DRG Category: 190
Mean LOS: 4.5 days
Description: Medical: Chronic Obstructive Pulmonary Disease With Major Complication or Comorbidity
Emphysema is a chronic obstructive pulmonary disease (COPD) characterized by permanent enlargement of the air spaces beyond the terminal bronchioles. In addition to this abnormality, destruction of respiratory walls occurs. The syndrome includes both chronic bronchitis and emphysema, both of which are airflow-limited states; some experts do not distinguish between the two conditions. COPD affects more than 15 million people and is the fourth leading cause of death overall in the United States. Approximately 5% to 6% of male adults and 1% to 3% of female adults in the United States have emphysema.
In emphysema, the affected terminal bronchioles contain mucous plugs that, when enlarged, eventually result in the loss of elasticity of the lung parenchyma, causing difficulty in the expiratory phase of respiration. The alveolar walls are destroyed by abnormal levels of enzymes (proteinases) that break down respiratory walls. Gas exchange is impaired by the reduced surface area that results from the destruction of alveolar walls.
Four types of emphysema have been identified: paraseptal emphysema, which affects the periphery of the lobule; panacinar or panlobular emphysema, which affects the lower anterior segments or the entire lungs; centriacinar or centrilobular emphysema, the most common form, which destroys respiratory bronchioles and is associated with chronic bronchitis and cigarette smoking; and bronchiectasis or chronic necrotizing infection that leads to abnormal and permanent bronchial dilation, which occurs rarely. Complications from emphysema include cor pulmonale, respiratory failure, pneumothorax, and recurrent respiratory tract infections. Emphysema is the most common cause of death from respiratory disease in the United States.
Cigarette smoking strongly contributes to emphysema and leads to neutrophil activation and retention in the lung functional tissue. Up to 80% of people with emphysema have a history of smoking. Approximately 20% of people with emphysema have chronic occupational exposure to inhaled mineral fumes or dusts, organic dusts from wood or grains, or fumes from exhausts or chemical vapors. Enzymes, primarily proteinases and elastases, destroy the extracellular matrix of the lung and cause emphysema. Normally, plasma proteinase inhibitors in lung tissue prevent proteolytic enzymes from digesting structural proteins of the lungs, but if an excess of proteinase occurs, the defenses cannot manage the enzymes, and destruction occurs. Emphysema is the result of an imbalance between the proteinases and antiproteinases, with an excess of proteinases.
Risk factors for the development of emphysema include cigarette smoking, living or working in a highly polluted area, a family history of pulmonary disease, and substance use (cocaine, IV drug use with methadone and methylphenidate). HIV infection is also an independent risk factor for emphysema.
Emphysema has both genetic and environmental components. Emphysema is most often caused by smoking, but susceptibility may be influenced by genetics. Heritability is estimated at 30% to 50%. There is also a rare form of familial emphysema caused by mutations in the SERPINA1 gene, leading to alpha-1 antitrypsin (AAT) deficiency. These mutations are inherited in an autosomal codominant pattern, meaning that two different gene alleles are expressed, and both contribute to the development of the trait. The M allele is most common and results in production of normal levels of AAT. People who are homozygous normal have copies of the M allele from both parents. The two variants (S and Z) cause production of low or moderately low amounts of AAT. Persons with the ZZ or SZ genotype are most likely to develop AAT deficiency. Persons with an MS or SS genotype usually produce enough AAT to protect the lungs. There is an increased risk of emphysema for those who carry the MZ alleles, particularly if they are smokers. Familial factors predisposing to emphysema in the absence of AAT deficiency are also likely.
Symptoms of emphysema may begin in the third or fourth decade of life but usually become severe during the fifth decade or later, which is when they are most likely to come to the attention of a healthcare provider. Previously, experts noted that emphysema occurs more often in males than in females, but as smoking rates have increased in women, the rates have equalized. Currently, women are dying from COPD more frequently than men and developing severe COPD earlier than men.
Black persons bear a significant burden of lung disease. Black patients with emphysema have worse symptoms, poorer quality of life, and increased complications as compared to White patients. Additionally, when neighborhood-level factors (neighborhood walkability, access to healthcare, socioeconomic level) were considered, Black patients continued to have increased risk of exacerbation and worse outcomes (Ejike et al., 2020). Multiple studies have indicated that education and income have a protective effect on lung health, but White persons have an even stronger protective effect from those factors than Black and Hispanic persons, creating additional disparities. Sexual and gender minority persons have higher odds for multiple chronic conditions, cancer, and poor quality of life and are more apt to have disabilities than cisgender males and females (cisgender is a term used to describe persons whose gender identity and gender expression are aligned with their assigned sex listed on their birth certificate) (Cacerese, Jackman, et al., 2020; Connelly et al., 2019). Gender and sexual minority persons also smoke more than their heterosexual and cisgender counterparts. While large-scale studies are not available, these factors place sexual and gender minority persons at risk for emphysema.
Research from Latin America, specifically, and likely around the globe, has shown higher prevalence of emphysema than the United States. Because more than 1.2 billion humans smoke cigarettes, prevalence is climbing. Results from pooled data suggest that the global prevalence of COPD is approximately 10% in adults over age 40 years; chronic bronchitis prevalence alone is 6%, and emphysema alone is 2% in adults.
