Chronic Obstructive Lung Disease

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Chronic obstructive pulmonary disease (COPD) is a broad term describing diseases of the lungs associated with limited airflow. The sub-categories of COPD include: emphysema, bronchitis, or a combination of these diseases. COPD starts years before it is diagnosed, damages the airways and decreases the amount of air that one can get in and/or out of the lungs. These limitations are not completely reversible, progressive and associated with inflammation of the lungs. COPD is made worse with smoking.

COPD is the fourth leading cause of death – and likely the third leading cause of death within the next 10 to 15 years. COPD has been diagnosed in about 12 million people and likely 12 million more are afflicted but have not been officially diagnosed (1). Part of the reason many are not diagnosed is because the disease is usually not diagnosed until it is advanced. The costs – mostly attributed to hospitalization – associated with COPD are high.

To have a full understanding of COPD, you need to have a basic understanding of breathing anatomy and physiology. Air, which contains oxygen, is breathed in through the nose and travels down a breathing tube known as the trachea. The trachea splits into two tubes known as the bronchus and enter into the left and right lungs. The air enters into the lungs into a series of tubes known as the bronchioles that terminate into the alveoli.

The alveoli, which are also known as the air sacs, are responsible for transferring oxygen from the lung into the blood. Carbon dioxide, which is the by-product of muscle metabolism, is transferred from the blood into the lungs and breathed out through the nose and mouth.

COPD is made up of emphysema and chronic bronchitis. Emphysema is a destruction of lung tissue that results in decreased oxygen to the body. In emphysema the alveoli are stretched out and are unable to transfer oxygen into the blood and have a decreased ability to release carbon dioxide from the blood.

Chronic bronchitis is characterized by excessive mucus production and chronic inflammation of the breathing passages. The presence of chronic productive cough for at least 3 consecutive months in 2 consecutive years is adequate for the diagnosis of chronic bronchitis. Overactive mucus glands produce excessive mucus.

Early in the disease the mucus can be coughed out but as the disease progresses the cough mechanism weakens and mucus cannot be coughed out as well. This leads to mucus pooling which is a breeding ground for bacteria. In addition, excessive mucus in the breathing passages results in the body being unable to get in enough oxygen or get rid of enough carbon dioxide. When mucus becomes very thick, as the disease progresses, the alveoli dilate and emphysema develops.



Causes

Causes of COPD are genetics, environmental exposure (especially smoking), and age. Smoking causes greater than 80% of the cases of COPD but only 10-15% of smokers develop COPD. Not everyone who smokes develops COPD. Scientists are currently unable to describe why certain individuals with exposure to smoking, environmental or occupational toxins develop COPD and others do not. Likely, there are other factors – in addition to the known genetic defect alpha – 1 – antitrypsin – that predispose individuals to COPD.

Pollution and occupational exposures are environmental factors associated with COPD. Being male, older age, increased frequency of respiratory problems as a child and low socioeconomic status are known risk factors associated with COPD. It takes 20-30 years of exposure before signs and symptoms of COPD are noted.

One particular genetic defect results in COPD. Alpha-1-antitrypsin deficiency is a genetic defect that results in a decrease in an enzyme named elastin. Elastin is an essential enzyme that stops the body from eating away healthy lung tissue. When this genetic deficit is present, the lung gets destroyed at fast rates. Those with alpha-1-antitrypsin deficiency develop emphysema at an early age. Emphysema typically starts at the age of 45-50 for non-smokers with this deficiency and 35 for smokers.

Symptoms

Symptoms of COPD vary depending on whether chronic bronchitis or emphysema predominates. A history of asthma as a child, frequent pneumonia, and frequent sinus infection are common in those with COPD. Cough, increased sputum production, shortness of breath and wheezing are the most prevalent symptoms of COPD.

Symptoms of emphysema include shortness of breath, fatigue, pursed lip breathing, and weight loss. Pure emphysema is characterized by dry cough without sputum production. Individuals with emphysema are typically thin, have a round chest and have a nice pink color to their skin.

