some people with bronchiectasis also have chronic obvious at birth, but there are forms of . This requires further evaluation since cystic fibrosis that may not be recognized bronchiectasis and sinusitis may be due to the same until adulthood. underlying disease.

Primary ciliary dyskinesia impairs the If left untreated, symptoms of bronchiectasis may ability of small hairs, called cilia, to clear progress. Further symptoms may include increasing mucus and bacteria from the airways. shortness of breath, worsening quality of life and Recurrent lung infections can occur and even . cause bronchiectasis. Alpha-1 antitrypsin deficiency. Alpha-1 How is Bronchiectasis Diagnosed? antitrypsin is a protein that moderates The evaluation for bronchiectasis often includes: inflammation that occurs during infection. A complete medical history and physical People who are deficient in alpha1 examination by a provider. antitrypsin or who have an abnormal A chest CT scan (a specialized X-ray protein may be more likely to have which produces detailed slice-like pictures) recurrent lung infections that cause of the lungs. bronchiectasis. tests, called pulmonary function tests. These determine the presence and Obstruction of the Airways severity of abnormal airflow out of the Obstructed airways trap mucus and infections behind lungs. the obstruction which can damage the airways and Specific screening or diagnostic tests for cause bronchiectasis. some of the possible underlying diseases Obstruction of the airways can be caused that may cause bronchiectasis, based on from a growth or tumor.

the history and physical examination. Spiriva (tiotropium)

How is Bronchiectasis Managed? Inhaled Steroids – Inhaled steroids reduce and Bronchiectasis management is long-term and is prevent swelling inside the airways. Common inhaled directed at: steroids include: Improving the clearance of sputum, also Flovent® () called bronchopulmonary hygiene Pulmicort® () Treatment of infections QVAR® (becolmethasone) Treatment of associated conditions (such Asmanex () as GERD and sinusitis) Azmacort® () Improving muscle strength and endurance Aerobid® () through pulmonary rehabilitation Identifying the need for surgical resection Inhaled Steroid and Long-Acting of affected segments or lobes of the lung Combinations Common combinations of inhaled steroid and long- Your health care provider will evaluate your history acting bronchodilator include: and recommend the best management plan for you. Advair® (Flovent® and Serevent®) Symbicort® (Pulmicort® and Foradil®) Bronchopulmonary Hygiene Therapy Improved clearance of mucus is the cornerstone of Inhaled hypertonic may be used to loosen the management of bronchiectasis and includes airway mucus for easier airway clearance. several components. They include: Inhaled (bronchodilator and/or Airway Clearance Measures inhaled steroid, saline) Airway clearance measures are treatments designed Airway clearance measures (oscillating to clear trapped mucus from the airways. positive expiratory pressure device, high- Oscillating positive frequency chest wall oscillation vest. expiratory pressure devices (OPEPD): These include Your health care provider may recommend one or devices such as the more of them depending on your individual needs. Aerobika®, Acapella or the Flutter Valve that help Inhaled Medication clear mucus from your lungs. These are Inhaled - An inhaled small devices you inhale and/or exhale bronchodilator medication opens the airways by into. relaxing the smooth muscles around the airways. This type of medication is available in a number of inhaled forms. Commonly used inhaled short-acting High-frequency chest wall oscillation vests: bronchodilators include: These include The Vest and the ProAir®, Proventil® HFA, Ventolin® HFA SmartVest® and are inflatable vests that (albuterol) you put on. The vest shakes your chest to Xopenex® (levalbuterol) help dislodge the mucus from the airway walls. Sometimes the Aerobika® or

Acapella is used after the inflatable vest. Inhaled long-acting bronchodilators may also be Once the mucus is dislodged, the device used. They include: can help clear the mucus. Serevent® () and clapping use gravity Foradil® ()

to promote drainage of mucus from the lungs. Pulmonary Rehabilitation may improve your overall health. A well-rounded rehabilitation program Each technique can be prescribed by your health includes education, exercise and eating well and can care provider. Correct technique using these devices help you stay healthy and feel good. is very important. Make sure a health care provider, often a , experienced in the use Resective is occasionally indicated – of the device shows you how to use it. It is also usually only if bronchiectasis is very localized in the important to have your technique checked lung and medical treatment and other therapies are periodically to make sure you continue to use it not effective. correctly to obtain the most benefit. What about a Healthy Lifestyle? Treating Infections A healthy lifestyle is important for everyone. Here are are used to treat bacteria and other some tips to consider: infectious organisms causing infection in the lungs to Exercise regularly as directed improve respiratory symptoms and prevent further by your health care provider. damage to the airways. For example, treating This helps you breathe easier pseudomonas auruginosa may entail 2-3 weeks of by improving your muscle intravenous antibiotics when symptoms are severe. strength and tone and helps Sometimes inhaled antibiotics are given to prevent improve clearing the mucus exacerbations of pseudomonas. Treating from the airways. mycobacteria may require multiple antibiotics taken Eat a well-balanced diet and drink plenty for a year of longer. Rotating or chronic antibiotics to prevent infections are not encouraged because this of fluids. promotes the development of drug-resistant Give up and avoid exposure to organisms. passive smoke. Ask your health care provider for techniques to help you give up Treating Associated Conditions smoking. Treating any identified specific causes, including Get a flu shot every year in the fall. Get a those listed under “Causes of Bronchiectasis” is pneumococcal vaccine every 5 or 6 years important. Examples include: as recommended by your health care Treating chronic infections with non- provider. tuberculous mycobacteria Treating antibody deficiency with immune Living with Bronchiectasis globulin if appropriate Living with bronchiectasis is a unique and Treating swallowing disorders and GERD special challenge that you and your family that cause chronic pulmonary aspiration. must deal with on a daily basis. But the The MEDFacts, Gastroesophageal Reflux more you know about bronchiectasis, the Disease, discusses this topic in more better suited you are in managing the detail various aspects of your disease. As you Prompt treatment or removal of any take control, your quality of life will foreign object, growth or tumor causing improve. obstruction of the airways Be sure to talk with your health care Treating chronic lung disease provider if you have questions or concerns Treating chronic sinusitis. The MEDFacts, about your plan. Write down any questions Sinusitis, discusses this topic in more you have and ask your health care detail provider at your next appointment.

Note: This information is provided to you as an educational service of (1.800.222.5864). It is not meant to be a substitute for consulting with your own doctor. ©Copyright 1987, Revised 1993, 2001, 2005, 2006, 2009, 2011, 2014. NATIONAL JEWISH HEALTH. PTE.029

Notes

The Mount Sinai − National Jewish Health Respiratory Institute was formed by the Icahn School of Medicine at Mount Sinai, a top ranked academic medical center in New York City, and National Jewish Health, the nation’s leading respiratory hospital, based in Denver, Colorado. Combining the strengths of both organizations into an integrated Respiratory Institute brings together leading expertise in diagnosing and treating all forms of respiratory illness and lung disease, including , chronic obstructive pulmonary disease (COPD), interstitial lung disease (ILD) and bronchiectasis. The Respiratory Institute is based in New York City on the campus of Mount Sinai.