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Chronic Respiratory Diseases

CHRONIC RESPIRATORY DISEASES

Chronic respiratory diseases include disorders that affect any part of the respiratory system, not only the lung but also the upper airway (nose, mouth, pharynx, larynx, and trachea), the chest wall and diaphragm, and the neuromuscular system that provides the power for breathing. Prolonged (chronic) diseases and disorders of the respiratory system in adults are those conditions that are present for months to years, and are treatable but generally not curable. Successful medical management of any chronic respiratory disease depends upon evaluating the patient as a whole and assessing the structure and function of the entire respiratory system.

The chronic diseases of the respiratory system collectively result in profound human suffering, mortality, and economic loss. For example, an estimated 163,000 Americans will die of cancer of the respiratory system in 2001. Chronic obstructive pulmonary disease (COPD) is the fourth leading cause of death in the United States (behind heart diseases, cancer, and stroke) and now kills about 113,000 Americans annually. The death rate from COPD rose 44.5 percent between 1979 and 1997, an increase that was the highest among the top ten causes of death in the United States. Experts estimate that about 30 million Americans have COPD, and only about half of these have been evaluated and diagnosed. The total economic impact of COPD in the country is estimated to be about $31.9 billion annually.

The huge impact of lung cancer, COPD, and other chronic respiratory diseases in our society is especially sobering in light of the fact that many of these conditions are preventable. The use of tobacco is the leading cause of preventable illness and death in the United States, accounting for about 430,000 deaths, or about 20 percent of all deaths, annually. Cigarette smoking is the primary risk factor for the development of COPD and lung cancer. About 25 percent of all Americans smoke cigarettes, and, tragically, 3,000 young Americans take up the smoking habit every day, greatly increasing their risk of dying prematurely from COPD, lung cancer, heart disease, or some other smoking-related disease. It is alarming that the percentage of high school and college students who smoke cigarettes increased during the early 1990s.

There are relatively few symptoms of respiratory disease, whether it be acute or chronic, as a diseased respiratory system has a limited number of clinical expressions. These include shortness of breath (dyspnea); cough, with or without phlegm (sputum) production; high-pitched continuous

Figure 1

breathing noise (wheezing); chest tightness; coughing up blood (hemoptysis); and chest pain. Uncommonly, patients with chronic respiratory disease are free of symptoms (asymptomatic) but have a disease process that is discovered incidentally, such as by a routine chest X-ray. Respiratory symptoms may be the early warning sign of chronic respiratory disease, but, unfortunately, they are commonly ignored, dismissed as being normal, or mistakenly attributed to aging or alternative disorders.

The medical evaluation of a patient with a suspected chronic respiratory disease starts with the physician's taking a detailed medical history, with particular attention to the symptoms listed above and their timing (see Figure 1). A thorough history of respiratory illness always includes attention to relevant factors such as tobacco use, occupational and environmental exposure to noxious respiratory agents, travel, hobbies, immunizations, family medical history, current and prior medications, general medical health, and comorbid conditions, to name just a few relevant components. The medical history is followed by a physical examination, which must be complete in order to detect both pulmonary and nonpulmonary clues to the presence of a disease process. It is important to emphasize that pulmonary disorders, whether acute or chronic, may originate in the lung or secondarily involve the lung after originating in another part of the body. Diagnostic testing supplements the medical history and physical examination. A wide range of diagnostic tests is now available to help the physician diagnose a specific respiratory system disease correctly.

There are hundreds of different chronic respiratory diseases. Table 1 provides an outline of their major headings and a few important examples of each category. The list is not intended to be complete, and the reader is referred to medical textbooks or electronic sources for a more complete listing. The remainder of this section will address briefly a few of the most important chronic respiratory diseases.

COPD. Chronic obstructive pulmonary disease is the most important and common of the chronic respiratory diseases. Remarkably, few Americans know what COPD is. This condition has been defined as "a disease state characterized by the presence of airflow obstruction due to chronic bronchitis or emphysema; the airflow obstruction is generally progressive, may be accompanied by airway hyperreactivity, and may be partially reversible" (American Thoracic Society, 1995). Most patients with COPD have smoked at least one pack of cigarettes daily for twenty years or longer. By their fifth or sixth decade of life they suffer from dyspnea, productive cough (often worse in the morning), difficulty in clearing sputum from the airways, wheezing, or any combination of these symptoms. Slowly and gradually the symptoms progress year by year and are often mistakenly attributed to increasing age or to another disease such as asthma. Initially, the dyspnea occurs only with extreme exertion, but eventually, in severe cases, it limits simple activities such as changing clothes, raising the arms above the head, bending over, or taking a shower. The diagnosis of COPD may be made by the medical history, physical examination, pulmonary function tests, and a chest X-ray, after exclusion of other conditions such as asthma, bronchiectasis, lung cancer, and congestive heart failur.

