Bronchiectasis and exacerbations or “flare ups”

What is Bronchiectasis?

Bronchiectasis is a long-term lung condition which affects the airways or ‘pipe work’ of your lungs. Air is carried into your lungs through these airways which divide into smaller and smaller tubes until they get to the little air sacs deep in the lung called alveoli.

In bronchiectasis, these airways have become inflamed and scarred. The scarring causes the airways to become much wider and floppier. The inflammation causes lots of phlegm to be produced.

What causes Bronchiectasis?

For the majority of patients, we don’t find one specific cause. When we are able to identify a cause, it is usually after having a severe lung infection such as pneumonia.

These infections cause the lungs and airways to become very inflamed and this inflammation heals with scarring. This is especially true if you have had these illnesses as a child. It can also happen if you have lots of infections as an adult and you have another lung condition such as asthma or COPD.

There are other causes including:

• Other conditions causing inflammation in your body such as Rheumatoid Arthritis, Systemic Lupus Erythematosus, Crohn’s disease and Ulcerative Colitis.
• Immunity-deficiency problems: These are illnesses in which your immune system is underactive and causes you to pick up infections more easily 
• Airway blockages: Occasionally food or objects can be accidentally inhaled into your airways. These will block an airway or group of airways. This blockage traps mucous and phlegm which causes inflammation and scarring.
• Gastro-Oesophageal Reflux Disease: This is also known as acid reflux. Some people are prone to acid washing out of their stomach and up the oesophagus (the gullet).
  The top of the gullet shares an opening with the top of your wind-pipe. If the acid reaches this opening, small amounts of it can travel down your wind pipe and into the airways in your lung. This acid can cause the airways to become inflamed.
• Allergy: You can become allergic to a fungus called aspergillus which is in the environment around us. This allergic response in your airways causes them to become inflamed. If this isn’t treated, in the longer term it can lead to bronchiectasis.
• Other causes: There are rare conditions of your airways and some other inherited conditions which can cause bronchiectasis.

You may also know that Cystic Fibrosis (CF) causes bronchiectasis. This is different from the bronchiectasis we are describing here

How will Bronchiectasis affect me?

The most common symptom of bronchiectasis is a chronic daily cough which produces sputum or ‘phlegm’. This sputum can change in colour and you can produce a lot of it over a day, up to the equivalent of a tea cup or more. This phlegm can be extremely sticky and difficult to cough up.

Other symptoms and problems:

• Chest infections: You may be more prone to picking up chest infections and these can take longer to get better.
• Fatigue: You may notice that you are much more easily tired or lacking in energy.
• Chest pain: During chest infections, you can get pain in the chest. This can be quite severe and limit your ability to take a deep breath or cough.
• Weight: If you get lots of infections, you can be prone to losing weight.

How is Bronchiectasis diagnosed?

Your team will listen carefully to your story and symptoms. They will examine you and may hear crackles in your chest when you’re breathing. They will then usually do the following tests:

• Chest X-ray: This is usually done to make sure that you haven’t had another problem causing your symptoms.
• Breathing tests: You may have a breathing test called spirometry. This is to look for narrowing in your airways. This is normally used to try and work out if you have asthma or COPD as a cause for your symptoms.
• Sputum sample: You might be asked for a sputum sample, usually if you have had courses of antibiotics but haven’t got better. This is to check if you have any bacteria in your lungs which aren’t sensitive to normal antibiotics.

These tests are normally done by GPs and they are very important to look for causes of your symptoms.

If these tests haven’t shown what is causing your symptoms or making you unwell, you may be referred to the hospital clinic for a further appointment. If your doctor believes you may have bronchiectasis, they will arrange for you to have a CT scan of your chest. This is a 3 dimensional X-ray of your chest. It shows the lungs in fine detail and is the best test to diagnose bronchiectasis.

What other tests might I have?

Blood tests: If you have bronchiectasis, you will usually have some other blood tests, such as:

• Immunoglobulin testing: This test measures the level of antibodies and proteins that your body is producing. If there are low levels, this can suggest a problem with your immune system as a cause for your bronchiectasis. We also check your immunity to tetanus and some bacteria which can cause pneumonia.
• IgE and Aspergillus level: IgE (Immunoglobulin E) is another protein produced by the immune system. It is involved in allergic conditions such as asthma.
  There is also a blood test for the fungus aspergillus which can test how sensitive you are to this. The combination of these two blood tests can help to work out whether aspergillus has potentially caused your bronchiectasis.
• Cystic Fibrosis gene testing: If you are young when you develop bronchiectasis, or have other conditions which might suggest you could have cystic fibrosis, your team will send a blood test to look for any of the genetic mutations which can cause cystic fibrosis.

