Emphysema

Definition

Emphysema is a chronic respiratory disease in which overinflation of the alveoli or air sacs causes a decrease in respiratory function and often dyspnea.

Description

Emphysema is the most common cause of death from respiratory disease in the United States, and is the fourth most common cause of death overall. There are1.8 million Americans with the disease, which ranks fifteenth among chronic conditions that cause limitations of activity. Around 44% of those with emphysema state that their activities of daily living have been affected by the disease.

Normally functioning lungs are elastic, efficiently expanding and recoiling as air passes freely through the bronchus to the alveoli, where oxygen is moved into the blood and carbon dioxide is filtered out. When a person inhales cigarette smoke, his or her immune system responds by releasing substances that are meant to defend the lungs against the smoke. These substances can also attack the cells of the lungs. Normally, the body inhibits such action by releasing other substances. In smokers and those with the inherited emphysema defect, however, no such prevention occurs, and the lung tissue is damaged in such a way that it loses its elasticity. The small passageways leading to the alveoli collapse, trapping air within the alveoli. The alveoli, unable to recoil efficiently and move the air out, overexpand and rupture. The smaller areas of alveoli destruction are known as blebs and the larger ones are called bullae. As the disease progresses coughing and dyspnea occur. In the later stages the lungs cannot supply sufficient oxygen to the blood. Emphysema often occurs with other respiratory diseases, particularly chronic bronchitis. These two diseases are often referred to as one—chronic obstructive pulmonary disease (COPD).

Emphysema is most common among people aged 50 years and older. Those with inherited emphysema may experience the onset as early as their 30s or 40s. Men are more likely than women to develop emphysema, but female cases are increasing as the number of female smokers rises.

Causes and symptoms

Heavy cigarette smoking causes about 80–90% of all emphysema cases. However, a few cases are the result of an inherited deficiency of alpha-1-antitrypsin (AAT). The number of Americans with this deficiency is relatively small, probably no greater than 70,000. Pipe, cigar, and marijuana smoking can also damage the lungs. While a person may be less likely to inhale cigar and pipe smoke, these types of smoke can also impair lung function. Marijuana smoke is even more damaging because it is inhaled deeply and held in the lungs longer by the smoker.

The symptoms of emphysema develop gradually over many years. It is a common occurrence for many emphysema patients to have lost 50–70% of their functional lung tissue before they become aware that something is wrong. Dyspnea, a chronic mild cough (which may be productive of large amounts of dark, thick sputum, and often dismissed as "smoker's cough"), and sometimes weight loss are associated with emphysema. Initially, a patient may notice shortness of breath only when he or she is exercising. However, as the disease progresses, it will occur during less exertion, and ultimately with no exertion at all. Emphysema patients may also develop an enlarged, or "barrel," chest. Other symptoms may include skipped breaths, insomnia, morning headaches, nasal flaring, increased difficulties breathing while lying down, chronic fatigue, and swelling of the feet, ankles, or legs. Those with chronic emphysema are at risk for other complications resulting from weakened lung function. These include pneumonia, pulmonary hypertension, cor pulmonale, and chronic respiratory failure.

Diagnosis

A history of heavy smoking alone is not enough for a physician to differentiate emphysema from other respiratory diseases. A physician will combine information on symptoms, medical history, physical examination, lung function tests, and chest x ray results to make a diagnosis of emphysema. One of the first clues may be a hollow sound heard through a stethoscope as the patient's chest is being tapped. The hollow sound is the result of the enlargement or rupture of the lungs' alveoli.

A variety of pulmonary function tests may be ordered. In the early stages of emphysema, the only result may be dysfunction of the small airways. Patients with emphysema may show an increase in the total amount of air that is in the lungs (total lung capacity), but a decrease in vital capacity. With severe emphysema, vital capacity is substantially below normal. Spirometry, a procedure that measures respiratory gases and resulting pulmonary function, aids in the diagnosis of emphysema.

A chest x ray is often ordered to aid in the diagnosis of emphysema, though patients in the early stages of the disease may have normal findings. Abnormal findings on the chest x ray include excessive inflation of the lungs and an abnormally increased chest diameter. The diaphragm may appear depressed or flattened. In addition, patients with advanced emphysema may show an enlargement of the heart. The physician may observe blisters in the lungs and bulging of the accessory muscles of the respiratory system. Late in the disease an EKG will show signs of right ventricular failure in the heart and increased hemoglobin due to lower oxygen in the patient's blood.

