Pulmonary Emphysema: Causes, Symptoms & Treatment

Pulmonary emphysema refers to irreversible damage to the lungs. Because existing damage cannot be reversed, therapy for emphysema can only slow or prevent the progression of the disease.

What is emphysema?

Infographic on the different lung diseases and their characteristics, anatomy, and location. Click to enlarge. In emphysema, overinflation of the air-filled small structures around the lungs occurs. Inflammatory processes in the lungs are caused by harmful substances in the air breathed or by the body’s own reactions. These also cause increased external pressure on the bronchioles, so that the alveoli inflate until they burst. This can cause large emphysema bubbles to form in emphysema. As these bubbles in the lungs have become nonfunctional, breathing becomes difficult.

Causes

There can be a variety of causes of emphysema. Normally, there is a balance between different enzymes within the lungs. Granulocytes are tissue-cleaving enzymes and alpha-1 proteinase inhibitors are protective enzymes that restore the balance. If there is a hereditary predisposition to a deficiency of this protective enzyme, emphysema can develop. However, only the severe form of this disease inevitably results in emphysema. In milder forms, the risk for emphysema exists only in connection with the ingestion of harmful substances. This is because harmful substances that are taken in with breathing can also lead to emphysema. For example, [smoking|smokers]] ingest substances with their smoke that lead to inactivation of the important alpha-1-proteinase inhibitor. In addition, smokers have an increased risk of infection. But chemical substances, such as aluminum or cadmium, and occupational dust exposure, such as in mines, animal feed or raw cotton, can also be the cause of emphysema.

Symptoms, complaints, and signs

The disease usually develops gradually. Signs of the onset of emphysema include a morning cough with sputum and shortness of breath during physical exertion. In some patients, there are noticeable rales when breathing, as is usually the case with bronchitis. External signs include a blue coloration of the lips and fingers and conspicuously curved fingernails. As a result of the lack of oxygen, pallor and dark circles under the eyes may also occur. In the late stages, as a result of the overinflation of the lungs, the chest becomes deformed, eventually resembling a barrel. Patients are usually physically and mentally depressed, with symptoms such as listlessness and fatigue. If left untreated, the lungs continue to enlarge, reducing the mobility of the chest. The result is difficulty breathing, especially exhaling. The increasing lack of oxygen also impairs organ functions, causing a variety of complaints. Typical symptoms are headaches, digestive problems, cardiac arrhythmias and jaundice. The initial shortness of breath now becomes permanent. In the long term, right heart failure may develop, which in turn is associated with serious symptoms. As it progresses, emphysema thus damages the entire body. If the disease progresses further, it inevitably leads to the patient’s death.

Disease progression

Pulmonary emphysema leads to reduced exercise capacity and, in advanced stages, even to severe shortness of breath. As emphysema progresses, instability of the airways and lung framework occurs, making exhalation in particular even more difficult. Overall, this prolongs the exhalation phase. For this reason, many affected persons use the so-called lip brake to exhale. This involves letting the breath out through the pursed lips. In more severe cases, there is also a strong inflation of the chest. As the disease progresses, emphysema can develop in two different directions. A distinction is made between “blue bloater” and “pink puffer”. The “blue bloater”, i.e. “blue cough“, tends to be overweight and has blue-red nails and lips due to the existing oxygen deficiency. He suffers less from shortness of breath than the “pink puffer”, but has an increased and expectorant cough. This course from emphysema easily develops right heart failure.The “pink puffer”, i.e. the “pink wheezer”, has a rather lean appearance. He suffers from severe shortness of breath and an occasional irritating cough without expectoration. His oxygen level is lower, but his carbon dioxide level is normal. In this course of emphysema, breathing may suddenly fail.

Complications

Several complications are threatened by emphysema. They can be both acute and chronic in nature. An acute sequela is the development of spontaneous pneumothorax. Physicians speak of this complication when emphysema bubbles burst. Air escapes into the gap between the chest wall and the lungs. Eventually, the lungs partially or even completely collapse. Also among the acute effects of emphysema are airway infections. They result in a deterioration of the patient’s health, who suffers from increasing cough, shortness of breath and sputum. For this reason, he receives additional treatment with antibiotic preparations. Furthermore, chronic complications can occur with emphysema. These include cor pulmonale. Due to the chronic lack of oxygen in emphysema, constriction of the pulmonary vessels occurs, which in turn causes the blood pressure in the pulmonary circulation to rise. In addition, the right heart is strained. In the further course, right heart failure is imminent. Another chronic consequence of emphysema is respiratory insufficiency. It is not uncommon for partial insufficiency to progress to global insufficiency. Underweight is also one of the effects of the disease. The heavy strain on the respiratory muscles results in a high consumption of calories, which can often lead to underweight. Furthermore, the patient feels shortness of breath and fullness when taking meals, so he eats less.

When should one go to the doctor?

