Definition
A.Emphysema is an abnormal dilation and destruction of alveolar ducts and airspaces distal to the terminal bronchioles. Lung function slowly deteriorates over many years before the illness develops. Emphysema is one of the chronic obstructive pulmonary diseases (COPDs)—a term that refers to conditions characterized by continued increased resistance to expiratory airflow. Chronic bronchitis, emphysema, and asthma comprise COPD. Chronic bronchitis and emphysema with airflow obstruction commonly occur together.
B.There are three morphological types of emphysema:
1.Centriacinar emphysema: Associated with long-term smoking; primarily involves the upper half of the lungs.
2.Panacinar emphysema, found predominantly in the lower half of the lungs; this is observed in patients with alpha 1-antitrypsin (AAT) deficiency.
3.Paraseptal emphysema involves the distal airway.
Incidence
A.Emphysema typically occurs in people older than age 50, with peak occurrence after the age of 65.
B.The prevalence of emphysema is 20.2 cases per 1,000.
C.The prevalence is greater in Caucasians than other ethnicities.
D.Emphysema is slightly greater in females (21.4/1,000) than males (19.0/1,000).
Pathogenesis
A.Decreased gas exchange occurs due to focal destruction limited to the airspaces distal to the respiratory bronchioles, causing airway obstruction, hyperinflation, loss of lung recoil, and destruction of alveolar–capillary interface.
Predisposing Factors
A.Long-term cigarette smoking.
B.Occupational and environmental exposure to toxic agents:
1.Dust.
2.Chemical fumes.
3.Secondhand smoke.
4.Air pollution.
5.Gases.
C.Alpha 1-protease inhibitor deficiency.
D.Intravenous (IV) drug use secondary to pulmonary vascular damage from the insoluble fillers (e.g., cornstarch, cotton fibers, cellulose, talc).
E.Connective tissue disorders (e.g., Marfan syndrome and Ehlers-Danlos).
F.HIV.
Common Complaints
A.Gradually progressing exertional dyspnea.
B.Chronic cough.
C.Wheezing.
D.Fatigue.
E.Weight loss.
Other Signs and Symptoms
A.Cough with mild to moderate sputum production and clear to mucoid sputum.
B.Early morning cough.
C.Shortness of breath (SOB).
D.Tachypnea.
E.Use of accessory muscles for breathing, pursed-lip breathing, prolonged expiration.
F.Barrel chest (increased anterior to posterior chest diameter).
G.Flushed skin.
H.Clubbed fingers.
I.Decreased libido.
J.Thin, wasted appearance.
K.Wheezing, particularly during exertion and exacerbations of emphysema.
L.Functional impairment related to dyspnea.
M.Depression.
Subjective Data
A.Elicit information about onset, duration, and course of symptoms.
B.Determine if the patient is a smoker. Determine cigar use and cigarette pack-year history (pack/day times the number of years smoked). If so, how much and for how long? Evaluate exposure to secondhand smoke. How much of the day? Are they interested in quitting?
C.Ask about current and previous work. Is the patient exposed to occupational and environmental irritants, such as dust, fumes, or gases? If so, what are the type, level, and duration of exposures?
D.Inquire about the cough’s characteristics. Is it productive, dry, bronchospastic, brassy, wheezy, strong, or weak?
E.Question the patient about episodes of tachypnea, frequency of respiratory infections, and incidence of angina during exertion.
F.Does the patient have a hereditary disease (e.g., cystic fibrosis (CF) or AAT deficiency), asthma, nasal abnormalities (e.g., deviated septum), or other respiratory problems?
G.Last tuberculin skin test and recent risk for tuberculosis (TB) exposure?
H.Find out the patient’s usual weight, and assess how much weight loss has occurred and over what time period.
I.Evaluate current vaccination status for pneumonia and influenza.
J.Review all medications, including over-the-counter (OTC) and herbal products.
