On completion of this article, readers should be able to:
- Recognize that spirometry is required for a definitive diagnosis of chronic obstructive pulmonary disease (COPD) and that alpha-1 antitrypsin deficiency should be excluded in patients with suspected COPD through an appropriate blood test.
- Properly assess the severity of a patient’s COPD using recommended indices, including the BODE index, and base treatment on this assessment of severity using international guidelines.
- Summarize the benefits of and appropriately refer COPD patients for pulmonary rehabilitation.
Respiration is the metronome of life, its rhythmic timekeeper.
Breathe in. Breathe out.
Most of us do not have to work at breathing—it just happens naturally. However, for patients with COPD, the next breath is far from certain. For them, breathing becomes a laborious process, punctuated by bouts of coughing and wheezing.
Breathe in. Breathe out.
Comprising chronic bronchitis (inflammation of the airways) and emphysema (damage to the alveoli of the lung), COPD is characterized by chronic lung obstruction that prevents proper gas exchange and leads to inadequate oxygen levels. Primarily caused by smoking, COPD is a progressive disease and, along with other chronic lower respiratory diseases, is currently the third leading cause of death and the twelfth leading cause of morbidity in the U.S.1 COPD-related mortality has decreased slightly in men in the past decade, but no such change has been seen for women or the population as a whole. An estimated 13.7 million Americans have been diagnosed with COPD (2011 data), and many more likely remain undiagnosed.2
Patients with COPD can experience acute exacerbations, which are characterized by a sudden worsening of symptoms and often result in costly hospitalizations. Total COPD-related costs for 2010 are estimated at $49.9 billion—$29.5 billion of which can be attributed to the direct costs of medical care.3
Breathe in. Breathe out.
Patients with COPD face a greatly reduced quality of life.
Much of their energy is spent on the very act of breathing.
Breathe in. Breathe out.
Indeed, many patients with COPD find it hard to maintain their weight because, according to Luca Paoletti, M.D., Assistant Professor in the Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine at MUSC, "they consume a lot of energy just breathing."
Breathe in. Breathe out.
Finding it hard to catch their breath, they can no longer participate fully in activities of daily living. The morning routine—getting out of bed, showering, having breakfast, travelling to work—which may have taken these patients an hour before COPD can now take several hours and become enough of an obstacle to render them house-bound.
Breathe in. Breathe out.
Too often, these limitations result in a very sedentary lifestyle for patients, leading to further loss of lung function and deterioration of health and mental well-being.
Breathe in. Breathe out.
Breathe in. Breathe out.
Breathe in. Breathe out.
Deadly But Preventable
In 2010, COPD was the underlying cause of 133,575 U.S. deaths and the primary reason for 10.3 million physician office visits, 1.5 million emergency department visits, and 699,000 hospitalizations.2 COPD can be deadly and places an enormous burden on health care systems, but it is also preventable.
Tobacco smoking is the primary risk factor for COPD. In the opinion of K. Michael Cummings, PhD, MPH, an internationally known investigator on tobacco policy who leads research on tobacco control, public policy, and smoking cessation within MUSC Hollings Cancer Center’s Cancer Prevention and Control program, "Quitting smoking is the single most important thing that COPD patients can do to manage their symptoms and extend their lives." Lynn Schnapp, M.D., Director of the Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine in the Department of Medicine at MUSC, concurs: "If patients continue to smoke, COPD will continue to get worse. If they stop, it will plateau. Patients can stop the progression by stopping smoking."
For providers in busy practices who are treating patients with multiple chronic diseases, taking time to counsel patients to quit smoking can be challenging. However, as little as three minutes of smoking cessation counseling by a physician has been linked to a 5% to 10% improvement in quit rates.3 Patients who are trying to stop smoking can also avail themselves of online resources, such as the American Cancer Association’s Quit Now program (https://www.quitnow.net/Program/) and the American Lung Association’s Freedom from Smoking program (http://www.lung.org/stop-smoking/how-to-quit/getting-help/).
Patients should not be duped by claims that modern cigarettes are safer than those of a generation ago. "Modern cigarette designs (i.e., filters and additives) have allowed smokers to take deeper, longer puffs of smoke into their airway," notes Dr. Cummings. "The cigarettes on the shelf today may actually be more dangerous than those sold 60 years ago."