Essential steps in managing COPD are outlined in Table 2. Smoking cessation slows the agerelated rate of loss of pulmonary function in middle-aged smokers with mild COPD. In COPD patients with low blood oxygen levels (hypoxemia), continuous oxygen therapy prolongs survival. All other treatment approaches are aimed at improving symptoms. No treatment intervention restores lung function to normal. Intense rehabilitation of patients with severe COPD provides temporary improvement in symptoms. Surgical treatment options are limited to a very small fraction of COPD patients and outcome benefits are the focus of ongoing research. Without treatment, COPD pursues a downhill course leading to premature disability and death.

Cancer. Cancer represents another broad category of chronic respiratory disease. Cancer may affect any part of the respiratory tract, including the larynx. Cancer that involves the lung is called "primary" if it originates in the lung and "secondary" if it spreads to the lung from another site. Primary lung cancer usually develops from the epithelial lining of the bronchi (bronchogenic carcinoma). Rarely it originates from the lung's soft tissues or the outer lining on the lung's surface (malignant mesothelioma). The layman's term "lung cancer" usually refers to bronchogenic carcinoma, a deadly chronic disease that in about 90 percent of cases is caused by cigarette smoking.

Patients with bronchogenic carcinoma have a wide variety of initial clinical manifestations, but a typical presentation is a new or changing respiratory symptom in combination with an abnormal chest X-ray. Loss of appetite and weight loss are common. Unfortunately, by the time most patients (about 75%) seek medical attention, the lung cancer cannot be entirely removed by surgery (unresectable) because it has spread in the chest or elsewhere in the body (the stage of the cancer is advanced). Or, the patient may be too ill to tolerate chest surgery because of the systemic effects of

Table 1

Selected Chronic Pulmonary Diseases
source: Courtesy of author.
Diseases of the airway
Chronic obstructive pulmonary disease (COPD)
Asthma, i.e., chronic bronchial asthma, factitious asthma
Bronchiectasis
Cystic fibrosis
Bronchiolitis
Miscellaneous, i.e., tracheal and bronchial obstruction, chronic aspiration, atelectasis secondary to airway obstruction
Lung cancer
Primary lung cancers
Bronchogenic carcinoma, i.e., bronchial carcinoid tumors, mesothelioma
Secondary lung cancer
Infiltrative diseases
Interstitial, i.e., idiopathic pulmonary fibrosis, interstitial pneumonitis
Alveolar, i.e., pulmonary alveolar proteinosis, alveolar hemorrhage
Disorders of the control of breathing, i.e., obstructive sleep apnea central sleep apnea
Infectious lung diseases, i.e., lung abscess, tuberculosis
Pleural diseases , i.e., chronic pleural effusion, pleural fibrosis
Chest wall and diaphragm diseases , i.e., kyphoscoliosis, ankylosing spondylitis
Mediastinal diseases , i.e., mediastinal tumors, mediastinal fibrosis
Neuromuscular diseases
Neurologic and neuromuscular transmission disorders, i.e., Guillain-Barré syndrome (acute idiopathic polyneuropathy,poliomyelitis)
Muscular, i.e., polymyositis and dermatomyositis, muscular dystrophies
Pulmonary vascular diseases , i.e., pulmonary thromboembolism, pulmonary hypertension
Occupational lung diseases , i.e., occupational asthma, pneumoconiosis, chronic hypersensitivity pneumonitis
Iatrogenic diseases , i.e., drug-induced lung disease, radiation-induced lung disease
Chronic respiratory failure

the cancer or another smoking-related condition such as COPD or heart disease. Anticancer drug therapy (chemotherapy), radiation therapy, and other treatment methods may provide temporary improvement in the size of the cancer (remission), diminution of symptoms (palliation), or slightly improved survival. Nevertheless, cure of lung cancer by nonsurgical approaches is rare, and most patients with unresected lung cancer die prematurely from the disease. Overall, only 14 percent of patients with lung cancer survive for five years.

