Pulmonary Emphysema, Alpha-1 Antitrypsin Deficiency Emphysema
What is Emphysema?
Emphysema is a type of chronic obstructive pulmonary disease (COPD) that primarily affects the air-exchanging spaces (alveoli) of the lungs. There is destruction of the walls of the alveoli, leading to enlarged irregularly shaped air spaces that are very inefficient in exchanging and absorbing oxygen. There is also a collapse of small airways on breathing out, resulting in airflow obstruction and air becomes trapped in the lungs. Emphysema is most often caused by chronic exposure to inhaled noxious gases; cigarette smoking is the number one cause. Of course, not all smokers develop emphysema, but there is no test that can predict which smokers are most likely to develop the disease.
Usually individuals with emphysema will also have some degree of chronic obstructive bronchitis or chronic asthmatic bronchitis. These three diseases combined represent the fourth leading cause of death in the US. Emphysema is generally diagnosed between the ages of 55 and 65. Emphysema caused by alpha-1 antitrypsin deficiency (an uncommon genetic defect) is usually diagnosed by age 40, without a history of smoking.
How is it diagnosed?
Emphysema signs and symptoms
- No symptoms in the early stages (often).
- Shortness of breath that increases in severity over several years.
- Occasional recurrent infections of the lungs or bronchial tubes.
History is of shortness of breath (dyspnea) that has worsened slowly over a long period of time, occurring with less and less exertion; in severe cases it may be present even at rest. Chronic cough, wheezing, and recurrent lung infections may also be mentioned.
Physical exam: Early in the disease the chest may appear normal, with only occasional wheezes or coarse breath sounds (rhonchi). As emphysema advances the breath sounds become very diminished. The individual may have labored breathing at rest or with minimal exertion.
Tests: Chest x-ray will reveal an expanded chest and a decrease in the normal lung markings. Large air spaces (blebs or bullae) may also be identified in advanced stages. The most accurate diagnosis of emphysema is with pulmonary function tests (PFTs) that measure the severity of airflow obstruction. Measurement of blood oxygen content is nearly normal, unless the individual is in respiratory failure. It is unusual for an individual to have pure emphysema without any clinical symptoms or pathology of asthma or bronchitis. Therefore, the treatment plan instituted for the individual with emphysema may appear similar to that of the individual with chronic obstructive bronchitis or chronic asthmatic bronchitis.
How is Emphysema treated?
It is most important for the individual to remove the inhaled irritants that cause or aggravate the disease, especially cigarette smoke. A heavy smoker will experience a steady decline in lung function. Bronchodilators are prescribed if the airway obstruction is partially reversible, as demonstrated during pulmonary function testing. Steroids may also be prescribed, if they are measurably helpful in decreasing airway obstruction. Supplemental oxygen therapy will be used if the blood oxygen levels are below normal while at rest.
Vaccination against pneumococcal pneumonia and influenza is recommended, due to the prolonged recovery time of individuals with emphysema from any type of lung infection. It is important that the individual be educated about his disease, how to conserve energy, how to avoid and recognize pulmonary infection, and how to breathe properly during exertion or severe dyspnea.
In certain individuals, surgical removal of large air spaces (bullae) may improve function of the remaining lung. Lung reduction surgery is a new treatment option. A lung transplant may be considered in severe cases. In individuals whose emphysema is caused by alpha 1-antitrypsin deficiency, intravenous administration of alpha-1 antitrypsin concentrate may slow down degeneration of lung function. Individuals with severe emphysema are cautioned to avoid high altitudes (over 4,000 feet) and to consider dry, pollution-free environments in which to live and work.
- Antibiotics to fight or prevent secondary infections.
- Bronchodilators to relax spasms of bronchial tubes.
- Arrange for immunizations against influenza and pneumonia.
- Oxygen in late stages.
Activity will be limited, but stay as active as your strength allows. Prolonged inactivity leads to increased disability.
Drink at least 8 glasses of fluid a day. This thins lung secretions so they can be coughed up more easily.
What might complicate it?
The occurrence of acute bronchitis, acute asthma, pneumonia or other lung disorder on top of the already compromised pulmonary system of the person with emphysema can lead to respiratory failure, requiring a mechanical ventilator until the acute illness passes; fatigue and exhaustion of the respiratory muscles is also a possibility. Right-sided heart failure (cor pulmonale) may also occur.
Emphysema is a chronic progressive disease that may ultimately lead to permanent disability. The signs of poor prognosis are increasing airway obstruction, a rapid heart rate (tachycardia) at rest, low blood oxygen levels, high blood carbon dioxide levels, and heart failure. Survival rates range from two to fifteen years depending on the degree of airway obstruction. Individuals living at high altitudes have a shorter survival in general.
Asthma and chronic bronchitis can coexist with emphysema. Other disorders that cause shortness of breath are a lung tumor, pulmonary embolus, pneumonia, heart failure, and occupational lung diseases such as silicosis.
Internist, pulmonologist and thoracic surgeon (for removal of large blebs, or lung reduction surgery).
Notify your physician if
You or a family member has symptoms of emphysema.
- The following occur after diagnosis:
- Blood in the sputum.
- Increased shortness of breath, or shortness of breath without coughing or when at rest.
- Chest pain.
- Sputum that increases, thickens or changes color, despite treatment.
Last updated 28 May 2012