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Emphysema is a type of chronic obstructive pulmonary disease (COPD) that primarily affects the air-exchanging spaces (alveoli) of the lungs. It destroys the walls of the alveoli, leading to enlarged, irregularly shaped air spaces that are very inefficient in absorbing oxygen into the blood and releasing carbon dioxide from the blood. The small airways also collapse on breathing out, resulting in airflow obstruction and air becoming trapped in the lungs.
Emphysema is most often caused by chronic exposure to inhaled noxious gases. Cigarette smoking is the primary 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.
Individuals with emphysema usually also have some degree of chronic obstructive bronchitis or chronic asthmatic bronchitis. In addition to smoking-related emphysema, there is also an inherited type of emphysema called alpha-1 antitrypsin (AAT) deficiency-related emphysema. This type of emphysema is caused by a gene mutation that results in a lack of the protective protein AAT. Under normal conditions, AAT shields the lungs from a natural enzyme called neutrophil elastase that helps fight bacteria and breaks down damaged lung tissue. Unless this enzyme is neutralized by AAT, it can eventually damage normal lung tissue. Since individuals with inherited AAT deficiency lack sufficient AAT to neutralize neutrophil elastase, enzyme-related damage can rapidly lead to emphysema. Smoking is believed to cause emphysema in part because tobacco smoke inactivates AAT.
Risk: Smoking is the prevalent risk factor for this disease and accounts for 80% to 90% of COPD, including emphysema (American Lung Association). Men older than age 40 who smoke are at highest risk of developing emphysema, although the rates for women increase each year (InteliHealth). Exposure to secondhand smoke and airborne toxins increase the chances of developing this disease.
Individuals born with a deficiency of alpha-1 antitrypsin (AAT) are at higher risk of developing emphysema. This mutation is most common in whites of northern European ancestry (InteliHealth). Incidence and Prevalence: There are an estimated 3 million cases of smoking-related emphysema diagnosed in the US. Approximately 50,000 to 100,000 individuals have AAT deficiency–related emphysema (American Lung Association). COPD (including emphysema and, to a lesser extent, asthmatic bronchitis) is the fourth leading cause of death in the US (Kleinschmidt). |
Source: Medical Disability Advisor
| History: The individual complains of shortness of breath (dyspnea) that has worsened slowly over a long period of time, occurring with progressively less exertion. In severe cases, dyspnea may be present even at rest. Chronic cough, wheezing, and recurrent lung infections may also be seen. Difficulty sleeping, fatigue, morning headaches, weight loss, swelling of the ankles, and lethargy may also be reported. Physical exam: A physical examination may show "pursed-lip" breathing, in that the individual exhales through minimally opened lips, which helps the individual to exhale more easily. Breath sounds are decreased, with minimal, if any wheezing, and prolonged exhalation (exhalation takes more than twice as long as inhalation). There may be an increased front-to-back diameter of the chest (barrel-shaped chest). There may be a bluish tint to the skin (cyanosis), which is a sign of insufficient oxygen levels in the blood. Early in the disease, the chest may appear normal with only slightly diminished breath sounds. As emphysema advances, the breath sounds become quite diminished, and exhalation becomes prolonged. The individual may have labored breathing at rest or with minimal exertion. Tests: A chest x-ray may reveal enlargement of the lungs, scarring, or the formation of bullae. CT scans may detect the disease earlier than other tests. Pulmonary function tests (PFTs), which include spirometry and measurement of lung volumes, may be helpful in measuring the severity of airflow obstruction. Blood tests may be done to measure the amount of oxygen and carbon dioxide in the blood. Other blood tests are used to check for low AAT levels, especially in a nonsmoker or young person who shows symptoms of emphysema. It is unusual for an individual to have pure emphysema without any clinical symptoms or pathology of asthma or bronchitis. An electrocardiogram (ECG) may be performed to discover signs of heart problems caused by emphysema. A small amount of mucus may be collected and tested for respiratory infection. An exercise stress test may be done to determine whether extra oxygen is necessary during exercise. |
Source: Medical Disability Advisor
| No treatment can reverse or stop the course of emphysema, but steps can be taken to relieve symptoms, treat complications, and minimize disability. The single-most critical factor for maintaining healthy lungs is the cessation of smoking. This is most effective at the early stages of emphysema. However, smoking cessation at any time can slow down the decline of lung function.
Bronchodilators are prescribed when the airway obstruction is partially reversible, as demonstrated during pulmonary function testing. Steroids may also be prescribed if they will measurably decrease airway obstruction. Supplemental oxygen therapy is used if the blood oxygen levels are below normal while at rest or during exercise or sleep.
Because individuals with emphysema take a long time to recover from any type of lung infection, vaccination against pneumococcal pneumonia and influenza is recommended. It is important that the individual be educated about the disease, how to conserve energy, how to avoid and recognize pulmonary infection, and how to breathe properly during exertion or severe dyspnea.
