Bronchiectasis

Introduction

Bronchiectasis is a condition that affects the lungs. It causes a persistent cough and an excess amount of sputum (phlegm).

The airways

Air passes in and out of the lungs through a series of branching airways called bronchial tubes. Usually, the bronchial tubes narrow smoothly towards the edges of each lung. However, in bronchiectasis, the airways of the lungs are irreversibly damaged and, instead of narrowing, they are abnormally widened.

The lung’s airways are covered in mucus and tiny, hair-like structures called cilia. The mucus acts as a protective ‘blanket’ against any unwanted particles that try to enter the lungs. The cilia waft the particles and any excess mucus up and out of the lungs. This is usually a tiny amount that is swallowed without noticing but, if there is a lot of mucus, it can cause coughing.

How bronchiectasis occurs

In bronchiectasis, the cilia that line the bronchial tubes are destroyed, causing a build-up of dust, mucus, and bacteria. Excessive amounts of mucus form on the back of the throat producing sputum. The sputum can be very thick, sticky, and difficult to dislodge through coughing.

The build up of sputum in the airways attracts bacteria that can live in the sputum and infect the lungs. Infections cause further irreversible damage to the bronchial tubes, and the increased damage makes another infection more likely, leading to a cycle of deterioration.

The bronchial tubes may be damaged as a result of a childhood illness, such as pneumonia, or whooping cough, or it may have no known cause.

Outlook

In the past, bronchiectasis was a condition that almost always started during childhood. However, with improved hygiene, childhood immunisation, and more frequent use of antibiotics, nowadays the condition does not often develop until middle age or later.

There is currently no cure for bronchiectasis, and the damage to the airways is usually permanent. However, early detection and treatment can help prevent the damage to the lungs from getting worse. Medication is effective at treating the recurrent infections, and there are a number of exercises that can help relieve the symptoms.

Lungs

Lungs are a pair of organs in the chest that control breathing. They remove carbon dioxide from the blood and replace it with oxygen.

Bacteria


Bacteria are tiny, single-celled organisms that live in the body. Some can cause illness and disease and some others are good for you.
Last updated: 04 October 2011

Symptoms of bronchiectasis

The main symptoms of bronchiectasis are:

  • a persistent and unpleasant cough,
  • producing a lot of green-yellow coloured phlegm (sputum), which can be thick and very sticky,
  • recurring chest infections (see below),
  • laboured, or difficulty, breathing (dyspnoea), with breathlessness and wheezing being common,
  • feeling tired and unwell,
  • loss of appetite which could result in weight loss,
  • a chronic infection of the nasal sinuses (the air-filled cavities inside your cheekbones) that lasts for weeks, or months, and
  • a constant runny nose (catarrh).

You may also have bad breath because your phlegm can smell unpleasant. Sometimes, the phlegm can be bloodstained due to tiny blood vessels breaking in your bronchial tubes.

Less common symptoms of bronchiectasis include:

  • coughing up a lot of blood, and
  • chest pain and joint pain.

Cycle of infections

Recurring chest infections are a common symptom of bronchiectasis. This is because your cilia (the tiny, hair-like structures that line your airways) cannot waft the mucus out of your lungs. As a result, there is a build up of mucus and phlegm in your airways that becomes infected with bacteria. The bronchial tubes become inflamed, leading to further damage, increasing the likelihood of another infection developing. 

If you have bronchiectasis, you are likely to experience the above symptoms in patterns of varying severity. For example, you may have a few weeks of only mild coughing, or breathlessness, followed by a couple of weeks of more intense and severe symptoms.

This is because the damage that is already done to your lungs causes the above symptoms in a mild and constant form, but the damage also makes you more vulnerable to infections. During an infection, the above symptoms will be worse, and/or more frequent. You may require treatment with antibiotics to limit any further possible damage to your airways.   

Last updated: 04 October 2011

Causes of bronchiectasis

In up to 50% of cases of bronchiectasis, the cause is unknown. However, there are some underlying conditions, or events, that can make the condition more likely to occur. This will normally be something that has caused damage to your lungs.

If your lungs have an abnormal inflammatory response to any damage that is done to them, your bronchial tubes may be irreversibly widened. This widening is what characterises bronchiectasis.

If your bronchial tubes are widened, they are more likely to produce mucus and sputum (phlegm) which cannot be dislodged. This attracts bacteria and starts a cycle of infection and further lung damage.

