Emphysema Research Paper
EMPHYSEMA
(CHRONIC OBSTRUCTIVE
PULMONARY DISEASE)
EMPHYSEMA -is a condition of the lung characterized by abnormal, permanent enlargement of the airspaces distal to the terminal bronchiole, accompanied by destruction of their walls. This over-inflation results from a breakdown of the walls of the alveoli, which causes a decrease in respiratory function and breathlessness. In emphysema, the lungs loose elasticity and are unable to fully expand and contract. This occurs because the air sacs cannot completely deflate, thus unable to fill with fresh air for adequate ventilation. Emphysema in children is usually caused by congenital abnormalities of the lung and alpha-1 antitrypsin deficiency.In emphysema, the patient can breathe in but breathing out is difficult and inefficient. The seriousness of emphysema varies greatly. Some persons with emphysema never reach a stage of incapacity and go through life with relatively little …show more content…
inconvenience, while in others, emphysema worsens until final degeneration of the ability to breath occurs.
PREVALENCE OF EMPHYSEMA
-prevalence generally refers to the number of patients suffering with a condition at any particular time, while incidence rate refers to newly diagnosed cases or annual rates. In the US, around 2 million persons are managing emphysema or about 17 per 1000 population or 1 in 136, with a lifetime incidence risk of 1.4%. Around the world, China and India lead in emphysema prevalence with nearly 10 million and 8 million cases respectively; nations with approximately 1 million emphysema patients include Pakistan, Indonesia, Brazil, Russia, Japan, and Bangladesh; nations with emphysema prevalence rates of approximately 1/2 million include Germany, Italy, UK, Iran, Thailand, Egypt, Vietnam, and the Philippines.
ECONOMIC BURDEN OF EMPHYSEMA
Emphysema affect approximately 1.9 million Americans and is one of the fastest growing causes of morbidity and mortality in the USA. Worldwide, the social burden of chronic obstructive pulmonary disease (COPD), in terms of days lost to disability, is expected to increase from twelfth to fifth among all chronic diseases from 1990–2020. Given the prevalence of this disease and the duration of illness for those affected, medical expenditures for treating COPD and the indirect costs of morbidity can represent a substantial economic and social burden for societies and for public and private payers. More importantly, because emphysema is highly prevalent, new treatments that are widely adopted for this condition, even if inexpensive at the individual patient level, can have a tremendous impact on the overall economic burden of the disease. In today’s cost-conscious environment, evaluating the economic impact of new therapies has become nearly as important as understanding their clinical impact. As healthcare costs continue to escalate, more emphasis is being placed on understanding the economic implications associated with disease processes and their treatments. Unfortunately, very little economic information concerning COPD is available, particularly outside of a few developed Western nations. Given the rising prevalence of COPD worldwide, it is urgently necessary to understand its economic burden and to provide more robust evaluations of healthcare interventions designed to reduce its incidence and impact. Studies designed for making decisions and policy must apply robust methods and report results in a standardized fashion.
FACTORS THAT INFLUENCE DISEASE DEVELOPMENT AND PROGRESSION
Smoking. The primary risk factor for the development of emphysema is tobacco abuse. Cigarette smoke contributes to this disease process in 2 ways. It destroys lung tissue, which is the cause of the obstruction, and it causes inflammation and irritation of airways that can cause the disease to get worse. It is not possible to predict in any individual smoker who will develop emphysema, how long it will take, or how many cigarettes one must smoke. In fact, most smokers do not develop symptomatic emphysema, suggesting that most people have sufficient reserve capacity in the lung to get by despite any damage.
