Mesothelioma Info

Emphysema is a chronic and serious disease that is characterized by severe shortness of breath. Damage to the lung tissue and the loss of elasticity in the lungs cause the changes that make breathing very difficult. Under normal conditions, air enters the nose or mouth and travels down the air tube (trachea) to the main air passages (bronchi). These passages allow air to go into the right and left lungs. Each bronchus branches into smaller passages (bronchioles) and eventually into tiny air sacs (alveoli). It is through the alveoli that oxygen enters the bloodstream when we inhale and that carbon dioxide is expelled when we exhale.


Figure 1. How emphysema damages the lungs
The air sacs (alveoli) in someone with emphysema lose their elasticity and form. The capillaries that surround the air sacs break apart. The destruction of the capillaries and the air sacs prevent the exchange of oxygen for carbon dioxide from occurring effectively.


Emphysema causes damage to the lungs that is irreversible. As air sacs are destroyed, the lungs become less able to transfer oxygen in the lungs for carbon dioxide in the bloodstream.


The lungs also lose their elasticity and the patient experiences great difficulty exhaling. This leads to over inflation of the lungs and very shallow breathing. The loss of elasticity results in lung enlargement. The enlarged areas are ineffective in eliminating carbon dioxide and in transferring oxygen from the air into the blood stream. Damage to the air sacs in the lungs not only results in difficulty breathing, but the heart also has to work harder to circulate blood through the lungs. All these changes make less oxygen available to the body.


Most people with this disease are men between 50 and 70 years of age who have been heavy smokers for most of their lives. However, as the rate of female smokers rises, the rate of emphysema among women will also increase. People with emphysema have permanently enlarged lung regions. Almost 44% of people with emphysema report that having this disease significantly affects their daily lives and limits their activities.


Emphysema develops very slowly. Shortness of breath during activity or exercise is usually the reason that prompts a person with this disease to see a doctor. Emphysema comes on very gradually—-usually after years of cigarette smoking. As the disease becomes worse, any amount of activity may cause difficulty breathing.
Causes

Smoking is the number one cause of emphysema. Smoking is responsible for 80% to 90% of all cases of emphysema. Environmental conditions, including air pollution containing sulfur dioxide and particulate matter or working with hazardous chemicals or substances can also contribute to emphysema.
An inherited, genetic disorder is responsible for causing emphysema in some patients. Deficiency in the protein AAT is an inherited disorder.



AAT is necessary to prevent an enzyme (neutrophil elastase) from destroying another protein (elastin) that is an essential part of the connective tissue of the lungs. People with this form of emphysema generally develop the disease in their thirties and forties. There are approximately 50,000 to 100,000 people in the United States with AAT deficiency; 20,000 to 40,000 of these people will develop emphysema.


People with AAT deficiency who smoke are at an increased risk for emphysema and for suffering severe consequences of the disease. Smoking can decrease the life span of someone with AAT deficiency by as much as 10 years.


Emphysema is classified according to the specific region of the lung that is affected by the disease. Emphysema is classified into two types:

• Centriacinar emphysema: The central or proximal parts of the acini are damaged. This type of emphysema accounts for 95% of all cases and is the type that is most often associated with smoking or environmental causes.
• Panacinar emphysema: The lower regions of the lung are damaged. The acini are uniformly enlarged. This type is most often associated with AAT deficiency.

The location of the disease may help determine whether smoking or AAT deficiency caused the disease. However, the cause of emphysema has little bearing on its outcome.
Symptoms

Shortness of breath is usually one of the first and most pronounced symptoms of emphysema. This caused by over inflation of the lungs. Shortness of breath, especially during activity, is one of the earliest symptoms of emphysema. In addition, patients may suffer from frequent colds accompanied by coughing. As the disease progresses, shortness of breath becomes constant, even during rest. To understand what it feels like to have the over inflated lungs of an emphysema patient, take a deep breath and then try to breathe in and out, on top of the first one. Breathing is very shallow and it’s easy to see how activity becomes impossible.
Other symptoms of emphysema include:

• Coughing
• Distress resulting from the inability to get enough air
• Wheezing
• Chronic mucus production
• The feeling of not being able to get enough air
• Weight loss
• Exhaling through pursed lips or grunting before exhaling
• Needing to lean forward to breathe while sitting
• Anxiety and/or depression

People with emphysema are often thin and have very pink skin. Patients with advanced disease may have the characteristic barrel chests from the increase in lung size.
Risk Factors

Almost all patients with emphysema are habitual, long-term, heavy smokers. Smoking is the number one risk factor for emphysema.
People in such occupations as farming or mining who are exposed to chemical fumes or biologically inactive dust, such as silica or cotton dust, are also at risk for emphysema. Factors that create the potential for high levels of indoor air pollution, such as outdoor pollution, cooking with wood or coal, or having indoor heating without proper ventilation, can place a person at risk for emphysema.