K.Assess the patient’s ability to perform activities of daily living (ADL) and instrumental activities of daily living (IADL), including grooming and personal hygiene, performing chores around the home, shopping, cooking, and driving.
L.Ask about alcohol use.
M.Screen for depression.
N.Ask about frequency of use of short-acting beta-2 agonists (SABA) and effectiveness.
O.Inquire about use of ED or need for hospitalization. Previous ventilation?
Physical Examination
A.Temperature (if indicated), pulse, respirations, blood pressure (BP), and weight for changes. Consider pulse oximetry.
B.Inspect:
1.Observe general appearance; note flushed skin color, use of accessory muscles, pallor around lips, pursed-lip breathing, barrel chest (lung hyperinflation), and thinness.
2.Assess for peripheral edema.
3.Dermal examination: Note finger clubbing and cyanosis.
4.Observe mental status and psychiatric concerns including depression
C.Auscultate:
1.Auscultate heart.
2.Auscultate lungs for wheezes, crackles, decreased breath sounds (generally diffuse decreased breath sound).
3.Assess for vocal fremitus (vibration) and egophony (increased resonance and high-pitch bleating quality). Air trapping causes air pockets that do not transmit sound well.
D.Percuss:
1.Percuss chest for presence of hyperresonance and signs of consolidation.
2.Percuss abdomen for organomegaly.
E.Palpate:
1.Palpate the neck, superior and inferior clavicular spaces for lymphadenopathy.
2.Palpate abdomen for organomegaly.
3.Grade dependent edema.
F.Further physical examinations are dependent on comorbidities.
G.Mental status: Assess for decreased level of consciousness (LOC).
Diagnostic Tests
A.Pulse oximetry: Blood gases if indicated. Baseline should be considered as early in disease process as patient presents.
B.AAT to rule out hereditary deficiency.
C.Tuberculin skin test.
D.Pulmonary function tests (PFTs) reveal increased total lung capacity with poor respiratory expulsion and increased respiratory volume.
E.ECG reveals sinus or supraventricular tachycardia.
F.Chest radiograph (CXR) reveals hyperinflation, flat diaphragm, and enlarged heart.
G.Sputum evaluation and/or culture.
Differential Diagnoses
A.Emphysema.
B.Chronic bronchitis.
C.Chronic asthma.
D.Bronchiectasis.
E.CF.
F.Chronic asthmatic bronchitis.
G.TB.
H.AAT deficiency.
I.Congestive heart failure (CHF).
Plan
A.Medical management: Supplemental oxygen therapy is indicated if the patient has a resting PaO2 less than 55 mmHg or a PaO2 less than 60 mmHg along with right heart failure or secondary polycythemia. Goals are to achieve a PaO2 of greater than 55 mmHg (usually 1–3 L/min).
B. See Section III: Patient Teaching Guide Emphysema.
C.Develop a smoking cessation plan: Assess readiness to quit. Set a quit date, and encourage a group smoking cessation program. A smoking cessation plan is an essential part of a comprehensive treatment plan:
The Global Initiative for Chronic Obstructive Lung Disease (GOLD) guidelines note a five-step program for smoking intervention:
1.ASK: Systematically identify all tobacco users at every visit, EVERY patient at EVERY clinic visit is queried about tobacco-use status.
2.ADVISE: Strongly urge all tobacco users to quit in a clear, strong, and personalized manner.
3.ASSESS: Determine willingness and rationale of the patient’s desire to make a quit attempt. Ask every tobacco user if he or she is willing to make a quit attempt at this time (e.g., within the next 30 days).
4.ASSIST: Aid in quitting, make a quit plan, provide practical counseling, provide educational materials, and help the patient to obtain social support. Pharmacotherapy if appropriate
5.ARRANGE: Schedule a follow-up contact either in person or by telephone.
