COPD (Chronic Obstructive Pulmonary Disease)

Chronic obstructive pulmonary disease is a slowly progressive obstruction of airflow into or out of the lungs. The incidence of COPD has almost doubled since 1982. Experts have estimated about 32 million persons in the United States have COPD. The disease occurs slightly more often in men than in women. The symptoms (for example, shortness of breath, coughing) come on slowly and many people are consequently diagnosed after age 40-50, although some are diagnosed at a younger age. COPD patients may exhibit symptoms of chronic bronchitis, emphysema, and asthma.

Chronic obstructive pulmonary disease is a slowly progressive disease so it is not unusual for the initial signs and symptoms to be a bit different from those in the late stages of the disease. There are many ways to evaluate or stage chronic obstructive pulmonary disease, often based on symptoms.

Usually the first signs and symptoms of COPD include a productive cough usually in the morning, with colorless or white mucus (sputum).

The most significant symptom of chronic obstructive pulmonary disease is breathlessness, termed shortness of breath (dyspnea). Early on, this symptom may occur occasionally with exertion, and eventually may progress to breathlessness while doing a simple task such as standing up, or walking to the bathroom. Some people may develop wheezing (a whistling or hissing sound while breathing). Signs and symptoms of chronic obstructive pulmonary disease include:

• Cough, with usually colorless sputum in small amounts
• Acute chest discomfort
• Shortness of breath (usually occurs in patients aged 60 and over)
• Wheezing (especially during exertion)

As the disease progresses from mild to moderate, symptoms often increase in severity:

• Respiratory distress with simple activities like walking up a few stairs
• Rapid breathing (tachypnea)
• Bluish discoloration of the skin (cyanosis)
• Use of accessory respiratory muscles
• Swelling of extremities (peripheral edema)
• Over-inflated lungs (hyperinflation)
• Wheezing with minimal exertion
• Course crackles (lung sounds usually with inspiration)
• Prolonged exhalations (expiration)
• Diffuse breath sounds
• Elevated jugular venous pulse

One way to stage chronic obstructive pulmonary disease is the Global Initiative for Chronic Obstructive Lung Disease program (GOLD). The staging is based on the results of a pulmonary function test. Specifically, the forced expiratory volume (how much air one can exhale forcibly) in one second (FEV1) of a standard predicted value is measured, based on the individual patient's physical parameters. The staging of chronic obstructive pulmonary disease by this method is as follows:

• Stage I is FEV1 of equal or more than 80% of the predicted value
• Stage II is FEV1 of 50% to 79% of the predicted value
• Stage III is FEV1 of 30% to 49% of the predicted value
• Stage IV is FEV1 of less than 30% of predicted value or an FEV1 less than 50% of predicted value plus respiratory failure

Other staging methods are similar but are based on the severity of the shortness of breath symptom that is sometimes subjective. The above staging is measurable objectively, providing the patient is putting forth their best effort.

The primary cause of chronic obstructive pulmonary disease is cigarette smoking or exposure to tobacco smoke. It is estimated that 90% of the risk for development of chronic obstructive pulmonary disease is related to tobacco smoke. The smoke also can be secondhand smoke (tobacco smoke exhaled by a smoker and then breathed in by a non-smoker).

Other causes of chronic obstructive pulmonary disease are:

• Prolonged exposure to air pollution, such as that seen with burning coal or wood and with industrial air pollutants
• Infectious diseases: Infectious diseases that destroy lung tissue in patients with hyperactive airways or asthma also may contribute to causing this COPD.

Damage to the lung tissue over time causes physical changes in the tissues of the lungs and clogging of the airways with thick mucus. The tissue damage in the lungs leads to poor compliance (the elasticity, or ability of the lung tissue to expand). The decrease in elasticity of the lungs means that oxygen in the air cannot get by obstructions (for example, thick mucus plugs) to reach air spaces (alveoli) where oxygen and carbon dioxide exchange occurs in the lung. Consequently, the person exhibits a progressive difficulty, first coughing to remove obstructions like mucus, and then in breathing, especially with exertion.