Infiltrative Lung Disease. Another broad category of chronic respiratory disease is infiltrative lung disease, in which shadows (infiltrates) appear in the lung tissue on the standard chest X-ray. Infiltrates are caused by the accumulation of cells or fluids in parts of the lung in excess of their normal amount. Infiltrates may be localized (focal) or widespread (diffuseinvolving all five lobes of the lung). They may involve the lung's tissue framework ("interstitial" infiltrate), airspace ("alveolar" infiltrate), or both. Well over one hundred specific lung diseases fall into the category of infiltrative disease, which may be acute or chronic; many of them are occupational in origin. The chronic infiltrative diseases share many similar findings, among which are unrelenting dyspnea (sometimes with a dry cough); abnormal lung sounds (crackles) on chest physical examination; reduced amount of air in the lung (restrictive dysfunction) and reduced gas transfer (diffusing capacity) on pulmonary function testing; and hypoxemia, especially with exercise.

Idiopathic Pulmonary Fibrosis (IPF). A lung scarring of unknown cause, IPF is the prototype of the chronic infiltrative pulmonary diseases, just as COPD is the prototype of the chronic airway diseases. IPF affects men and women between the ages of fifty and seventy. The prevalence of IPF has been estimated to be three to six cases per 100,000 people. Although the exact cause of this disease is not known, cigarette smoking is a suspected risk factor. Progressive dyspnea, dry cough, and crackles on physical examination are typical clinical features of this disease. Patients with suspected IPF are often evaluated with high-resolution computed tomography (HRCT) imaging, which displays patchy scarring below the lung surface, especially in the lower lung zones. Biopsy of lung tissue via the airway (flexible fiberoptic bronchoscopy)

Table 2

Comprehensive Medical Management of COPD
source: courtesy of author.
  • Establishing a sound relationship with a medical health care provider and medical institution
  • Patient education about COPD
  • Drug therapy
    Bronchodilators, i.e., anticholinergic drugs
    Anti-inflammatory agents, i.e., corticosteroids
    Antibioticsonly for active airway infection (e.g., acute exacerbation of COPD)
  • Smoking cessation
    Reduces the rate of decline in pulmonary function
  • Immunizationpreventive therapy for selected adult patients particulary annual influenza vaccine and vaccination against infection by Streptococcus pneumoniae every five years
  • Physical therapy measures, e.g., general aerobic conditioning, postural drainage with or without chest percussion
  • Supplemental oxygen
    Generally for patients whose partial pressure of oxygen in arterial blood is low
    Benefits
    Improved quality of life, improved survival, reduced hospitalization needs
  • Psychological and social support
  • Pulmonary rehabilitation
    Maintaining a healthy lifestyle
    Benefits
    Improvement in dyspnea, health-related quality of life, and walking distance
    Reduced hospitalization needs
  • Nutrition especially adequate protein and calorie intake daily and maintenance of ideal body weight
  • Home care and assistance with activities of daily living
  • Surgical options for a few selected patients, e.g., bullectomy
  • Care at the end of life including treatment of respiratory failure and following advance medical directives

or via the chest wall (video-assisted thoracic surgery) is often employed to confirm the clinical impression. Treatment of this condition is mainly supportive, as the scarring itself is not reversible. Continuous supplemental oxygen is helpful in reducing dyspnea. Anti-inflammatory therapy with corticosteroids or immunosuppressive agents is frequently attempted, but results are usually disappointing. Treatment with interferon has been investigated. Lung transplantation is an option for a few patients with IPF. Most patients with IPF have a poor prognosis, and median survival is about five years.

Ventilatory Control Disorders. The rate, depth, and rhythm of normal breathing are exquisitely controlled by a complex interplay of regulatory mechanisms in the brain, the respiratory system, the great blood vessels, and other parts of the body. Disturbances in any of these mechanisms may lead to altered breathing (ventilatory) control, sometimes with disastrous consequences. A number of chronic respiratory disorders may be attributed to abnormal control of ventilation, including sleep apnea and obesity-hypoventilation syndrome.