Other tests: These tests are performed only in a small number of patients:

• Sweat testing: If you have a positive Cystic Fibrosis blood test, or if you have other conditions which suggest you may have Cystic Fibrosis then you may be asked to have a sweat test. This is a simple test which measures the amount of chloride in your sweat and can help diagnose Cystic Fibrosis.
• Primary Ciliary Dyskinesia: This is a very rare condition. If your team suspect you have this, they will send you to a specialist centre for assessment.

How is Bronchiectasis treated?

Bronchiectasis can’t be cured but the symptoms and their effects can be treated or managed. The main goal is to get rid of as much of the phlegm in your chest as possible; things which can help are:

Chest physiotherapy

This is the most important thing for your day-to-day symptoms. You will usually be referred to a physiotherapist who specialises in chest illnesses. They will then teach you various different breathing and coughing techniques to help clear the mucous and phlegm which accumulate in your chest.

Once you have been taught these we also ask you to do them on a daily basis. Keeping your chest clear of phlegm reduces your symptoms and your chance of infections or flare ups.

If the physiotherapist thinks that you aren’t able to clear the phlegm in your chest with the coughing or breathing techniques alone, they may provide you with a device such as a flutter or acapella device for you to use as well.

Anti-mucous medicine

You will usually be prescribed a medicine known as a mucolytic. The most common of these is known as carbocisteine. This is usually taken 2-3 times daily. It helps to make your chest mucous much runnier and wet, making it easier to cough up.

Nebulisers

A nebuliser is a device which delivers medicine in a fine mist through a face mask or mouth-piece. If you are still having problems removing the phlegm from your chest despite the coughing techniques, devices and anti-mucous medicine may be given using a nebuliser.

This will contain a medicine called hypertonic saline which is very salty water. This is taken twice a day and helps draw water into your airways and make the phlegm much runnier and easier to cough up.

There are other things and treatments which may also help:

Stopping smoking

In addition to clearing the phlegm regularly, stopping smoking is the single most important thing you can do. This helps reduce the inflammation in your airways, which cuts down the amount of mucous and phlegm that is being produced. Over time, it will also help some of the narrowing in your airways to improve.

If you want help (which we really encourage, stopping smoking on your own is hard) then we will be able to assist you to access the help you need.

Inhalers

Inhalers are not usually used for bronchiectasis itself, however if you have another chest problem, such as asthma or COPD, you may be given inhalers for these conditions.

Exercise

Exercise is really important. People with bronchiectasis can become more breathless because of the narrowing in the airways. Breathlessness may make you more reluctant to exercise which causes you to lose fitness and muscle strength. This loss of fitness and strength can, in itself, make you more breathless. It’s important to realise that it is still safe for you to exercise and getting breathless will not cause you or your lungs harm.

We encourage you to continue to exercise, especially doing things that you enjoy. Your team may offer you the chance to attend a class called Pulmonary Rehabilitation. This is an exercise training class for people who are chronically breathless due to a lung condition. It is run by specialist physiotherapists who are experienced in working with people who get easily breathless.

Over the course of the class, which runs for 6-8 weeks, they will help you to exercise and gradually build up your fitness. People who complete the class are generally fitter, less breathless and in a better position to live a normal life.

What long term effects can Bronchiectasis cause?

Long term colonisation of your lungs with bacteria

Over time, certain bacteria may take up residence in your lungs. These can be found by providing regular sputum samples. The more common bacteria are Haemophilus influenzae and Moraxella catarrhalis. Usually, these bacteria will not cause you any problems, but from time to time they may cause flare ups.

Another bacterium called Pseudomonas Aeruginosa can colonise your lungs if you have more extensive bronchiectasis. This bacterium produces a very sticky film in your airways. This can make your mucous a very strong colour and much stickier.

Again the majority of time these bacteria will not cause day-to-day problems, but can give you more frequent and longer lasting flare ups. It is important that we know if it is present in your lungs as treating it often requires different antibiotics we don’t normally use for chest infections.

Other infections

Very rarely, some patients with severe bronchiectasis can become colonised with other bacteria know as non-tuberculous mycobacteria. These bacteria come from the same family as tuberculosis. They are usually picked up from the environment and cannot be passed from person to person.