How the bronchial tubes can be damaged

Possible ways that your bronchial tubes may be damaged include;

  • acid from your stomach being regurgitated (brought up) and inhaled into your bronchial tubes,
  • inhaling poisonous gases,
  • an allergy to fungus spores may inflame your bronchial tubes, leading to permanent damage,
  • a severe respiratory infection - for example, pneumonia, whooping cough, or measles, particularly during childhood, can damage the airways and cause bronchiectasis in early adulthood, and
  • inhaling (breathing in) a foreign body - for example, inhaling a peanut into your lungs.

If you have had a choking incident (where a piece of food falls to back of your throat, causing you to gag and cough), it is possible that the object you were choking on has ended up in your lungs. Afterwards you may notice a new problem with your breathing. If the item is removed quickly, any damage (including bronchiectasis) can usually be avoided.

Alternatively the damage to your bronchial tubes may be caused by an underlying health condition such as:

  • cystic fibrosis,
  • primary ciliary dyskinesia, or
  • an immune system defect.

Cystic fibrosis

Cystic fibrosis is a genetic disorder where internal bodily secretions become thick and sticky, hindering the function of certain organs, such as the lungs and digestive system.

Cystic fibrosis is caused by a faulty gene that controls the movement of salt and water in and out of the body’s cells. When cystic fibrosis occurs, too much salt and not enough water passes into the cells and turns the body's secretions, which usually act as a lubricant, into thick mucus. The mucus in the lungs, that usually forms a protective layer over the airways, clogs them up, making them ineffective and prone to infection.

The infections can damage the airways resulting in bronchiectasis.

Primary ciliary dyskinesia

Primary ciliary dyskinesia is a genetic disorder that causes defects in the cilia (the tiny, hair-like structures that cover the airways in the lungs).  The condition is sometimes referred to as Kartagener’s syndrome.

The cilia are made up of microtubles (tiny arms) that allow them to bend so that they can waft away mucus and unwanted foreign particles from your lungs. If some of the microtubles are missing, shortened, in the wrong order, or have some other kind of defect, they are unable to waft away mucus. The mucus then builds up in the airways.

This attracts bacteria which can lead to infections that can cause lung damage and bronchiectasis.    

Immune system defects

Any problems that affect your immune system will make you more vulnerable to developing infections. For example, if you have an antibody deficiency syndrome, your immune system will be unable to produce enough antibodies to fight off an infection. Antibodies are proteins that are produced by the body to neutralise, or destroy, disease-carrying organisms and toxins.

Most immune system defects are genetic, but some, such as HIV, can be the result of an infection.

Your lungs are more vulnerable to infections than other parts of your body because it is easy for bacteria, or viruses, to enter your bronchial tubes when you breathe in. If you have an immune system defect, it may lead to recurrent lung infections. These could damage your lungs and cause bronchiectasis. 

Infections

The above three conditions can increase the likelihood of causing lung infections, or making them more severe. Infections can, in turn, cause the damage to the lungs that characterises bronchiectasis.

Research has shown that, in people with bronchiectasis, there are three main types of bacteria that cause infections in the lungs. These are:

  • Haemophilus influenzae,
  • Pseudomonas aeruginosa, and
  • Streptococcus pneumoniae.

Among other conditions, the above bacteria can cause pneumococcal infections, influenza, epiglottitis, and meningitis. If these are severe, or if they keep returning, they can result in permanent lung damage, which can cause bronchiectasis.

Last updated: 04 October 2011

Diagnosing bronchiectasis

If your GP suspects that you have bronchiectasis, you may be asked about your history of respiratory (breathing) problems or childhood diseases. This is to find out if there is anything in your past that might have damaged your bronchial tubes (the branching airways that carry air in and out of your lungs).

You will be referred for lung and sputum (phlegm) tests to confirm if you have bronchiectasis and to find out how it has affected your lungs.

HRCT scan

The use of high resolution computed tomography (HRCT) scans to detect bronchiectasis is now widespread, and some doctors regard them as the best diagnosis method. During a HRCT scan, a series of highly sensitive X-rays will be taken of your chest at slightly different angles, and a computer will be used to put all the images together. This produces a very detailed picture of the inside of your body.

