What is emphysema

Emphysema is pathologically defined as an abnormal permanent enlargement of air spaces distal to the terminal bronchioles, accompanied by the destruction of air sacs (alveolar walls) in the lungs and without obvious fibrosis¹. Clinically, the term emphysema is used interchangeably with chronic obstructive pulmonary disease (COPD). Chronic obstructive pulmonary disease (COPD), which includes chronic bronchitis and emphysema, is a chronic lung disease that makes it hard to breathe ². The disease is increasingly common, affecting millions of Americans, and is the third leading cause of death in the U.S. The good news is chronic obstructive pulmonary disease (COPD) is often preventable and treatable.

The bronchi, bronchioles, and alveoli of the lungs are normally very elastic. They easily stretch and then return to their original size during the process of breathing. The alveoli fill up with air like tiny balloons when you breathe in, and then easily deflate with each exhalation. With emphysema, the walls between many of the alveoli are destroyed and the airway loses much of its normal shape.

As a result, the airways and alveoli are no longer able to bounce back to their original shape, especially during exhalation. Some of the airways collapse, and consequently some alveoli don’t properly deflate. Collapsed airways become narrow, which obstructs normal airflow. When airways collapse or alveoli are destroyed, air can get trapped in the lungs. This causes parts of the lung to become enlarged (hyperinflated). Destruction of the alveoli also hampers the exchange of oxygen and carbon dioxide. This leads to too little oxygen and too much carbon dioxide in the blood.

Emphysema (COPD) can happen due to smoking (and exposure to secondhand smoke). Cigarette smoking is the leading cause of emphysema (COPD). Most people who have emphysema (COPD) smoke or used to smoke. However, up to 25 percent of people with chronic obstructive pulmonary disease (COPD) never smoked. Long-term exposure to other lung irritants—such as air pollution, chemical fumes, or factory dusts—also may contribute to COPD. A rare genetic condition called alpha-1 antitrypsin deficiency can also cause the disease, especially if they also smoke.

You may notice that you’re always tired and short of breath (this is one of the main emphysema symptoms, but it can occur so gradually you may not be aware of it), and that your lips and fingertips look bluish (this is due to lack of oxygen).

Emphysema vs COPD

In the United States, the term COPD (chronic obstructive pulmonary disease) includes two main conditions ³:

1. Emphysema and
2. Chronic bronchitis.

In emphysema, the walls between many of the air sacs are damaged. As a result, the air sacs lose their shape and become floppy. This damage also can destroy the walls of the air sacs, leading to fewer and larger air sacs instead of many tiny ones. If this happens, the amount of gas exchange in the lungs is reduced.

In chronic bronchitis, the lining of the airways stays constantly irritated and inflamed, and this causes the lining to swell. Lots of thick mucus forms in the airways, making it hard to breathe.

Most people who have COPD have both emphysema and chronic bronchitis, but the severity of each condition varies from person to person. Thus, the general term COPD is more accurate.

Epidemiology of Emphysema

The National Health Interview Survey reports the prevalence of emphysema at 18 cases per 1000 persons and chronic bronchitis at 34 cases per 1000 persons ⁴. While the rate of emphysema has stayed largely unchanged since 2000, the rate of chronic bronchitis has decreased. This prevalence is based on the number of adults who have ever been told by any health care provider that they have emphysema or chronic bronchitis. This is felt to be an underestimation because most patients do not present for medical care until the disease is in its later stages.

The Burden of Obstructive Lung Disease (BOLD) study showed that the worldwide prevalence of COPD (stage II or higher) was 10.1% ⁵. This figure varied by geographic location and by sex with a pooled prevalence among men of 11.8% (8.6-22.2%) and among women of 8.5% (5.1-16.7%). The differences can, in part, be explained by site and sex differences in the prevalence of smoking. These rates are similar to rates observed in the Proyecto Latino Americano de Investigacion en Obstruccion Pulmonar study, which studied 5 countries in Latin America ⁶.

In the past, COPD was more prevalent among men; however, this was attributed to the difference in smoking rates of men versus women. With the increase in smoking among women over the past 30 years, the sex difference has declined. Some studies have suggested women may be even more susceptible to the development of emphysema ⁷, ⁸.

Figure 1. Normal Lungs and Lungs With Emphysema (COPD)

Figure 2. Frontal Chest radiograms (X-ray) between A. Normal patient and B. Emphysema (COPD) patient

Note: A, Frontal posteroanterior (PA) chest radiograph shows no abnormality of the pulmonary vasculature, with normal intercostal spaces and a diaphragmatic dome between the 6th and 7th anterior ribs on both sides. B, Image in a patient with emphysema demonstrating reduced pulmonary vasculature resulting in hyperlucent lungs. The intercostal spaces are mildly enlarged, and the diaphragmatic domes are straightened and below the extremity of the seventh rib

As a result, your body does not get the oxygen it needs. Emphysema makes it hard to catch your breath. You may also have a chronic cough and have trouble breathing during exercise.

