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Bronchiectasis

Introduction

Bronchiectasis is a long-term lung condition where the airways of the lungs become abnormally widened, leading to a build-up of excess mucus.

This in turn can make the airways of the lungs more vulnerable to infection.

The most common symptom of bronchiectasis include:

  • a persistent cough that usually brings up sputum (phlegm)
  • breathlessness
  • coughing up or spitting out blood

Symptoms can vary widely. Some people have only a few symptoms that don't appear often, while others have wide-ranging symptoms that occur daily.

Read more about the symptoms of bronchiectasis.

The lungs are full of tiny branching airways, known as the bronchi, whose role is to move oxygen into even smaller sacs called alveoli, which transfer the oxygen into the blood so it can then be transported around the body.

The lining of the bronchi are coated with a sticky fluid called mucus (the same fluid that can come out of your nose when you sneeze), which protects against particles moving down into the lungs and damaging them.

With bronchiectasis, one or more of the bronchi become abnormally widened. This can then trigger a vicious circle of mucus gathering in the bronchi, making them prone to infection and further widening the bronchi, leading to a build-up of more mucus.

During an infection, the symptoms of bronchiectasis will usually get worse.

Causes

Bronchiectasis has a wide range of possible causes. The three most commonly reported causes in England are:

  • The bronchi became damaged due to a previous childhood lung infection.
  • Underlying problems with the immune system (the body’s defence against infection) has led to the bronchi becoming damaged due to infection, or in some cases, they have mistakenly been attacked by the immune system.
  • The patient has allergic bronchopulmonary aspergillosis (ABPA) – an allergy to a certain type of fungi that damages the lungs.

However, in over half of all cases of bronchiectasis no obvious reason can be found to explain why the bronchi became widened.

Read more about the possible causes of bronchiectasis.

Treatment

Treatment for bronchiectasis involves using a number of different methods to help improve symptoms and quality of life, such as:

  • medication to help improve airflow within the lungs
  • physiotherapy and exercise to help clear the lungs of mucus
  • antibiotics, when necessary, to treat infection

If an infection is severe you may be admitted to hospital as a precaution.

Surgery is occasionally used when other treatments fail to improve symptoms, although not everyone is a suitable candidate for surgery.

Read more about the treatment of bronchiectasis.

Complications

Complications of bronchiectasis are rare but when they do occur they can be serious. Probably one of the most serious complications is coughing up large amounts of blood which can be life-threatening and require emergency surgery to treat.  

Read more about the complications of bronchiectasis.

Who is affected?

Bronchiectasis is thought to be relatively rare and can affect anyone at any age. 

One estimate is that around 1 in every 1,000 adults and 1 in every 10,000 children have bronchiectasis in England and Wales.  Around 9,000 people are admitted to hospital each year due to problems arising from bronchiectasis.

Outlook

The outlook can be highly variable and often depends on the underlying cause.

For most people the outlook is good, although living with bronchiectasis can be distressing and frustrating.

In people with very severe symptoms, bronchiectasis can disrupt normal lung function, which can sometimes be fatal.

In 2010, there were just over 1,000 deaths reported in England and Wales where bronchiectasis was thought to be an underlying factor. 

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Symptoms

The most common symptoms of bronchiectasis is a cough, which affects around 9 out of 10 people with bronchiectasis.

In three out of four cases the cough will bring up sputum (phlegm), which can be clear, pale yellow or yellow-greenish in colour.

In the remaining cases the cough may only occasionally bring up sputum, or bring up no sputum at all.

Other symptoms include:

  • shortness of breath
  • coughing up blood or blood-stained sputum
  • chest pain

Infection

If you contract a lung infection then your symptoms will usually get worse. This is known as an infective exacerbation and could mean that:

  • You cough up even more sputum and often the sputum has an unpleasant smell.
  • Your shortness of breath worsens.

You may also experience additional symptoms, such as:

  • feeling very tired
  • coughing up blood – if you had not already done so
  • a sharp chest pain that is made worse when breathing – this is known as pleurisy
  • a general sense of feeling unwell

When to seek medical advice

If you haven't previously been diagnosed with bronchiectasis and you begin to experience a persistent and unexplained cough, visit your GP for advice.

While persistent coughing may not necessarily be the result of bronchiectasis, it will require further investigation.

If you have been previously diagnosed with bronchiectasis and you begin to experience symptoms that suggest you have contracted a lung infection, such as worsening of your present symptoms or coughing up foul-smelling sputum, contact your GP.

