Disclaimer: This fact sheet is for educational use only. Please consult your doctor or other health professional to make sure this information is right for your child.

Key points to remember

Bx (bronchiectasis) is a lung condition
the breathing tubes (airways or bronchi) in the lungs of a child with Bx have become damaged and scarred
mucus (phlegm or sputum) can collect in the scarred area and infections occur in this mucus
the main symptoms of Bx are:
– a long-standing wet sounding cough
– lots of chest infections
Bx is not the same as asthma but some children with Bx have asthma as well; asthma doesn’t cause Bx
Bx is not catching (contagious)
once your child has Bx, they usually have it for life
good care and treatment can help your child to stay well with Bx
Bx is common in New Zealand, especially in Maori and Pacific Island children and young people

What is Bx?

Bx is a chest disease in which the breathing tubes in the lungs become damaged and scarred.

Normally, the inside of the breathing tubes is smooth and covered with tiny hairs (cilia). These hairs help to clear phlegm or mucus from the lungs.

If your child has Bx, their lungs make more mucus (phlegm or sputum) than normal. The tiny hairs that should clear the mucus stop working properly and the mucus gets stuck in the scarred areas in the breathing tubes.

This extra mucus is a great place for bacteria or viruses to grow and cause infection.

These infections cause damage and more scarring to the breathing tubes 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.

Is Bx different from asthma?

Bx is different from asthma, however some children with Bx have asthma as well. Asthma cannot cause Bx.

What causes Bx?

Most children get Bx after having a very bad or repeated infection/s in the lungs. Possible causes include:

a severe bronchiolitis or pneumonia requiring hospitalisation (see Pneumonia and Bronchiolitis on this website)
less severe chest infections which carry on for a long time
a long-standing wet sounding cough

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 diifculty 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 breathing tubes 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. This condition is called PCD (primary ciliary dyskinesia¹)

You can’t catch Bx or give Bx to anyone - it’s not contagious (catching).

How long does Bx last?

Once a person has Bx, they usually have it for life, so keeping well is very important. See How to keep well with Bx.

Who gets Bx?

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 ten days
at least 80 percent of children affected by Bx are Maori or Pacific Island
at least one in 1,600 Maori children get Bx
at least one in 625 Pacific Island children get Bx

What are the signs and symptoms?

Children with Bx feel well most of the time.

The main symptom is a long-standing wet sounding cough which is caused by the extra mucus (phlegm or sputum) in the breathing tubes.

This cough can get worse:

during infections
first thing in the morning
during exercise (but playing sport is an important part of keeping well with Bx)

When children with Bx become unwell, they cough more and the mucus can change colour from clear to yellow or dark green. Sometimes, the mucus can be very 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 (general practitioner). If there is a large amount, or if the bleeding continues, then you should take your child to see a doctor urgently (see When should I seek help).

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
are coughing more than normal
have more mucus (plegm or sputum) which is darker in colour and / or thicker and / or smells bad
have a temperature / fever
are breathing faster than normal
have pain in their chest
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 Bx diagnosed?

Chest x-ray

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.

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 six years of age).

What tests may be needed?

Sputum culture
Mucus (phlegm or sputum) is often collected and tested for the viruses or bacteria that cause lung infections. This is so that the right antibiotic can be given to your child.

Blood tests
Blood tests are done to try and find out why your child has Bx.

Bronchoscopy
This test involves the doctor using a special telescope to look into the lungs. The test checks the condition of the breathing tubes 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 when they wake up, if you want. Child breathing into a machine during a lung function test

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 30 minutes.

Other tests

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 Bx treated?

All children with Bx need to manage their condition in the following ways: Physiotherapy

Chest physiotherapy (pictured at right). This 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 three to four 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 huff and puff.

Some children with Bx may benefit from inhalers because they also have asthma.

How to keep well with Bx?

Things that will help your child stay well:

Exercising is very important

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 Immunisation: Flu 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)
going to all their outpatient clinic appointments

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, you can:

call the Quitline on 0800 778 778 0800 778 778 for free help
check out the website Quit/Me Mutu²
ask your health professional

How often should my child be seen at an outpatient clinic?

Children with Bx should be reviewed three-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 six months, depending on how well they are.

What will happen at the outpatient clinic appointment?

If your child has Bx and is older than five 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 30 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. This is 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 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. (See Drips (cannula or IV) on this website). 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.

Acknowledgements

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

Diagram 1

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.

Links (these are the web addresses for the numbered links in the text above)

[1] http://www.pcdsupport.org.uk/

[2] http://www.quit.org.nz/page/index.php

Your notes

Endorsement

This fact sheet was endorsed by PSNZ - 16/03/2010

Copyright

Fact sheets are subject to copyright. In the interests of information sharing they may be copied but acknowledgement must be given to PSNZ and Starship Foundation.
© The Paediatric Society of New Zealand and Starship Foundation 2005 - 2010

The Paediatric Society of New Zealand
http://www.paediatrics.org.nz

Starship Foundation
http://www.starship.org.nz