A. Patient with Suspected or Established Diagnosis of COPD - Chronic obstructive pulmonary disease (COPD) is defined by the American Thoracic Society (ATS) in Standards for the Diagnosis and Care of Patients with Chronic Obstructive Pulmonary Disease (1995). Further detail on lung function testing is found in another ATS publication (1991).

1. Definitions 


a. Chronic obstructive pulmonary disease is a disorder 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.

b. Chronic bronchitis is defined as the presence of chronic productive cough for 3 months of each of two successive years in a patient in whom other causes of chronic cough have been excluded.

c. Emphysema is defined as abnormal permanent enlargement of the air spaces distal to the terminal bronchioles, accompanied by destruction of their walls and without obvious fibrosis.

d. Asthma is by definition associated with reversible airflow obstruction. Patients with asthma whose airflow obstruction is completely reversible are not considered to have COPD. The obstruction in many patients with COPD may include a significant reversible component. Some patients with asthma may develop irreversible airflow obstruction indistinguishable from COPD.

B. Perform Clinical Assessment, History, Physical Examination, Lab Tests, and Spirometry

1. History 


a. Smoking - Age at initiation, quantity smoked per day, whether or not still smoker and if not, date of cessation

b. Environmental (chronological) - May disclose important risk factors

c. Cough (chronic, productive) - Frequency and duration, whether or not productive (especially on awakening)

d. Wheezing

e. Acute chest illnesses - Frequency, productive cough, wheezing, dyspnea, fever

f. Dyspnea

2. Physical Examination of Chest 


a. Airflow obstruction evidenced by:
• Wheezing during auscultation on slow or forced breathing
• Forced expiratory time of > 6 seconds
 

b. Severe emphysema indicated by:

• Overdistention of lungs in stable state, low diaphragmatic position
• Decreased intensity of breath and heart sounds
 

c. Severe disease suggested by (characteristics not diagnostic):

• Pursed-lip breathing
• Use of accessory respiratory muscles
• Indrawing of lower interspaces

3. Other - Unusual positions to relieve dyspnea at rest, digital clubbing (suggests possibility of lung cancer or bronchiectasis), and mild dependent edema that may be seen in absence of right heart failure

4. Laboratory 


a. Chest radiography - Diagnostic only of severe emphysema but essential to exclude other lung diseases

b. Spirometry (pre- and postbronchodilation) - Essential to confirm presence and reversibility of airflow obstruction and to quantify maximum level of ventilatory function (ATS 1995)

c. Lung volumes - Measurement of more than forced vital capacity is not necessary except in special circumstances (e.g., presence of giant bullae)

d. Carbon monoxide diffusing capacity - Unnecessary except in special instances (e.g., dyspnea out of proportion to severity of airflow limitation)

e. Arterial blood gases are not needed in staged mild disease, but remain the standard for determining the need for oxygen therapy

f. Alpha₁-antitrypsin (AAT) - AAT deficiency accounts for less than 1 percent of COPD. Obtain a serum AAT level to screen for AAT deficiency in the following situations:

• Chronic bronchitis with airflow obstruction in a never smoker
  
  
• Bronchiectasis, especially in the absence of clear risk factors for the disease
  
  
• Premature onset of COPD with moderate or severe impairment before age 50
  
  
• A predominance of basilar emphysema; development of unremitting asthma, especially in a patient under age 50
  
  
• A family history of AAT deficiency or of COPD onset before age 50
  
  
• Cirrhosis in a patient without apparent risk factors (ATS 1995). If diagnosis of COPD or asthma is made, refer to specialist for recommendations for therapy.

C. Stage COPD on the Basis of FEV₁ as Mild, Moderate, or Severe - Grading or staging, based on severity of airflow obstruction, facilitates the application of clinical recommendations and attempts to offer a composite picture of disease severity. Forced expiratory spirometry is used in the diagnosis of COPD as well as in the assessment of its severity, progression, and prognosis. Once airflow limitation has been diagnosed by a reduction in FEV₁:VC (vital capacity) or FEV₁:FVC (forced vital capacity), the severity can be graded by the patient's percentage of predicted FEV₁. Stages of mild, moderate, and severe as adopted in the 1995 ATS document are used in the attached Chronic COPD algorithm. The severe stage in the VA's COPD population (predominantly middle-aged and male) is usually determined by a reduction in the FEV₁ below one liter. 