Other tests that may be performed include peak flow measurements, arterial blood gases, and pulse oximetry.

Treatment

Treatment methods for emphysema do not cure or reverse the damage to the lungs. However, they can slow the progression of the disease, relieve symptoms, and help control possibly fatal complications. The first step in treatment for smokers is to quit smoking to prevent any further deterioration of breathing ability. Smoking cessation programs may be effective. Consistent encouragement, along with the help of health care professionals as well as family and friends, can help increase the quit rate.

If the patient and the health team develop and maintain a complete program of respiratory care, disability can be decreased, acute episodes of illness may be prevented, and the number of hospitalizations reduced. However, only smoking cessation has been shown to slow down the progression of the disease; and among all other treatments, only oxygen therapy has exhibited an increase in survival rate.

Home oxygen therapy may improve the survival times in those patients with advanced emphysema who also have hypoxemia, or low blood oxygen levels. It may improve the patient's tolerance of exercise, as well as improve their performance in certain aspects of brain function and muscle coordination. The functioning of the heart may also improve with an increased concentration of oxygen in the blood. Oxygen may also decrease insomnia and headaches. Some patients may receive oxygen only at night, but studies have illustrated that it is most effective when administered for at least 18, but preferably, 24 hours per day. Those patients just beginning the therapy may wish to postpone continuous oxygen administration until it becomes absolutely necessary because of inconvenience and decreased mobility. Portable oxygen tanks prescribed to patients carry a limited supply and must be refilled on a regular basis by a home health care provider. Medicare and most insurance companies cover a large proportion of the cost of home oxygen therapy. Patients should be instructed regarding special safety issues involving the transport and presence of oxygen in the home.

A variety of medications may be used in the treatment of emphysema. Usually the patient responds best to a combination of medicines rather than one single drug. Bronchodilators are sometimes used to help alleviate the patient's symptoms by relaxing and opening the airways. There are three primary categories of bronchodilators:

sympathomimetics (isoproterenol, metaproterenol, terbutaline, albuterol), which can be inhaled, taken by mouth, or injected; parasympathomimetics (atropine); and methylxanthines (theophylline), which may be administered intravenously, orally, or rectally.

Another category of medication often used is corticosteroids or steroids (beclomethasone, dexamethasone, triamcinolone, flunisolide). These help to decrease the inflammation of the airway walls. They are occasionally used if bronchodilators are ineffective in preventing airway obstruction. Some patients' lung function improves with corticosteroids, and inhaled steroids may be beneficial to patients with few side effects.

A variety of antibiotics are frequently given at the first sign of a respiratory infection, such as increased amounts of sputum or a change in the color of the sputum. Expectorants can help loosen respiratory secretions, enabling the patient to more easily expel them from the airways.

Many of the medications prescribed involve the use of a metered dose inhaler (MDI) that may require special instruction to be used correctly. MDIs are a convenient and safe method of delivering medication to the lungs. However, if they are used incorrectly the medication will not get to the right place. Proper technique is essential for the medication to be effective.

For some patients, surgical treatment may be the best option. Lung volume reduction surgery is a surgical procedure


KEY TERMS


Alveoli—Small cells or cavities. In the lungs, these are air sacs in which oxygen enters the blood and carbon dioxide is filtered out.

Arterial blood gases—A test to analyze blood for oxygen, carbon dioxide, and bicarbonate content, as well as blood pH. Used to test the effectiveness of respiration.

Cor pulmonale—A disease characterized by an increase in bulk of the right ventricle of the heart that can lead to heart failure.

Hypoxemia—A condition characterized by deficient oxygen supply in the blood.

Peak flow measurement—Measurement of the maximum rate of airflow attained during a forced vital capacity determination.

Pulmonary—Related to or associated with the lungs.

Pulse oximetry—The noninvasive monitoring or determination of oxygen-hemoglobin saturation of the blood.


in which the most diseased parts of the lung are removed to enable the remaining lung and breathing muscles to work more efficiently. Preliminary studies suggest improved survival rates and better functioning with the surgery. Another surgical procedure used for emphysema patients is lung transplantation. Transplantation may involve one or both lungs. However, it is a risky and expensive procedure and donor organs may not be available.