The most common cause is cigarette smoking. If you smoke, quitting can help prevent you from getting the disease. If you already have emphysema, not smoking might keep it from getting worse. Treatment is based on whether your symptoms are mild, moderate or severe. Treatments include inhalers, oxygen, medications and sometimes surgery to relieve symptoms and prevent complications.

Figure 3. Emphysema

Note: Chest radiograph of an emphysematous patient shows hyperinflated lungs with reduced vascular markings. Pulmonary hila are prominent, suggesting some degree of pulmonary hypertension

Lung emphysema

Pathologically defined as permanent enlargement of airspaces distal to the terminal bronchioles, emphysema creates an environment leading to a dramatic decline in the alveolar surface area available for gas exchange. Loss of individual alveoli with septal wall destruction leads to airflow limitation via two mechanisms. First, loss of alveolar wall results in a decrease in elastic recoil, which subsequently limits airflow. Second, loss of alveolar supporting structures is indirectly responsible for airway narrowing, again limiting airflow ⁹.

Though the paradigm for classification continues to evolve, the described morphological pathology of region-specific emphysema remains in three types ¹⁰:

• Centriacinar (centrilobular) emphysema
• Panacinar (panlobular) emphysema
• Paraseptal emphysema

Centrilobular emphysema

Centrilobular emphysema is the most common type of pulmonary emphysema mainly localized to the proximal respiratory bronchioles with focal destruction and predominantly found in the upper lung zones. The surrounding lung parenchyma is usually normal with untouched distal alveolar ducts and sacs. Also known as centrilobular emphysema, this entity is associated with and closely-related to long-standing cigarette smoking and dust inhalation ¹¹.

Figure 4. Centrilobular emphysema

Panacinar emphysema

Panacinar emphysema destroys the entire alveolus uniformly and is predominant in the lower half of the lungs. Panacinar emphysema generally is observed in patients with homozygous (Pi ZZ) alpha1-antitrypsin deficiency. In people who smoke, focal panacinar emphysema at the lung bases may accompany centriacinar emphysema ¹¹.

Figure 5. Panacinar (panlobular) emphysema

Paraseptal emphysema

Paraseptal emphysema, also known as distal acinar emphysema, preferentially involves the distal airway structures, alveolar ducts, and alveolar sacs. The process is localized around the septae of the lungs or pleura. Although airflow is frequently preserved, the apical bullae may lead to spontaneous pneumothorax. Giant bullae occasionally cause severe compression of adjacent lung tissue ¹¹.

Figure 6. Paraseptal emphysema

Emphysema life expectancy

Various measures have been shown to correlate with prognosis in COPD, including forced expiratory volume in 1 second (FEV1), diffusion capacity for carbon monoxide (DLCO), blood gas measurements, body mass index (BMI), exercise capacity, and clinical status. A correlation has also been established between radiographic severity of emphysema and mortality ¹².

A widely used simple prognostication tool is the BODE index, which is based on the body mass index (BMI), obstruction (FEV1), dyspnea (using Medical Research Council Dyspnea Scale), and exercise capacity (i.e., 6-minute walk distance).

You can make an estimated calculation for your BODE Index for COPD life expectancy with a BODE Index calculator (https://reference.medscape.com/calculator/bode-index-copd). Here’s what you’ll need before you enter information into the calculator:

1. FEV1 percentage predicated after using a bronchodilator to open airways from your pulmonary function test
2. Your 6-minute walk test distance
3. Body mass index
4. Level of dyspnea or difficulty breathing.

After you enter the information, an approximate survival prediction will show. Of course, it’s important to remember that this is a tool to help estimate life expectancy, and you need to discuss your BODE Index and COPD with your doctor.

BODE index

Body mass index (BMI) is scored as follows:

• Greater than 21 kg/m² = 0 points
• Less than 21 kg/m²= 1 point

FEV1 (postbronchodilator percent predicted) is scored as follows:

• Greater than 65% = 0 points
• 50-64% = 1 point
• 36-49% = 2 points
• Less than 35% = 3 points

Modified Medical Research Council (MMRC) dyspnea scale is scored as follows:

• MMRC 0 = Dyspneic on strenuous exercise (0 points)
• MMRC 1 = Dyspneic on walking a slight hill (0 points)
• MMRC 2 = Dyspneic on walking level ground; must stop occasionally due to breathlessness (1 point)
• MMRC 3 = Dyspneic after walking 100 yards or a few minutes (2 points)
• MMRC 4 = Cannot leave house; dyspneic doing activities of daily living (3 points)

Six-minute walking distance is scored as follows:

• Greater than 350 meters = 0 points
• 250-349 meters = 1 point
• 150-249 meters = 2 points
• Less than 149 meters = 3 points

BODE’s approximate 4-year survival is as follows:

• 0-2 points = 80%
• 3-4 points = 67%
• 5-6 points = 57%
• 7-10 points = 18%

Mortality/morbidity

A US Centers for Disease Control and Prevention (CDC) Morbidity Mortality Weekly Report study of the National Vital Statistics System reported an age-standardized death rate from COPD in the United States for adults older than 25 years of 2.3 deaths per 100,000 population ¹³. This rate varied by location, with the lowest rate in Hawaii (27.1 deaths per 100,000 population) and the highest rate in Oklahoma (93.6 deaths per 100,000 population).

