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Non-cystic fibrosis bronchiectasis fact sheet

Non-cystic fibrosis bronchiectasis

bronchiectasisBronchiectasis is a condition in which some of the breathing tubes (bronchi) are damaged by repeated infections and inflammation, and become wider and weaker than normal. Excessive mucus produced by the lining of the breathing
tubes also results in a wet or phlegmy cough. This situation is worsened by colds and flus (virus infections) and, particularly, exposure to cigarette smoke. During these periods bacterial infections (bronchitis and pneumonia) may also occur and children will need antibiotics and sometimes even hospitalisation for intravenous antibiotics.

Paediatric bronchiectasis differs from adult bronchiectasis in terms of causes, natural course and severity. With early detection and effective treatment the process can be halted and even reversed in children.

Bronchus diagram

Possible causes of bronchiectasis

  • Respiratory infections such as previous pneumonia, whooping cough, influenza, adenovirus, tuberculosis (TB) or aspergillosis (a fungal infection).
  • Bronchial obstruction caused by an inhaled object (foreign body) or enlarged
    lymph glands.
  • Hereditary conditions such as primary ciliary dyskinesia (PCD), Marfan syndrome and immuno-deficiency states, and autoimmune or hyper-immune disorders (eg. rheumatoid arthritis).
  • Inhalation injury (e.g. breathing in noxious fumes or gases)
  • Airway inflammation or swelling.

In up to 40 per cent of child patients, no cause may be found.

Symptoms

Bronchiectasis can develop at any age. Symptoms include chronic (lasting more than four weeks) wet or productive cough, shortness of breath, abnormal chest sounds, weakness, weight loss
and fatigue. Recurrent chest infections may produce coloured mucus. A chronic wet cough is often the only symptom present for some children with bronchiectasis.

Diagnosis

Bronchiectasis is diagnosed via a chest computed tomography (CT) scan. A chest X-ray, bronchoscopy, lung function, blood tests, sputum culture, and other tests (e.g. immune function, sweat test and genetics) for associated diseases may also be performed. Before a diagnosis of bronchiectasis is made, symptoms are often attributed to asthma or a viral infection.

If a chronic wet cough fails to respond to four weeks of appropriate antibiotics it increases the probability of underlying bronchiectasis and is an indication for referral to a specialty respiratory service.

Treatment

Treatment requires a multi-disciplinary approach with regular reviews. The aim is to control symptoms, improve quality of life and prevent disease progression. Treatment includes:

  • Early use of antibiotics for acute flare-ups or epsiodes
  • Daily airway clearance techniques (chest physiotherapy – see below)
  • Appropriate vaccinations
  • Avoiding irritants such as cigarette smoke
  • Bronchodilators (only if asthma is also present)

Chest physiotherapy

Chest physiotherapy aims to mobilise, shift and clear mucus to prevent infections.
A physiotherapist will advise on the most appropriate techniques for your child’s age and disease. Techniques may include breathing exercises, chest percussion or positive expiratory pressure (PEP) devices. Regular exercise is also important.

Contact us

Respiratory and Sleep Unit (5a)
Queensland Children’s Hospital
Level 5, 501 Stanley Street, South Brisbane 4101
t: 3068 2300 (Mon- Fri, 8.30am -4pm)
t: 07 3068 1111 (general enquiries)
e: Lcch_respiratory@health.qld.gov.au

In an emergency, always call 000.

If it’s not an emergency but you have any concerns, contact 13 Health (13 43 2584). Qualified staff will give you advice on who to talk to and how quickly you should do it. You can phone 24 hours a day, seven days a week.

Resource No: FS121. Developed by Respiratory and Sleep Unit, Children’s Health Queensland. Updated: January 2017. All information contained in this sheet has been supplied by qualified professionals as a guideline for care only. Seek medical advice, as appropriate, for concerns regarding your child’s health.

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