Symptoms of bronchitis include cough with mucus production especially in morning, frequent respiratory infections, and occasional shortness of breath. Mucus volume is initially small and is clear to slightly off colored. When the mucus increases in volume or the color changes than infection is more likely, especially if it is accompanied shortness of breath. Monitoring mucus volume and color is a good indicator of infection. Inability to shake a cold is a common presentation of chronic bronchitis. Late problems associated with bronchitis include: decreased oxygen levels, swelling and respiratory failure. Those with bronchitis are typically robust and have a more dusky appearance to the skin.



Diagnosis

Health care providers base the diagnosis of COPD on history and physical examination. Presenting to the doctor and reporting a history of smoking with cough, sputum production, shortness of breath and fatigue point to the diagnosis. Chest X-rays rule out other diseases – such as pneumonia - and confirm the diagnosis of COPD as evidence by specific patterns on the X-ray that are characteristic of COPD. Computed axial tomography (CAT) scans, though usually not done to diagnosis COPD, can help confirm the diagnosis of COPD and are extremely helpful in ruling out other lung diseases.

Lung function tests, also called spirometry, are the gold standard in the diagnosis of COPD. Spirometry measures lung capacity. Spirometry may be obtained in persons with exposure to cigarettes and/or environmental or occupational pollutants, a family history of lung problems, or the presence of cough, sputum production, or shortness of breath. COPD severity is classified based on spirometry reading.

Blood tests, especially arterial blood gases, can help diagnosis and predict severity of the disease. Arterial blood gas readings measure how much acid, oxygen and other gases are in the blood. This test assists in the diagnosis of COPD.

Other diseases – congestive heart failure, heart disease, hypertension, and arrhythmia – are associated with COPD and worsen the prognosis. These diseases need to be sought in those with COPD, as optimal treatment of these diseases improves quality and quantity of life.

Treatment

No treatment exists to reverse the process of COPD, but treatments exist to control the signs and symptoms of the disease. Treatment goals for COPD are to maintain open airways, decrease airway constriction, decrease signs and symptoms, make the patient as functional as possible and prevent hospitalizations.

The most important step is to stop smoking. Smoking leads to progression of the disease. Starting at age 25 everyone starts to lose lung function but the rate is doubled for those who smoke and are susceptible to COPD. Stopping smoking will not reverse the process but slows the loss of lung function. Stopping smoking is the most important step in those who practice health care responsibility.

Cough is often a disabling symptom but it provides a valuable function in clearing the airway and therefore completely suppressing it is not recommended. Coughing that is disabling and associated with rib fracture, passing out or inability to sleep can be suppressed with Dextromethorphan (Robitussin) or a prescription medication such as a narcotics if something stronger is needed. Thinning out the mucous is helpful and can be accomplished by drinking more water or the use of the over the counter product guaifenesin (Mucinex).

Pulmonary Rehabilitation

Exercise/Pulmonary rehabilitation, which includes exercise and education, is an essential step in improving patient function. It helps the patient understand the disease process, treatment options, and develop coping strategies. Multiple professionals – nurses, exercise physiologists, dietitians and doctors – run pulmonary rehabilitation.

Pulmonary rehabilitation has many benefits including improving baseline level of physical activity, decreasing risk of hospitalization and improving quality of life. Pulmonary rehabilitation added to standard therapy improves function and symptoms by helping patients increase activity through exercise training.

Pulmonary rehabilitation is recommended for those patients with reduced exercise tolerance and restricted activity because of shortness of breath. Pulmonary rehabilitation results in improvement in multiple outcomes including health status and healthcare utilization. Nutritional screening is recommended because weight loss, muscle wasting contribute to disability and death in COPD. Nutritional supplementation, exercise, and some medication may be effective in reversing this complication.

Pulmonary rehabilitation teaches exercises to patients with COPD. Traditional exercise training is accomplished with the help of exercise specialists and nurses. Exercises to help improve breathing function – pursed lip breathing and diaphragmatic breathing – are also included as part of pulmonary rehabilitation.