What causes emphysema

Over time, exposure to irritants that damage your lungs and airways can cause chronic obstructive pulmonary disease (COPD), which includes chronic bronchitis and emphysema. The main cause of COPD is smoking, but nonsmokers can get COPD too.

Smoking

About 85 to 90 percent of all COPD cases are caused by cigarette smoking. When a cigarette burns, it creates more than 7,000 chemicals, many of which are harmful. The toxins in cigarette smoke weaken your lungs’ defense against infections, narrow air passages, cause swelling in air tubes and destroy air sacs—all contributing factors for COPD.

Your Environment

What you breathe every day at work, home and outside can play a role in developing COPD. Long-term exposure to air pollution, secondhand smoke and dust, fumes and chemicals (which are often work-related) can cause COPD.

Alpha-1 Deficiency

A small number of people have a rare form of COPD called alpha-1 deficiency-related emphysema. This form of COPD is caused by a genetic (inherited) condition that affects the body’s ability to produce a protein (Alpha-1) that protects the lungs.

Intravenous drug user

Emphysema occurs in approximately 2% of persons who use intravenous drugs. This is attributed to pulmonary vascular damage resulting from the insoluble filler (eg, cornstarch, cotton fibers, cellulose, talc) contained in methadone or methylphenidate.

The bullous cysts found in association with intravenous use of cocaine or heroin occur predominantly in the upper lobes. In contrast, methadone and methylphenidate injections are associated with basilar and panacinar emphysema.

Immune deficiency syndromes

Human immunodeficiency virus (HIV) infection was found to be an independent risk factor for emphysema, even after controlling for confounding variables such as smoking, intravenous drug use, race, and age ¹⁴.

Apical and cortical bullous lung damage occurs in patients who have autoimmune deficiency syndrome and Pneumocystis carinii infection. Reversible pneumatoceles are observed in 10-20% of patients with this infection.

Vasculitis

Hypocomplementemic vasculitis urticaria syndrome (HVUS) may be associated with obstructive lung disease.

Other sequellae include angioedema, nondeforming arthritis, sinusitis, conjunctivitis, and pericarditis.

Connective-tissue disorders

Cutis laxa, a disorder of elastin that is characterized most prominently by the appearance of premature aging. The disease is usually congenital, with various forms of inheritance (ie, dominant, recessive). Precocious emphysema has been described in association with cutis laxa as early as the neonatal period or infancy. The pathogenesis of this disorder includes a defect in the synthesis of elastin or tropoelastin.

Marfan syndrome is an autosomal dominant inherited disease of type I collagen characterized by abnormal length of the extremities, subluxation of the lenses, and cardiovascular abnormality. Pulmonary abnormalities, including emphysema, have been described in approximately 10% of patients.

Ehlers-Danlos syndrome refers to a group of inherited connective-tissue disorders with manifestations that include hyperextensibility of the skin and joints, easy bruisability, and pseudotumors.

Salla disease

Salla disease is an autosomal recessive storage disorder described in Scandinavia; the disease is characterized by intralysosomal accumulation of sialic acid in various tissues. The most important clinical manifestations are severe mental retardation, ataxia, and nystagmus.

Emphysema/COPD Risk Factors

Smoking is the biggest risk factor for chronic obstructive pulmonary disease (COPD), which includes chronic bronchitis and emphysema. It increases your risk of both developing and dying from COPD. Approximately 85 to 90 percent of COPD cases are caused by smoking. Female smokers are nearly 13 times as likely to die from COPD as women who have never smoked; male smokers are nearly 12 times as likely to die from COPD as men who have never smoked.

Other risk factors for COPD include:

• Exposure to air pollution
• Breathing secondhand smoke
• Working with chemicals, dust and fumes
• A genetic condition called Alpha-1 deficiency
• A history of childhood respiratory infection

5 Steps to Reduce Your Risk for COPD

If you are concerned about getting COPD, there are steps you can take to protect yourself.

1. If you are a smoker, STOP SMOKING. Quitting smoking is the single most important thing a smoker can do to live a longer and healthier life. The American Lung Association ¹⁵ has many programs to help you quit for good.
2. If you don’t smoke, don’t start. Smoking causes COPD, lung cancer, heart disease and other cancers.
3. Avoid exposure to secondhand smoke. Make your home smokefree. You’ll not only protect yourself, but your family too. Learn about your rights to a smoke free environment at work and in public places.
4. Be aware of other dangers. Take care to protect yourself against chemicals, dust and fumes in your home and at work.
5. Help fight for clean air. Work with others in your community to help clean up the air you and your family breathe.