For those patients with advanced emphysema, keeping the air passages reasonably clear of secretions can prove difficult. Some common methods for mobilizing and removing secretions include:

  • Postural drainage. This technique helps to remove secretions from the airways. The patient lies in a position that allows gravity to aid in draining different parts of the lung. This is often done after the patient inhales and aerosol medication. The basic position involves the patient lying on the bed with chest and head over the side and forearms resting on the floor.
  • Chest percussion. This technique involves a caregiver lightly clapping the back and chest of the patient. It may help to loosen thick secretions.
  • Coughing and deep breathing. These techniques may aid the patient in bringing up secretions.
  • Aerosol treatments. These treatments may involve solutions of saline, often mixed with a bronchodilator, which are then inhaled as an aerosol. The aerosols thin and loosen secretions. A treatment normally takes 10–15 minutes and is given three or four times a day.

Patients with COPD can be instructed to perform a variety of self-help measures that can help improve their symptoms and ability to participate in activities of daily living. These measures include:

  • Avoiding any exposure to dust and fumes.
  • Avoiding air pollution, including secondhand cigarette smoke.
  • Avoiding other people who have infections like the cold or flu, and getting a pneumonia vaccination and a yearly flu shot.
  • Drinking plenty of fluids to help loosen respiratory secretions so they can be coughed up more easily.
  • Avoiding extreme heat or cold and high altitudes (special precautions can be taken that may enable the emphysema patient to fly on a plane).
  • Maintaining adequate nutritional intake; normally, a high-protein diet taken in many small feedings, is recommended.

Many patients are interested in whether any alternative treatments for emphysema are available. Some practitioners recommend supplements of antioxidant nutrients. There have also been some studies indicating a correlation between a low vitamin A status and COPD, with suggestions that supplements of vitamin A might be beneficial. Aromatherapists have used essential oils like eucalyptus, lavender, pine, and rosemary, to help relieve nasal congestion and make breathing easier. The herb elecampane may act as an expectorant to help patients clear mucus from the lungs. The patient should discuss these remedies with their health care practitioner prior to trying them, as some may interact with the more traditional treatments already being given.

Prognosis

Emphysema is a serious and chronic disease that cannot be reversed. If detected early effects and progression can be slowed, particularly if the patient ceases smoking immediately. Complications of emphysema include higher risks for pneumonia and acute bronchitis. Overall, the prognosis for patients with emphysema is poor, with a medical survival rate for all COPD patients of four years, and even less for emphysema patients. However, individual cases vary, and many patients can live much longer with supplemental oxygen and other treatment measures.

Health care team roles

Many members of the health care team may treat the patient with emphysema. The patient usually seeks help from a physician first, who will make the diagnosis. In the course of the diagnostic workup, x-ray technicians and respiratory therapists may treat the patient. The nurse plays an important role in assessing the patient, administering medications, in teaching the patient how best to cope with and understand the disease, and—in some cases—provides home care. The physical therapist may assist the patient to find ways of increasing their strength and activity tolerance.

Prevention

The best way to prevent emphysema is to avoid smoking. Even patients with inherited emphysema should avoid smoking, as it hastens onset and worsens severity of the disease. If patients quit smoking as soon as evidence of small airway obstruction begins, they can significantly improve their prognosis.

Resources

BOOKS

Beers, Mark H., and Robert Berkow. The Merck Manual of Diagnosis and Therapy, 17th ed. Whitehouse Station, NJ: Merck and Company, Inc., 1999.

PERIODICALS

"Data Mounting on Merits of Lung Volume Reduction Surgery." Family Practice News (February 15, 2001): 5.

Lewis, Laurie. "Optimal Treatment for COPD." Patient Care (May 30, 2000): 60.

ORGANIZATIONS

American Lung Association. 1740 Broadway, New York, NY 10019. (212) 315-8700. <http://www.lungusa.org>.

The National Emphysema Foundation. 15 Stevens St., Norwalk, CT 06856. <http://www.emphysemafoundation.org>.

National Heart, Lung and Blood Institute. <http://www.nhlbi.nih.gov>.

Deanna M. Swartout-Corbeil, R.N.