D.Pharmacotherapy and nicotine replacement reliably increase long-term smoking abstinence rates. The effectiveness and safety of e-cigarettes as a smoking cessation aid is uncertain at this time. See Section III: Patient Teaching Guide Nicotine Dependence
:
1.Nicotine chewing gum produces better quit rates than counseling alone.
2.Transdermal nicotine patches have a long-term success rate of 22% to 42%.
3.The use of vapor nicotine products should be discussed, as their long-term adverse effects on lung function is unknown.
4.The use of an antidepressant such as bupropion sustained release (Zyban) has been shown to be effective for smoking cessation by reducing the enjoyment of smoking, reducing withdrawal symptoms, and limiting weight gain. Bupropion may be used in combination with nicotine replacement therapy (NRT). Begin dosing 1 week before quit date:
a.Day 1 to 3 of treatment: 150 mg every morning.
d.Medication doses and/or frequency intervals is required for renal and mild hepatitis impairment.
e.Moderate to severe hepatic impairment: 150 mg every other day.
5.Varenicile (Chantix) is a partial agonist selective for alpha-4, beta-2 nicotinic acetylcholine receptors. Varenicline mimics the effect of nicotine; therefore it reduces the urge to smoke and relieves withdrawal symptoms. It blocks or blunts the effect of nicotine if the person relapses and has a cigarette. Review the patient’s history of renal impairment, psychiatric disorders, seizure history/risk, alcohol use, and cardiovascular disease.
E.Pharmaceutical therapy.
Oral and inhaled medications are used for patients with stable emphysema to reduce dyspnea and improve exercise tolerance. Prior to any medication/dosage changes, monitor the patient’s compliance. (Refer to Table 12.1.) A spacer/chamber device should be used to improve drug delivery and reduce adverse effects:
1.Bronchodilators decrease muscle tone in both the small and large airways thereby increasing ventilation. This category includes beta-adrenergic agents, methylxanthines, and anticholinergics:
a.Albuterol (Proventil, Ventolin), a beta-2 agonist, relaxes smooth muscle with little effect on cardiac muscle contractility. Albuterol is available as a liquid for nebulizers, and as a metered-dose inhaler (MDI), and dry powder inhaler (DPI).
b.Metaproterenol (Alupent), a beta-2 agonist, relaxes smooth muscle with little effect on cardiac muscle contractility. Metaproterenol is available as liquid for nebulizer, MDI, and DPI.
c.Levalbuterol (Xopenex) is a selective beta-2 agonist that is also used for treatment or prevention of bronchospasm.
d.Ipratropium (Atrovent) is chemically related to atropine with antisecretory properties. It is used on a fixed schedule with a beta agonist.
e.Salmeterol (Serevent) relaxes smooth muscles and can relieve bronchospasm. It may also facilitate expectoration. The bronchodilating effect lasts longer than 12 hours. It is used on a fixed schedule in addition to regular use of anticholinergic agents.
f.Formoterol (Foradil) is a beta agonist with rapid onset and long duration. The bronchodilating effects last longer than 12 hours. Formoterol is used on a fixed schedule in addition to regular use of anticholinergic agents.
g.Iotropium (Spiriva) is a quaternary ammonium compound that elicits anticholinergic/antimuscarinics effects with inhibitory effects on M3 receptors on airway smooth muscles, leading to bronchodilation. It is available as a capsule dosage form containing dry powder for oral inhalation via HandiHaler.
h.Theophylline (Aminophylline, Theo-24, Theo-Dur, Slo-bid) potentiates exogenous catecholamines and diaphragmatic muscular relaxation, which stimulates bronchodilation. Theophylline has a narrow therapeutic range and frequent toxicity:
The target theophylline concentration is 5 to 10 μg/mL. Prescribing theophylline requires weight-based dosing. Dosage adjustment therapy is based upon serum theophylline concentrations. Monitor theophylline level whenever there is a new worsening chronic illness, or a change in medications that may alter theophylline clearance ., fever > 102°F sustained for ≥24 hours or altered liver function including hepatitis). Check drug interaction using an online checker; www.rxlist.com/drug-interaction-checker.htm.