Emphysema stages

The FEV1 is used to stage the severity of COPD. It is normalized as a percentage of predicted for healthy controls. The following Global Initiative for Chronic Obstructive Lung Disease staging system is widely used (note that the postbronchodilator FEV1 is used):

• Stage I (mild) – FEV 1 of 80% or more of predicted
• Stage II (moderate) – FEV 1 of less than 80% and 50% or more of predicted
• Stage III (severe) – FEV 1 less than 50% and 30% or more of predicted
• Stage IV (very severe) – FEV 1 less than 30% of predicted or FEV 1 less than 50% and chronic respiratory failure

Respiratory failure is defined as a PaO2 less than 60 mm Hg (kPa 8.0) or a PaCO2 higher than 50 mm Hg (kPa 6.7).

Emphysema signs and symptoms

Many people don’t recognize the symptoms of COPD until later stages of the disease. Sometimes people think they are short of breath or less able to go about their normal activities because they are “just getting older.” But shortness of breath is never normal. If you experience any of these symptoms, or think you might be at risk for COPD, it is important to discuss this with your doctor.

• Chronic cough
• Shortness of breath while doing everyday activities (dyspnea)
• Frequent respiratory infections
• Blueness of the lips or fingernail beds (cyanosis)
• Fatigue
• Producing a lot of mucus (also called phlegm or sputum)
• Wheezing

Remember: Don’t wait for symptoms to become severe because valuable treatment time could be lost. Early detection of COPD is key to successful treatment.

How Emphysema/COPD Is Diagnosed

Chronic obstructive pulmonary disease (COPD) includes both chronic bronchitis and emphysema but you may have only one of these diseases. Your doctor will ask questions about your family history with respiratory illnesses, your symptoms and the medicines you use, and ask questions about your lifestyle. He or she will conduct a physical exam and order some laboratory tests to diagnose and assess the severity of your COPD.

Health History

Your doctor will want to know if you:

• Smoke or have a history of smoking
• Are exposed to secondhand smoke, air pollution, chemicals or dust
• Have symptoms such as shortness of breath, chronic cough or lots of mucus
• Have family members who have had COPD

Testing for COPD

Spirometry: If you are at risk for COPD or have symptoms of COPD, you should be tested through spirometry. Spirometry is a simple test of how well your lungs work. For this test, you blow air into a mouthpiece and tubing attached to a small machine. The machine measures the amount of air you blow out and how fast you can blow it.

Spirometry can detect COPD before symptoms develop. Your doctor also might use the test results to find out how severe your COPD is and to help set your treatment goals.

Other tests: Your doctor may also want you to have a chest x-ray and/or other tests, such as an arterial blood gas test, which measures the oxygen level in your blood. This test can show how well your lungs are able to move oxygen into your blood and remove carbon dioxide from your blood.

Emphysema treatment

When you are diagnosed with chronic obstructive pulmonary disease (COPD), which includes chronic bronchitis and emphysema, you likely will have many questions and the answers may not always be clear at first. Not all people with COPD have the same symptoms and treatment may differ from person to person. It is important to talk to your doctor about your treatment options and to get answers to all of your questions.

Quit Smoking

The most essential step in any treatment plan for COPD is quitting smoking and to stop all smoking. It’s the only way to keep COPD from getting worse — which can eventually reduce your ability to breathe. But quitting smoking isn’t easy. And this task may seem particularly daunting if you’ve tried to quit and have been unsuccessful.

Talk to your doctor about nicotine replacement products and medications that might help, as well as how to handle relapses. Your doctor may also recommend a support group for people who want to quit smoking. It’s also a good idea to avoid secondhand smoke exposure whenever possible.

Nicotine Replacement Therapy

Supervised use of pharmacologic agents is an important adjunct to self-help and group smoking cessation programs. Nicotine is the ingredient in cigarettes primarily responsible for the addiction of smoking. Withdrawal from nicotine may cause unpleasant adverse effects (ie, anxiety, irritability, difficulty concentrating, anger, fatigue, drowsiness, depression, and sleep disruption). These effects usually occur during the first weeks after quitting smoking. Nicotine replacement therapies after smoking cessation reduce withdrawal symptoms. A person who smokes and who requires the first cigarette within 30 minutes of waking is likely to be highly addicted and would benefit from nicotine replacement therapy. Several nicotine replacement therapies are available.

Nicotine polacrilex is a chewing gum and produces improved quit rates compared to counseling alone. Transdermal nicotine patches are readily available for replacement therapy. Long-term success rates have been 22-42%, compared with 2-25% with placebos. These agents are well tolerated, and the adverse effects are limited to localized skin reactions. The use of an antidepressant medication, bupropion at 150 mg bid has been shown to be effective for smoking cessation and may be used in combination with nicotine replacement therapy.

The most recent drug to receive approval for smoking cessation is varenicline. Varenicline is a partial agonist selective for alpha4, beta2 nicotinic acetylcholine receptors. Its mechanism of action is believed to be binding the nicotinic subtype receptor, producing agonist activity while simultaneously preventing nicotine binding. Varenicline’s agonistic activity is significantly lower than nicotine’s.

COPD Medications

A variety of medicines are used to treat COPD and there is no “best” medicine for all people. Each person’s COPD is different and your doctor and healthcare team will work with you to set up the best plan to address your symptoms and needs. Doctors use several kinds of medications to treat the symptoms and complications of COPD. You may take some medications on a regular basis and others as needed.