Tobacco smoking contributes to emphysema by:
1. Recruiting neutrophils into the lung by factors from smoke-activated alveolar macrophages.
2. Activating alveolar macrophages release elastase which injures elastic tissue of the lung.
3. Alveolar macrophage also stimulates the release of elastase from neutrophils.
4. Inactivation of alpha-1-antitrypsin by oxidants in tobacco smoke or free radicals released by activated neutrophils.
Age. Although the lung damage that occurs in emphysema develops gradually, most people with tobacco-related emphysema begin to experience symptoms of the disease between the ages of 40 and 60. Older age is a risk factor for emphysema. Lung function normally declines with age. Therefore, it stands to reason that the older the person, the more likely they will have enough lung tissue destruction to produce emphysema. In a few individuals, genetic factors such as a reduced level or activity of protective enzymes in the lung (such as occurs in alpha1-antiprotease deficiency) may result in emphysema even in the absence of smoking, but this is much rarer.
Exposure to secondhand smoke. Secondhand smoke, also known as passive or environmental tobacco smoke, is smoke that you inadvertently inhale from someone else's cigarette, pipe or cigar. Being around secondhand smoke increases your risk of emphysema.
Occupational exposure to fumes or dust. If you breathe fumes from certain chemicals or dust from grain, cotton, wood or mining products, you're more likely to develop emphysema. This risk is even greater if you smoke.
Exposure to indoor and outdoor pollution. Breathing indoor pollutants, such as fumes from heating fuel, as well as outdoor pollutants — car exhaust, for instance — increases your risk of emphysema. Air pollution acts in a similar manner to cigarette smoke. The pollutants cause inflammation in the airways, leading to lung tissue destruction. Abnormal airway reactivity, such as bronchial asthma, has been shown to be a risk factor for the development of emphysema. Men are more likely to develop emphysema than women. The exact reason for this is unknown, but differences between male and female hormones are suspected.
Genes. Alpha–1 antitrypsin (AAT), also called alpha-1 protease inhibitor, is an enzyme (protein) that helps to protect lung tissue and allow the alveoli to function properly. People who have a genetic (hereditary) deficiency of alpha-1 antitrypsin are at increased risk for developing alpha-1 antitrypsin deficiency emphysema or familial emphysema.
In this condition, damage to the structure and elasticity of the air sacs occurs unchecked. It is important that people who have this deficiency never smoke. AAT is manufactured in the liver, and patients who have this deficiency also may experience liver damage (e.g., cirrhosis, hepatitis).
PATHOLOGY, PATHOGENESIS, PATHOPHYSIOLOGY
PATHOLOGY
It is classified according to anatomical distribution of the lesion within the acinus.
Types of emphysema:
1.Centriacinar (centrilobular) emphysema: This is the commonest form of emphysema and is usually associated with clinical symptoms. It involves the cluster of terminal bronchioles near the end of bronchiolar tree, more exactly the proximal part of the acinus.
It is characterized by:
i) There is destruction and enlargement of central or proximal parts of respiratory unit, formed by respiratory bronchioles, whereas distal alveoli are spared. ii) The lesions are more common and severe in the upper lobes and superior segment of lower lobe. iii) Severe lesions are seen primarily in male smokers, often in association with chronic bronchitis. A. Cut surface from a lung with centriacinar emphysema showing holes in the center of lobules surrounded by relatively normal parenchyma. The severity varies among lobules. B. Microscopic section showing that the airspace enlargement in centriacinar emphysema is most marked adjacent to the abnormal respiratory bronchiole, corresponding to the center of the lobule. Also, some of the alveolar walls of the abnormal airspaces are thickened and fibrotic (H&E × 16).
2.Panacinar(panlobular)emphysema:
This is characterized by:
i) Initial involvement of alveolus and alveolar duct which extends to respiratory bronchioles. ii) Uniform destruction and enlargement of the acinus from the respiratory bronchiole to the terminal blind alveoli. iii) Mainly seen in lower basal zones. iv) Strong association with alpha-1-antitrypsin deficiency. C . Cut surface of a lung slice showing how the entire lobule is uniformly affected in panacinar emphysema. D. Microscopic section demonstrating that in panacinar emphysema, the airspaces adjacent to the lobular septa are enlarged to the same degree as those in the center of the lobule (H&E × 16).
