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Learn About Bronchiectasis

Bronchiectasis is when the airway walls, known as bronchi, thicken or enlarge. This is caused by chronic inflammation and/or repeated infections. In fact, bronchiectasis means “dilated airways.” (“Bronchi-” means the airways of the lungs, and “-ectasis” means an organ that is enlarged or dilated.) Bronchiectasis damages your airways, which makes it hard for mucus to leave the body. As a result, mucus builds up in the lung. This attracts bacteria and microbes that lead to infection. Chronic infections lead to chronic inflammation, and more mucus. Chronic inflammation causes additional thickening and widening of the airways.

Over time, it gets harder to breathe. But there’s good news—the process can be slowed down by catching inflammation and infections early.

This guide will introduce you to bronchiectasis – its causes, symptoms and risk factors; how it is diagnosed; how it is treated; and how to manage the condition to minimize its impact.

This patient education guide, infographic, and other collateral pieces are generously supported by Bayer.

Key Facts

• More than 110,000 people in the United States have been diagnosed with bronchiectasis.
• While people of all ages can get it, the risk increases with age. Fewer than 1 in 20,000 people ages 18 to 34 have the disease. But 1 in 350 have it by age 75.
• The costs of treating bronchiectasis are about $630 million per year.
• It occurs more in women than in men.
• Treatment includes:
  • clearing mucus
  • preventing infections
  • reducing inflammation

What Is Bronchiectasis

Bronchiectasis is when the airway walls, known as bronchi, thicken or enlarge. This is caused by chronic inflammation and/or repeated infections.

In a normal lung, there are little hair-like structures, known as cilia, on the airways. Cilia sweep mucus and particles out of the lungs. But in people with bronchiectasis, cilia are destroyed. Mucus and particles can’t be cleared from the lungs.

As a result, mucus builds up in the lung. This attracts bacteria and microbes that lead to infection. Chronic infections lead to chronic inflammation, and more mucus. Chronic inflammation causes additional thickening and widening of the airways. Over time, it gets harder to breathe.

The Chronic Cycle of Bronchiectasis

1. Mucus builds up. It attracts bacteria and microbes.
2. Bacteria multiply. This causes additional lung infections and inflammation.
3. New lung infections and inflammation do additional damage to the airways.
4. Over time, enlarged and widened airways make it harder and harder to breathe.
5. There are repeated infections. The airways can’t clear mucus out of the lungs.

How Bronchiectasis Affects Your Body

Bronchiectasis is a long-term condition that can be treated but never cured. Lung function gradually declines over years. People with bronchiectasis have good and bad periods. During bad times, they have flare-ups, known as exacerbations. Flare-ups can last days or weeks. They vary in severity. The sooner people go to the doctor for treatment, the less damage to the lungs from the infection. Along with medications, other measures can help prevent declining lung function.

Bronchiectasis Symptoms, Causes, and Risk Factors

What Are the Signs and Symptoms of Bronchiectasis?

The most common symptoms are:

• coughing up a lot of mucus
• shortness of breath that’s worse during flare-ups
• feeling run-down or tired, especially during flare-ups
• fevers and/or chills, usually during flare-ups
• wheezing or a whistling sound while you breathe
• coughing up blood or mucus mixed with blood, a condition called hemoptysis
• chest pain from increased effort to breathe
• thickening of skin under nails, known as clubbing

Causes of Bronchiectasis

There are two kinds of bronchiectasis: cystic fibrosis (CF)-bronchiectasis and non-CF bronchiectasis. Between one-third and one-half of bronchiectasis cases in the United States are associated with CF.

Bronchiectasis is often a part of diseases that affect the whole body. Non-CF bronchiectasis can develop from:

• Low levels of infection-fighting proteins in the blood. This is known as “humoral immunodeficiency.”
• Recurring or chronic infections, such as tuberculosis or nontuberculous mycobacteria (NTM).
• Inflammatory bowel disease, including Crohn’s disease and ulcerative colitis
• Rheumatologic diseases, including rheumatoid arthritis and Sjögren’s disease
• Alpha₁-antitrypsin deficiency. This is the genetic cause of COPD in some people.
• Chronic obstructive pulmonary disease or COPD
• Asthma
• Immune system weaknesses, such as HIV or uncontrolled diabetes
• An allergic lung inflammation, allergic bronchopulmonary aspergillosis, that causes airway swelling
• Recurring or chronic pulmonary aspiration. This is when a person inhales food, liquids, saliva, or stomach acids into the lungs.
• Damage to the hair-like structures lining the airway, making them not work properly. This is known as primary ciliary dyskinesia.
• Something people are born with (or congenital).

