Emphysema

Emphysema is a long-term lung condition that makes breathing difficult and gets worse over time, but with the right treatment and lifestyle changes, many people can manage their symptoms and maintain a good quality of life.

Table of contents

• What is emphysema?
• What causes emphysema?
• Who is at risk?
• Symptoms of emphysema
• Stages of emphysema
• How is emphysema diagnosed?
• Treatment options
• Living with emphysema

What is emphysema?

Emphysema is a lung disease that causes shortness of breath and makes it harder to breathe over time^[1]. It is one of two main types of a group of lung diseases called chronic obstructive pulmonary disease (COPD), which are conditions that make breathing difficult and get worse over time^[3]. The other main type is chronic bronchitis. Most people with COPD have both emphysema and chronic bronchitis, though the severity of each can vary from person to person^[3].

In emphysema, the walls of the tiny air sacs in your lungs, called alveoli, become damaged and eventually break^[1]. Healthy lungs contain about 300 million of these small, thin-walled air sacs arranged in clusters at the end of the airways^[2]. When you breathe in, these sacs stretch and fill with air like small balloons. They draw in oxygen and transport it to your blood. When you breathe out, the sacs shrink and push carbon dioxide out of your body^[2].

In emphysema, the inner walls of the air sacs are damaged, causing them to rupture. Instead of many tiny air sacs, this creates one larger air space, reducing the surface area available for the exchange of oxygen and carbon dioxide^[1]. Think of your lungs as bubble wrap. The alveoli are like individual bubbles. If you have emphysema, your alveoli eventually break, creating a large air pocket like a big shipping air pillow instead of many tiny bubbles^[2]. This damage makes it hard to get fresh air in and out of your lungs, making breathing difficult and causing shortness of breath^[2].

Over 3 million people in the United States have emphysema^[2]. In 2016, there were 6,977 deaths from emphysema in the United States, accounting for 2.2 per 100,000 people^[6]. Globally, it accounts for 5% of all deaths^[6].

• Lungs
• Alveoli (air sacs)
• Airways
• Bronchial tubes

What causes emphysema?

The main cause of emphysema is long-term exposure to irritants that damage your lungs and airways^[3]. In the United States, cigarette smoking is the leading cause. Between 80% to 90% of patients with COPD are identified as cigarette smokers^[4]. However, only 10% to 15% of smokers develop COPD^[4]. Pipe, cigar, and other types of tobacco smoke can also cause emphysema, especially if you inhale them^[3].

Smoking damages the thin walls of the air sacs in your lungs over time. The damaged sacs break open to create larger spaces, which limits the amount of oxygen your lungs can take in^[4]. In smokers, the onset and severity of symptoms depend on factors such as the intensity of smoking, the number of years of exposure, and baseline lung function. Typically, symptoms begin to appear after at least 20 pack-years of tobacco exposure^[4].

Other factors that can contribute to emphysema include exposure to secondhand smoke, air pollution, and chemical fumes or dusts from the environment or workplace^[3]. In developing countries, biomass fuels used for cooking and heating can also play a significant role^[4].

Rarely, a genetic condition called alpha-1 antitrypsin deficiency can play a role in causing emphysema^[3]. The liver produces a protein called alpha-1 antitrypsin that protects the lungs from damage. In people with this deficiency, there isn’t enough of this protein to prevent damage to lung tissue, resulting in emphysema. Individuals with this condition may get emphysema even if they do not smoke, though smokers with this deficiency typically develop emphysema earlier and with much higher severity^[8].

Who is at risk?

Several factors increase the risk of developing emphysema^[3]:

• Smoking: This is the main risk factor. Up to 75% of people who have emphysema smoke or used to smoke.
• Long-term exposure to lung irritants: This includes secondhand smoke, air pollution, and chemical fumes and dusts from the environment or workplace.
• Age: Most people who have emphysema are at least 40 years old when their symptoms begin. About 15% of smokers are diagnosed with emphysema, typically in their 40s or 50s^[8].
• Genetics: This includes alpha-1 antitrypsin deficiency. Smokers who get emphysema are also more likely to develop it if they have a family history of COPD.

Emphysema usually develops over many years, making it more common in middle-aged or older people because it takes time to develop with the effects of tobacco smoking and other risk factors^[6].