D. Acute Exacerbation of COPD - Acute exacerbation is defined as a recent deterioration of the patient's clinical and functional state that is due to worsening of his or her COPD. Typical symptoms and signs of COPD exacerbation are listed below (adapted from the European Respiratory Society Consensus Statement, Siafakas, 1995):

• Worsening dyspnea, sometimes at rest
• Increased cough
• Increased sputum production, often with change in character from mucoid to purulent
• Development or increase in wheezing
• Loss of energy
• Fever
• Increased respiratory rate
• Tachycardia
• Increased cyanosis
• Use of accessory muscles
• Peripheral edema
• Loss of alertness
• Worsening airflow obstruction as determined by FEV₁ or peak expiratory flow
• Worsening of arterial blood gases or pulse oximetry O₂ saturation.

F. If on Therapy, Reevaluate or Begin Trial of Therapy with Inhaled Anticholinergic (IAC) - A trial of IAC therapy is recommended in apparently asymptomatic patients with an FEV₁ of less than 50 percent of predicted, since this degree of obstruction is usually associated with dyspnea. This is based on the well-known phenomenon of patients "adapting to their disability." Such a lack of symptoms may result from the patient's avoiding activities or simply thinking along the lines of "Doesn't everyone get short of breath doing this activity at my age?"

Ipratropium (without prn inhaled short-acting beta2-agonist, since it is not needed for rescue medication) is generally the first choice in a trial of therapy, with improvement in function or activities of daily living being used to guide therapy (See annotation G). If ipratropium is ineffective or produces a less-than-optimal effect, add a short-acting inhaled beta2-agonist on a regular schedule (i.e., not prn) as combination therapy. A long-acting inhaled beta2-agonist may be substituted for the short-acting inhaled beta2-agonist if usage warrants. For further details on use of ipratropium and beta2-agonists, see Pharmacotherapy Module A4. If there is no improvement or if symptoms worsen, the trial should be discontinued.

Ipratropium and short-acting inhaled beta2-agonist in typical doses (2 to 4 inhalations) on a scheduled rather than prn use are generally equally as effective as bronchodilators, although some studies suggest that ipratropium has a greater peak and a longer duration of action. The side effects of each are similar (except for increases in heart rate and tremor neither of which is typical at these doses) that occur almost exclusively with beta2-agonist. Dyspnea may be improved to a greater extent with an inhaled beta2-agonist. Some patients will have a response to one but not the other, so in any trial of therapy, both should be tried if improvement is not optimal with the first choice. There is evidence that ipratropium improves baseline pulmonary function (after withholding ipratropium for 6 to 12 hours) whereas beta2-agonist do not. 

Symptoms may improve without substantial improvement in FEV₁, indicating that continuation of therapy does not depend on routine assessment with spirometry. For example, short-acting inhaled beta2-agonist and ipratropium can improve exercise performance without necessarily improving FEV₁. Also, it may be difficult to reduce high-dose ipratropium, since such doses may be needed to improve exercise performance. Inhaled short-acting beta2-agonists can improve dyspnea, and the benefit may be related to this action. Short-acting inhaled beta2-agonists but not ipratropium increase the alveolar-arterial oxygen difference, and this may be a reason to decrease the dose of beta2-agonist in titrating a patient's medication.

Should symptoms not resolve, the short-acting inhaled beta2-agonist can be increased to regularly scheduled dosing qid, with additional prn dosing for rescue. The typical dose is 2 to 4 inhalations, not to exceed 12 per day. However, as much as 1 mg of albuterol (or the equivalent of other short-acting beta2-agonists) may be required acutely to provide maximal bronchodilation for rescues.