By taking the right medicine at the right time, you can:

• Breathe better
• Do more of the things you enjoy
• Have fewer flare-ups or exacerbations

Most people with COPD take long-acting medicine every day to help shortness of breath. In addition, short-acting medicine is used on an only-when-needed basis to decrease shortness of breath. For many, a combination of medicines is prescribed.

Types of medicines often prescribed for COPD:

Bronchodilator

Bronchodilators relax the muscles around the airways which helps to keep them open and makes breathing easier. Most bronchodilators are often delivered through an inhaler or can be nebulized so you breathe the medicine straight into your lungs. Inhalers operate in different ways, so make sure to ask your healthcare team to show you how your specific inhaler works. This ensures you will get a full dose of medicine with each breath. Other bronchodilators are swallowed as a pill.

Bronchodilators can be short-acting or long-acting. Short-acting bronchodilators work quickly so that you get relief from symptoms fast, but they wear off in a few hours. Long-acting bronchodilators provide relief for many hours, but the effect is slower. Short- and long-acting bronchodilators include beta2-agonists and anticholinergics.

• Beta2-Agonists are very potent at relaxing tightened muscles around your airways. This opens the airway and makes breathing easier. Short-acting beta-agonists work within minutes but last only 4-6 hours. Long-acting beta-agonists are slow to start working but can last up to 12 hours so are used to maintain open airways throughout the day or the night.

• Anticholinergics prevent the muscles around your airways from tightening so keep the airways open and help clear mucus from your lungs. This combination allows your cough to expel mucus more easily. There are short-acting and long-acting anticholinergics. Beta-agonists and anticholinergics can be delivered through an inhaler or a nebulizer.

Depending on the severity of your disease, you may need a short-acting bronchodilator before activities, a long-acting bronchodilator that you use every day or both.

Short-acting bronchodilators include albuterol (ProAir HFA, Ventolin HFA, others), levalbuterol (Xopenex HFA), and ipratropium (Atrovent). The long-acting bronchodilators include tiotropium (Spiriva), salmeterol (Serevent), formoterol (Foradil, Perforomist), arformoterol (Brovana), indacaterol (Arcapta) and aclidinium (Tudorza).

Anti-Inflammatory

Decreasing inflammation leads to less swelling and mucus production in the airways and that makes it easier to breath. These medicines are known as corticosteroids or steroids. They are usually inhaled with an inhaler device. Make sure you rinse your mouth with water immediately after using a steroid inhaler to prevent thrush, a yeast infection of the throat.

Corticosteroids can also be swallowed as a pill and are usually delivered for short periods of time in special circumstances when your symptoms are getting more severe. Steroids have serious side effects, such as weight gain, diabetes, osteoporosis, cataracts and increased risk of infection so they must be monitored carefully. Your doctor will talk with you about these side effects.

For people who have a moderate or severe acute exacerbation, short courses (for example, five days) of oral corticosteroids prevent further worsening of COPD. However, long-term use of these medications can have serious side effects, such as weight gain, diabetes, osteoporosis, cataracts and an increased risk of infection.

Combination Medicines

A corticosteroid, an anticholinergic and a beta-agonist can be combined into one inhaler or nebulizer solution. The most common combinations contain two of these medicines as a:

• Short-acting beta-agonist and short-acting anticholinergic
• Long-acting beta-agonist and corticosteroid
• Long-acting anticholinergic and corticosteroid

Some medications combine bronchodilators and inhaled steroids. Salmeterol and fluticasone (Advair) and formoterol and budesonide (Symbicort) are examples of combination inhalers.

Antibiotics

People with COPD do experience flare-ups with more coughing, more mucus and more shortness of breath. This is often caused by bacterial or viral infections. Your doctor may give you an antibiotic or an anti-viral prescription to keep on hand and fill for when you have an infection.

Make sure you take all of the antibiotic exactly as prescribed. You may start to feel better during the antibiotic course but make sure you take the antibiotic for the prescribed duration. Short cutting the antibiotic course may allow the infection to come back or become resistant to the antibiotic.

In patients with COPD, chronic infection or colonization of the lower airways with S pneumoniae, H influenzae, and/or Moraxella catarrhalis is common. Patients with severe disease have a higher prevalence of Gram-negative organisms such as Pseudomonas. The use of antibiotics for the treatment of acute exacerbations is well supported ¹⁶. The patients who benefited most from antibiotic therapy were those with exacerbations that were characterized by at least two of the following: increases in dyspnea, sputum production, and sputum purulence (The Winnipeg criteria).

Inpatient management of acute exacerbations of COPD includes empiric antibiotic coverage with a macrolide, a beta-lactam, or doxycycline.

