Key points to remember
- bronchiectasis (Bx) is a type of scarring in the lungs
- the airways in the lungs have become distorted and scarred
- mucus (phlegm or sputum) can collect in the scarred area
- infections occur in this mucus
- the main symptoms of Bx are:
- a wet sounding cough lasting for weeks or recurring frequently
- lots of chest infections
- Bx is not the same as asthma but some children with Bx have asthma as well
- Bx is not catching (contagious) - it is not spread between children
- once your child has Bx, they usually have it for life
- early recognition and treatment can improve the scarring
- good care and treatment can help your child to stay well with Bx
- Bx is common in New Zealand, especially in Māori and Pacifica children
What is it?
Bx is a chest disease in which the airways in the lungs become damaged and scarred.
The airways become widened and mucus can be trapped in pockets within the airway. Having extra mucus in the airways means bacteria and viruses can grow quickly and cause new or chronic infection.
These infections cause damage and more scarring to the airways and lungs. The breathing tubes become baggy and holes form in the lungs. Once this has happened, the scarring and damage cannot be fixed. However, with good treatment, further damage can be prevented.
Diagram 1: Figure A shows a cross-section of the lungs with normal airways and with widened airways. Figure B shows a cross-section of a normal airway. Figure C shows a cross-section of an airway with bronchiectasis. See Acknowlegements below.
Is bronchiectasis different from asthma?
Bx is different from asthma, however some children with Bx have asthma as well.
What causes it?
Most children get Bx after having a very bad infection or repeated infections in the lungs. Possible causes include:
- a severe bronchiolitis or pneumonia requiring hospitalisation
- less severe chest infections which carry on for a long time
- a wet sounding cough which lasts for weeks or recurs frequently
There are also other less common causes:
- some people get Bx because they cannot fight infection very well. In some children it can be because they are born with a problem in their immune (infection-fighting) system. Some medications cause difficulty in fighting off infection
- Bx can also start if an object, such as a nut or a piece of a toy goes down the “wrong way”, gets stuck in the airways and stays there
- children can have very bad reflux of acid from their stomachs or have food that goes down their airway as well as their food pipe when they eat (aspiration). These children sometimes cough or choke when feeding
- some children’s Bx is caused by the tiny mucus-clearing hairs (cilia) in the lungs not working properly. The hairs line all the airways and move mucus along. This condition is called PCD (primary ciliary dyskinesia)
You can’t catch Bx or give Bx to anyone - it’s not contagious and cannot spread between children.
How long could it last?
Once a person has Bx, they usually have it for life, so keeping well is very important.
Who gets it?
- at least one in 3,000 New Zealand children gets Bx
- there is approximately one new case of Bx diagnosed in a New Zealand child every week
- at least 80 percent of children affected by Bx are Māori or Pacifica
- at least one in 1,600 Māori children and one in 625 Pacifica children get Bx
What are the signs and symptoms?
Children with Bx feel well most of the time.
The main symptom is a wet sounding cough lasting for weeks which is caused by the extra mucus (phlegm or sputum) in the airways.
This cough can get worse:
- during infections
- first thing in the morning
- during exercise (but playing sport is also an important part of keeping well)
When children with Bx become unwell, they cough more and the mucus can change colour from clear to yellow or dark green and sometimes the mucus can be smelly. When children with Bx get sick, they may lose their appetite and feel tired.
Children with Bx can occasionally cough up blood. This is scary but is not always serious. However you should take your child to your GP especially If there is a large amount, or if the bleeding continues, which makes it urgent.
When should I seek help?
If your child has Bx, you should take them to see your family doctor when:
- they have a bad cold with a runny green nose
- they are coughing more than normal
- their cough becomes more wet sounding than usual
- they have more mucus (plegm or sputum) which is darker in colour and / or thicker and / or smells
- they have a fever
- they are breathing faster than normal
- they have pain in their chest
- they are tired and not eating or drinking as well as normal
If your child has the following symptoms, they are very sick and need to see a doctor urgently:
- a high fever which doesn’t go down with paracetamol
- gets suddenly more unwell
- coughs up a large amount of blood
Dial 111 for urgent medical help if your child
- is having difficulty talking because they are so short of breath
- has blue lips or tongue
How is it diagnosed?
One of the first things a doctor will do if they are worried about your child’s chest is get a chest x-ray. Chest x-rays are useful but do not always show Bx damage very well. If the doctor is concerned about Bx, they will go on to get a chest CT scan.
Chest CT scans are special x-rays. They show Bx damage well and so are the best way to diagnose this condition. The image to the right is of a chest CT scan showing Bx damage in one lung.
Having a CT scan does not hurt. Your child may need a general anaesthetic for this if they are unable to lie very still for a short time (such as children under 6 years of age).
What tests may be needed?
Mucus (phlegm or sputum) is often collected and tested for the viruses or bacteria that cause lung infections so that the right antibiotic can be given to your child.
Blood tests are done to try and find out why your child has Bx.