Another predisposing factor for emphysema is AAT deficiency, an inherited disorder that primarily affects white people of northern European descent. People with this form of emphysema generally develop the disease in their thirties and forties. There are approximately 50,000 to 100,000 people in the U.S. with AAT deficiency, but only 20,000 to 40,000 of these people will develop emphysema. This deficiency is found in just 3% of all emphysema patients.
Diagnosis

A diagnosis of emphysema is made on the results of a physical examination, a medical history, blood tests, and other tests. Emphysema generally does not become a significant problem until a person reaches 50 years of age or older. However, the damage from the disease can begin much earlier. Patients often notice breathing difficulties, which is the symptom that brings them to their doctors for a check-up. The doctor will make a diagnosis of emphysema based on the results of the following:

• A physical examination: Your physician may take special note of the color of your lips and fingernails. In very advanced cases, they may be bluish, which can indicate low oxygen levels in your blood. Another sign of seriously advanced disease is if the jugular veins in your neck stick out (distended); this may indicate that lung damage has affected your cardiovascular system.
• A medical history: Your doctor will be interested to know whether you smoke now or have in the past, or whether anyone in your family smokes. The physician will ask whether anyone in your family developed emphysema, especially at a young age. Whether you have been exposed to hazardous chemicals or substances at work and whether you live in an area with heavy air pollution will also be discussed.
• Lung function test: In a test called spirometry, the amount of air that can be blown out in 1 second as well as the total amount of air that can be exhaled from the lungs is measured. This is one of the better ways to determine the amount of damage that has occurred in the lungs. The test is easy to perform and painless. You simply will blow into a tube connected to a machine, which records the airflow and lung capacity.
• An arterial blood gas test: This test will be conducted to measure the amount of oxygen and carbon dioxide in your blood. This test is helpful in diagnosing more advanced stages of the disease and can help determine whether you require oxygen therapy.
• A blood test for AAT deficiency: For patients with a premature onset of emphysema, and those without a smoking history, a blood test can measure the level of AAT in their blood. Depending on the test results, another test may be conducted to determine the genetic make-up of the patient.
• Chest x-ray: A chest x-ray is generally good for diagnosing moderate and severe stages of emphysema. A chest x-ray can also help determine whether a case of emphysema is due to AAT deficiency by revealing the location of the damage.

Prevention and Screening

Quitting smoking or never starting to smoke is the most significant preventive measure a person can take to reduce their risk for emphysema. Quitting smoking helps to slow the progression of the disease and is even valuable for patients who may be in the late stages of the disease.
Avoiding the source of environmental pollutants, which may require relocating or changing jobs, is beneficial.


Once emphysema has been diagnosed, prevention mainly focuses on slowing down the progression of the disease. Slowing down the disease process includes the following:

• Getting rapid treatment for any respiratory infection
• Getting vaccinated against respiratory infections, such as the flu and pneumococcal pneumonia
• Eating a balanced diet with lots of fruits and vegetables
• Exercising daily

Treatment

Urgent Care

If you are having extreme difficulty catching your breath (acute breathing crisis) and you notice a bluish tinge to your fingernails or lips, your body is not getting enough oxygen and you should seek medical attention immediately. Consult your doctor or go to the emergency room. If you are wheezing or coughing and your usual medicine is not helping, consult your doctor or go to an emergency room.
Self Care

Emphysema cannot be cured. The goal of treatment is to help people with the disease live more comfortably and to prevent progression of the disease. Quitting smoking is the single most important step people can take toward treating their emphysema. Quitting smoking—even in the late stages of disease—is beneficial. The rate of breathing dysfunction in patients with emphysema slows after quitting, and even people with advanced disease in their sixties who have stopped smoking have been shown to live longer than people who continue to smoke.