This is because it's likely that you will require treatment with antibiotics (although some people with bronchiectasis are given a stock of antibiotics as a precaution in case they suddenly develop a lung infection).

When to seek immediate medical advice

Some people with bronchiectasis develop a severe lung infection that may need to be treated in hospital.

Signs and symptoms of serious lung infection include:

  • Your skin takes on a bluish tinge due to lack of oxygen – this is known as cyanosis.
  • You (or someone in your care) are mentally confused.
  • You have a high temperature of or above 38C (100.4F).
  • You are breathing rapidly, with more than 25 breaths a minute.
  • You have severe chest pains that are making it too painful to cough and to clear any mucus out of your lungs.

If you experience any of the above, phone the healthcare professional in charge of your care for advice immediately. This may be your GP, a doctor who specialises in lung conditions (pulmonologist) or a specialist nurse.

If this isn't possible then phone NHS Direct Wales on 0845 46 47 or your local out-of -hours service.

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Causes

Bronchiectasis is a progressive condition that gets worse over time in a pattern often referred to as a vicious circle.

Your lungs are continually exposed to germs so your body has a number of sophisticated defence mechanism that are designed to keep the lungs sterile (germ free). However, occasionally these defences can be breached.

Your immune system will attempt to stop the spread of any infection by sending infection-fighting white blood cells to the location of infection.

These cells release chemicals that can cause surrounding tissue to become inflamed.

In some cases, inflammation can destroy the elastic-like tissue and muscles that surround the bronchi, leading to the bronchi to becoming wider.

This abnormal bronchi then becomes filled with an excess amount of mucus which can trigger persistent coughing and make the lung more vulnerable to infection. This in turn can result in further inflammation, and further widening of the bronchi, leading to more mucus gathering and so it continues. However, the levels of damage to the lungs can differ widely from case to case.

In around half of all cases of bronchiectasis, no obvious cause can be found to explain what triggered the symptoms. Other triggers are described below.

Childhood infections

Around a third of cases of bronchiectasis currently found in adults are associated with a previous severe childhood lung infection, such as:

As there are now vaccinations available for these types of conditions, the rates of these types of infection-related bronchiectasis are expected to fall in the future.

Immunodeficiency

Around 1 in 12 cases of bronchiectasis are caused because a person has a weakened immune system – the body’s defence against infection – making their lungs more vulnerable to tissue damage. The medical term for having a weakened immune system is immunodeficiency.

Some people are born with an immunodeficiency due to problems with the genes that they inherit from their parents. It is also possible to acquire an immunodeficiency due to an infection such as HIV.

Allergic bronchopulmonary aspergillosis (ABPA)

Around 1 in 14 people develop bronchiectasis as a complication of an allergic condition known as allergic bronchopulmonary aspergillosis (ABPA).

People with ABPA have an allergy to a type of fungi known aspergillus, which is found in a wide range of different environments across the world.

If a person with ABPA breathes in fungal spores it can trigger an allergic reaction and persistent inflammation which in turn can progress to bronchiectasis.  

Aspiration

The medical term for accidentally swallowing the "wrong way" into your lungs rather than down into your stomach is aspiration, which is responsible for around 1 in 25 cases of bronchiectasis.

The lungs are very sensitive to the presence of foreign objects, such as small samples of food or even stomach acids, so this can trigger inflammation leading to bronchiectasis.

Young’s syndrome

Young’s syndrome is a rare disorder that accounts for around 1 in 33 cases of bronchiectasis and only affects males. The condition damages little tiny, hair-like structures that cover the airways in the lung known as cilia. The cilia are designed to protect the airways of the lungs, as well as helping move away any excess mucus.

Damage to the cilia can lead to the symptoms of bronchiectasis.

Young’s syndrome is thought to be caused by childhood exposure to mercury. As the regulations regarding the use of mercury are now much stricter than they were in the past it is expected that Young’s syndrome will become a much less common cause of bronchiectasis. 

Cystic fibrosis

Cystic fibrosis is a relatively common genetic disorder where the lungs become clogged up with mucus. The mucus then provides an ideal environment for a bacterial infection to take place, leading to the symptoms of bronchiectasis.

It is estimated that cystic fibrosis is responsible for around 1 in 35 cases of bronchiectasis.

Rheumatoid arthritis

Rheumatoid arthritis is a common condition in which the immune system goes wrong and starts attacking healthy tissue, causing inflammation which in most cases is confined to the joints. However, in a small number of cases the inflammation can spread to the lungs, triggering the symptoms of bronchiectasis.

