Bronchiectasis

Bronchiectasis is a long-term condition in which the airways in your lungs become permanently widened and scarred, usually after infection or inflammation has damaged their walls. Damaged airways cannot clear mucus properly, so it collects, bacteria settle in it, and chest infections keep coming back. Day to day it often means a daily cough that brings up phlegm, tiredness that is hard to explain to other people, and several courses of antibiotics a year.

What bronchiectasis is, and what it is not

Bronchiectasis is often mistaken for COPD or asthma, and people are sometimes treated for those for years first. The difference is structural. In COPD the airways are narrowed and the small air sacs are damaged, usually after long-term exposure such as smoking. In bronchiectasis the airways themselves have been stretched wide and scarred, which is why mucus pools and infections return. Some people live with more than one of these conditions at once, and that is part of why a CT scan is used when repeated infections do not fit a straightforward picture.

It is also not a single disease with a single cause. Bronchiectasis is the end result of many different processes, including past severe infections, inherited conditions, immune deficiencies and autoimmune disease. Finding the underlying cause is not an academic exercise: Canadian guidance in CMAJ notes that identifying it changes management in as many as 37 percent of adults.

Signs and symptoms

• A daily cough that brings up phlegm, often large amounts
• Phlegm that is thick and may be yellow, green or brown
• Repeated chest infections that need antibiotics several times a year
• Breathlessness and wheezing
• Feeling very tired much of the time
• Chest pain or discomfort
• Coughing up blood, or blood-streaked phlegm, in some people

Symptoms differ a great deal between people and often run in a cycle: a stable stretch, then a flare-up with more phlegm and less energy, then a slow recovery.

What raises the risk

Some things cannot be changed:

• A previous severe chest infection such as pneumonia, whooping cough or tuberculosis
• Inherited conditions including cystic fibrosis and primary ciliary dyskinesia
• Immune system deficiencies, including low antibody levels
• Autoimmune and inflammatory conditions such as rheumatoid arthritis and inflammatory bowel disease
• Allergic bronchopulmonary aspergillosis, an allergic reaction to a common mould

Others can often be worked on, usually with support from a health care team:

• Smoking, and exposure to second-hand smoke, dusts and fumes, which add to airway irritation
• Untreated acid reflux and repeated aspiration of stomach contents into the lungs

How bronchiectasis is diagnosed in Canada

Bronchiectasis is confirmed with a high-resolution CT scan of the chest, which shows airways that are wider than the blood vessel beside them and airway walls that are thickened. Canadian guidance in CMAJ notes the scan is ideally done when you are clinically stable rather than in the middle of a flare-up. Once bronchiectasis is confirmed, further tests look for a cause and guide care: spirometry and other lung function tests, sputum cultures for bacteria and for nontuberculous mycobacteria, blood tests of immune function, and sweat chloride or genetic testing for cystic fibrosis where that is relevant.

Treatment and day-to-day management

Clinical research for Bronchiectasis is enrolling. See the current studies ↓

Care for bronchiectasis is built around clearing mucus, treating and spacing out infections, and dealing with whatever is driving the damage. Depending on your scan, your sputum results and how often you have flare-ups, a health care team may discuss:

• Airway clearance techniques taught by physiotherapists and respiratory therapists, sometimes with devices or nebulised saline
• Antibiotics given by mouth, by vein or by inhalation, used for flare-ups and, in some people, on a long-term basis
• Investigation and treatment of the underlying cause, such as immune deficiency, allergic bronchopulmonary aspergillosis or nontuberculous mycobacterial infection
• Pulmonary rehabilitation and supervised exercise programmes
• Care through specialist respirology clinics, including the 12 Canadian sites taking part in the national bronchiectasis and NTM registry

Because the causes differ so much, two people with bronchiectasis can end up with quite different plans. These are decisions for you and your own clinician, and this page is not a recommendation about any of them.

When to talk to a doctor

• Call 911 if you cough up a large amount of blood, have severe difficulty breathing or chest pain, or become confused or drowsy.
• Seek medical care within a day if your phlegm changes colour, thickness or volume, if you develop a fever, or if your breathlessness or tiredness worsens noticeably, since these can signal a flare-up.
• Contact your respirology team or family doctor if you are needing antibiotics more often than usual, losing weight without trying, or your usual airway clearance routine has stopped working.

Why clinical research matters for bronchiectasis

There is no approved medicine in Canada specifically for bronchiectasis, so care is largely borrowed from cystic fibrosis and COPD practice. Clinicians cannot yet predict who will deteriorate, or which people gain from long-term antibiotics. Canada also lacks basic figures on how many people are affected, which is why the Canadian Bronchiectasis and NTM Registry was set up and linked with the European EMBARC and United States registries. Studies now underway in Canada and internationally include neutrophil serine protease inhibitors such as DPP-1 inhibitors, inhaled antibiotic formulations, new regimens for nontuberculous mycobacterial lung disease, and airway clearance and rehabilitation strategies.

Registries and trials are different things, and joining one does not commit you to the other. Both are voluntary, both begin with informed consent, and you can withdraw at any time without affecting the care you get from your own team. No one can say in advance whether a study will help you personally.

Learn more from Canadian sources

• Canadian Bronchiectasis and NTM Registry (BXConnect)
• Canadian Lung Association: Lung Health Information Line
• CMAJ: diagnosis and management of bronchiectasis
• Canadian Institute for Health Information: hospital stays in Canada