Postural drainage is a technique used by patients with excess mucus and an inability to expel it by cough. It is a technique that can be done by anyone but usually, at least initially, by a physical therapist, nurse or respiratory therapist. It involves placing the patient in twelve different positions and using clapping and vibration to help remove secretions. It is helpful if a large glass of water is drunk before the session. A hot shower and an aerosol treatment are also helpful before a treatment to help enhance mucus clearance.

Medicines

Medications in COPD improve quality of life and prevent exacerbations of disease but no medication modifies the disease process. Medicines are given to dilate the lungs known as bronchodilators. Other medications are administered to reduce the swelling in the lungs thereby improving oxygen transportation. Others are used to improve the ability of the diaphragm, which aids in breathing. Directly giving oxygen is another strategy to improve oxygen levels and the only proven way to decrease death rates from COPD.

Bronchodilators, inhaled medicines that open up the airways, are usually the first medicines used in the treatment of COPD. Two common bronchodilators are ipratropium bromide and albuterol. Bronchodilators increase exercise capacity.

Long-acting formulations are more convenient than the short-acting form. The long-acting formulation of albuterol and the long-acting form of ipratropium (Spiriva) or short-acting form of ipratroprium (Atrovent) improve health status.

Ipratropium is a more effective drug in the treatment of COPD with the two most common medicines being Atrovent and Spiriva. They prevent or reduce exacerbations. Bronchodilators are formatted as short acting, lasting 4 hours or long acting forms lasting 12-24 hours. The short acting forms do not provide a sustained benefit but the onset of action is quicker. The long acting forms keep the breathing tubes opened for longer periods of times but have a longer onset of action and should not be used for quick relieve.

Theophylline, given as a pill, is a bronchodilator and can increase the strength of the diaphragm muscle. It is not a first-line agent in COPD because it has many side effects and requires a very specific blood level to avoid toxicity. The blood levels need to be monitored in those taking these medicines. Side effects of theophylline include nausea and increased heart rate. Toxic levels of theophylline result in heart rate rhythm abnormalities, restlessness, and nausea. If this medication is used in COPD than the slow-release preparation should be used.

Oral steroids are often used in COPD patients but not the steroids that athletes use to build muscle mass and strength. Steroids work by decreasing inflammation in those with COPD. They can be used when someone has an acute flare or can be used chronically in those who have severe disease. There are many long-term side effects with the use of oral steroids including thinning bones, cataracts, and increased blood sugar levels.

Inhaled steroids, which are used as first line therapy in asthma, can also be used in COPD. The benefits of inhaled corticosteroids are not as universal in COPD but those with severe disease do benefit. Those with severe disease and frequent flares benefit from treatment with inhaled steroids, especially in combination with long acting bronchodilators.

Oxygen therapy, which is the only therapy shown to prolong life, is recommended for patients with low levels of blood oxygen. The goal is to maintain oxygen saturation greater than 90% during rest, sleep, and exertion. Long-term oxygen, even though it is associated with prolonging life expectancy, has a risk of reducing physical activity and increases risk of patients staying at home.

Sleep apnea – recurring occurrences of upper airway obstruction during sleep – presents a special risk in those with COPD. Although, many patients have decreased oxygen levels at night due to COPD and not sleep apnea, when the two conditions coexist, the degree of low oxygen is considerably worse. In COPD, sleep quality is often markedly impaired, your doctor should evaluate for the presence of sleep apnea.

Surgery

Surgery may be considered for the symptomatic patients with end-stage COPD. Bullectomy, lung volume reduction surgery (LVRS), and lung transplantation are the current surgical options. There is risk of death or disability with lung surgery so they are typically a not considered in those with less than severe disease.



Prevention

Those with COPD are at high risk for developing pneumonia and influenza. If pneumonia or influenza develops in patients with COPD, death rates are increased when compared to the population without COPD. It is essential to prevent pneumonia and influenza in these patients. If they do develop it is important to treat these conditions as early as possible.