What Are the Risk Factors?

The most common risk factors for bronchiectasis are:

• Being diagnosed with cystic fibrosis (CF)
• Having a chronic inflammatory lung disease
• Chronic or severe lung infections (such as tuberculosis)
• Aspirations that chronically damage the lungs

 

Diagnosing and Treating Bronchiectasis

How Is Bronchiectasis Diagnosed?

Health-care providers may use various tests to diagnose bronchiectasis and find the cause. These tests include:

• Blood tests. They check for:
  • conditions associated with bronchiectasis
  • an infection
  • low levels of infection-fighting blood cells
• Chest CT (or CAT) scan and/or radiograph. A CT scan can show the extent and location of lung damage. It can also show abnormal thickness and irregular airways.
• Mucus (sputum) culture. This checks for growth of bacteria or other microbes.
• Lung function tests. These tests measure your breathing for:
  • how much air you take in
  • how much air you expel
  • how well your lungs get oxygen to your blood
• Bronchoscopy. A flexible, narrow tube (known as a bronchoscope) is inserted into the airways. In more severe or resistant cases, it helps find blockages and sources of infection and inflammation.

Treating Bronchiectasis

Common Medications

The three goals of bronchiectasis treatment are:

• Fight infections
• Thin mucus (making it easier to clear from the lungs)
• Open the airways (using bronchodilators)

Doctors have many options for treating bronchiectasis. Your doctor will select the most appropriate one, or a combination, based on your condition and health status. Generally, medications fall into three categories:

• Antibiotics
• Macrolides
• Mucolytics

Antibiotics

Antibiotics are typically the first line of attack against infections. Based on the severity of your condition, your doctor will give you antibiotics that you either take by mouth or receive through your veins (intravenously). Some antibiotics can be inhaled using a hand-held device called a nebulizer. It converts the medicine into a mist.

Macrolides

Macrolides are a type of antibiotic that not only kill certain types of bacteria but also reduce inflammation in the airways. Azithromycin and clarithromycin are examples of macrolides. They are sometimes used over several months. This may be beneficial for some people but also may have serious side effects, including:

• diarrhea
• nausea
• hearing loss
• dizziness
• development of resistant bacteria in the lungs

Mucus-Thinning Medication

Mucus-thinning medications, known as mucolytics, help people with bronchiectasis get mucus out of their lungs. A nebulizer turns the medicine into a mist. The mist is inhaled deep into the lungs.

Medicine given through a nebulizer helps to dissolve mucus in the airways. The mucus then can be coughed up more easily. In cases of bronchiectasis caused by CF, another inhaled mucolytic may be prescribed.

Nebulizers 

Nebulizers turn medicines into a mist that you can breathe into your lungs.
There are three basic parts:

1. the nebulizer cup that holds the medicine;

2. a machine that converts the medicine into a mist; and

3. a mask or mouthpiece for breathing in the mist.

Nebulizers are small and quiet,  and it usually takes from 8 to 10 minutes to receive a full dose of medicine.

Airway Clearance Devices

Some patients use devices to clear out mucus. Some of these devices have the patient exhale into a hand-held device, for example. This causes air to enter the airway, helping to break up mucus. Other devices are wearable, like a vest. They shake the chest to help loosen mucus. Talk to your doctor about whether any of the following devices are right for you.

• Acapella®
• AerobiKa®
• Flutter®

• Oscillating Positive Expiratory Pressure (PEP): Patients blow into a portable hand-held PEP device. It contains a small ball that moves up and down. This creates vibrations in the lungs. The vibrations help release mucus from airway walls. That makes it easier to cough out of the lungs. Several examples of PEP devices are:
• Intrapulmonary Percussive Ventilation (IPV): This device is a pressurized aerosol machine that turns medications into a mist. The mist is inhaled as the machine oscillates. This vibrates the chest and loosens mucus.
• Postural Drainage: Gravity helps drain mucus from the lungs. Patients lie in a position with the head and chest facing down. This can be effective along with percussion and/or vibration.

Oxygen Therapy

Some people with bronchiectasis may need supplemental oxygen. This can:

• Make your breathing easier
• Enhance your quality of life
• Help you live longer

Oxygen therapy also:

• Improves sleep, mood, and mental alertness
• Better allows the body to conduct everyday functions
• Prevents heart failure

There are three types of oxygen:

• Compressed gas oxygen in steel or aluminum containers of different sizes for home use and traveling
• Liquid oxygen that changes into a breathable gas.
• Oxygen concentrators, electrical devices that remove nitrogen from the air.

