Bronchiectasis: the 'other' obstructive lung disease

Bronchiectasis is a relatively rare condition that affects the lungs. In this disorder the bronchial tubes become enlarged and distended forming pockets where infection may gather. The walls themselves are damaged which results in impairment to the lung’s complex cleaning system. The tiny hairs, called cilia - which line the bronchial tubes and sweep them free of dust, germs and excess mucus - are destroyed. When this cleaning system is not working effectively dust, mucus and bacteria accumulate. Infection develops and is difficult to remove.

The Causes

Bronchiectasis is caused by various types of infections which damage and weaken the bronchial walls and interfere with the action of the cilia. Patients may be predisposed to get this condition with various congenital or inherited deficiencies such as immunological deficiency or cystic fibrosis. Rarely patients inherit a primary abnormality of the hair cells or cilia which renders them more prone to develop bronchiectasis.

Certain pneumonias which may be associated with measles and whooping cough, usually occurring in childhood may predispose to this condition by weakening the walls of the bronchial tubes and causing pockets of infection to form.

An obstruction of some sort - anything that presses on the bronchial tubes from the outside or blocks them from the inside - may also cause bronchiectasis. In childhood this most commonly results from choking on food such as a peanut which is small enough to go down the windpipe and large enough to block off one of the air tubes. When this happens the wall of the tube is injured and air is prevented from passing beyond the obstruction. The bronchial tube, below the obstruction, balloons out to form a perfect hiding place for infection and pus.

Symptoms

The main symptom of patients with bronchiectasis is a cough. This cough occurs with great regularity every day. Generally patients with bronchiectasis cough up large quantities of phlegm which resembles green or yellow pus. It would not be uncommon for somebody to cough up more than a cup of this phlegm per day. This phlegm may have an objectionable odour and can be a source of great embarrassment to the patient suffering from bronchiectasis. This occurs because pus is produced in the enlarged bronchial tubes which are chronically infected and can only be removed by coughing.

Although chronic infection exists within the tubes the patients generally feel well - without fever or pain. However, they are prone to getting worse infections which may go on to pneumonia if not promptly treated.

Spitting up blood may occur from time to time in patients with bronchiectasis. Tiny blood vessels near the surface of the thinned walls of the bronchial tubes are easily ruptured. The bleeding is generally minor and self limited but occasionally can present as a major emergency. Coughing up blood usually indicates added infection.

Symptoms

Chronic cough with large amounts of foul-smelling sputum production
Coughing up blood
Cough worsened by lying on one side
Shortness of breath worsened by exercise
Weight loss
Fatigue
Clubbing of fingers may be present (abnormal amount of tissue in the fingernail beds)
Wheezing
Skin discoloration, bluish
Paleness
Breath odour

Treatment

The treatment of bronchiectasis is designed to prevent the complications of pneumonia and blood spitting and to allow patients with this condition to live as normal a life as possible. The most important aspect of the treatment is done at home by the patient often with the help of family members. Because the usual mechanism for cleaning the lung is not effective a helping hand is necessary. As a result patients with bronchiectasis must learn to position themselves so that the damaged areas of the lungs can drain by gravity. This is usually done by hanging one’s head over the side of the bed with the affected part of the lung upper most. This is usually necessary one to three times a day and can be taught to the patient by a physician or physiotherapist. Clapping the chest to help the mucus run out is also very helpful. This can be done by a family member by hand or using a mechanical percussor.

Bronchiectasis is often caused by recurrent inflammation or infection of the airways. It may be present at birth, but most often begins in childhood as a complication from infection or foreign body aspiration.

Cystic fibrosis causes about 50% of all bronchiectasis in the United States today. Recurrent, severe lung infections (pneumonia, tuberculosis, fungal infections), abnormal lung defenses, and obstruction of the airway by a foreign body or tumor are some predisposing factors.

Symptoms often develop gradually, and may occur months or years after the event that causes the bronchiectasis.

Complications

Cor pulmonale
Recurrent pneumonia
Hemoptysis (coughing up blood)

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