PLANNING AND IMPLEMENTATION
Collaborative
Viral or bacterial infections may lead to bronchospasm or increased mucous secretions. Acute exacerbations are accompanied by dyspnea, fatigue, and even respiratory failure. Low-flow oxygen therapy based on arterial blood gas results is often administered to treat hypoxemia. For the patient with increasing airway obstruction and plugging, noninvasive or invasive mechanical ventilation may be needed to maintain adequate airway and breathing, followed by pulmonary rehabilitation. Adequate hydration is also necessary to help liquefy secretions. A smoking cessation program is the most important collaborative intervention that is needed for long-term health and the most effective intervention for most patients. Refer the patient to a smoking cessation counselor.
Surgical options exist for patients with emphysema with the goal of restoring function and improving symptoms. Surgery is discussed carefully because it is linked with significant morbidity, and patients are chosen who will receive the greatest benefit. Types of procedures that are considered include bullectomy (removal of giant bullae), lung volume reduction surgery (resection of the most diseased portions of the lungs), and lung transplantation, which improves quality of life but does not prolong it.
| Medication or Drug Class | Dosage | Description | Rationale |
|---|
| Bronchodilators: Anticholinergic agents | Varies by drug | Atropine sulfate, ipratropium bromide | Reversal of bronchoconstriction |
| Bronchodilators: Beta2-adrenergic agents | Varies by drug | Inhaled beta2-adrenergic agonists by metered-dose inhaler (MDI) such as albuterol, metaproterenol, or terbutaline | Reversal of bronchoconstriction |
| Tiotropium | Two oral inhalations of an 18-mcg capsule daily with inhaler | Quaternary ammonium compound; long-acting muscarinic agent with anticholinergic and antimuscarinic effects; has an inhibitory effect on M3 receptors in airway smooth muscle | Long-lasting bronchodilation (24 hr) |
| Systemic corticosteroids (oral and inhaled) | Varies by drug | Methylprednisolone IV; prednisone PO; may also add inhaled fluticasone, inhaled budesonide | Decrease inflammatory response and improve airflow in some patients for a few days during acute exacerbations; should only be added if patient is also on a long-acting bronchodilator |
Other Drugs: Bronchodilators, which are used for prevention and maintenance therapy, can be administered as aerosols or oral medications. Generally, inhaled anticholinergic agents are the first-line therapy for emphysema, with the addition of beta-adrenergic agonists added in a stepwise fashion. Antibiotics are ordered if a secondary infection develops. As a preventive measure, influenza and pneumonia vaccines are administered.
Independent
Smoking cessation is the most effective therapy for patients. The healthcare team needs to coordinate the approach to smoking cessation, often introduced by the physician but implemented by the nurse, who may develop the smoking cessation plan. Experts suggest multiple interventions and multiple settings such as group programs and one-to-one counseling and teaching sessions. When the patient is acutely ill, maintaining a patent airway is a priority. Use a humidifier at night to help the patient mobilize secretions in the morning. Encourage the patient to use controlled coughing to clear secretions that might have collected in the lungs during sleep. Instruct the patient to sit at the bedside or in a comfortable chair, hug a pillow, bend the head downward a little, take several deep breaths, and cough strongly.
Place patients who are experiencing dyspnea in a high Fowler position to improve lung expansion. Placing pillows on the overhead table and having the patient lean over in the orthopneic position may also be helpful. Teach the patient pursed-lip and diaphragmatic breathing. To avoid infection, screen visitors for contagious diseases and instruct the patient to avoid crowds.
Conserve the patient's energy in every possible way. Plan activities to allow for rest periods, eliminating nonessential procedures until the patient is stronger. It may be necessary to assist with the activities of daily living and to anticipate the patient's needs by having supplies within easy reach. Refer the patient to a pulmonary rehabilitation program if one is available in the community. Patient education is vital to long-term management. Teach the patient about the disease and its implications for lifestyle changes, such as avoidance of cigarette smoke and other irritants, activity alterations, and any necessary occupational changes. Provide information to the patient and family about medications and equipment.
Evidence-Based Practice and Health Policy
Lim, E., Sousa, I., Shah, P., Diggle, P., & Goldstraw, P. (2020). Lung volume reduction surgery: Reinterpreted with longitudinal data analyses methodology. Annals of Thoracic Surgery, 109, 1496–1502.
- The authors reevaluated the National Emphysema Treatment Trial (NETT) in order to report longer-term outcomes of lung volume reduction surgery (LVRS). The authors analyzed trial data released by the U.S. National Heart, Lung, and Blood Institute to ascertain the difference in lung function variables between patients receiving LVRS and patients receiving medical care out to 5 years.
- For the outcome of forced expiratory volume in 1 second in patients randomized to LVRS, there was an immediate improvement compared with medical therapy, with an estimated decline to baseline approximately 5 years after randomization. Five years after surgery, physiological function improved in the areas of shortness of breath, well-being, and exercise performance in the surgical group. LVRS improved lung function and exercise workload that returned to baseline within a 5-year period. Although LVRS may not improve survival, the procedure improved dyspnea as far out as 5 years after surgery.