A variety of options are available to help people stop smoking, such as nicotine-containing patches, gum and nasal spray and perhaps most important, support groups. Certain anti-depressants can also be helpful. Quitting smoking can be extremely difficult. Remember that concerned professionals and other people are available to help you through this tough change.


Avoiding air pollution and environmental contaminants is also beneficial.
Certain exercises and dietary supplements can be helpful. There are exercises that strengthen the diaphragm and abdominal muscles used in breathing. Learning how to control your breathing can also make you feel better. Establishing a general exercise regimen, such as a walking program, under your doctor’s supervision can help you build strength and improve your overall well-being.


A ten minute exercise called “pursed-lip breathing” can improve lung function, especially right before beginning an activity. Lie flat on a bed with your head on a pillow and inhale through your nose, consciously moving your abdominal muscles so that your lungs fill with air. Then exhale through the mouth with the lips pursed, so that you make a hissing sound. The exhalation should last twice as long as the inhalation. Try to push all the air out of the lungs. You should feel pressure in your chest and windpipe. Repeat several times. Once you are accustomed to this exercise, you can perform it anytime you need more air, even while standing.


Learning special positions for sleeping and lying down can help clear your lungs of mucus. A technique known as postural drainage may help some patients. It is usually taught by a physical therapist and can help to drain the upper, middle and lower portions of your lungs. Each position is help approximately 5 to 10 minutes. To drain the middle and lower sections, you should be positioned with your chest above your head. Placing pillows under your hips or using a tilt bed, if one is available, will enable you to achieve this position. To drain the upper lungs, sit up at a 45 degree angle. When you are in the proper postural drainage position, change your position in sequence: turn side to side, lay on your stomach and then lay on your back.


Dietary supplements can help you maintain your weight and nutritional balance if you are becoming too thin. Products such as Ensure (a nutritional drink) may help add calories to the daily intake. Starches and sugars do not need to be limited in stable patients.


Maintaining good hydration to keep secretions loose also assists in eliminating mucus. Try to drink eight glasses of water per day, unless instructed otherwise. Using a humidifier in your home may help as well.
Drug Therapy

Your doctor is the best source of information on the drug treatment choices available to you.
Other Therapies

People with emphysema often need oxygen therapy to keep their blood oxygen at normal levels. Administered oxygen may be needed all the time, only at night, or while traveling on an airplane.


Bronchopulmonary drainage may also be recommended. This procedure helps remove mucus in patients who are unable to cough it out themselves, thereby helping to keep the lungs clear. People with emphysema may find pulmonary rehabilitation programs helpful. Although these programs are not intended to significantly improve lung function, they help the patient cope and learn strategies of living with emphysema.
Surgery

Surgery is often a drastic option in the care of someone with emphysema. Two extensive surgical procedures can be done to help people with emphysema. Newer, less invasive techniques are also in development. The current extensive surgical techniques are:

• Lung-volume reduction surgery: In this procedure, the most severely diseased parts of the lung are removed to allow the remaining lung tissue and muscles to work better. This surgery often eliminates the need for additional oxygen and improves breathing function.
• Lung transplantation: This procedure is often a last resort for people with emphysema.

Lung researchers are also developing less invasive surgical techniques to help treat emphysema. One technique involves putting small valves in different parts of the lung using a tube called a bronchoscope. Researchers are evaluating whether this helps people with advanced emphysema. Special Circumstances

Emphysema is one of the most common forms of chronic obstructive pulmonary disease (COPD). Another common form of COPD is chronic bronchitis, which is an inflammation and swelling of the bronchial wall that obstruct airflow. Emphysema and bronchitis often occur together.
Prognosis

Emphysema lasts a lifetime. If caught in the early stages and treated properly, disease progression can be slowed. However, this requires lifestyle changes, including quitting smoking.
The more severe the damage to the lungs, the poorer the prognosis will be. Only 20% to 30% of patients diagnosed with severe lung damage and carbon dioxide retention will survive for more than five years.
Follow-up

Once emphysema has been diagnosed, routine medical care is necessary. People with emphysema require medical care for the rest of their lives. These patients need to be monitored to evaluate lung function and to alter therapy, when necessary.