The most common and important disorder of ventilatory control is sleep apnea syndrome. An apnea is defined as cessation of airflow at the nose and mouth for more than ten seconds, and an hypopnea is a drop of oxyhemoglobin saturation of more than 4 percent with reduced air-flow. Obstructive apneas occur because of temporary closure of the throat (pharynx) and central apneas occur because of a transient reduction in breathing effort. The consequences of apneas and hypopneas during sleep are nocturnal hypoxemia and poor sleep quality. Sleep apnea is confirmed by performing recordings of physiological variables during sleep (polysomnography). An excessive number of apneas and hypopneas during a night of sleep defines sleep apnea. Sleep apnea syndrome is present when sleep apnea is accompanied by associated symptoms, including loud and cyclical snoring, excessive daytime sleepiness and daytime sleep attacks, morning sluggishness, daytime fatigue or tiredness, neuropsychological impairment, and declines in personality.

Obstructive sleep apnea is very prevalent, being found in 24 percent of middle-aged men and 9 percent of middle-aged women. Obstructive sleep apnea syndrome (OSAS) occurs in 4 percent of men and 2 percent of women. Overweight middle-aged and older men are most commonly affected. Hypoxemia during sleep and sleep disruption may be so severe as to cause daytime problems, particularly excessive and inappropriate sleepiness. Patients with OSAS may fall asleep while driving a car, operating machinery, or performing a job. Substantial psychosocial consequences may follow, including personality changes, marital stress, and loss of employment. The medical consequences of OSAS include high blood pressure in the lung (pulmonary hypertension), and in the body as a whole (systemic hypertension); failure of the right side of the heart; heart rhythm disturbances; and cardiovascular complications (stroke, myocardial infarction, and sudden death).

Treatment of OSAS consists of general measures such as patient education, weight loss, avoidance of alcohol and hypnotic medication, surgical relief of mechanical upper airway obstruction, and improved sleep habits. Many patients with OSAS are treated with nocturnal use of nasal continuous positive airway pressure (nasal CPAP) masks that stent the airway open during sleep, precluding pharyngeal obstruction. Dramatic improvement in symptoms is commonly observed. Results of treatment of central sleep apnea syndrome are less encouraging.

Obesity hypoventilation ("Pickwickian") syndrome is a disorder of ventilatory control in patients with moderate to severe obesity. This condition is thought to be caused by blunted breathing effort (ventilatory drive) and the mechanical load placed on the chest wall and abdomen by obesity. This disorder occurs in only a small percent of patients with obesity. The daytime hypoxemia and elevated blood carbon dioxide levels (hypercapnia) found in this condition may improve with significant weight loss. Patients with this condition also suffer from OSAS.

In conclusion, chronic respiratory diseases are very prevalent in our society. They cause untold suffering, premature death, and economic harm to patients, their families, and the nation. Chronic obstructive pulmonary disease, lung cancer, idiopathic pulmonary fibrosis, and obstructive sleep apnea syndrome are common examples of these chronic respiratory diseases. Treatment for these conditions is difficult and expensive, but successful management provides substantial symptomatic benefit for most patients. Many of the chronic respiratory diseases could be prevented by elimination of cigarette smoking.

John L. Stauffer

(see also: Asthma; Bronchitis; Lung Cancer; Occupational Lung Disease; Pulmonary Function; Smoking Behavior; Smoking Cessation; Tobacco Control )

Bibliography

American Thoracic Society (1995). "Standards for the Diagnosis and Care of Patients with Chronic Obstructive Pulmonary Disease." American Journal of Respiratory and Critical Care Medicine 152 (5):S77 S120.

(2000). "Idiopathic Pulmonary Fibrosis: Diagnosis and Treatment. International Consensus Statement." American Journal of Respiratory and Critical Care Medicine 161 (2):646664.

Centers for Disease Control and Prevention (1998). "Tobacco Use Among High School StudentsUnited States, 1997." Morbidity and Mortality Weekly Report 47 (12):229233.

(1999). "Mortality PatternsUnited States, 1997." Morbidity and Mortality Weekly Report 48 (30):664668.

Chesnutt, M. S., and Prendergast, T. J. (2001). "Lung." In Current Medical Diagnosis & Treatment 2001, 40th edition, ed. L. M. Tierney et al. Stamford, CT: Lange Medical Books/McGraw-Hill.

Fiore, M. C.; Bailey, W.C.; Cohen, S. J. et al. (2000). Treating Tobacco Use and Dependence: A Quick Reference Guide for Clinicians. Rockville, MD: U.S. Department of Health and Human Services.

