Emphysema is a respiratory disease in which millions of the lungs' tiny air sacs (alveoli) stretch out of shape or rupture. As these thin, fragile air sacs become damaged or destroyed, the lungs lose their natural elasticity and are unable to empty easily. Emphysema is a progressive disease, which means it continues to get worse. As the condition progresses, the lungs also lose their ability to absorb oxygen and release carbon dioxide. Breathing becomes more difficult, and a person feels easily short of breath, like he or she is not getting enough air.

Emphysema and chronic bronchitis are the two most common forms of chronic obstructive pulmonary disease (COPD), and they often occur together. A person with chronic bronchitis (an inflammation and swelling of the bronchial walls) typically has a daily cough with phlegm that lasts for months at a time over several years. Both emphysema and chronic bronchitis are caused by damage to the lungs and bronchial tubes, and this damage is usually permanent. When the damage is caused by smoking, symptoms may improve after a smoker quits.

Smoking is responsible for up to 90% of cases of emphysema. Exposure to secondhand smoke and airborne toxins also can contribute to emphysema, although these factors are much less important than smoking. Smokers exposed to high levels of air pollution, including sulfur dioxide and particulates, appear to be at higher risk of developing COPD.

About 1% of people in the United States develop emphysema from an inherited disease known as alpha 1-antitrypsin deficiency. In this genetic condition, the body doesn't make enough of a protein called alpha 1-antitrypsin (AAT), which protects the lungs from damage by enzymes. When levels of AAT are low, the lungs are prone to being damaged by these enzymes. In its most severe form, emphysema can develop in people in their 30s or 40s. Because smoking interferes with the function of AAT, people with AAT deficiency who smoke develop more severe emphysema at an earlier age than those who don't smoke. Most people with AAT deficiency are Caucasians of northern European descent.

COPD is the most common cause of death from respiratory disease in the United States. Most people with emphysema are cigarette-smoking men older than age 40, who live in areas where pollution is a constant problem. However, because of the dramatic increase in smoking among women over the past few decades, the rate of emphysema in women continues to climb.

During the early stages of emphysema, most people will have few symptoms. The disease usually progresses slowly, and changes in breathing may be hardly noticed. A typical person will not experience symptoms until they have smoked a pack of cigarettes per day for more than 20 years.

However, over time, almost all people with emphysema will develop shortness of breath. At first, this may be noticed only during strenuous activities, such as climbing several flights of stairs or playing sports. As time goes on, the shortness of breath may occur with daily activities, such as housework or walking short distances. Eventually, the person may be short of breath for much of the day, even while at rest or sleeping. At its worst, emphysema can cause "air hunger," the constant feeling of being unable to catch one's breath.

These respiratory symptoms are the same regardless of the cause of the emphysema. However, two people with the same degree of lung damage may have different symptoms. One person with mild emphysema may feel very short of breath, while another person with more advanced stages of the disease may be hardly bothered by symptoms. These differences might result from other medical conditions and how well a person is able to treat and condition their lungs.

Other symptoms caused by emphysema include:

• Wheezing, coughing or bringing up phlegm (if chronic bronchitis also is present)
• Tightness feeling in the chest
• Barrel-like distended chest
• Constant fatigue
• Difficulty sleeping
• Morning headaches
• Weight loss
• Swelling of the ankles
• Lethargy or difficulty concentrating

Your doctor will ask for details about your smoking (how long you've smoked, and how many cigarettes per day). Other questions may include:

• Do you breathe passive (secondhand) smoke at work or at home?
• Do you live or work in an area where you are exposed to airborne irritants or noxious materials?
• Do you live in an area with significant air pollution?
• Is there a family history of AAT deficiency, early onset of emphysema or non-smokers who developed emphysema?

Your doctor also will ask about your respiratory symptoms, particularly if and when you develop shortness of breath. He or she also may wish to ask about respiratory allergies, recurrent bad colds or a persistent, heavy cough.

Your doctor then will examine you to look for typical signs of emphysema. This may include:

• Watching for shortness of breath when you perform simple activities, such as walking into the exam room
• Looking at the size and shape of your chest as well as how it moves when you breathe.
• Listening to your lungs for wheezing or loss of the normal breath sounds
• Checking your ears, nose and throat for reasons why you may be coughing
• Listening to your heart
• Checking your skin, lips and fingernails for a bluish tint that indicates low blood oxygen levels (your doctor also may directly measure your blood oxygen level with a special finger probe known as an oximeter)
• Checking your fingernails for an unusual curvature ("clubbing") that sometimes occurs with chronic lung disease
• Feeling your ankles for swelling that indicates fluid accumulation

It is important to keep in mind that the results of this examination may be totally normal in many people in the earliest stages of emphysema.

In most people, emphysema will be diagnosed by X-ray or lung-function tests. A regular chest X-ray may show typical changes of emphysema including enlargement of the lungs, scarring or the formation of holes (bullae). However, these changes may not appear until significant damage has occurred. Computed tomography (CT) scans are better for detecting the earliest changes of emphysema, and may help to diagnose the disease in younger people or those who have never smoked.

Pulmonary function testing (also known as spirometry) is useful both to diagnose emphysema and to determine the stage of the disease. In this test you will blow forcefully through a tube that is connected to a machine designed to measure your lung capacity. Your doctor also may order specialized lung tests that may require you to sit inside a glass box, or slowly breathe in a mixture of different gases.