Of course, COPD is best prevented by convincing people not to begin smoking in the first place. Legislative clean air acts, which designate smoke-free zones, have proven effective in dissuading youth from smoking. "Why I’m a big advocate of these initiatives is that children don’t start smoking," remarks Timothy P.M. Whelan, M.D., Associate Professor in the Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine. "My dad was a heavy smoker, and I thought that was the norm. For my kids, not smoking is the norm. That’s huge."
Myths About COPD Impede Guideline Implementation
Although evidence-based international guidelines (i.e., The Global Initiative for Chronic Obstructive Lung Disease [GOLD],4 American Thoracic Society/European Respiratory Society1) have been established for COPD diagnosis and management, physicians admit to spending less time in short clinic visits on COPD than on other chronic diseases such as hypertension, diabetes, and hyperlipidemia. Many mistakenly believe that there are fewer effective interventions for COPD and that early intervention provides little benefit. Such myths about COPD, whether about its natural history, diagnosis, or management, impede the implementation of guideline-based care.
Myth 1: Decline in lung function begins slowly and accelerates as COPD becomes more severe.
Fletcher and Peto were among the first to describe the natural history of COPD5 and are credited with recognizing the risks of smoking and demonstrating that smoking cessation is crucial to prevent disease progression. However, one of their fundamental assumptions—that COPD progresses slowly with most of the damage occurring in later stages of the disease—had the unintended consequence of undercutting the value of early diagnosis and intervention. Recent research, however, suggests that the most rapid and preventable lung decline occurs in the early stages of COPD,6 and the value of early intervention has been demonstrated in clinical trials (UPLIFT,7 TORCH8). Early intervention can include intensive smoking cessation efforts, pharmacotherapy, and pulmonary rehabilitation.
Diagnosis and Assessment
Myth 2: Only smokers develop COPD.
Those with alpha-1 antitrypsin deficiency are prone to develop advanced emphysema. In patients with this deficiency, the enzyme neutrophil elastase, typically kept in check by alpha-1 antitrypsin, breaks down the elastin of the lungs, eventually resulting in emphysema. Because the treatment for this deficiency (i.e., plasma-derived alpha-1 augmentation therapy) differs markedly from usual COPD management, all patients with suspected COPD should be screened once in their lifetime for this deficiency using a simple blood test. Since 3% of the U.S. population carries an abnormal gene for alpha-1, family genetic testing in these patients is important.
Myth 3: COPD can be diagnosed accurately on the basis of patient symptoms and history.
Although international guidelines advise that a definitive diagnosis of COPD can be established only via spirometry,1,4 some physicians continue to believe that they can arrive at a diagnosis based solely on symptoms (e.g., progressive shortness of breath that worsens with exercise, a chronic cough, and production of sputum) and history (e.g., smoking history, family history of COPD, secondhand smoke exposure). However, research has shown that, when diagnosis relies on symptoms and history alone, many cases of early-stage COPD are missed and a number of other diseases (e.g., interstitial lung disease and heart failure) are misdiagnosed as COPD and do not receive appropriate treatment.9 If post-bronchodilator spirometry does not confirm COPD (FEV1/FVC ≤70% in patients with shortness of breath, chronic cough, and sputum production), other underlying pathologies should be investigated.
Myth 4: Pulmonary rehabilitation is not widely available, and patients are unlikely to commit to such a program.
Although most patients with COPD would benefit from pulmonary rehabilitation (PR), only 3% to 16% of suitable patients with COPD are referred for PR, with only 1% to 2% eventually receiving treatment.10 Pulmonary rehabilitation programs, which typically last about six weeks, teach patients breathing exercises to maximize their oxygen intake, strategies to manage exacerbations, and exercises that improve their stamina. Patients also benefit from the social support provided by their peers and the PR staff. Strong evidence shows that PR reduces the number and intensity of exacerbations, improves quality of life, and increases exercise tolerance.10 Patients who are short of breath may at first find it counterintuitive to exercise, but they soon see its value as their stamina and quality of life improve. To maintain these gains, patients must continue to exercise regularly after graduating from the PR program. Certified PR programs are available throughout South Carolina (for a list, see http://scacvpr.org/~scacvpro/?q=content/certified-programs-0). Pulmonary rehabilitation is reimbursed by Medicaid and Medicare and by most private payors. Patients with severe resting hypoxemia should also receive supplemental oxygen, which is the only intervention to date that has been shown to confer a survival benefit.4
Myth 5: Systemic corticosteroids, effective for treating asthma attacks, also offer a long-term treatment option for COPD.