In individuals who meet certain criteria, lung volume reduction surgery may be a treatment option. This procedure involves removing diseased portions of the individual's lung to allow the remaining lung to function more effectively. A lung transplant may be considered in severe cases. In individuals whose emphysema is caused by alpha-1 antitrypsin deficiency, supplemental AAT may be administered by vein. AAT therapy is not appropriate for individuals who develop emphysema as a result of smoking or environmental factors.
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. They are also cautioned about air travel; special arrangements may be needed to obtain oxygen during the flight. |
Source: Medical Disability Advisor
| Survival rates depend on the degree of airway obstruction. Individuals who follow treatment instructions and adopt good health habits can enjoy a fairly normal lifestyle for a long time. Even individuals whose emphysema is severe (those with a significant decrease in airflow rates and increased levels of carbon dioxide in the blood) have a good chance of surviving for 5 years or more. Unfortunately, in those individuals with emphysema who continue to smoke, research indicates that smoking dramatically increases the severity of the illness and may reduce life span by 10 years or more. Living at high altitudes reduces the time an individual with emphysema will survive.
When a lung transplant is successful, the individual is expected to regain normal life activities. |
Source: Medical Disability Advisor
| Pulmonary rehabilitation combines exercise training and behavioral and educational programs designed to help individuals with emphysema control symptoms and improve day-to-day activities. It is a team approach. Individuals work closely with their doctors; nurses; respiratory, physical, and occupational therapists; psychologists; exercise specialists; and dietitians. The main goals of pulmonary rehabilitation are to help individuals improve their day-to-day lives and to restore their ability to function independently. Pulmonary rehabilitation can help reduce the number and length of hospital stays and increase the chances of living longer.
Pulmonary rehabilitation involves exercise training and ventilatory muscle training. It also includes psychosocial support and educational programs.
Since smoking is well known to be the primary risk factor for the onset and progression of emphysema, many pulmonary rehabilitation programs provide educational sessions and counseling to help individuals stop smoking. |
Source: Medical Disability Advisor
| Enlargement and strain on the right side of the heart (cor pulmonale) may occur, resulting in swelling of the feet and legs. Respiratory infections are frequent, and may often result in hospitalization. |
Source: Medical Disability Advisor
| Individuals need to stay away from inhaled irritants and extremes of air temperatures in the work environment. The amount of physical work they can do depends on their lung function. Individuals wearing continuous oxygen must work in areas where there is no danger of explosion from the gas or open flames or sparks. Individuals must not work in areas that require respirator use.
The ideal work environment for individuals with emphysema is a dry atmosphere, free of pollution. |
Source: Medical Disability Advisor
| If an individual fails to recover within the expected maximum duration period, the reader may wish to consider the following questions to better understand the specifics of an individual's medical case. Regarding diagnosis:
- How old is individual?
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Is individual a smoker?
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Does individual have alpha-1 antitrypsin (AAT) deficiency-related emphysema?
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Does individual have chronic obstructive bronchitis or chronic asthmatic bronchitis?
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Does individual complain of dyspnea that has worsened slowly over a long period of time and occurs with less and less exertion? Is it present at rest?
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Does individual report chronic cough, wheezing, and recurrent lung infections?
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On physical exam, does individual have decreased breath sounds and prolonged exhalation? Does individual have a barrel-shaped chest?
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Does individual show signs of chronic sleep deprivation or insufficient oxygen levels?
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Has individual had a chest x-ray, pulmonary function testing, arterial blood gases, and AAT levels, if appropriate?
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Were conditions with similar symptoms ruled out?
Regarding treatment:
- Is individual compliant with smoking cessation?
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Is individual being treated with bronchodilators, steroids, and oxygen therapy?
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If necessary, is individual receiving supplemental AAT?
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Has individual received vaccinations against pneumococcal pneumonia and influenza? Has individual been educated about the disease, how to conserve energy, how to avoid and recognize pulmonary infection, and how to breathe properly during exertion or severe dyspnea?
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Was surgery necessary?
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Does individual live and work in a dry, pollution-free, low-elevation environment?
Regarding prognosis:
- Is individual active in pulmonary rehabilitation? Is a home exercise program in place?
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Can individual's employer accommodate any necessary restrictions?
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Does individual have any conditions that may affect ability to recover?
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Have any complications developed such as cor pulmonale or recurrent respiratory infections?
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Source: Medical Disability Advisor
| "Emphysema." American Lung Association. Oct. 2003. 25 Oct. 2004 <http://www.lungusa.org/site/pp.asp?c=dvLUK9O0E&b=35043#whatis>. "Emphysema." InteliHealth. 26 Jul. 2003. 25 Oct. 2004 <http://www.intelihealth.com/IH/ihtIH/WSIHW000/9339/9885.html>. Kleinschmidt, Paul. "Chronic Obstructive Pulmonary Disease and Emphysema." eMedicine. Eds. David F. M. Brown, et al. 18 Oct. 2004. Medscape. 25 Oct. 2004 <http://emedicine.com/EMERG/topic99.htm>. |
Source: Medical Disability Advisor
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