People who smoke tobacco are at the highest risk for developing chronic obstructive pulmonary disease. Other risk factors include exposure to secondhand smoke from tobacco and exposure to high levels of air pollution, especially air pollution associated with wood or coal. In addition, individuals with airway hyper-responsiveness such as those with chronic asthma are at increased risk.

There is a genetic factor called alpha-1 antitrypsin deficiency that places a small percentage (less than 1%) of people at higher risk for COPD (and emphysema) because a protective factor (alpha-1 antitrypsin protein) for lung tissue elasticity is decreased or absent.

Other factors that may increase the risk for developing chronic obstructive pulmonary disease include

• intravenous drug use,
• immune deficiency syndromes,
• vasculitis syndrome,
• connective tissue disorders, and
• genetic problems such as Salla disease (autosomal recessive disorder of sialic acid storage in the body).

What other diseases or conditions contribute to COPD?

In general, three other non-genetic problems related to the lung tissue play a role in chronic obstructive pulmonary disease. 1) chronic bronchitis, 2) emphysema, and 3) infectious diseases of the lung.

• Chronic bronchitis and emphysema, are thought by many to be variations of chronic obstructive pulmonary disease and considered part of the progression of chronic obstructive pulmonary disease by many researchers. Chronic bronchitis is defined as a chronic cough that produces sputum for three or more months during two consecutive years.
• Emphysema is an abnormal and permanent enlargement of the air spaces (alveoli) located at the end of the terminal bronchioles in the lungs.
• Infectious diseases of the lung may damage areas of the lung tissue and contribute to chronic obstructive pulmonary disease.

Doctors make a preliminary diagnosis of COPD in a person with chronic obstructive pulmonary disease symptoms by noting

• his/her breathing history,
• the history of tobacco smoking or exposure to secondhand smoke, and/or
• exposure to air pollutants, and/or a history of lung disease (for example, pneumonia).

Other tests to diagnose COPD

Other tests to diagnose COPD include:

• Chest X-rays
• CT scan of the lungs
• Arterial blood gas or a pulse oximeter to look at the saturation level of oxygen in the patient's blood

In addition, the person may be sent to a lung specialist (pulmonologist) to determine their FEV1 level that is used by some physicians to stage COPD as described above in the section that describes the stages of COPD.

There are many treatments for chronic obstructive pulmonary disease. The first and best is to stop smoking immediately.

Medical treatments of chronic obstructive pulmonary disease drugs, for example, nicotine replacement therapy, beta-2 agonists and anticholinergic agents (bronchodilators), combined drugs using steroids and long-acting bronchodilators, mucolytic agents, oxygen therapy, and surgical procedures such as bullectomy, lung volume reduction surgery, and lung transplantation.

The treatments are often based on the stage of chronic obstructive pulmonary disease, for example:

• Stage I - short-acting bronchodilator as needed
• Stage II - short-acting bronchodilator as needed and long-acting bronchodilators plus cardiopulmonary rehabilitation
• Stage III - short-acting bronchodilator as needed long-acting bronchodilators cardiopulmonary rehabilitation and inhaled glucocorticoids for repeated exacerbations
• Stage IV - as needed, long-acting bronchodilators, cardiopulmonary rehabilitation, inhaled glucocorticoids, long-term oxygen therapy, possible lung volume reduction surgery and possible lung transplantation (stage IV has been termed "end-stage" chronic obstructive pulmonary disease)

The three major goals of the comprehensive treatment and management of chronic obstructive pulmonary disease are:

1. Lessen airflow limitation
2. Prevent and treat secondary medical complications (for example, hypoxemia, infection)
3. Decrease respiratory symptoms and improve quality of life

Nicotine Replacement Therapy

The first line of therapy that involves medication is related to smoking cessation with nicotine replacement therapy. Nicotine replacement therapy can help patients quit smoking tobacco because it can help reduce the withdrawal symptoms due to nicotine. Replacement therapies include nicotine-containing chewing gum and patches that allow nicotine to be absorbed through the skin. In these types of therapy, nicotine is gradually reduced. This medication can work well for those patients who are seriously attempting to quit tobacco.