From this picture, your GP will be able to detect the severity of the bronchiectasis, the pattern and distribution of it in your lungs, and also any other infections that you may have as a result of the condition.

Bronchiectasis can be identified by looking at your bronchi (the small tubes that carry air in and out of your lungs). Instead of tapering (getting narrower) as they go further into your lungs, the bronchi are dilated (wider).

The width of the bronchi is compared to the width of your pulmonary arteries (the blood vessels that carry blood from your heart to your lungs). This provides a standard for how wide your bronchi should be. If the bronchi are wider than your pulmonary arteries, it is usually confirmation of bronchiectasis.  

Additional tests

There are several other tests that your GP may perform. These are explained below.

  • Sputum test - a sample of sputum is taken from your lungs and tested to confirm the diagnosis. It can also be tested to see which kinds of bacteria might have infected your lungs. This will help your GP to decide on the best course of treatment.
  • Bronchography - a standard chest X-ray that is used to identify any widening of your bronchial tubes and any areas of infection.
  • Pulmonary function test - a peak flow meter (a small, hand-held device that you blow in to) is used to measure how hard and how quickly you can expel air from your lungs. This will give your GP more information about your lung capacity and volume.
  • Bronchoscopy - a flexible tube with a camera at one end is used to look into your lungs. This will normally only be necessary if your GP suspects that you have inhaled (breathed in) a foreign object.

Other tests may be recommended if an underlying cause of your bronchiectasis is suspected. For example, you may have a blood test to help diagnose a genetic disorder.

Last updated: 04 October 2011

Treating bronchiectasis

There is currently no cure for bronchiectasis. Treatment for the condition mainly consists of using medication to break the cycle of infection and worsening lung damage. You will have an individual course of medicine that will be tailored to the characteristics of your bronchiectasis.

Other therapies (see below) are aimed at managing the symptoms of bronchiectasis, particularly the coughing and over-production of sputum (phlegm). However, these should only be tried after discussions with your GP, or healthcare professional.

In addition, you should consider the two points below.

  • Smoking  makes the symptoms of bronchiectasis worse. Therefore, if you smoke, you should stop immediately if you are diagnosed with bronchiectasis. You should also try to avoid second hand smoke as much as possible.
  • You may need to be vaccinated against influenza (flu) and pneumonia. This will depend on your GP’s assessment of how at risk you are from these infections.

Medication

Your GP will prescribe courses of medication to you as and when they are necessary. This will depend on the symptoms that you are experiencing and the frequency and severity of your infections. The most common medicines used are:

  • antibiotics, and
  • corticosteroids.

Antibiotics

Antibiotics are widely used to target the bacteria responsible for the recurrent chest infections which are a predominant symptom of bronchiectasis. By limiting the amount of infections, and lessening their severity when they do occur, further damage to your lungs can be minimised.

Depending on the type of infections that you have, your GP will decide on your individual course of antibiotics. A sputum sample can be taken and tested to find out which bacteria are causing your infections and to identify any patterns of infection.

An antibiotic treatment regime can be created that is specific to your cycle of infections in order to limit their frequency and severity. Your GP should  take sputum samples from you periodically to test for any changes in your infection cycle and the bacteria present. 

Antibiotics can be taken in a number of different ways:

  • orally (as a tablet that is swallowed),
  • intravenously (a liquid injected into a vein),
  • intramuscularly (a liquid injected into a muscle),
  • subcutaneously (a liquid injected under the skin), or
  • inhaled with a nebuliser (a device that converts liquid into mist so that it can be breathed in).

The length of time antibiotics are taken for can also vary. For example, you may be prescribed antibiotics for:

  • a short course of 7-10 days, or 10-14 days - to treat the symptoms of an infection, and reduce the amount of sputum in your airways, or
  • a longer course, possible daily, or for several weeks - to prevent an infection occurring and to maintain low levels of sputum.

If your symptoms do not respond to the antibiotics after two weeks, your GP may change your antibiotics to a different type. The type of antibiotics you take should also be changed regularly to prevent the bacteria developing a resistance to them.

Antibiotic resistance

Bacteria can adapt and ?nd ways to survive the effects of an antibiotic. They become ‘antibiotic resistant’ so that the antibiotic no longer works. The more often an antibiotic is used, the more likely bacteria will become resistant to it. For this reason, GPs will only prescribe you antibiotics when they believe it is necessary, and may vary your prescriptions with slightly different antibiotics.