This test involves the doctor using a special telescope to look into the lungs. The test checks the condition of the airways and involves taking a sample of mucus. This allows the doctor to find out what bugs are there so that the right antibiotics can be given. Photos of the inside of the lungs will be taken and the doctor will show them to you and your child.
Lung function test
This is a test (pictured at right) that involves your child breathing into a machine to measure how their lungs are working. It is an easy test that does not hurt. It usually takes about 15 minutes.
This looks for cystic fibrosis - an inherited condition causing frequent infections.
Sometimes other tests, such as a sweat test (pictured below), are needed. These will be explained by your doctor.
A child's arm being prepared for a sweat test:
How is it treated?
All children with Bx need to manage their condition in the following ways:
Chest physiotherapy usually needs to be done once or twice a day. Your action plan will say how often. There are different ways to do chest physiotherapy - either with breathing and percussion or by using a physiotherapy breathing device (PEP, acapella, bubble). The aim is to clear the extra mucus or phlegm from the lungs and keep your child well. The physiotherapist will show you the best method to use with your child.
Antibiotics if your child starts to get sick. Antibiotic treatment for children with Bx needs to be longer than for other children (10 to 14 days).
Exercise or play sport 3 to 4 times a week for at least half an hour (for example, walking, trampoline, school sport). All children need exercise but it is especially important for children with Bx. Any type of exercise is good, especially if it makes your child take deep breaths.
Some children with Bx may benefit from inhalers because they also have asthma.
How to keep well with bronchiectasis?
Things that will help your child stay well:
- doing their physiotherapy regularly to keep their lungs clear
- exercising regularly
- eating a healthy balanced diet
- avoiding tobacco smoke, especially in a house or car
- never smoking themselves
- having their flu vaccine each year (see Flu immunisation on this website)
- visiting the family doctor as soon as your child is sick. If you have not met them before, it is important you tell them that your child has Bx (bronchiectasis)
- regular hospital clinic reviews for assessment and update of your child's treatment
All children and young people with Bx should have an action plan for when they are sick. This should be completed by your physiotherapist or doctor and reviewed regularly. If your child does not have an action plan, you should ask for one from your doctor.
If you want to give up smoking:
- call the Quitline on 0800 778 778 0800 778 778 for free help
- check out the website Quit/Me Mutu
- ask your health professional
How often should my child be seen at an outpatient clinic?
Children with Bx should be reviewed 3 monthly by the most appropriate local doctor, in consultation with other health care providers. Your child should be reviewed by a paediatrician or respiratory paediatrician at least once every 6 months, depending on how well they are.
What will happen at the outpatient clinic appointment?
If your child has Bx and is older than 5 years, they will be asked to do a breathing test called lung function testing. This is a test that involves your child breathing into a machine to measure how their lungs are working. It is an easy test that does not hurt. It usually takes about 15 minutes.
If your child can, they will be asked to cough up some mucus into a clean pot so it can be tested for bacteria or viruses so that the right antibiotic can be given to them.
Your child will be seen by the doctor. They will ask you and your child questions, and look and listen to your child’s chest. They will also check on growth and nutrition.
If your child takes puffers and inhalers, bring them to clinic, so that the doctor or nurse can check how well your child is using them.
Sometimes a physiotherapist will review your child to check techniques for chest physiotherapy.
Other people your child may see in clinic are – the respiratory nurse, the dietitian, and the social worker.
What will happen if my child needs to come into hospital?
Sometimes an antibiotic given at home and regular chest physiotherapy are not enough to keep your child well. Sometimes your child needs to go to hospital where they will usually need to stay 10 to 14 days.
Your family doctor will refer you to the emergency department of your local hospital. After looking and listening to your child’s chest, hospital doctors and nurses may do some extra tests. They may do a chest x-ray and if possible may collect some of your child’s mucus to see which bacteria or viruses are making them sick.
If your child has to stay in hospital, they may need to have a “drip” (IV or special plastic tube) put into their hand or arm to give them stronger antibiotics. Sometimes, if they are going to have antibiotics for more than a few days, the doctors arrange for a special drip called a “PICC line” to be put into their arm. They will need to have an anaesthetic for this so they are asleep and to make sure it doesn’t hurt.
Your child will need to have physiotherapy much more regularly.
Starship Foundation and the Paediatric Society of New Zealand acknowledge the co-operation of the Starship Respiratory Service at Starship Children's Health in making this fact sheet available to patients and families.
Acknowledgements for images
The image of a normal airway and an airway with bronchiectasis comes from
http://www.nhlbi.nih.gov/health/dci/Diseases/brn/brn_whatis.html. Thank you to the National Heart, Lung, and Blood Institute (NHLBI), part of the National Institutes of Health and the U.S. Department of Health and Human Services, for use of this image.
© Paediatric Society of New Zealand and Starship Foundation 2005 – 2014
Printed on 24 April 2014. Content is regularly updated so please refer to www.kidshealth.org.nz for the most up-to-date version