If your health-care provider prescribes oxygen, be sure to ask about:

• The oxygen flow rate or setting, so you receive the right amount
• When you should wear it—during activity, while sleeping, or all the time
• Which type best suits your lifestyle

Chest Physical Therapy

Chest physical therapy, also known as chest physiotherapy, stands for a variety of physical techniques for loosening mucus from the lungs so that it can be expelled. The typical techniques used for bronchiectasis include:

• Chest percussion:

  This is controlled tapping or clapping on the body. Clapping is most common. A percussive vest is a convenient and portable option. It automatically tightens then loosens around the chest.

• Controlled coughing:

  This is often used with chest percussion. This kind of coughing provides a way to loosen mucus to be pushed out of the lungs.

Surgery

Bronchoscopy: Sometimes people get a blockage in the airway. Left unchecked, it could lead to severe lung damage. To remove the blockage, you may need a bronchoscopy. A bronchoscope, a long thin tube with a light and camera at the end, is inserted either through the mouth or nose. It examines and removes whatever is in the airway.

Lung Surgery: This is a last resort. In cases where lung damage and/or serious symptoms don’t respond to other treatments, surgery may involve:

• Removal of part or all of a single lung. This is an option when only one lung is severely damaged, or only one lung has a severe infection that does not improve with antibiotics.
• Embolzation. This stops bleeding when a lot of blood is being coughed up.
• Lung transplantation.

 

Living Well with Bronchiectasis

Bronchiectasis is chronic. It usually develops slowly over time. It can never be cured. However, it is possible to slow the progress and preserve lung function. Effective management is possible when self-care is part of your daily routine, such as:

• Taking oral and inhaled medications
• Clearing mucus
• Staying well-nourished and hydrated

What To Expect

Bronchiectasis is a long-term condition. Its symptoms need to be managed every day. This includes:

• Taking medicine to keep airways open and reduce mucus
• Taking antibiotics for 2 to 3 weeks during flare-ups
• Getting lung function tests, chest radiographs, or CT (CAT) scans
• Providing mucus samples to check for infection
• Occasional hospital stays for IV antibiotics, in severe cases
• Possibly bronchoscopy, performed under sedation

Exercise and Nutrition

One of the best ways to manage bronchiectasis is by strengthening your overall health—through both exercise and nutrition. Your doctor may suggest an exercise program that can be done at home 2 to 3 times per week.

Nutrition is also important. It is recommended that people with bronchiectasis meet with a nutritionist. A nutritionist can help you improve your diet and maintain a healthy weight.

Hydration

Drinking lots of fluids, especially water, helps keep mucus thinner and easier to clear from the body. It is highly recommended for people with bronchiectasis.

Immunizations

Typically, the seasonal influenza vaccine and pneumococcal vaccine are recommended for patients with bronchiectasis.

Pulmonary Rehabilitation

Your doctor may order a program of exercise, education, and support. It can improve your exercise capacity and quality of life. It also may reduce flare-ups.

Finding Support

There are several online support groups. They can be found through a simple Internet search. The American Lung Association’s Living with Lung Disease Support Community connects patients and caregivers with others facing this disease.

You can call the Lung Association’s Lung Helpline at 1-800-LUNGUSA. A trained respiratory professional can answer your questions and connect you with support.

If you have cystic fibrosis, visit the website for the Cystic Fibrosis Foundation.

Questions to Ask Your Doctor About Bronchiectasis

Patients with bronchiectasis should get regular checkups. Making notes before your visits, as well as taking a family member or friend, can help.

Questions include:

• Why did I get this disease?
• Is it contagious?
• Is my type of bronchiectasis genetic?
• How often should I provide a mucus sample?
• What are the signs and symptoms of a flare-up?
• What should I do when I have a flare-up?
• What can I do to prevent a flare-up?
• What vaccinations should I have to prevent lung infections?
• What if prescribed medications don’t help me, even though I take them as directed?
• Should I take an over-the-counter cough and cold product?
• What can help with the emotional toll of having lung disease?
• What can I do to improve my lung condition?
• What type of exercise might help?
• What changes should I make to my diet?
• Are there support groups?

Authors

Doreen Addrizzo-Harris, MD, FCCP
NYU School of Medicine

Christopher Barrios, MD
Wake Forest Baptist Medical Center

Sheetal Gandotra, MD
Wake Forest Baptist Medical Center

Alex Gifford, MD, FCCP
Geisel School of Medicine at Dartmouth

Malvika Sagar, MBBS
Baylor Scott & White Health

Gilbert Seda, Jr., MD, PhD, FCCP
Naval Medical Center

 

Date Last Reviewed

January 2018