Oral Medications to Quit Smoking (Smoking Cessation)

• Varenicline (Chantix) is an oral medication that is prescribed to promote cessation of smoking. This is also an alternative to try to quit smoking.
• Bupropion (Zyban) is an antidepressant that helps reduce symptoms of nicotine withdrawal.
• Some medications are used "off label" (that is, they are normally prescribed for another condition) to help people quit smoking. These drugs are recommended by the Agency for Healthcare Research and Quality to help smokers kick the habit, but have not been approved by the FDA for this use. These medications include nortriptyline (Pamelor), an older type of antidepressant. It's been found to help smokers double their chances of quitting compared to taking no medicine. Another drug used off label is clonidine (Catapres). Normally used to treat high blood pressure it can help smokers quit.

Bronchodilators

Bronchodilators are used for COPD treatment because they open up the airway tubes and allow air to more freely pass in and out of the lung tissue. There are both short-term (several hours) and long-term (12 or more hours) types of bronchodilators.

Examples of short-term bronchodilators

• albuterol (Ventolin, Proventil)
• metaproterenol (Alupent)
• levalbuterol (Xopenex)
• pirbuterol (Maxair)

Examples of long-term bronchodilators

• salmeterol (Serevent)
• formoterol (Foradil)
• arformoterol (Brovana)
• indacaterol (Arcapta)

Anticholinergicbronchodilators

• ipratropium (Atrovent)
• tiotropium (Spiriva)
• aclidinium (Tudorza)

Other bronchodilators such as theophylline (Elixophyllin, Theo-24) are occasionally used, but are not favored because of unwanted side effects including anxiety, tremors, seizures, and arrhythmias.

Also on the market are combined to drugs using steroids and long-acting bronchodilators. Roflumilast (Daxas, Daliresp) is a new drug that inhibits the enzyme phosphodiesterase type 4, has been utilized in patients with symptoms of chronic bronchitis.

Acute exacerbation of chronic obstructive pulmonary disease is one of the major reasons for hospital admission in the United States.

You may need to be hospitalized if you develop severe respiratory dysfunction, if the disease progresses, or if you have other serious respiratory diseases (for example, pneumonia, acute bronchitis). The purpose of hospitalization is to treat symptoms and to prevent further deterioration. You may be admitted to an intensive care unit (ICU) if they require invasive or noninvasive mechanical ventilation or if you have these symptoms:

• Confusion
• Lethargy
• Respiratory muscle fatigue
• Worsening hypoxemia (not enough oxygen in the blood)
• Respiratory acidosis (retention of carbon dioxide in the blood)

There are three types of surgery generally available to treat certain types of patients with COPD that include

1. Bullectomy
2. Lung volume reduction surgery
3. Lung transplant surgery

Surgery may not be available or desirable for many people with COPD.

• Bullectomy surgery is the removal of giant bullae. Air-filled spaces usually located in the lung periphery that occupy lung space most often in people with emphysema are termed bullae. Giant bullae may occupy over 33% of the lung tissue, compress adjacent lung tissue, and reduce blood flow and ventilation to healthy tissue. Surgical removal can allow compressed lung tissue that is still functional to expand.
• Lung volume reduction surgery is removal of lung tissue that has been most damaged by tobacco smoking, usually the 20% to 30% of lung tissue located in the upper part of each lung. This procedure is not done often; it is usually done on people who have severe emphysema and marked hyperinflation of the airways and air spaces.
• Lung transplantation is surgical therapy for people with advanced lung disease. People with COPD are the largest single category of people who undergo lung transplantation. In general, these people with COPD usually are at COPD stage three or four with severe symptoms and generally, without transplantation, have a life expectancy of about two years or less.