The symptoms of an asthma attack and a COPD exacerbation can be very similar: shortness of breath, coughing, and wheezing. However, airflow obstructions are reversible in asthma but not in COPD, meaning that lung function returns to near normal after an asthma attack, but that symptoms continue, albeit in a lesser form, after a COPD exacerbation subsides. Short-term systemic corticosteroids can be used effectively to treat the symptoms of both asthma attacks and COPD exacerbations. Physicians who prescribe systemic corticosteroids for an exacerbation may be tempted to continue to do so to control chronic COPD symptoms. Guidelines strongly recommend against doing so because long-term use has been associated with increased morbidity (e.g., diabetes and osteoporosis).4
Pulmonary rehabilitation programs can improve patients' quality of life and provide needed social support.
Instead, bronchodilators (e.g., ß2-agonists and anticholinergics) should be the mainstay of treatment for COPD patients.4 Inhaled bronchodilators are preferred over oral agents because they have better efficacy and a more attractive side effect profile. Lower-dose combination regimens often prove more effective and result in fewer adverse effects than higher-dose monotherapy. Although short-acting bronchodilators can be effective for short-term symptom relief, long-acting bronchodilators provide a more sustained benefit. A combined regimen of a long-acting bronchodilator and an inhaled corticosteroid has also been shown to be effective for moderate-to-severe COPD that does not respond to bronchodilators alone. However, monotherapy with long-term inhaled corticosteroids is not recommended because of possible side effects (e.g., pneumonia).4
Lung Volume Reduction Surgery
Myth 6: Little can be done to improve breathing function in patients whose lungs have been damaged by COPD.
In patients with emphysema, the alveoli or air sacs of the lung are enlarged and less efficient at oxygenation. The elasticity of the lungs is also reduced as the small airways in them weaken, making exhalation more difficult and leading to hyperinflation. Removing areas of diseased lung, whether by surgical or nonsurgical means, can improve breathing efficiency.
Lung volume reduction surgery (LVRS), which typically removes 20% to 35% of diseased tissue from each lung, has been shown to improve survival in select patients with medication-refractory COPD. The National Emphysema Treatment Trial, a prospective, multicenter trial that randomized patients to either LVRS or medical therapy, showed that patients with upper lung involvement and poor exercise tolerance had the greatest benefit from surgery.11 Patients with more diffuse emphysema and those with extremely poor lung function are not candidates for this surgery. Potential complications include prolonged air leakage (40% of patients), pneumonia (15%), bleeding (2% to 5%), heart attack (1%), stroke (<1%), and death (3% to 8%). Because LVRS is available only at centers with resources sufficient to undertake this complex procedure and because it has relatively high complication and mortality rates, nonsurgical procedures for lung volume reduction are under development.
Nonsurgical Alternatives for Lung Volume Reduction
Charlie Strange, M.D., Professor in the Division of Pulmonary, Critical Care, Allergy, and Sleep Medicine at MUSC, is leading the U.S. RENEW trial (Lung Volume Reduction Coil Treatment in Patients With Emphysema; NCT01608490), which is seeking to assess the safety and efficacy of emphysema coils for treating patients with COPD (specifically emphysema) compared with standard care. Approximately ten coils are inserted via bronchoscopy into each lobe of the lungs; the coils are straight when inserted but recoil once in place, compressing damaged tissue to improve breathing function. The coils also tether open some of the small airways of the lung, help restore their elasticity, and improve patients’ ability to exhale fully.
