Non-Cystic Fibrosis Bronchiectasis
Air moves through your lungs through your bronchi (airways). When you have bronchiectasis, these airways get thick and damaged. When your bronchi are damaged, mucus builds up, and your airways become an environment for germs. This further damages the lung.
Bronchiectasis is a chronic condition, meaning it’s persistent and long-lasting. Because it’s chronic, bronchiectasis needs to be monitored and managed. If it’s not, it can significantly affect your quality of life.
We don’t always know the exact cause of bronchiectasis. It is sometimes a result of another condition. For example, an airway blockage, like a growth or a noncancerous tumor, can lead to bronchiectasis.
What is Non-Cystic Fibrosis Bronchiectasis?
Non-cystic fibrosis bronchiectasis (NCFB) is a type of bronchiectasis that isn’t caused by cystic fibrosis. With irreversibly and abnormally dilated (narrow) airways, NCFB can cause someone to have a persistent cough, excessive mucus production and recurring pulmonary (lung) infections. NCFB often occurs with other respiratory conditions, such as chronic obstructive pulmonary disease (COPD)
Referrals to the Baystate Health Bronchiectasis and Nontuberculous Mycobacteria Clinic
Treating and managing NCFB requires input from experts in areas including pulmonology, infectious disease and physical therapy. Baystate Health’s Bronchiectasis and Nontuberculous Mycobacteria Clinic can help you determine the severity of your condition and develop the right treatment plan.
To be seen at our clinic, you’ll need a referral from your doctor. You can start the process by completing the referral form below..
Learn More About Non-Cystic Fibrosis Bronchiectasis
Bronchiectasis is most often linked to cystic fibrosis, but there are other conditions that can bring it on. These fall into the non-cystic fibrosis (NCFB) category, and can be caused by:
- Autoimmune diseases
- Allergic bronchopulmonary aspergillosis, an allergic or hypersensitive response to a fungus
- Immunodeficiency disorders, including HIV or diabetes
- Chronic pulmonary aspiration, where problems with swallowing brings food or fluids into the lungs and leads to inflamed airways
- COPD and alpha-1 antitrypsin deficiency (which causes COPD in some people)
- Diseases that affect the small, hair-like structures that clear mucus and line your airways (cilia)
- Inflammatory bowel disease, including Crohn’s and ulcerative colitis
It can also be caused by recurring infections that cause damage to airway walls, like:
- Pneumonia
- Pertussis (Whooping cough)
- Tuberculosis
- Fungal infections
- Women are at higher risk for bronchiectasis, although men also get it
- Another health condition or recurring infection
- An airway blockage, like a growth, noncancerous tumor or the result of inhaling something
Symptoms of bronchiectasis can take months or years to develop. And once they start, they gradually get worse.
The two primary symptoms are a daily cough and daily production of mucus. But other symptoms can include:
- Chest pain
- Coughing up blood or mucus mixed with blood (hemoptysis)
- Fatigue, feeling run-down or tired
- Fevers and/or chills
- Shortness of breath
- Thickening of the skin under fingernails and toenails (clubbing)
- Wheezing or whistling sound while you breathe
Early diagnosis is important to prevent further lung damage, but bronchiectasis is under-diagnosed. People are often incorrectly diagnosed with other lung diseases.
To diagnose NCFB, your doctor will start with a detailed family history and blood tests. They might also recommend tests like:
- Chest X-ray or CT scan, which is the most common way to diagnose bronchiectasis
- Lung function test to see how well your lungs are working
- Sputum (mucus) culture to see if you have bacteria in your lungs
- Sweat test, to help determine if you have cystic fibrosis
Treating Bronchiectasis
The goals of bronchiectasis treatment include:
- Managing underlying conditions
- Preventing lung infections
- Removing excess mucus
- Keep flare-ups from happening
Treatment usually includes a combination of medication, hydration and chest physical therapy. If your blood oxygen levels are low, your doctor might recommend oxygen therapy. Surgery may also be an option if your bronchiectasis is isolated in a section of your lung or if you have excessive bleeding.
Managing Bronchiectasis
Because it’s chronic, bronchiectasis doesn’t go away. You’ll need to follow the plan you and your doctor create, which could include making some lifestyle changes, like:
- Quitting smoking and avoiding secondhand smoke
- Eating a healthy diet low in sodium, added sugars, saturated fats and refined grains
- Staying hydrated, as drinking plenty of water can help prevent mucus build-up
- Taking prescribed oral and inhaled medications
- Performing daily mucus clearance techniques
- Getting all recommended vaccinations
- Taking prescribed antibiotics during flare-ups
Even with all these changes, you might have flare-ups. This can happen because of a new respiratory infection or an overgrowth of bacteria.
Symptoms of a Flare-Up
Symptoms of a flare-up can include:
- Increased mucus that’s bloody or a different color
- Fever
- Fatigue
- Weight loss
- Worsening shortness of breath
If you experience any of these, contact your provider who will determine if you need to adjust your treatment.
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