The prophylactic use of antibiotics, in particular azithromycin, to prevent COPD exacerbations has been explored over the past 20 years. In 2011, Albert et al ¹⁶ reported on the use of azithromycin to prevent exacerbations of COPD; they showed that among 1,142 patients with severe COPD (defined as an FEV1 of less than 40% predicted), those randomized to take 250 mg of daily azithromycin for 1 year had fewer clinical exacerbations, longer time to first exacerbation, and higher quality of life scores when compared with placebo. Adverse effects include hearing loss and prolongation of the QT interval.

In 2013, the FDA released an announcement of sudden death associated with azithromycin, stating that patients at particular risk for developing torsades de pointes were known to have preexisting prolonged QT interval, low levels of potassium or magnesium, bradycardia, history of antiarrhythmics, or known arrhythmias.

Managing exacerbations

Even with ongoing treatment, you may experience times when symptoms become worse for days or weeks. This is called an acute exacerbation, and it may lead to lung failure if you don’t receive prompt treatment.

Exacerbations may be caused by a respiratory infection, air pollution or other triggers of inflammation. Whatever the cause, it’s important to seek prompt medical help if you notice a sustained increase in coughing, a change in your mucus or if you have a harder time breathing.

When exacerbations occur, you may need additional medications (such as antibiotics, steroids or both), supplemental oxygen or treatment in the hospital. Once symptoms improve, your doctor will talk with you about measures to prevent future exacerbations, such as quitting smoking, taking inhaled steroids, long-acting bronchodilators or other medications, getting your annual flu vaccine, and avoiding air pollution whenever possible.

Vaccinations

COPD increases your risk for infection with influenza (flu) and pneumonia. Vaccines are available to protect you against the flu and you should get immunized every year. You need a yearly flu shot because the influenza virus changes slightly every year and you must get the latest and newest vaccine. Find where you can get vaccinated in your area. Pneumococcal pneumonia is a bacterial infection of the lung that often follows an influenza infection. There are two vaccines to protect against this infection. You only need to get vaccinated once but you need to get each one. Your doctor can provide these to you.

Mucolytic agents

Viscous lung secretions in patients with COPD consist of mucus-derived glycoproteins and leukocyte-derived DNA. Mucolytic agents reduce sputum viscosity and improve secretion clearance. Although mucolytic agents have been shown to decrease cough and chest discomfort, they have not been shown to improve dyspnea or lung function ¹⁷.

However, in 2009-2010, Chinese investigators designed and implemented a prospective, randomized, double-blind placebo-controlled trial, studying the effects of long-term oral N-acetylcysteine at 600 mg twice daily in subjects with GOLD stage I COPD. They found long-term use (over a year and a half) can actually prevent exacerbations in moderate disease. Interesting enough, exacerbations of COPD were the most significant adverse effect of the trial ¹⁸. The study was published in The Lancet in March of 2014.

Alpha1-antitrypsin deficiency treatment

The treatment strategies for alpha1-antitrypsin deficiency involve reducing the neutrophil elastase burden, primarily by smoking cessation, and augmenting the levels of alpha1-antitrypsin. Available augmentation strategies include pharmacologic attempts to increase endogenous production of alpha1-antitrypsin by the liver (ie, danazol, tamoxifen) or administration of purified alpha1-antitrypsin by periodic intravenous infusion or by inhalation. Tamoxifen can increase endogenous production of alpha1-antitrypsin to a limited extent, so this may be beneficial in persons with the PIZZ phenotype.

Intravenous augmentation therapy is the only available approach that can increase serum levels to greater than 11 mmol/L, the protective threshold. Studies show that the infusions can maintain levels of more than 11 mmol/L, and replacement is administered weekly (60 mg/kg), biweekly (120 mg/kg), or monthly (250 mg/kg). The ability of intravenous alpha1-antitrypsin augmentation to alter the clinical course of patients with alpha1-antitrypsin deficiency has not been demonstrated. Uncontrolled observations of patients suggest that the FEV1 may fall at a slower rate in patients who receive alpha1-antitrypsin replacement ¹⁹.

Pulmonary Rehabilitation

Pulmonary rehabilitation is an outpatient program and may be based in a hospital or a clinic. You may also be able to receive certain forms of pulmonary rehabilitation in your own home. Pulmonary rehabilitation is a program of education and exercise to increase awareness about your lungs and your disease. You will learn to achieve exercise with less shortness of breath. The classes are offered in a group setting so you get the chance to meet others with your condition, give support to them, as well as receive support from them. The skills and knowledge learned in the program will help you to feel better and manage your chronic lung disease. You become stronger by increasing your level of fitness. Exercising your lungs and your muscles helps you be more active so you can do the things you enjoy with your loved ones. Pulmonary rehabilitation may even decrease the need for hospital visits.

Pulmonary rehabilitation is recommended for patients with COPD who experience shortness of breath frequently and are not able to do daily activities despite daily medication use. Many patients in rehabilitation programs have a diagnosis of COPD, but these programs also help people with other types of chronic lung disease that limits breathing and activity.