• For people with mild COPD (stage I) the prognosis is very good and they may have a relatively normal life expectancy but this decreases as the severity of staging increases.
• People with COPD who are admitted to an ICU have an estimated death rate of about 24% and this rate can double for people over age 65.
• The average life expectancy of a COPD patient who undergoes a lung transplant is about five years.
• People who have COPD and continue to smoke, have a rapid decline in FEV1, who develop severe hypoxemia, develop right-sided heart failure and/or have poor ability to do daily functions usually have a poor prognosis.

The most effective and preventative therapy for chronic obstructive pulmonary disease is to avoid contact with tobacco smoke. If you use tobacco products - quit.

Exercise for COPD

• If a person with chronic obstructive pulmonary disease has mild to moderate symptoms, often they can benefit from exercise programs that can increase their stamina and slow the advancing pace of COPD disease.

Diet, Supplements, Therapy, and Complementary Medicine for COPD

A number of over-the-counter (OTC) supplements and foods are reportedly helpful in reducing symptoms of chronic obstructive pulmonary disease. Home remedies for COPD include:

• Vitamin E to improve lung function
• Omega-3 fatty acids to decrease inflammation (found in supplements or foods such as salmon, herring, mackerel, sardines, soybeans, canola oil)
• Antioxidants to reduce inflammation (found in kale, tomatoes, broccoli, green tea, red grapes)
• Breathing techniques relaxation therapy, meditation
• Acupuncture COPD symptom reduction by needle placement

Other supplementary therapies such as treatment with antibiotics to reduce pathogen (viral, fungal, bacterial) damage to lung tissue, mucolytic agents to help unblock mucus-clogged airways, or oxygenation therapies to increase the available oxygen to lung tissues may also reduce the symptoms of COPD.

In some people, oxygen therapy will increase his/her life expectancy, and improve the quality of life. This is especially true with people with COPD who have chronically low oxygen levels in the blood. It may also help exercise endurance. Oxygen delivery systems are now easily portable and have reduced in cost in comparison to earlier designs.

Yoga may be another form of beneficial exercise that helps with breathing efficiency and breathing muscle control.

The person with COPD should discuss the use of any home remedies or supplements with their physician before beginning such treatments because some treatments may interfere with ongoing therapy.

A person should see their doctor if they experience any of the signs and symptoms of COPD and are members of a high-risk group for developing COPD, such as people who smoke.

In general, patients who notice an increasing shortness of breath that wasn't present recently, especially with any minor exertion should make an appointment to see their doctor. People already diagnosed with COPD who notice an increase in symptoms or have emphysema or chronic bronchitis that worsen should also see their doctor quickly.

Except for COPD due to genetic problems, this health condition can be prevented in many people by simply never using tobacco products.

Other preventive measures include

• Avoiding wood, oil, and coal-burning fumes
• Limiting one's exposure to lung irritants such as air pollutants
• Receiving recommended vaccines to avoid infections (for example, the flu) can help reduce lung damage and the COPD symptoms that accompany lung damage.

• COPD is a long-term disease that can be progressive, so along with the patient's primary care physician, a pulmonologist specialist who treats lung disease is consulted to manage COPD symptoms with medications and other diagnostic tests. In addition, pulmonary rehabilitation specialists that can help with teaching the patient breathing exercises, physical and muscle strengthening along with nutritional counseling can help reduce COPD symptoms.
• Depression and anxiety are common problems in individuals with COPD so having a mental health professional such as a psychiatrist or psychologist on the patient's medical team can provide treatment for symptoms of depression or anxiety, provide counseling for the patient and/or family members, and help with setting up support networks.
• Other medical professionals such as surgical specialists may be consulted if the patient qualifies for a lung transplant or requires lung reduction surgery for severe emphysema or needs other lung surgery.
• In emergencies, people will COPD may be treated by emergency medicine physicians or medical critical care doctors (intensivists).
• Your physician may suggest consultation with individuals to help you stop smoking, a common problem for people with COPD.