If the affected person experiences coughing attacks immediately after waking up, this is a sign of irregularity. A visit to the doctor should be made if the symptoms continue unabated for several days or weeks. A doctor is needed if there is palpitations, disturbances in the heart rhythm, or irregularities in breathing. If there is discoloration of the skin, poorly perfused lips, fingers or toes, and a feeling of shortness of breath, a doctor’s visit is needed. Blue discolorations indicate a lack of oxygen in the organism and should therefore be clarified by a doctor as soon as possible. Without timely medical attention, a life-threatening condition may develop, which must be prevented in time. Fatigue, tiredness, internal weakness or a general feeling of illness are indications that should be followed up. A visit to the doctor is necessary if the usual level of performance drops, everyday tasks can no longer be performed or sleep disturbances begin. If anxiety or behavioral problems occur, a doctor should be consulted. Since untreated emphysema can lead to premature death, a visit to the doctor is advisable at the first signs and irregularities. If existing symptoms increase or spread, a doctor should be consulted as soon as possible. Headaches, digestive disorders or a yellow complexion should also be examined and treated.

Treatment and therapy

Because the changes to the lungs in emphysema are irreversible, the disease cannot be cured. All that can be done is to try to prevent further progression of the disease. First, contact with the harmful substances, such as cigarette smoke, must be stopped immediately. Respiratory infections should be treated consistently and early to prevent further damage to the lungs. Physiotherapeutic exercises can be used to strengthen the respiratory muscles and thus facilitate breathing. If there is a congenital enzyme deficiency, this deficiency should be compensated for by a weekly infusion of this enzyme. If large emphysema bubbles already exist, it may be necessary to remove them surgically. If emphysema is already more advanced, existing shortness of breath can be alleviated by administering oxygen. In particularly severe cases, however, emphysema may require a lung transplant.

Outlook and prognosis

Noticeably often, emphysema occurs in smokers. However, gases and dusts could also cause the disease.According to current scientific knowledge, the best way to alleviate the symptoms is to stop smoking immediately. However, this and other therapies do not lead to a cure. Patients have to live with sometimes difficult restrictions. Future life expectancy sometimes depends on their willingness to cooperate. Advanced age and other underlying respiratory diseases significantly worsen the outlook. If the affected person already suffers from smoker’s cough, a life expectancy of five to seven years results if the therapy is carried out consistently. With this prognosis, upward and downward swings represent a common variation. Those who omit treatment sooner or later accept their death. There is a risk of lung failure. Patients also forgo existing therapies such as surgery, respiratory exercise, and oxygenation. The destruction of the tissue progresses irreversibly. Depending on the basic condition at diagnosis, life expectancy ranges from a few months to a few years. Difficulties in everyday life and the typical complaints of emphysema increase more and more.

Prevention

To prevent the development of emphysema, the first priority should be to avoid inhalation of the harmful substances mentioned. Infections of the respiratory tract should also be prevented if possible. On the one hand, this can be done with flu and pneumococcal vaccinations; on the other hand, this can also be achieved through respiratory therapies and exercise in the fresh air. In the case of congenital enzyme deficiency, the same points should be observed. Furthermore, the enzyme deficiency can be compensated to prevent damage to the lungs and the development of emphysema.

Follow-up

One of the goals of follow-up care is to keep the shortness of breath caused by emphysema under control as much as possible. This includes abstaining from stimulants that irritate the lungs, such as smoking. Smoking is considered to be the primary cause of obstruction or narrowing of the airways. It is scientifically proven that stopping nicotine addiction contributes to healing, insofar as the consumption is actually responsible for the typical complaints. Preventive measures, which the patient can take himself, are also effective in many other cases. Inhalation of certain substances and baths with essential oils can prevent disease under certain circumstances. A physician will provide information about suitable measures. Basically, immunity does not build up after a one-time illness. Affected persons therefore have to struggle permanently with respiratory complaints, which can take different forms depending on the severity of the underlying disease. Follow-up care becomes an ongoing issue. Imaging procedures such as CT scans or X-rays provide information about the progress of the airway obstruction. Sufferers obtain relief through certain inhalations or breathing techniques. The attending physician prescribes medication on an ongoing basis or orders therapies as needed. Complications are thus prevented.

What you can do yourself

There is no cure for emphysema, but active management of the disease can contribute significantly to maintaining quality of life. Above all, this includes eliminating harmful influences as much as possible: Stopping smoking immediately can slow down the progression of the disease, and those affected should avoid spending time in polluted air as far as possible. To prevent infections from placing an additional burden on the diseased lungs, it is important to wear appropriate clothing and wash one’s hands thoroughly, especially during the typical cold season. Preventive vaccination against influenza and pneumococci is advisable. Physical activities strengthen the lungs and the cardiovascular system. The duration and intensity must be tailored to the severity of the disease and the individual’s ability to cope with stress – ideally, a training plan is drawn up with the attending physician before exercise training is started. In addition, respiratory and physiotherapy can help to improve the symptoms. In the advanced stage of the disease, it is important to take into account the decreasing performance capacity in everyday work and home life: Routine tasks can also take more time, and regular breaks provide the necessary rest. In the household, aids make many activities easier – but those affected should not be afraid to accept outside help when needed. Exchanging experiences in a self-help group can be helpful in coming to terms with the disease psychologically.