The side effects include tremor, increase in heart rate, and reduced arterial oxygen saturation. When SAIBA is used as rescue medication, doses that are generally considered large (up to 10 inhalations) when used over a short time (minutes) may be necessary to alleviate acute shortness of breath or wheezing. In such cases, patients should seek medical advice to determine whether other measures are needed (see also Module A3, Acute Exacerbation).

Some patients can be maintained on a regularly scheduled short-acting inhaled beta2-agonist and ipratropium, such as 2 to 4 puffs of each four to five times a day.

Should the number of inhalations of short-acting beta2-agonist exceed 12 per day (the usually recommended maximum dose), the addition of long-acting inhaled beta2-agonist is recommended (see Pharmacotherapy Module A4). Short-acting inhaled beta2-agonist is continued as prn rescue medication.

The sequence of administration of ipratropium and short-acting inhaled beta2-agonist does not generally make any difference in the bronchodilator benefit.

If symptoms do not resolve, 6 and possibly 8 puffs of ipratropium may be needed qid. Improvement in pulmonary function is maximal at 6 to 14 puffs of ipratropium. Improvement in exercise performance generally requires a minimum of 6- to 8-puffs (108 to 144 g).

I. Does the Patient Have Symptoms of Sleep Apnea?

Symptoms of sleep apnea include:
• Excessive daytime sleepiness (EDS)
• Heavy snoring
• Observed apnea during sleep
• Choking during sleep
• Morning headaches
• Memory loss
• Cardiac arrhythmias during sleep
• Unexplained impotence

1. Smoking Cessation - All smokers should be strongly advised to quit. Smoking cessation results in a small improvement in lung function and a slowing of the rate of decline to approximately that seen in never smokers of the same age. Patients not willing to quit should receive motivational intervention to promote subsequent quitting attempts. The smoker willing to make a quit attempt should be assisted by being asked to set a quit date, providing self-help materials, encouraging nicotine replacement therapy, and referring to intensive treatments when appropriate. All patients attempting to quit should have follow-up contact scheduled. (For additional details, see Module D, Collaborative Self-Management for patients with COPD/Asthma, and see also DHHS Guidelines on Smoking Cessation (1996), Agency for Health Care Policy and Research, Centers for Disease Control and Prevention (1996).

2. Pneumoccocal Vaccination is Recommended - The Advisory Committee on Immunization Practices (CDC 1997) recommends vaccination for all patients with chronic obstructive pulmonary disease and emphysema. Patients age 65 or older who were vaccinated more than 5 years previously should be revaccinated. When the status of previous vaccination is unsure, vaccination is indicated.

However, the evidence for the efficacy of pneumococcal vaccination in patients with COPD is inconclusive. One small, randomized controlled trial failed to demonstrate vaccine efficacy for pneumococcal infection-related or other medical outcomes in the heterogeneous group of subjects labeled as high-risk. Case-controlled trials suggest an effectiveness of 65 to 84 percent among high-risk patients including those with COPD.

3. Annual Influenza Vaccination - Annual influenza vaccination is recommended for individuals with chronic pulmonary disease unless contraindicated due to severe anaphylactic hypersensitivity to egg protein. Influenza vaccination has been shown to be 30 to 80 percent effective in preventing illness, complications, and death in high-risk populations. Pneumococcal and influenza vaccines can be administered concurrently at different sites without increasing side effects.

Patients with COPD should avoid environmental exposures that exacerbate their symptoms (e.g., occupational exposures, second-hand smoke, air pollution).

4. Nutritional Counseling - Should include good dietary habits and encourage adjustment of weight to approximate ideal body weight. If malnourished, attempts should be made to restore nutritional balance with several small meals a day to help maintain caloric needs but avoid undue dyspnea. Forced nutrition or special diets are not recommended.

5. Pulmonary Rehabilitation - Referral is indicated in patients on optimal medical therapy and who:

• Continue to display severe respiratory symptoms, including dyspnea
• Have had several emergency room or hospital admissions per year
• Exhibit limited functional status, restricting activities of daily living
• Experience impairment in quality of life

K. Be Aware of Precautions and Recommendations for Use of Medications and Aerosols
 

1. Precautions when Using Beta2-Agonists

• Watch for nonimprovement or paradoxical deterioration with aerosol use.
  
