Emphysema

What is it?

Emphysema is a respiratory disease. In this condition, 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. They become unable to empty easily.

Emphysema is a progressive disease, which means it continues to get worse. As the condition progresses, the lungs lose their ability to absorb oxygen and release carbon dioxide. Breathing becomes more difficult. 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). They often occur together. Bronchitis is an inflammation and swelling of the bronchial walls. A person with chronic bronchitis typically has a daily cough with phlegm that lasts for months at a time over several years.

Smoking is responsible for the vast majority of cases of emphysema. Exposure to secondhand smoke and airborne toxins also can contribute to emphysema, though to a much lesser degree. Smokers exposed to high levels of air pollution appear to be at higher risk of developing COPD.

Both emphysema and chronic bronchitis are caused by damage to the lungs and bronchial tubes. When the damage is caused by smoking, symptoms may improve after a smoker quits.

A small number 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). AAT protects the lungs from damage by enzymes. When levels of AAT are low, the lungs are prone to being damaged by these enzymes. Smoking makes this condition worse.

Symptoms

During the early stages of emphysema, most people will have few symptoms. The disease usually progresses slowly. 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." This is 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. Another person with more advanced stages of the disease may be hardly bothered by symptoms.

Other symptoms caused by emphysema include:

• Wheezing
• Coughing
• 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

Diagnosis

Your doctor will ask for details about your smoking. He or she will ask 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
  • Non-smokers who developed emphysema

Your doctor also will ask about your respiratory symptoms. He or she will want to know if and when you develop shortness of breath. The doctor also may wish to ask about:

• Respiratory allergies
• Recurrent bad colds
• 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
• Looking at how your chest 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 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

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. These include:

• Enlargement of the lungs
• Scarring
• 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. CT scans may help to diagnose the disease in younger people or those who have never smoked.

Pulmonary function testing is useful both to diagnose emphysema and to determine the stage of the disease. This test is also known as spirometry. In this test you will blow forcefully through a tube. The tube is connected to a machine that measures your lung capacity.

Your doctor also may order specialized lung tests. These 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 in your blood. The blood is taken by a needle from a small artery in the wrist.
• Electrocardiogram (EKG). Looks for evidence of heart problems that may cause more shortness of breath than emphysema alone. An EKG also looks for heart strain caused by emphysema.

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

Expected duration

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

Prevention

If you smoke, stop. If you don't smoke, don't start. By quitting smoking you can either prevent emphysema or slow its progression.

You also should limit your exposure to air pollution. Restrict 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, consult with a specialist in occupational medicine.

If you have emphysema, ask your doctor about vaccinations against influenza (flu) and pneumococcal pneumonia. These vaccinations can help to prevent life-threatening respiratory infections in people with lung disease.

Treatment

No treatment can reverse or stop emphysema. But treatment can help to:

• Relieve symptoms
• Treat complications
• Minimize disability

Doctors' top treatment advice is to quit smoking. This is 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 AAT deficiency may be candidates for replacement therapy. This is done with infusions of natural AAT obtained from donors. This form of treatment appears effective. But it is time consuming and very expensive.

Your doctor may prescribe a number of different medications. These can help to relieve symptoms. Medications may include:

• Bronchodilators Inhalers.
  • Tiotropium (Spiriva)
  • Ipratropium (Atrovent)
  • Albuterol (Proventil, Ventolin, others)
  • Salmeterol (Serevent)
  • Formoterol (Foradil)

These medications are taken through hand-held inhalers or machine-driven nebulizers. These create a fine mist that can be inhaled.

Bronchodilators help to open the bronchial tubes in your lungs. By doing so, they reduce 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. They help control the inflammation of chronic bronchitis.

• Combination Corticosteroid/ Long-Acting Bronchodilator Inhalers.
  • Budesonide and formoterol (Symbicort)
  • Fluticasone and salmeterol (Advair)
  • Mometasone and formoterol (Dulera)

These combination products provide both anti-inflammatory action and airway opening in one inhaler.

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

Oxygen therapy increases 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 may be stored in metal cylinders. Or, it may be purified from air by an oxygen concentrator.

A number of lightweight, portable devices are available. They allow those who need oxygen to leave their homes for hours at a time.

Some people with emphysema need oxygen only at night.

Supplying oxygen at home is very expensive. As a result, most medical insurance companies have strict requirements to qualify for home oxygen.

People with emphysema also are at risk of becoming malnourished. It is important to see your doctor regularly about an appropriate diet. They are also at risk for developing psychological problems such as anxiety or depression. Counseling or medications can help.

Pulmonary rehabilitation is a form of physical therapy. It includes a structured exercise program combined with special breathing techniques, psychological support and education. It helps people with emphysema:

• Conserve energy
• Improve stamina
• Reduce breathlessness
• Have a better sense of well-being

Other treatments may be considered for people with advanced stages of emphysema.

• Lung volume reduction surgery. In this technique, parts of diseased lung are removed to improve the function of remaining, healthier lung.

• Lung transplant. A transplant may be considered in certain individuals with severe disease.

When to call a professional

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 can increase your likelihood of success compared to "going cold turkey." These include medications and counseling.

You also should see your doctor if anyone in your family has been diagnosed with AAT deficiency.

Prognosis

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 people with emphysema who continue to smoke, smoking dramatically increases the severity of the illness. It may reduce life span by 10 years or more.

Additional info

American Lung Association
https://www.lung.org/

National Heart, Lung, and Blood Institute (NHLBI)
https://www.nhlbi.nih.gov/