This technology has been standard of care for patients with severe emphysema in Europe since 2010 and has received CE mark approval, indicating that it complies with health, safety, and environmental protection legislation of the European Union. CE mark approval was based on the results of a multicenter feasibility study (NCT01328899) conducted at 11 investigational sites12 showing significant improvements in lung function, exercise capacity, and quality of life at six months with minimal risk. At one year, gains in exercise capacity and improved quality of life were maintained in 74% and 96% of study participants, respectively. Emphysema coils are being trialed in patients with localized and diffuse emphysema and, if proven efficacious for both, could mark an important therapeutic advance over LVRS, which is typically restricted to patients with localized emphysema.
Other investigational alternatives to reducing lung volume include bronchoscopic thermal vapor ablation to burn away diseased and poorly functioning tissue in patients with predominantly upper lobe COPD (Bronchoscopic Thermal Vapor Ablation [BTVA] for Lung Volume Reduction; NCT01041586)13 and bronchoscopic placement of unidirectional valves that allow passage of gas out of but not into the diseased areas of the lungs.14
Lung transplant is often the best option for patients at highest risk of death over the next four years (BODE index [body mass index, airflow obstruction, dyspnea index, and exercise tolerance] score >7) who meet transplant criteria. According to Dr. Whelan, the Medical Director of MUSC’s Lung Transplant program: “It is clear that lung transplant improves quality of life for these patients.” After successful lung transplant, patients can lead a fuller social life and resume most activities of daily living, including housework, shopping, hobbies, and other leisure activities. The BODE index, an increasingly popular tool for predicting mortality in patients with COPD, relies on spirometric assessment of airflow obstruction, along with six-minute walk test results and scores on the Modified Medical Research Council Dyspnea scale. For a BODE index calculator, visit http:/reference.medscape.com/calculator/bode-index-copd
Not a Death Sentence
Although incurable, COPD does not have to be a death sentence. Available therapies can help control symptoms, slow disease progression, and improve quality of life and stamina. Early diagnosis of COPD using spirometry allows for prompt intervention that further improves outcomes. The time physicians spend with patients counseling smoking cessation, encouraging pulmonary rehabilitation, explaining the proper use of bronchodilators, and discussing the various treatment options available for more advanced disease can dramatically improve these patients’ lives and begin to reduce the high morbidity and mortality currently associated with COPD.
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2 Ford ES, et al. COPD Surveillance—United States, 1999-2011.Chest 2013; 144(1):284-305.
3 National Heart Lung and Blood Institute. Morbidity and Mortality: 2009 Chart Book on Cardiovascular, Lung, and Blood Diseases. Bethesda, MD: National Heart, Lung, and Blood Institute; 2009.
4 Global Initiative for Chronic Obstructive Lung Disease. Pocket guide to COPD diagnosis, management, and prevention. A guide for health care professionals. (Updated 2014). Available at http://www.goldcopd.org
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8 Jenkins CR, et al. Efficacy of salmeterol/fluticasone propionate by GOLD stage of chronic obstructive pulmonary disease: analysis from the randomised, placebo-controlled TORCH study. Respir Res. 2009;10:59. Available at http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2714501/.
9 Walters JA, et al. Factors associated with misdiagnosis of COPD in primary care. Prim Care Respir J 2011; 20(4): 396-402.
10 Johnston K, Grimmer-Somers K. Pulmonary rehabilitation: overwhelming evidence but lost in translation? Physiother Can. 2010;62(4):368–373.
11 Fishman A, et al. A randomized trial comparing lung-volume-reduction surgery with medical therapy for severe emphysema. N Engl J Med 2003:348(21):2059-2073.
12 Slebos DJ, et al. Bronchoscopic lung volume reduction coil treatment of patients with severe heterogeneous emphysema. Chest 2012 Sep;142(3):574-582.
13 Hopkins PM, et al. The efficacy of bronchoscopic thermal vapor ablation (BTVA) In patients with upper lobe emphysema. Am J Respir Crit Care Med 183;2011:A6089.
14 Berger RL, et al. Lung volume reduction therapies for advanced emphysema: an update. Chest 2010; 138(2):407-417.
15 Smeritschnig B, et al. Quality of life after lung transplantation: a cross-sectional study. J Heart Lung Transplant 2005; 24(4):474-480.