Bronchiectasis

What is bronchiectasis?

Bronchiectasis is abnormal damage and dilation (widening) of the large airways. A person may be born with it (congenital bronchiectasis) or may acquire it later in life as a result of another disorder such as cystic fibrosis or recurrent infections. The bronchi are the tubes that transport air to the lung tissue (alveoli). They produce mucus which keeps the tubes moist and prevents bacteria settling. The mucus is normally brushed away by tiny hairs (cilia) that line the walls of the bronchi. In some people, the airways become damaged and the naturally produced mucus is not removed as efficiently.

Subsequently this mucus becomes chronically infected, resulting in airway inflammation and further airway damage.

What causes it?

In over half the cases in the UK the cause is unknown. Other causes are:

• Genetic disorders such as cystic fibrosis or primary ciliary dyskinesia.
• Inhaled foreign object, for example, a peanut
• Gastro oesophageal reflux immunodeficiency

If an associated condition is identified it may be necessary to treat this in addition to the bronchiectasis.

Signs and symptoms

These vary in degree and severity between individuals. Not all symptoms apply to every child.

• Persistent productive cough
• Wheeze
• Shortness of breath especially on exercise
• Weight loss or poor weight gain
• Finger / toe clubbing (clubbing is an increase in the soft tissue of the end of the fingers or toes)
• Recurrent chest infections

How is it diagnosed?

There are several methods of diagnosing bronchiectasis:

• Chest x-ray
• Chest scan CT
• Lung function tests

In addition, other investigations may support the diagnosis:

• Sputum cultures – sputum is taken to the laboratory where it is placed in a medium under conditions that allow the organisms to grow.
• Blood tests

Treatment

Treatment will depend on the severity of the condition. It is a long term condition. The existing damage to the airways cannot be reversed but with treatment further damage may be prevented:

• Antibiotics (oral or intravenous) may be used to treat chest infections
• You may be taught how to perform chest physiotherapy to help clear secretions and mucus from the airway by a physiotherapist.