Greenlee, R. T.; Hill-Harmon, M. B.; Murray, T.; and Thun, M. (2001). "Cancer Statistics, 2001." CA. A Cancer Journal for Clinicians 51 (1):1536.

Hoffman, P. C.; Mauer, A.M.; and Vokes, E. E. (2000). "Lung Cancer." Lancet 355 (9202):479485.

McGinnis, J. M., and Foege, W. H. (1993). "Actual Causes of Death in the United States." Journal of the American Medical Association 270 (18):22072212.

National COPD Awareness Panel (2000). "Guidelines for Early Detection and Management of COPD." The Journal of Respiratory Diseases 21 (9):S5S21.

Petty, T. L. (1947). "A New National Strategy for COPD." The Journal of Respiratory Diseases 18 (4):365369.

Pierce, J. P.; Fiore, M. C.; Novotny, T. E.; Hatziandreu, E.; and Davis, R. M. (2000). "Trends in Cigarette Smoking in the United States. Projections to the Year 2000." Journal of the American Medical Association 261 (1):6165.

Stauffer, J. L., and Reynolds, H. Y. (1998). "Approach to the Patient with Respiratory System Disease." In Internal Medicine, 5th edition, ed. J. H. Stein et al. St. Louis, MO: Mosby.

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Wechsler, H.; Rigotti, N. A.; Gledhill-Hoyt, J.; and Lee, H. (1998). "Increased Levels of Cigarette Use Among College Students. A Cause for National Concern." Journal of the American Medical Association 280 (19): 16731678.

Young, T.; Palta, M.; Dempsey, J.; Skatrud, J.; Weber, S.; and Badr, S. (1993). "The Occurrence of Sleep-disordered Breathing Among Middle-aged Adults." New England Journal of Medicine 328 (17):12301235.

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Chronic Obstructive Lung Disease

Chronic Obstructive Lung Disease

Definition

Chronic obstructive lung disease, also known as chronic obstructive pulmonary disease (COPD), is a general term for a group of conditions in which there is persistent difficulty in expelling (or exhaling) air from the lungs. COPD commonly refers to two related, progressive diseases of the respiratory system, chronic bronchitis and emphysema. Because smoking is the major cause of both diseases, chronic bronchitis and emphysema often occur together in the same patient.

Description

COPD is one of the fastest-growing health problems. Nearly 16 million people in the United States, 14 million with chronic bronchitis and two million with emphysema, suffer from COPD. COPD is responsible for more than 96,000 deaths annually, making it the fourth leading cause of death. Although COPD is more common in men than women, the increase in incidence of smoking among women since World War II has produced an increase in deaths from COPD in women. COPD has a large economic impact on the healthcare system and a destructive impact on the lives of patients and their families. Quality of life for a person with COPD decreases as the disease progresses.

Chronic bronchitis

In chronic bronchitis, chronic inflammation caused by cigarette smoking results in a narrowing of the openings in the bronchi, the large air tubes of the respiratory system, and interferes with the flow of air. Inflammation also causes the glands that line the bronchi to produce excessive amounts of mucus, further narrowing the airways and blocking airflow. The result is often a chronic cough that produces sputum (mainly mucus) and shortness of breath. Cigarette smoke also damages the cilia, small hair-like projections that move bacteria and foreign particles out of the lungs, increasing the risk of infections.

Emphysema

Emphysema is a disease in which cigarette smoke causes an overproduction of the enzyme elastase, one of the immune system's infection-fighting biochemicals. This results in irreversible destruction of a protein in the lung called elastin which is important for maintaining the structure of the walls of the alveoli, the terminal small air sacs of the respiratory system. As the walls of the alveoli rupture, the number of alveoli is reduced and many of those remaining are enlarged, making the lungs of the patient with emphysema less elastic and overinflated. Due to the higher pressure inside the chest that must be developed to force air out of the less-elastic lungs, the bronchioles, small air tubes of the respiratory system, tend to collapse during exhalation. Stale air gets trapped in the air sacs and fresh air cannot be brought in.