It is estimated that rheumatoid arthritis is responsible for around 1 in 35 cases of bronchiectasis. 

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Diagnosis

Your GP will ask you about the pattern of your symptoms and will want to know how often you cough, whether you bring up any sputum and whether you smoke. 

They may also listen to your lungs with a stethoscope as you breathe (the lungs of people with bronchiectasis often make a distinctive crackling noise as a person breaths in and out) and take a sample of your sputum to be checked for bacteria.

You will also probably be given a chest X-ray to rule out other, more serious causes of your symptoms, such as lung cancer.

If your GP suspects that bronchiectasis could be likely then you will be referred to a doctor who specialises in treating lung conditions (a respiratory consultant) for further testing.

The maximum time you should have to wait for referral is 18 weeks, although you may not have to wait as long as this. Read more about waiting times.

HRCT scan

Currently the most effective test available to diagnose bronchiectasis is called a high resolution computed tomography (HRCT) scan.

A HRCT scan will be taken of your chest at slightly different angles, and a computer is used to put all the images together. This produces a very detailed picture of the inside of your body and the airways inside your lungs (the bronchi) should show up very clearly.

In a healthy pair of lungs the bronchi should become narrower and narrower as they move down deeper in your lungs, in the same way that a tree branch will then separate into narrower branches and then twigs.

If the scan shows that a section of bronchi are actually getting wider, this usually confirms that you have bronchiectasis.

Other tests

Other tests can be used to assess the state of your lungs and to try to determine what the underlying cause of your bronchiectasis may be.

These tests may include:

  • blood tests – which can be used to check how well your immune system is working and check for infectious agents, such as bacteria, viruses and fungi
  • a sample of your blood can be tested to see how much salt is in it – high levels of salt can be caused by cystic fibrosis (if this test is positive then a more detailed genetic test can be carried out: read more about diagnosing cystic fibrosis)
  • pulmonary function test – a small, hand-held device that you blow into is used to measure how hard and how quickly you can expel air from your lungs; this can assess how well your lungs are working
  • bronchoscopy – a flexible tube with a camera at one end is used to look into your lungs; this test is usually only required if it is suspected that you could have breathed a foreign object into your lungs
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Treatment

Bronchiectasis is treated using a combination of:

  • a series of exercises and, in some cases, specially designed devices to help clear your lungs of mucus
  • medication to help improve the flow of air through the airways (bronchi) of your lungs; these are usually delivered using a device known as an nebuliser
  • antibiotics to treat lung infections as and when they occur – in some cases antibiotics may also be used to help prevent infection

In a small number of cases surgery may also be recommended.

Exercise

There are a range of exercises, known as airway clearway techniques, which can help remove mucus from your lungs. This can often help improve symptoms of coughing and breathlessness. 

You can be referred to a physiotherapist who can give you several airway clearway techniques.

The most widely used in the UK is called "active cycle of breathing techniques" (ACBT).

Active cycle of breathing techniques (ACBT)

ACBT involve you repeating a cycle made up of a number of different steps. These include a period of normal breathing, followed by deep breaths to loosen the mucus and force it up; then you cough the mucus out.

You then repeat the cycle for 20 to 30 minutes.

Don't attempt ACBT if you have not first been taught the steps by a suitably trained physiotherapist, as performing the techniques incorrectly could damage your lungs.

If you are otherwise in good health you will probably only need to perform ACBT once or twice a day.

If you develop a lung infection you may need to perform ACBT on a more frequent basis.

Postural drainage

Changing your position can also make it easier to remove mucus from your lungs. This is known as postural drainage.

Each technique can involve several complex steps but to summarise, most techniques involve you leaning or lying down while the physiotherapist or a carer uses their hands to vibrate certain sections of your lungs as you go through a series of "huffing" and coughing.

Devices

There are also devices that can help remove mucus from your lungs. These include:

  • the flutter © – a handheld advice, shaped like an asthma inhaler, which delivers vibration to the airway of your lungs, making it easier to cough out mucus
  • RC cornet © – shaped like a small section of garden hosing, which works in much the same way as the flutter
  • the Acapella © – a device, shaped like a small torch, that uses a combination of vibration and air pressure to help remove mucus

These devices are not always available on the NHS  so you may have to pay for one. They cost in the region of around £45 to £60.

The British Lung Foundation has complied a list of medical equipment suppliers that people with a chronic lung condition may find useful (note – the fact that a company has been included on the list does not mean that they have been endorsed by either NHS Direct Wales or the British Lung Foundation).