Prevention methods include avoiding patients who are acutely ill with any type of respiratory infections. Frequent hand washing is a critical step. The most important measure may be keeping your immunizations up to date. Make sure you get the pneumococcal and influenza vaccine to reduce the risk of death from these two diseases.

Treat infections early to prevent complication of the disease. This is accomplished by starting antiviral agents at the first sign of the flu. Antiviral medication needs to be started within 48 hours of the onset of symptoms – so reporting symptoms and getting to your doctor is essential. In addition, at the first signs of a flare of bronchitis it is essential that you are treated.

Acute flares of chronic bronchitis are indicated by increased mucus production, change in color of the mucous and shortness of breathe. It is not always associated with a fever. Flares of COPD are caused by a variety of viruses and bacteria. There is no uniform definition for the acute exacerbation of COPD but it is characterized by an increase in the patient’s daily symptoms of shortness of breath, cough, and/or sputum beyond normal. Frequent exacerbations, typically a marker of more severe disease, are associated with reduced levels of physical activity and increased incidence of depression, anxiety, and isolation. Health Care Responsibility

1. Know your disease. Fill out the COPD questions form. If you do not know the answer to these questions ask your doctor. Knowing the answers to these questions will help assure your doctor is appropriately managing your disease.
2. Track your disease. Make sure you are able to accurately report signs and symptoms to your health care provider. This can be accomplished by filling out the COPD flow sheet.
3. Know when you are due to have your immunizations.
4. Call the doctor at the first sign of an acute flare of COPD or other respiratory infection.

   Questions to ask you health care provider

   What disease of my lungs do I have? Do I have emphysema or bronchitis? Do I have a combination or these diseases? Which one predominates?

   How did you diagnose my disease? Did you diagnose it based on my symptoms or did you use any diagnostic tests such as x-ray, CAT scan or pulmonary function tests.

   How severe is my disease? Mild, moderate or severe? This is usually based on the severity of your signs and symptoms as well as the pulmonary function tests.

   How fast will this disease progress and what can I expect? It is impossible to know the exact answer to this question but your doctor may be able to give you some guidance as to what you can expect with your disease.

   Do I need lung function tests? When do I need them repeated? Patients with COPD benefit from lung function tests. This helps the doctor assess the severity of the disease as well as determine response to therapy.

   If you are still having problems with your breathing or with excessive coughing: is there any other medicines or interventions that will reduce symptoms and make me more comfortable? Keeping a journal of your signs and symptoms helps you accurately disseminate the symptoms to your doctor and allow him/her to individualize your treatment to maximize function/comfort.

   What type of follow-up do I require? How often should I visit the doctor?

   What types of medicines are being used to treat my lung disease? Do I need oxygen? There are strict insurance guidelines for oxygen therapy. Your doctor will order a test called an oxygen saturation or an arterial blood gas to determine if your quality. Monitoring oxygen levels is a simple test where an infrared sensor is placed on the finger and the percentage of oxygen in the blood is determined. The arterial blood gas is a blood draw from your artery.

   Are there any non-drug treatments to treat my disease? Could I benefit from pulmonary rehabilitation? There a very few individuals who should not exercise with pulmonary disease, but not everyone qualifies for the structured pulmonary rehabilitation program. The program, in addition to exercise, provides nutrition advice, lifestyle advice and moral support.

   Should I see a lung doctor? Lung doctors, also known as a pulmonologist, are experts in diseases of the lungs and may be able to provide additional guidance of how to manage your disease.

   Should I get a pneumococcal vaccine/flu vaccine? Unless you are allergic, you should get these vaccines as recommended.

   1. National Heart, Lung and Blood Institute. It has a Name: COPD. Available from: http://www.nhlbi.nih.gov/health/public/lung/copd/index.htm. Accessed 5/26/2009.

    

    

    

    

    

    

    

    

    

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