Causes and symptoms

There are several important risk factors for COPD:

  • Lifestyle. Cigarette smoking is by far the most important risk factor for COPD (80% of all cases). Cigar and pipe smoking can also cause COPD. Air pollution and industrial dusts and fumes are other important risk factors.
  • Age. Chronic bronchitis is more common in people over 40 years old; emphysema occurs more often in people 65 years of age and older.
  • Socioeconomic class. COPD-related deaths are about twice as high among unskilled and semi-skilled laborers as among professionals.
  • Family clustering. It is thought that heredity predisposes people in certain families to the development of COPD when other causes, such as smoking and air pollution, are present.
  • Lung infections. Lung infections make all forms of COPD worse.

In the general population, emphysema usually develops in older individuals with a long smoking history. However, there is also a form of emphysema that runs in families. People with this type of emphysema have a hereditary deficiency of a blood component, an enzyme inhibitor called alpha-1-antitrypsin (AAT). This type of emphysema is sometimes called "early onset emphysema" because it can appear when a person is as young as 30 or 40 years old. It is estimated that there are between 75,000 and 150,000 Americans who were born with AAT-deficiency. Of this group, emphysema afflicts an estimated 20,000-40,000 people (1-3% of all cases of emphysema). The risk of developing emphysema for an AAT-deficient individual who also smokes is much greater than for others.

The first symptoms of chronic bronchitis are cough and mucus production. These symptoms resemble a chest cold that lingers on for weeks. Later, shortness of breath develops. Cough, sputum production, and shortness of breath may become worse if a person develops a lung infection. A person with chronic bronchitis may later develop emphysema as well. In emphysema, shortness of breath on exertion is the predominant early symptom. Coughing is usually minor and there is little sputum. As the disease progresses, the shortness of breath occurs with less exertion, and eventually may be present even when at rest. At this point, a sputum-producing cough may also occur. Either chronic bronchitis or emphysema may lead to respiratory failurea condition in which there occurs a dangerously low level of oxygen or a serious excess of carbon dioxide in the blood.

Diagnosis

The first step in diagnosing COPD is a good medical evaluation, including a medical history and a physical examination of the chest using a stethoscope. In addition, the doctor may request one or more of the following tests:

Pulmonary function test

Using a spirometer, an instrument that measures the air taken into and exhaled from the lungs, the doctor will determine two important values: (1) vital capacity (VC), the largest amount of air expelled after the deepest inhalation, and (2) forced expiratory volume (FEV1), the maximum amount of air expired in one second. The pulmonary function test can be performed in the doctor's office, but is expensive.

Chest x ray

Chest x rays can detect only about half of the cases of emphysema. Chest x rays are rarely useful for diagnosing chronic bronchitis.

Blood gas levels

Blood may be drawn from an artery (more painful than drawing blood from a vein) to determine the amount of oxygen and carbon dioxide present. Low oxygen and high carbon dioxide levels are often indicative of chronic bronchitis, but not always of emphysema.

Tests for cause of infection

If infection is present, blood and sputum tests may be done to determine the cause of infection.

Electrocardiogram (ECG)

Many patients with lung disease also develop heart problems. The ECG identifies signs of heart disease.

Treatment

The precise nature of the patient's condition will determine the type of treatment prescribed for COPD. With a program of complete respiratory care, disability can be minimized, acute episodes prevented, hospitalizations reduced, and some early deaths avoided. On the other hand, no treatment has been shown to slow the progress of the disease, and only oxygen therapy increases survival rate.

Drugs

Medications frequently prescribed for COPD patients include:

  • Bronchodilators. These agents open narrowed airways and offer significant symptomatic relief for many, but not all, people with COPD. There are three types of bronchodilators: Beta2 agonists, anticholinergic agents, and theophylline and its derivatives. Depending on the specific drug, a bronchodilator may be inhaled, injected, or taken orally.
  • Corticosteroids. Corticosteroids, usually inhaled, block inflammation and are most useful for patients with chronic bronchitis with or without emphysema. Steroids are generally not useful in patients who have emphysema.
  • Oxygen replacement. Eventually, patients with low blood oxygen levels may need to rely on supplemental oxygen from portable or stationary tanks.
  • Antibiotics. Antibiotics are frequently given at the first sign of a respiratory infection, such as increased sputum production or a change in color of sputum from clear to yellow or green.
  • Vaccines. To prevent pulmonary infection from viruses and bacteria, people with COPD should be vaccinated against influenza each year at least six weeks before flu season and have a one-time pneumococcal (pneumonia ) vaccine.
  • Expectorants. These agents help loosen and expel mucus secretions from the airways.
  • Diuretics. These drugs are given to prevent excess water retention in patients with associated right heart failure.
  • Augmentation therapy (for emphysema due to AAT-deficiency only). Replacement AAT (Prolastin), derived from human blood which has been screened for viruses, is injected weekly or bimonthly for life.