GPs follow guidance from the National Institute of Clinical Excellence (NICE) about when and how often to prescribe antibiotics. If your GP believes that antibiotics will make little difference to your symptoms, they may not be prescribed at all. If your symptoms do not improve, or they get worse, your GP may prescribe antibiotics. If there is a possibility of further complications with your infection, antibiotics will usually be prescribed immediately.

Your GP will explain their decision to you and you should discuss any concerns that you have.

Corticosteroids

Corticosteroids may be prescribed by your GP as they act directly on your immune system, helping to calm the swelling and inflammation of your airways. They are sometimes prescribed as tablets that are swallowed, or as an inhaler which you breathe in (your GP will show you how to use this). Corticosteroids are routinely prescribed for other conditions, such as sinusitis and chronic asthma, but their use for bronchiectasis still needs more research.

Some studies have shown positive results for the use of inhaled corticosteroids in reducing the amount of daily sputum produced, easing coughing and improving lung function. However, one trial of people with chronic obstructive pulmonary disease (COPD) found that the incidence of pneumonia increased with the use of inhaled corticosteroids. Your GP will advise you about the possible benefits of corticosteroids and whether they will be suitable for you.

Other therapies

Depending on the severity of your symptoms and what you find most helpful, your GP, or healthcare professional, could advise you about some of the following. However, these will not be suitable for everyone and should only be used with medical advice.

Chest physiotherapy

If you are producing a lot of sputum, and antibiotics are not helping to reduce it, you may benefit from chest physiotherapy. The aim is to help clear the sputum from your lungs as soon as it forms, so that there is less available for the bacteria to infect. Your GP may refer you to a physiotherapist who can advise you about the best methods.

Physiotherapy involves exercising your arms and legs to help strengthen your chest muscles to improve your breathing. A ‘head down’ position is often adopted with someone clapping on your chest and back to clear your lungs of mucus. This is usually done once, or twice, a day. This method requires a lot of time and effort but, if it is done regularly, it can be very effective. You should drink plenty of fluids during the exercises because this makes your sputum less sticky and easier to clear.

Daily exercise

Gentle, daily exercise - for example, swimming or walking - can help to clear your lungs of mucus. The movement helps the mucus work its way up from your lungs, making it easier for you to breathe. If you find the positions recommended in physiotherapy hard to maintain, gentle exercise may be a suitable alternative for you.

Make sure that you stay hydrated during any exercise by drinking plenty of water and do not push yourself too hard.

Cycle breathing

Cycle breathing is a technique that is sometimes used alongside physiotherapy. The pattern of breathing that is used is likely to be specific to you but, could, for example, involve taking four deep, gentle breaths, a pause to relax, and then two deep breaths where you force the air out as you exhale. This is then followed by a pause during which you may cough to expel the loosened mucus or sputum.

Some people find this technique uncomfortable but, for others, it can be useful several times a day to help clear the airways.

Postural drainage

Postural drainage uses vibrations to loosen the build up of mucus, making it easier to clear your airways by coughing it up. This is sometimes done using an inflatable vest that has air pulsing through it to apply pressure to your chest. This pressure loosens the mucus in your airways and encourages it to move up and out of your lungs. Other types of mechanical devices are also sometimes used.

Postural drainage can be time consuming as it may need to be done twice a day. It should not be attempted without proper guidance from a healthcare professional.

Salt solutions

Salt solutions - known as hypertonic saline - can be used to reduce the amount of mucus in your lungs, or to make the mucus less sticky. The solution is inhaled using a nebuliser (a device that converts liquid into mist so that it can be breathed in). More research is still needed but initial tests suggest salt solutions can help improve lung function.

Surgery

Sometimes, it may be possible to remove the part of your lung that is affected by bronchiectasis. Provided the condition is not present elsewhere in your lungs, surgery can be a highly effective treatment. However, unfortunately, surgery is rarely an option because bronchiectasis tends to be present in both lungs and can be extensively spread around your airways.

In very severe cases, a lung transplant might be considered, but this carries its own risks. For example, it is possible that the new lung could fail, or that the donor organ could be rejected. You will also need to take immunosuppressant medicines (medicines that lower your immunity) for the rest of your life in order to lower the risk of the donor organ being rejected. These will leave you more vulnerable to infections.

Last updated: 04 October 2011