Causes and Risks: Cigarette smoking is the major cause of emphysema. Tobacco smoke and other pollutants are thought to cause the release of chemicals within the air sacs of the lung that damage the walls of the air sacs. The damage becomes worse over time, affecting the exchange of oxygen and carbon dioxide in the lungs.

A substance called alpha-1 antitrypsin may protect against the damage. People with alpha-1 antitrypsin deficiency are at a higher risk for severe disease. The incidence is 2 out of 1,000 people.

Emphysema in children is caused by congenital abnormalities of the lung and alpha-1 antitrypsin deficiency.

Prevention: Avoiding exposure to tobacco smoke and other pollutants will decrease the risk of the disease.

Symptoms:

• a chronic cough
• a bluish coloration of the skin caused by lack of oxygen
• shortness of breath (chronic)
• shortness of breath with mild exertion
• wheezing

• nasal flaring
• eyes, bulging
• vision abnormalities
• dizziness
• breathing, absent temporarily
• breathing difficulty, lying down
• anxiety, stress, and tension
• ankle, feet, and leg swelling
• unintentional weight loss
• headache (may be worse in the morning)
• impotence
• fatigue
• impaired ability to concentrate
• excessive daytime sleepiness
• difficulty falling asleep or staying asleep (insomnia)
• awakening many times at night
• awakening from sleep not rested
• irritability or poor temper control
• memory loss
• clubbing of the fingers or toes

Signs and Tests: A physical examination shows decreased breath sounds, wheezing, abnormal breath sounds (rales), and prolonged exhalation (exhalation takes more than twice as long as inspiration). The chest may appear enlarged on the right side, or there may be an increased front-to-back diameter of the chest (barrel shaped chest). There may be signs of chronic sleep deprivation, or signs of chronically insufficient oxygen levels in the blood.

These tests help confirm the diagnosis:

• arterial blood gases showing reduced oxygen
• serum alpha-1 antitrypsin level showing a deficiency
• chest X-ray
• pulmonary function tests

• urine pH
• a pulmonary ventilation/perfusion scan
• a chest MRI
• a CEA level (not useful in children)

Treatment: Medications used to improve breathing include bronchodilators (hand-held inhaler or nebulizer), diuretics, and corticosteroids. Antibiotics are prescribed when respiratory infections occur. Influenza (flu) vaccines and Pneumovax are recommended for people with this diagnosis.

Thinning and removal of lung secretions can be enhanced by an adequate fluid intake and chest physical therapy (postural drainage and chest clapping).

Many people use low-flow oxygen at home. Avoidance of air pollution and smoking is important.

EXPERIMENTAL THERAPY:
An experimental surgery, called lung reduction, is currently being used to treat some patients with severe emphysema. Lung reduction surgery removes the damaged portions of the lung, thus allowing the normal portions of the lung to expand more fully and take advantage of increased areation.

Prognosis: The outcome is usually better for those who quit smoking early. The outcome is likely to be poor for those who have extensive lung damage; death occurs from respiratory failure or heart failure.

Complications:

• recurrent respiratory infections
• pulmonary hypertension
• cor pulmonale (enlargement and strain on the right side of the heart)

Call for an appointment with your health care provider if symptoms of emphysema develop.

If you have emphysema, call your health care provider if breathing difficulty is increased or other new symptoms develop.