Symptoms of emphysema

You can have emphysema for many years without noticing any symptoms. Symptoms usually begin gradually, and you may not realize you have the condition because the disease is silent at first. Symptoms only appear after more than half of the lung tissue has been damaged or destroyed^[1][8]. At first, you may have no symptoms or only mild symptoms. As the disease gets worse, your symptoms usually become more severe^[3].

Common symptoms include^[1][3]:

• Shortness of breath: This is the main symptom of emphysema, especially with physical activity. You may start avoiding activities that cause you to be short of breath.
• Frequent coughing or wheezing: A cough that produces a lot of mucus.
• Wheezing: A whistling or squeaky sound when you breathe, particularly when breathing out.
• Chest tightness or heaviness.
• Feeling very tired: Fatigue that affects daily activities.

You may start by noticing these symptoms during physical exertion, but as the condition progresses, they can also happen during rest^[7].

Some people with emphysema get frequent respiratory infections such as colds and the flu^[3]. In severe cases, emphysema can cause additional symptoms such as^[1][3]:

• Weight loss and reduced appetite
• Weakness in lower muscles
• Swelling in ankles, feet, or legs
• A blue tinge to the skin (called cyanosis) due to lack of oxygen
• Barrel-shaped chest from expansion of the ribcage to accommodate enlarged lungs^[9]

Stages of emphysema

Healthcare providers use a staging system to describe emphysema and help guide treatment. The Global Initiative for Chronic Obstructive Lung Disease (GOLD) categorizes COPD in four stages based on how well the lungs function^[2]:

• Stage 1 (Mild): This is the mildest stage of emphysema. Your lungs operate at least 80% as well as the healthy lungs of someone who’s the same age, height, and sex.
• Stage 2 (Moderate): Your lungs operate between 50% and 79% as well as the healthy lungs of someone who’s the same age, height, and sex.
• Stage 3 (Severe): Your lungs operate between 30% and 49% as well as the healthy lungs of someone who’s the same age, height, and sex.
• Stage 4 (Very Severe): Your lungs operate less than 30% as well as the healthy lungs of someone who’s the same age, height, and sex.

How is emphysema diagnosed?

To find out if you have emphysema, your doctor asks about your medical and family history, smoking habits, and whether you’re often around other lung irritants^[10]. Your healthcare professional does a physical exam that includes listening to your lungs. If you have shortness of breath or a long-term productive cough (a cough that produces mucus or phlegm), see your doctor for a lung function test^[9].

Diagnostic tests may include^[3][10]:

Imaging tests:

• Chest X-ray: This test may show some lung changes caused by emphysema and can rule out other causes of your symptoms. However, a chest X-ray may not show changes even if you have emphysema.
• Computerized tomography (CT) scan: A CT scan combines X-ray images taken from many different angles to create detailed images of structures inside the body. A CT scan gives much greater detail of changes in your lungs than a chest X-ray does and can show emphysema. It also can help in deciding if you might benefit from surgery.

Lung function tests:

• Spirometry: This is the most common test to diagnose emphysema. During spirometry, you blow into a large tube connected to a small machine. This measures how much air your lungs can hold and how fast you can blow the air out of your lungs. Spirometry tells how much airflow is limited.
• Other tests include measurement of lung volumes and diffusing capacity, six-minute walk test, and pulse oximetry.

Blood tests: These can help determine if your lungs are transferring oxygen to your bloodstream and removing carbon dioxide effectively.

Treatment options

There is no cure for emphysema, and the damage to your lungs and airways cannot be reversed. However, treatments can help with symptoms, slow the progress of the disease, and improve your ability to stay active^[3][8]. The goal of therapy is to relieve symptoms, prevent disease progression, improve exercise tolerance and health status, and prevent and treat complications^[15].

Lifestyle changes

Quitting smoking: If you smoke, stopping is the single most effective therapy and the most important step you can take to treat emphysema^[3][15]. Although any damage done to your lungs and airways cannot be reversed, giving up smoking can help prevent further damage and slow how fast the condition gets worse^[1][16]. Even people with advanced emphysema will benefit from quitting. Your doctor can help you by recommending programs and products that can help you quit.

Avoiding lung irritants: You should also avoid secondhand smoke and places where you might breathe in other lung irritants such as dust, fumes, or toxic substances^[3]. When going out in cold weather, wear a scarf or mask over your mouth to keep chilly air from causing airway spasms^[21].