Usually, these bacteria are found due to the phlegm sample becoming contaminated on the way to the laboratory. If any of these bacteria are found in your phlegm sample, your team will ask you for further samples of your phlegm to check it is a genuine result.

Alternatively, you may be asked to have a camera test (called a bronchoscopy) so that a sample of your phlegm can be obtained directly from your lungs.

If these bacteria are genuinely present in your lungs, you will only be treated if the team looking after you think they are causing your lungs problems.

Coughing up blood

Occasionally, the arteries and veins supplying the parts of your lung with bronchiectasis can become swollen or fragile. This can cause you to cough up blood.

If this is happening frequently or it is a significant amount of blood, we may arrange for you to have an X-ray procedure known as a bronchial angiogram. This may allow us to identify the artery or vein causing the problem and block it off.

What is an exacerbation of Bronchiectasis?

An exacerbation of bronchiectasis is also known as a ‘flare-up’. You will get to know what your normal day-to-day symptoms are from your chest. You will also be able to tell the difference between what good and a bad days feel like. You will also know how long these bad days usually last for.

A flare up is when your symptoms get much worse than your usual bad days and for longer.

What causes a flare up of Bronchiectasis?

The most common causes of flare ups are infections. Bacteria are most common but they can be caused by viruses or a combination of both.

As mentioned before some people have bugs colonising their lungs which can cause more frequent flare ups, this is particularly true if you have pseudomonas in your lungs.

There are other causes of exacerbations which are not infections. These include worsening air pollution, pollen and dust, particularly hot or cold weather, smoking and forgetting to take your regular bronchiectasis treatment. If you stop doing your daily mucous clearing exercises, this may also increase your chances of having a flare up.

What symptoms could I get during a flare up of my Bronchiectasis?

During a flare up, your usual symptoms will tend to be worse than normal. You may also get different symptoms:

• Breathlessness: You may be more breathless doing things you usually manage. The breathlessness can be worse at night.
• Cough and phlegm: You will probably cough more than you usually do. The volume and colour of your phlegm might change. It may become harder to cough up as it is stickier.

Other symptoms:

• Coughing up blood: Some people do cough up blood when they have a flare up. It is usually a small amount.
• Wheezing: You may be wheezier than normal and you may need to use your ‘reliever’ inhaler more often.
• Fever: You may get a fever or feel hot and shivery.
• Tiredness: You may feel tired or fatigued much more easily than normal.
• Chest pain: You may have chest pain during a flare up. This can be sharp and sometimes may make it more difficult to cough or breathe deeply.

What tests will diagnose a flare up?

The most important way your team will diagnose you is to listen to you describe your symptoms and to examine your chest. It will be important to them to understand what your chest is like on a normal day.

If they are concerned or you are sent to hospital, they may do other tests including:

• X rays: You may have an X-ray of your chest. This is usually done to make sure there isn’t another reason for your symptoms. The team are looking for things such as pneumonia, fluid in the chest or collapsed lungs.
• Heart tracings: These are known as ECGs and are recordings of the electricity being conducted through your heart. These are done to make sure that there hasn’t been a problem with your heart that has caused your symptoms.
• Blood tests: If you are admitted to hospital, you will usually have blood tests. These check for evidence of infection in your body and whether there are other problems such as kidney or liver problems which may be contributing to your symptoms.
  If your oxygen level is low when it is checked by a finger probe, you might also have a sample of blood taken out of the artery in your arm, this is called an arterial blood gas (ABG).
• Sputum (phlegm) samples: If you are able to cough up phlegm, we will ask you for a sample of this. This can identify if bacteria are causing the infection and allow us to choose an antibiotic which is tailored towards this.

What treatment will I need?

Antibiotics

You will usually be given a course of antibiotics for 10 – 14 days. If you are well enough, this will be given as tablets. The antibiotics may be chosen depending on what bacteria have previously been found in your phlegm, or on what experience you and your team have had.

If you are more unwell, or if you have had courses of tablet antibiotics recently which haven’t made you better, we may give the antibiotics through a drip (a small plastic tube with a needle attached) into your arm.

In this case, you may need up to two weeks of this type of treatment. This doesn’t mean that you would need to be in hospital for this time. If you are well enough not to come to hospital, or your team think you can get home very soon, we can organise this treatment to be done at home.

This will mean that you have a cannula in your arm (you will be shown how to protect it) and a team known as the Out Patient Antibiotic Team will visit you twice a day and give you an injection of antibiotics.