Pulmonary rehab programs vary in format and length. Most programs use a small group format and include:

• Education about your condition, symptoms, medications and oxygen
• Supervised exercise classes and instruction
• Breathing techniques
• Nutritional counseling
• Emotional health support

Education

In pulmonary rehab, patients learn everything they need to know about their specific chronic lung disease. Sawchuk says her clients go over anatomy, physiology, breathing techniques and medication management—which includes things like the best time of day to take medications.

Exercise

Because of breathing challenges, people with chronic lung disease tend to avoid exercise. However, the right amount and type of exercise has many benefits. It can improve your heart and muscle strength, increase energy levels and help you use oxygen more efficiently.

In pulmonary rehab, patients are monitored and taught to exercise safely by paying attention to their breathing and oxygen levels, as well as learning modifications such as using a chair for weight lifting and yoga.

Breathing and Oxygen

All pulmonary rehab patients are taught pursed lipped breathing and diaphragmatic breathing exercises, and other breathing strategies, to help increase oxygen levels and better manage symptoms—and fears.

In a pulmonary rehab program, people can learn how to keep breathing issues in check and avoid supplementary oxygen. Others learn how and when to use their oxygen correctly to maximize quality of life.

Nutritional Counseling

Achieving and maintaining a healthy weight is an important part of an overall healthy lifestyle, and it’s no different for people with chronic lung disease. Excessive body weight can increase shortness of breath, and low body weight can decrease your ability to fight infections. Plus, when short of breath it can be hard to eat. People learn to prepare nutritious food without getting stressed or too tired.

Emotional Health Support

Because many people with chronic lung disease, or any chronic illness, often experience depression, anxiety and other emotional problems, many pulmonary rehab programs offer counseling services, or partner with an organization that offers them.

Maintenance

The information acquired during pulmonary rehab needs to be used throughout life in order to avoid hospitalizations, complications and other issues. Plus, importantly, people who use those tools feel better. Many programs offer a maintenance option that allows clients to return for exercise classes and support groups after the program is over.

Sleep and COPD

Patients with COPD may develop substantial decreases in nocturnal PaO2 during all phases of sleep but particularly during rapid eye movement sleep. These episodes are associated with rises in pulmonary arterial pressures and disturbance in sleep architecture initially, but patients may develop pulmonary arterial hypertension and cor pulmonale if the hypoxemia remains untreated. Therefore, patients who have a daytime PaO2 greater than 60 mm Hg but demonstrate substantial nocturnal hypoxemia should be prescribed oxygen supplementation for use during sleep.

Supplemental Oxygen

Chronic obstructive pulmonary disease (COPD) can damage the way that our lungs work to the extent that extra oxygen is needed in order to do daily tasks.

Your body needs oxygen to do everything from digesting food, daily household chores, to going to the grocery store. Sometimes with COPD, your breathing becomes restricted so much that the body needs extra oxygen or supplemental oxygen (also called oxygen therapy).

When the lung alone is unable to provide adequate oxygen, supplemental oxygen benefits the body by:

• Improving sleep and mood
• Increasing mental alertness and physical strength
• Maintaining normal body function
• Preventing heart failure in people with severe lung disease

Providing Oxygen Therapy at Home

• Oxygen can be delivered to your home as compressed oxygen gas or as liquid oxygen. Compressed oxygen gas is stored in steel and aluminum tanks or cylinders. Larger tanks are stationary and used at home. Smaller tanks are more portable for use when you leave the house. Liquid oxygen is made by cooling oxygen gas and converting it to a liquid. Since liquids can be stored in a smaller volume, liquid oxygen is more convenient than compressed oxygen gas for active people. A disadvantage of liquid oxygen is the time of usage as it is more prone to evaporate than compressed oxygen gas.
• Oxygen concentrators also are available for home use. An oxygen concentrator is an electrical device about the size of an end table. It concentrates oxygen in the air by removing nitrogen. This method is less expensive and easier to maintain as it requires no refilling of tanks. However, oxygen concentrators may give off heat and be noisy. Usage may also increase your electricity bill. You will still need a back-up source of oxygen in case of a power failure. There are several reliable portable oxygen concentrators that allow you to leave your home to work, enjoy recreational activities and travel.

You may need oxygen therapy for all or just part of the day. A doctor’s prescription is required for supplemental oxygen.

Safety Tips

There are important safety factors to keep in mind when using oxygen. Oxygen is a safe gas and is non-flammable, however it supports combustion. Materials burn more readily in an oxygen-enriched environment. Follow these tips for safe oxygen use:

• Post “No Smoking” and “No Open Flames” signs in and outside your home to remind people not to smoke
• Avoid open flames in the presence of oxygen such as matches, cigarette lighters, candles and burning tobacco. Insist that people who wish to smoke step outside your home.
• Use caution around other sources of heat, such as electric or gas heaters and/or stoves—at least 5 feet is a recommended distance between oxygen and other heat sources.
• Avoid using lotions or creams containing petroleum, which are more likely to catch fire in the presence of oxygen. Use water-based products instead.
• Store cylinders safely in an upright position, secured in an approved cart or storage device.
• Remember that oxygen supply valves should be turned off when not in use.
• Always follow the instructions of your oxygen supply company regarding safe usage.
• Losing access to oxygen can be very dangerous if you need oxygen continually. Make sure to have backup equipment (normally a large oxygen tank) and tell your power company that you have life-sustaining equipment in your home.