Other tests your doctor may order include:

• Arterial blood gases — Measures the levels of oxygen and carbon dioxide from blood taken by a needle from a small artery in the wrist.
• Electrocardiogram (EKG) — Looks for evidence of heart problems that may cause breathing trouble or heart strain caused by emphysema

If suspected, your doctor can order a specific blood test to confirm the diagnosis of alpha-1 antitrypsin deficiency. If this test is positive, your doctor may recommend screening for your entire family.

Regardless of the cause, lung damage in emphysema cannot be reversed. If the disease is not treated, damage and symptoms of emphysema will continue to get worse. If treated, the symptoms of emphysema can improve.

If you smoke, stop. If you don't smoke, don't start. Studies have shown that by quitting smoking you can either prevent emphysema or slow its progression. You also can limit your exposure to air pollution by restricting your outdoor activity when there are reports of high smog levels. People exposed to harmful chemicals at work should speak to their employers about respirator masks or should consult with a specialist in occupational medicine.

People diagnosed with emphysema should ask their doctors about vaccinations against influenza (flu) and pneumococcal pneumonia. These vaccinations can help to prevent life-threatening respiratory infections in people with lung disease.

No treatment can reverse or stop emphysema, but treatment can help to relieve symptoms, treat complications and minimize disability. Topping the list of treatment advice from a doctor is to quit smoking, the single most important factor for maintaining healthy lungs. Stopping smoking is most effective at the early stages of emphysema, but it also can slow the loss of lung function in later stages of the disease.

People with alpha-1 antitrypsin deficiency may be candidates for replacement therapy with infusions of natural AAT obtained from donors. While this form of treatment appears effective, it is time consuming and very expensive. Genetically engineered forms and an inhaled form are under development.

Your doctor may prescribe a number of different medications to relieve the symptoms of chronic bronchitis that often accompanies emphysema. These may include:

• Bronchodilators — Including tiotropium (Spiriva), ipratropium (Atrovent), albuterol (Proventil, Ventolin and other brand names) and salmeterol (Serevent). These medications are taken through hand-held inhalers or machine-driven nebulizers, which create a fine mist that can be inhaled. These medicines are called bronchodilators, and they help to open the bronchial tubes in your lungs and reduce the shortness of breath, wheezing and cough. Theophylline (sold under several brand names) is a pill form of a bronchodilator. Because it can interact with medications and cause side effects, it is used less often than the inhaler medications.
• Corticosteroids — These medications help to reduce inflammation in the lungs. During an acute flare-up of symptoms, they often are given in pill form or by injection. Inhaled corticosteroids or pills may be prescribed for daily use to help control the inflammation of chronic bronchitis.



• Antibiotics — These typically are used for acute flare-ups of COPD triggered by respiratory infections.

Oxygen therapy has been proven to increase life expectancy in people with emphysema who have below-normal levels of oxygen in the blood. Oxygen usually is given through a plastic tube (nasal cannula) worn under the nostrils. The oxygen either is stored in metal cylinders, or is purified from air by an electrical machine (an oxygen concentrator). A number of lightweight, portable devices are now available that allow those who need oxygen to leave their homes for hours at a time. Some people with emphysema need oxygen only at night. Because supplying oxygen at home is very expensive, most medical insurance companies have strict requirements to qualify for home oxygen.

People with emphysema also are at risk of becoming malnourished, and developing psychological problems such as anxiety or depression. Therefore, it is important to see your doctor regularly about an appropriate diet, as well as counseling or medications to help with psychological issues.

A number of other treatments are available for people with advanced stages of emphysema.

• Pulmonary rehabilitation — This is a form of physical therapy that teaches patients with emphysema to conserve energy, improve stamina and reduce breathlessness.
• Lung volume reduction surgery — In this controversial technique, parts of diseased lung are removed to improve the function of remaining, healthier lung.
• Lung transplant — A transplant usually is considered only in people whose emphysema is so severe that life expectancy is felt to be less than two to three years.

Call your doctor if you develop:

• New shortness of breath
• A persistent cough, with or without phlegm
• A decrease in your usual ability to exercise
• Frequent respiratory infections

If you smoke, see your doctor about ways to quit. Several different types of treatment (including medications and counseling) can double your likelihood of success compared to "going cold turkey."

You also should see your doctor if anyone in your family has been diagnosed with alpha-1 antitrypsin deficiency.

There is no cure for emphysema, but the condition can be controlled. People with mild emphysema who quit smoking have a normal life expectancy. Those who adopt good health habits can enjoy a fairly normal lifestyle for a long time. Even people whose emphysema is severe have a good chance of surviving for five years or more.

In those people with emphysema who continue to smoke, research indicates that smoking dramatically increases the severity of the illness and may reduce their life span by 10 years or more.

American Lung Association
61 Broadway, 6th Floor
New York, NY 10006
Phone: (212) 315-8700
Toll-Free: (800) 548-8252E-Mail: info@lungusa.org
http://www.lungusa.org/

National Heart, Lung, and Blood Institute (NHLBI)
P.O. Box 30105
Bethesda, MD 20824-0105
Phone: (301) 592-8573
TTY: (240) 629-3255
Fax: (301) 592-8563
E-Mail: nhlbiinfo@rover.nhlbi.nih.gov
http://www.nhlbi.nih.gov/