Alternatively, you may be given an antibiotic bottle and pump which runs for 24 hours. The bottle is changed once a day. At the end of the course of antibiotics we take the cannula out. This approach is not suitable for everyone and your team will tell you if they think you are suitable.

Chest physiotherapy

When you notice the symptoms of a flare up, we ask you to increase how often you do your coughing and breathing techniques. This can be up to three or four times a day.

You may also be asked to increase the dose of your anti-mucous medicine (carbocistene) to help these techniques be more effective.

If you are admitted to hospital you may be visited by a physiotherapist so that they can help you to clear your chest.

Nebuliser

If you are admitted to hospital, you may also be given a regular nebuliser. This will usually be salty water (a much less potent version of the one described previously) to help make your phlegm runnier and easier to cough up.

If you have asthma or COPD, you may also be given other medicines through the nebuliser. You will not normally go home with a nebuliser (unless you had one at home already).

Steroid tablets

You won’t normally be given these during flare ups of your bronchiectasis, unless you also have asthma or COPD.

Oxygen

If you are admitted to hospital and your oxygen levels are low, you will usually be given oxygen, either through a mask on your face or a tube with prongs which sit inside your nose.

Where will I be admitted to?

If you are admitted to hospital, you will normally be seen in the Emergency Admissions Unit or the Ambulatory Care unit. If you need to stay in hospital for more than 24 – 48 hours, you will usually move to one of our medical wards.

When will I get home?

We will get you home as soon as you are well enough.

Is there anything that can stop me having more flare ups?

Chest infections are a part of normal day-to-day life, even in people who don’t have lung conditions. Because of this, you will still get flare ups. However, there are things which can make these flare ups less frequent:

• Ensuring you are up to date with your pneumonia vaccination.
  
• Self-management plans: Once you have got to know what your normal day-to-day symptoms are and what happens when you are getting a flare up, you may get a written management plan to help you deal with these.
  

These plans usually involve when to increase your normal physiotherapy and anti-mucous medicines and when you need to start antibiotics.

If you are suitable for one of these plans you may have an antibiotic on your repeat prescription and a supply at home for you to start when you have a flare up. When you use a course of your antibiotics, you can order another with your prescription.

Some people will continue to get frequent flare ups despite all of the usual treatment described previously. In this situation you will most likely be referred to see a chest consultant in the hospital clinic. If you are getting more than 2 – 3 flare ups a year, they may think of trying the following:

Long term antibiotics

Your doctor may prescribe you an antibiotic which you will take for at least 6 months. The most common antibiotic that is used is called Azithromycin. It is taken three times a week. This antibiotic reduces the inflammation in your airways which allows them to fight off infections more effectively.

This will hopefully cut down the frequency of flare ups you get. Before you start this, your doctor would do some special tests on your sputum and take a heart tracing. They would explain what possible side-effects you might get and what tests you will need while you are on the treatment.

If the treatment is successful and you have less flare ups over the initial six months, you would very likely stay on this long term. If you are allergic to this antibiotic or have had a trial of this and it hasn’t been successful, we may consider other tablet antibiotics, such as Doxycycline or Co-amoxiclav instead.

Nebuliser antibiotics

If you have grown pseudomonas in your sputum and are getting frequent flare ups, we may consider prescribing an antibiotic called Colomycin, which is taken through a nebuliser twice a day.

This antibiotic suppresses the pseudomonas and prevents it from growing. It may help reduce the number of flare ups you get. Occasionally, nebulised antibiotics are used if you are colonised with other bacteria and they don’t seem to be responding to tablet or intravenous antibiotics. This is much less common, but your doctor would consider this.

Regular intravenous antibiotics

Occasionally, if the other strategies have been tried and not been successful, your consultant may decide to give you regular courses of intravenous antibiotics every 8-12 weeks to keep you well.

Will I need any follow up?

We will usually ask you to come back to a hospital clinic if you are having frequent flare ups. If we decide to try any other methods to cut down your flare ups, this is usually only done in hospital clinics or by hospital based teams.

Contact numbers

Lung Health
Telephone: 01642 624270

Department of Respiratory Medicine
Monday to Friday
09:00am-05:00pm
Telephone: 01642 624936

Emergency Department
24 hours a day, 7 days a week
Telephone: 01642 382899

Where can I get more information?

https://www.blf.org.uk/support-for-you/bronchiectasis

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Leaflet Reference : PIL1211

Date for Review: 28/08/2023