Other breathing treatments or assisted breathing devices are being used more frequently for people with COPD. These treatments are known as CPAP, BiPAP or nasal positive pressure ventilation.

Surgery

Some people with very severe COPD symptoms may have a hard time breathing all of the time. In some of these cases, doctors may suggest lung surgery to improve breathing. Not everyone is a candidate for lung surgery.

Are You a Candidate for Lung Surgery ?

Some people with COPD will experience fewer or less severe symptoms and be more active after surgery, but others will not benefit. Some considerations for surgery candidates include:

• You must be strong enough to have the surgery.
• You must participate in a pulmonary rehabilitation program.
• You cannot be a current smoker.

Some lung surgeries require that the lung damage must be in an area that is localized (a specific area) and can be removed. The decision for surgery is based on the results of many tests. Talk to your doctor to find out if lung surgery is right for you.

Types of Lung Surgery

There are two types of lung surgery performed to address COPD:

• Bullectomy is a procedure where doctors remove one or more of the very large bullae or blebs from the lungs. Bullae are large air sacs that form from hundreds of destroyed alveoli. These air spaces can become so large that they crowd out the better functioning lung and interfere with breathing. For those people, removing the destroyed air sacs improves breathing.

• Lung Volume Reduction Surgery is a procedure to help people with severe emphysema affecting the upper lung lobes. Lung Volume Reduction Surgery is not a cure for COPD but can improve one’s exercise capacity and quality of life. The goal of the surgery is to reduce the size of the lungs by removing about 30 percent of the most diseased lung tissues so that the remaining healthier portion can perform better. Lung Volume Reduction Surgery also can allow the diaphragm to return to its normal shape, helping you breathe more efficiently. The surgery has been shown to help improve breathing ability, lung capacity and overall quality of life among those who qualify for it.
• Endobronchial valve placement through bronchoscopy is under investigation as an alternative to Lung Volume Reduction Surgery. These valves are unidirectional and allow exhalation but do not allow inhalation. This results in a deflated lung distal to the valve. Bronchi are chosen to isolate segments of the lung that show the greatest emphysema and hyperinflation. The benefit, similar to Lung Volume Reduction Surgery, is obtained by decreasing the volume of most diseased portions of the lung. The Endobronchial Valve for Emphysema Palliation Trial (VENT) studied the safety and efficacy of this approach in a nonblinded, prospective, randomized multicenter study. Results showed a modest but significant improvement in both the FEV 1 (relative increase, 6.8%) and 6-minute walk test (relative increase, 19.1 m) in the study group. Analysis revealed that the greatest benefit was obtained by those patients with greater heterogeneity of emphysema and intact interlobar fissures. The study group unfortunately also showed significantly higher rates of COPD exacerbations and hemoptysis ²⁰.

Lung Transplants

Sometimes COPD can cause severe damage to the lungs, causing them not to function normally. Your doctor may recommend a lung transplant if the damage is not repairable. Lung transplants can improve your ability to breathe and be active. However, like any major operation, you should consider the risks and complications, such as organ rejection or needing to take immune suppressing medications daily. Talk to your doctor to find out more information about how and where to receive a lung transplant evaluation or the process for becoming an organ donor.

Lung transplantation provides improved quality of life and functional capacity but does not result in survival benefit. The lack of survival benefit makes the timing of transplant difficult. The patients selected to receive transplants should have a life expectancy of 2 years or less. Current guidelines by the International Society of Heart and Lung Transplantation recommends referring for transplantation when the BODE index (body mass index, obstruction [FEV1], dyspnea [ie, Medical Research Council Dyspnea Scale], and exercise capacity [ie, 6-min walking distance]) is greater than 5 ²¹.

Palliative Care and COPD

Palliative care is a specialty in medicine focused on treating the symptoms, pain and stress that accompany serious illnesses like COPD. It is available to you from the moment you are diagnosed and through the entire course of your illness. The goal of palliative care is to help you and your family, achieve the highest quality of life. Palliative care is appropriate for all people living with COPD regardless of stage or prognosis.

If you or a loved one has COPD, palliative care can help you in several ways including:

• Therapies to relieve the discomfort of shortness of breath or anxiety
• Education about lifestyle changes during COPD
• Medication and disease management

What Are the Benefits of Palliative Care ?

Palliative care improves quality of life by relieving overwhelming physical and emotional symptoms and improving communication with your healthcare providers.

Who Administers Palliative Care ?

Palliative care specialists include a team of doctors, nurses and social workers. Your other doctors and nurses will work to alleviate your symptoms, but palliative care specialists ensure that the goals of COPD care match your specific expectations.

How Do I Get Palliative Care ?

Anytime is the best time to discuss palliative care during your journey in COPD care. Never fear that it is too late to seek out a palliative care specialist. Ask your doctor for a palliative care referral, or you can search for a palliative care team at GetPalliativeCare.org ²².

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