i.Dosage should be titrated up. Titration steps include the following: the starting dose; after 3 days, if tolerated, increase dose; and after 3 more days, if tolerated and if needed, increase dose
ii.Common side effects of theophylline include loss of appetite, abdominal pain, diarrhea, weight loss, restlessness, tremor, insomnia, headache, lightheadedness, dizziness, and sweating.
iii.Instruct the patient to call 911 and get emergency medical help for severe headache, hyperglycemia, severe or persistent vomiting, cardiac arrhythmias, seizures, or confusion.
i.Aclidinium (Tudorza Pressair) is a M1-M3 muscarinic agonist (longacting muscarinic agonists [LAMAs]). It inhibits M3 receptors, leading to muscle relaxation and subsequent bronchodilation.
2.Corticosteroids attempt to moderate the inflammatory component. They should only be added to a regimen that includes a long-acting bronchodilator:
a.Fluticasone inhaled: Vasoconstrictive and anti-inflammatory activity.
b.Budesonide inhaled.
3.Phosphodiesterase 4 (PDE4) inhibitors: Selective PDE4 inhibitors reduce exacerbations, improve dyspnea, and increase lung function in patients with severe COPD.
4.Oral steroids should be used to treat outpatients with acute exacerbations. Corticosteroids reduce mucosal edema, inhibit prostaglandins that cause bronchoconstriction, and increase responsiveness to bronchodilators. Taper dose as soon as bronchospasm is controlled. A minority of patients who respond to oral steroids can be maintained on long-term inhaled steroids.
5.In patients with emphysema, chronic infection or colonization of the lower airways is common. The goal of antibiotic therapy is not to eliminate organisms, but to treat acute exacerbations. If infection is present, give one of the following:
a.Augmentin 500 mg orally twice daily.
b.Doxycycline 100 mg orally twice daily.
c.Trimethoprim-sulfamethoxazole 80 to 160 mg orally every 12 hours.
d.If pseudomonas is considered: Levofloxacin 500 mg daily.
6.Mucolytic agents in clinical practice are not recommended currently because of a lack of evidence for their benefit.
7.Trivalent influenza vaccine is essential for all COPD patients.
Administer yearly flu vaccine. High-dose trivalent or quadrivalent influenza vaccine is essential for all COPD patients. Those 65 years of age or older should receive highdose trivalent vaccine. Give the patient the vaccine in late September, at least 6 weeks before the onset of flu season.
8.Administer pneumonia vaccine for patients 65 years and older, or beginning at age 19 if immunocompromised or chronic respiratory condition is present. Consider revaccination every 5 to 10 years for high-risk patients. Download a guide to sequential administration according to individual health considerations here: www.cdc.gov/vaccines/vpd/pneumo/downloads/pneumo-vaccine-timing.pdf.
a.Sequential administration of the pneumococcal conjugate vaccine (PCV13) and pneumococcal polysaccharide vaccine (PPSV23) is recommended. (download Centers for Disease Control and Prevention’s [CDC] guide here: www.cdc.gov/vaccines/vpd/pneumo/downloads/pneumo-vaccine-timing.pdf ). In the vaccine naïve patient, give PCV13 first, then the PPSV23 12 months later.
b.In older adults who received PPSV23 at age 65 or older, give PCV13 in 1 year or more.
c.In older adults who received PPSV23 BEFORE age 65, give PCV13, then 5 years after the
initial PPSV23 give a second PPSV23.
d.Give a one-time second dose of PPSV23 for persons with chronic renal failure or nephrotic syndrome, functional or anatomic asplenia, or immunocompromising conditions.
9.AAT is needed for significant antitrypsin deficiency (less than 80 mg/dL). Patients get weekly or monthly infusions. Consult with a physician before therapy. A history of smoking rules out candidacy.