Improving the airflow

As well as the exercises discussed above there are other treatments that can help improve the flow of air in and out of your lungs. These are discussed below.

Nebuliser

A nebuliser is a device, consisting of a:

  • face mask or mouthpiece
  • chamber – designed to convert a medication into a fine mist that is then pumped into your lungs
  • compressor – a power source designed to pump the medication into your lungs

The face mask, mouthpiece and chamber, and the medication designed to go in the chamber, are usually available on prescription on the NHS. However, you will have to pay for the compressor which can cost anywhere between around £40 to £120 depending on the manufacturer.

Medications that can be administered using a nebuliser include:

  • normal saline – which is water that contains a small amount of salt (the salt helps ensure that the water is free of germs)
  • hypertonic saline – this is similar to normal saline but contains higher levels of salt as well as a number of other medications; the added minerals can be more effective in clearing out mucus from the airways of your lungs
  • terbutaline – a type of medication known as a beta2-adrenergic agonist which works by helping to relax the airways; a dose of terbutaline is often given before a session of exercise to make the effects of exercise more effective

Other medications

You may be prescribed medications on a short-term basis if you have a particualry severe flare-up of symptoms, although these aren’t routinely used in most cases.

These could include:

  • steroid inhalers (corticosteroids) – which help reduce inflammation inside the lungs
  • bronchodilators – which help relax the muscle in the lungs making breathing easier; beta2-adrenergic agonist is one type of bronchodilator and other types include anticholinergics and theophylline

Antibiotics

If you experience a worsening of symptoms because of a bacterial infection (an infective exacerbation) then you will need to be treated with antibiotics.

A sample of sputum will be taken to determine what type of bacteria is causing the infection. But as testing can take a few days you will be initially treated with an antibiotic known to be effective against a number of different bacteria (a broad spectrum antibiotic).

Depending on the test results, you may be prescribed a different antibiotic, or in some cases, a combination of antibiotics.

If you are well enough to be treated at home you will probably be prescribed two to three antibiotic tablets a day for a 14-day course. It is important to finish the course even if you feel better as stopping the cause prematurely could cause the infection to reoccur quickly.

If your symptoms are more severe (see symptoms of bronchiectasis for a detailed description) then you may need to be admitted to hospital and treated with antibiotic injections.

Preventative treatment

If you have three or more infective exacerbations in any one year or your symptoms during an infective exacerbation were particualry severe then it may be recommended that you take antibiotics on a long-term basis. This can help prevent further infections and give your lungs the chance to recover.

This could involve taking low-dose antibiotic tablets (to minimise the risk of side effects) or using an antibiotic nebuliser.

Using antibiotics in this way does increase the risk that one or more types of bacteria will develop a resistance to the antibiotic. Because of this risk you may be asked to give regular sputum samples to check for any resistance. If bacteria do show signs of developing a resistance then your antibiotic may need to be changed.

Surgery

Surgery is usually only recommended where bronchiectasis is only affecting a single section of your lung (focal bronchiectasis) and your symptoms aren't responding to other treatment.

The lungs are made up of sections known as lobes – the left lung has two lobes and the right lung has three lobes.

Surgery for focal bronchiectasis would usually involve removing the lobe affected by the bronchiectasis in a type of operation known as a lobectomy. (If more than one lobe is affected then it surgery won’t go ahead as it’s dangerous to remove so much lung tissue.)

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Complications

Coughing up large amounts of blood (massive haemoptysis)

A rare but serious complication of bronchiectasis is coughing up large amount of blood (the medical term for this haemoptysis). This can occur when a section of the blood vessels that supply the lungs is suddenly split open.

Symptoms that may indicate massive haemoptysis include:

  • coughing up more than 100ml of blood in a 24-hour period – 100ml is roughly equivalent to a third of a can of coke
  • blood obstructing your airways causing breathing difficulty
  • experiencing symptoms associated with rapid blood loss – such as feeling lightheaded, dizzy and having cold clammy skin

Massive haemoptysis is a medical emergency. If you suspect that you or someone in your care is experiencing massive haemoptysis then call 999 and ask for an ambulance.

A person with massive haemoptysis will need to be admitted to hospital so a tube can be placed into their throat to assist them with their breathing.

Surgery will then be required to stop the bleeding. This is usually done using a type of surgery known as a bronchial artery embolisation (BAE). During a BAE a special dye is injected into your arteries so that they show up clearly on X-rays.

Then, using X-ray scans as a guide, the source of the bleeding is located and injected with tiny particles, around the size of a grain of sand, that will help clog the vessel up and stop the bleeding.

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