Surgery

Surgical procedures for emphysema are very rare. They are expensive and often not covered by insurance. The great majority of patients cannot be helped by surgery, and no single procedure is ideal for those who can be helped. In January of 1996, the government temporarily suspended Medicare payments for lung reduction surgery.

  • Lung transplantation. Lung transplantation has been successfully employed in some patients with end-stage COPD. In the hands of an experienced team, the one-year survival rate is over 70%.
  • Lung volume reduction. These procedures remove 20-30% of severely diseased lung tissue; the remaining parts of the lung are joined together. Mortality rates can be as high as 15% and complication rates are even higher. When the operation is successful, patients report significant improvement in symptoms.

Pulmonary rehabilitation

A structured, outpatient pulmonary rehabilitation program improves functional capacity in certain patients with COPD. Services may include general exercise training, administration of oxygen and nutritional supplements, intermittent mechanical ventilatory support, continuous positive airway pressure, relaxation techniques, breathing exercises and techniques (such as pursed lip breathing), and methods for mobilizing and removing secretions.

Alternative treatment

For both chronic bronchitis and emphysema, alternative practitioners recommend diet and nutritional supplements, a variety of herbal medicines, hydrotherapy, acupressure and acupuncture, aromatherapy, homeopathy, and yoga.

Prognosis

COPD is a disease that can be treated and controlled, but not cured. Survival of patients with COPD is clearly related to the degree of their lung function when they are diagnosed and the rate at which they lose this function. Overall, the median survival is about 10 years for patients with COPD who have lost approximately two-thirds of their lung function at diagnosis.

Prevention

Lifestyle modifications that can help prevent COPD, or improve function in COPD patients, include: quitting smoking, avoiding respiratory irritants and infections, avoiding allergens, maintaining good nutrition, drinking lots of fluids, avoiding excessively low or high temperatures and very high altitudes, maintaining proper weight, and exercising to increase muscle tone.

Resources

PERIODICALS

Cordova, Francis C., and Gerard J. Griner. "Management of Advanced Chronic Obstructive Pulmonary Disease." Comprehensive Therapy 23, no. 6: 413-424.

Lefrak, Stephen S., et al. "Recent Advances in Surgery for Emphysema." Annual Review of Medicine 48: 387-398.

ORGANIZATIONS

American Association for Respiratory Care. 11030 Ables Lane, Dallas, TX 75229. (214) 243-2272. http://www.aarc.org.

American Lung Association. 1740 Broadway, New York, NY 10019. (800) 586-4872. http://www.lungusa.org.

National Heart, Lung and Blood Institute. P.O. Box 30105, Bethesda, MD 20824-0105. (301) 251-1222. http://www.nhlbi.nih.gov.

National Jewish Medical and Research Center. 1400 Jackson St., Denver, CO 80206. (800) 222-LUNG (Lung Line). http://www.njc.org.

KEY TERMS

Alpha-1-antitrypsin (AAT) A blood component that breaks down infection-fighting enzymes such as elastase.

Alveoli Terminal air sacs of the respiratory system, where gas (oxygen and carbon dioxide) exchange occurs.

Bronchi Large air tubes of the respiratory system.

Bronchioles Small air tubes of the respiratory system.

Bronchodilators Drugs that open wider the bronchial tubes of the respiratory system.

Corticosteroids A group of hormones that are used as drugs to block inflammation.

Forced expiratory volume (FEV1) The maximum amount of air expired in one second.

Spirometer An instrument used by a doctor to perform a breathing test.

Vital capacity (VC) The largest amount of air expelled after one's deepest inhalation.

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chronic obstructive pulmonary disease

chronic obstructive pulmonary disease (COPD, chronic obstructive airways disease, COAD) n. a disease of adults, especially those over the age of 45 with a history of smoking or inhalation of airborne pollution. It has features of emphysema and chronic bronchitis and is diagnosed, according to the GOLD guidelines, at different stages (0–4) based on the value of the forced expiratory volume in 1 second (FEV1). Different treatment regimens are recommended for different stages: inhaled corticosteroids and long-acting beta agonists can improve quality of life and survival in the later stages.

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