  
• Use spacers to improve compliance.
  
  
• Avoid overuse; check number of metered dose inhalers (MDIs) used per month against number of puffs per MDI (200 to 300+, depending on brand).
  
  
• Instruct client on maximum number of puffs per day (usually 8 to 12) and on number allowed during an exacerbation (e.g., 12 to 24 over 3 to 4 hours) before additional intervention is required.
  
  
• If a long-acting agent is used, caution client that frequent use must be avoided.
  
  
• Home updraft nebulizers with inhalant solutions providing large dosages are rarely needed.
  
  
2. Precautions when Using Theophylline
 
• Initiate treatment with a low dose (e.g., 400 mg/day) and adjust after a few days.
  
  
• Reduce dosage if drug clearance is likely to be impaired because of illness, liver malfunction, or concomitant drugs.
  
  
• Do not allow any additional theophylline preparation to be taken.
  
  
• Drug must be taken at the same time each day with respect to meals.
  
  
• When symptoms change, acute illness develops, new drugs are added, or symptoms suggestive of toxicity develop, check serum level of theophylline.
  
  
• Aim for a serum level of 5 to 12 g/ml; adjust dosage and follow serum level when indicated.
  
  
3. Precautions when Using Ipratropium
 
• Clients should generally use a spacer and should avoid spraying into eyes.
  
  
• Be prepared to increase dose if necessary from 2 to 3 puffs 3 to 4 times a day to 6 to 8 puffs 3 to 4 times a day, if tolerated.
  
  
• Caution client that onset of effect is relatively slow; additional doses should not be taken for acute symptom relief.
  
  
• Monitor for side effects, e.g., tachycardia, dry mouth, glaucoma, prostatism, or bladder neck obstruction.
  
  
4. Precautions when Using Oral Steroids
 
• Reduce dosage to lowest effective daily dose or to alternate-day dosing as quickly as symptoms allow.
  
  
• Monitor for hypertension, diabetes, weight gain, mental changes, infections, central polar cataracts, skin thinning, purpura, osteoporosis, and osteonecrosis.
  
  
• Distinguish psychological benefit from true pulmonary benefit by following forced expiratory volume in 1 second (FEV₁) for 2 weeks after initiating therapy

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• Administer prophylactic calcium therapy to women; treat osteoporosis appropriately.
  
  
• Steroid-dependent clients require steroid coverage during any crisis for many months after stopping steroids.
  
  
• Repeatedly evaluate client to determine whether steroid therapy can be discontinued.
  
  
5. Precautions when Using Aerosol Steroids
 
• Seek objective evidence of the value of this therapy, because its use may decrease compliance with other aerosol usage.
  
  
• Use a spacer; monitor for oral thrush and laryngeal dysfunction.
  
  
• Be aware that aerosol steroid side effects may occur in skin, bone, etc.
  
  
• When introducing aerosol steroids in a client on an oral steroid, wean slowly off the oral drug.
  
  
TABLE OF EVIDENCE



Intervention	References	Grade of Evidence	Strength of Recommendation
Precautions when using pharmacotherapy.	ATS 1995	C	1


6. Recommendation - The fewest inhalers with the fewest inhalations at the least frequent interval consistent with a client's inhaler needs should be used to improve compliance. A similar principle applies to oral medications.

7. Recommendation - MDI with, if necessary, a spacer is preferred unless not recommended by manufacturer; a hand-held nebulizer should be used only if client unable to use an MDI.

L. Follow-Up as Indicated, Including Education - For mild COPD, spirometry is the test used for measuring disease progression. As the disease becomes more severe, oximetry and ABG assume greater importance. The frequency of obtaining these measures is based on clinical symptoms and status. In general, patients with mild COPD should be seen annually; moderate COPD, 6 months to 1 year, depending upon status; and severe COPD, every 6 months at a minimum.