Follow-Up
A.If the patient is acute, contact by phone in 24 to 48 hours and consider immediate referral.
B.Monitor the patient’s body weight.
C.Serial PFTs may help guide therapy and offer prognostic information.
D.Monitor theophylline levels because of the drug’s potential for toxicity. Adverse effects, including nausea and nervousness, are the most common. Other adverse effects include abdominal pain with cramps, anorexia, tremors, insomnia, cardiac arrhythmia, and seizures. Theophylline doses: 100 to 200 mg every 6 to 8 hours.
Emergent Issues/Instructions
A.Monitor theophylline levels because of the drug’s potential for toxicity:
1.Instruct the patient to call 911 and get emergency medical help for severe headache, hyperglycemia, severe or persistent vomiting, cardiac arrhythmias, seizures, and confusion.
Consultation/Referral
A.If the patient’s condition remains acute after 48 hours of treatment, consider immediate referral to a physician.
B.Refer the patient to a social worker for help in getting Meals on Wheels, handicapped parking, and finding other community resources.
C.A consultation with a pulmonary specialist is recommended.
D.Pulmonary rehabilitation should be considered early in the disease process. Pulmonary rehab programs vary in format and length. Most programs use a small group format and include the following:
1.Education about symptoms, medications, and oxygen.
2.Supervised exercise classes and instruction.
3.Breathing techniques.
4.Nutritional counseling.
5.Emotional health support.
Individual Considerations
A.Adults: Sexual dysfunction is common in patients with COPD.
B.Geriatrics:
1.Older adults are at higher risk for restrictive lung diseases. Therapy follows the underlying cause. These diseases fall into four broad categories:
a.Intrinsic: Idiopathic pulmonary fibrosis, radiation-induced, drug-induced, postinflammatory lung fibrosis, connective tissue disease.
b.Extrinsic: Kyphosis/kyphoscoliosis, obesity.
c.Neuromuscular: Amyotrophic lateral sclerosis (ALS), thyroid disorders, adrenal disorders, postpolio syndrome, vitamin D deficiency.
d.Central nervous system disorders: Parkinson’s disease, multisystemic atrophy, multiple sclerosis, progressive supranuclear palsy.
2.Discuss course of disease, living wills, advanced directives, and resuscitation status early, before a crisis occurs. Encourage patients to discuss their preferences with their family.
3.Older adults may be unable to use standard inhalers for the following reasons:
a.Decreased manual dexterity due to arthritis, reduced grip strength, tremors, muscle weakness, and poor hand–eye coordination.
b.Cognitive impairment: Difficulty coordinating the required activation/inhalation steps to achieve proper penetration of medication into the lungs.
c.Patients should demonstrate use of their inhaler. If concerns, nebulizer therapy should be prescribed to improve the benefit of pharmacotherapy.
4.Beers list caution: Theophylline—theophylline is on the Beers list of drugs to use with caution in the geriatric population related to cardiovascular, renal, hepatic, insomnia, and peptic ulcers:
a.Theophylline has a drug–drug interaction with multiple medications, increasing the risk of theophylline toxicity. For example, theophylline has a drug–drug interaction with cimetidine. (Refer to Appendix C [Beers Criteria], Table C.4.)
Resources
A Patient’s Guide to Aerosol Drug Delivery is available at www.copd-alert.com/AerosolDrug.pdf
Drug interaction checker: www.rxlist.com/drug-interaction-checker.htm
Global Initiative for Chronic Obstructive Lung Disease (GOLD) guidelines: www.goldcopd.org.
National Emphysema Foundation: www.emphysemafoundation.org
Pulmonary Disease Aerosol Delivery Devices: A Guide for Physicians, Nurses, Pharmacists, and Other Health Care Professionals is available at www.aarc.org/wp-content/uploads/2018/03/aersol-guides-for-hcp.pdf