Nutrition: Ask your healthcare provider for an eating plan that will meet your nutritional needs^[3]. If you have trouble eating enough because of symptoms such as shortness of breath and fatigue, you may not get all the calories and nutrients you need. Your doctor may suggest eating smaller meals more often throughout the day, resting before eating, and taking vitamins or nutritional supplements. Four to six small meals is better than three larger ones because a less-full stomach makes breathing easier^[21].

Physical activity: Ask your provider about how much physical activity you can do. Physical activity can strengthen the muscles that help you breathe and improve your overall wellness^[3].

Medications

Bronchodilators: These medicines relax the muscles around your airways, which helps to open the airways and makes breathing easier^[3][12]. Bronchodilators can be short-acting (lasting about 4-6 hours and used on an as-needed basis) or long-acting (lasting 12 hours or more and used every day)^[16].

Inhaled corticosteroids: These medications can reduce inflammation of the airways and help prevent flare-ups (called exacerbations)^[16]. Steroid inhalers are normally prescribed as part of a combination inhaler that also includes a long-acting bronchodilator. If your medication includes a steroid, always rinse your mouth after you use it^[21].

Antibiotics: Respiratory infections such as acute bronchitis, pneumonia, and influenza can aggravate emphysema symptoms. Antibiotics treat these infections^[12].

Other medications: Theophylline tablets may be prescribed to help reduce swelling in the airways and relax the muscles lining them^[16]. Mucolytic medicines like carbocisteine can make the phlegm in your throat thinner and easier to cough up^[16].

Vaccines

Influenza and pneumonia can cause serious problems for people who have emphysema. For this reason, it’s important to reduce your risk of getting these illnesses by getting your flu shot each year and asking your doctor about the pneumonia vaccine^[21].

Oxygen therapy

Oxygen therapy can help people with severe emphysema that have low levels of oxygen in their blood to breathe better^[12]. The treatment involves administering oxygen either through a mask or through two small tubes that enter the nose. Some people who receive oxygen therapy need it all the time, while others only need it at certain times.

Pulmonary rehabilitation

Pulmonary rehabilitation is a comprehensive program that offers many resources in one place^[21]. You can get nutrition counseling and learn breathing techniques. If you need help to stop smoking, healthcare professionals there can offer advice. They also can show you safe ways to exercise. These programs aim to improve your overall well-being and quality of life.

Surgery

Surgery may be considered in severe cases. Options include lung volume reduction surgery, which removes damaged lung tissue, or lung transplant for very severe emphysema^[8]. Bronchoscopic interventions may also be used in select cases.

Living with emphysema

Emphysema is a chronic disease with no cure yet. However, lifestyle changes and treatments can help you feel better, stay more active, and slow the progress of the disease^[20]. Managing emphysema actively is key to maintaining a good quality of life.

Daily management tips:

• Drink plenty of fluids: You need about 8 cups a day to keep your body hydrated and your mucus thin. Water is a great choice, but you can also drink fruit juice, decaf coffee, and tea. Limit caffeine and alcohol, as they can dry you out^[21].
• Pace your eating: Take your time with meals. Rest before you eat, and don’t lie down right after you finish. Try eating earlier in the day when you’re less tired^[21].
• Dress comfortably: Close-fitting clothes can make breathing harder. Try drawstring pants and avoid things that fasten in the back or are tight around the neck. Wear slip-on shoes^[21].
• Use your inhaler correctly: Know which medications to use and exactly how to use them. Check how many doses you have left^[21].
• Get rest: Try to avoid napping so you are tired at bedtime. Try to get 30 minutes of exercise three times a week. Keep your bedroom cool, dark, and quiet^[23].
• Protect yourself from infections: During cold and flu season, wash your hands often and use hand sanitizer if you’re going to be out in a crowd^[21].

When to call your healthcare provider: Contact your provider if you notice that your symptoms are worsening or if you have signs of an infection, such as a fever^[20]. Your provider may change or adjust your treatments to relieve and treat symptoms.

Support and education: Joining a COPD self-management program can help you learn about the disease and interventions that can make it easier to manage and have a better quality of life^[20]. Support groups with other people managing the condition can also be very helpful.

With proper management and care, many people with emphysema continue to live full, healthy lives. The key is working closely with your healthcare team, following your treatment plan, and making necessary lifestyle changes^[22].