3.Paraseptal(distal)emphysema: It involves mostly the distal acinus, proximal portion of the acinus is normal. It is found near the pleura along the lobular septa and at the margin of the lobules. It also occurs adjacent to fibrosis, scars or atelectasis and usually more severe in the upper half of the lungs. They show multiple, continuous, enlarged air spaces from less than 0.5 mm to more than 2.0 cm in diameter, often forming cyst-like structures. This type probably underlies many of the cases of spontaneous pneumothorax in young adults.
4. Irregular emphysema -is the irregular involvement of the acinus and is associated with scarring. It is usually asymptomatic and is the commonest autopsy finding.
Other emphysemas are:
i) Bullous emphysema: These are blebs or bullae larger than 1 cm in diameter and mostly subpleural near the apex. They are the localized form of one of the four types of emphysema. Sometimes they are seen in relation to tuberculous scarring.
ii) Interstitial emphysema: This is characterized by entry of air into the connective tissue of lung, mediastinum or subcutaneous tissue. Alveolar tears allow the entry of air into the connective tissues. Progressive accumulation of air in the connective tissue may cause marked swelling of the head and neck, chest wall with cracking crepitation all over the chest. In most instances such air is promptly reabsorbed as soon as the point of entrance is sealed.
Common causes of interstitial emphysema:
Lung penetrating chest injury.
Fracture rib that punctures the lung substance.
Patients being artificially ventilated.
Individuals who suddenly inhale irritant gases.
Combination of coughing with bronchiolar obstruction, which increase alveolar-sac pressure.
Children with whooping cough and airway obstruction by blood clots, foreign body etc.
iii) Compensatory emphysema: This is the dilation of alveoli not due to destruction of septal walls but compensatory expansion of the residual lung parenchyma after surgical removal of a diseased lung or lobe. iv) Senile emphysema - is the over- distended, voluminous lungs found in the aged. This is due to age-related changes in the lung, i.e. larger alveolar ducts and smaller alveoli without loss of elastic tissue or destruction of lung substance.
PATHOGENESIS
Protease-antiprotease hypothesis holds that destruction of alveolar walls in emphysema is due to an imbalance between proteases and their inhibitors in the lung.
This is evidenced from:
i) Hereditary deficiency of the major protease inhibitor, alpha-1-antitrypsin, invariably develop emphysema. ii) Pulmonary instillation of proteolytic enzyme, and neutrophil elastase, results in emphysema in experimental animals.
PATHOPHYSIOLOGY
In emphysema, recurrent inflammation is associated with the release of proteolytic enzymes from the lung cells. This causes irreversible enlargement of the airspaces distal to the terminal bronchioles. Enlargement of the airspaces destroys the alveolar walls, which results in a breakdown of elasticity and loss of fibrous and muscle tissue, thus making the lungs more compliant.
In patients with emphysema, recurrent pulmonary inflammation damages and eventually destroys the alveolar walls creating large spaces. The alveolar septa are initially destroyed, eliminating a portion of the capillary bed and increasing air volume in the acinus. This breakdown leaves the alveoli unable to recoil normally after expanding and results in bronchiolar collapse on expiration. The damage or destroyed alveolar walls can’t support the airways to keep them open. The amount of the air that can be expired passively is diminished, thus trapping air in the lungs and leading to over distension.
Hyperinflation of the alveoli produces bullae (air spaces) adjacent to the pleura (blebs). Septal destruction also decreases airway calibration. Part of each inspiration is trapped because of increased residual volume and decreased calibration. Septal destruction may affect only the respiratory bronchioles and alveolar ducts, leaving alveolar sacs intact (centriacinar emphysema), or it can involve the entire acinus (panacinar emphysema), producing damage that’s more random and involves in the lower lobes of the lungs.
Associated pulmonary capillary destruction usually allows a patient with severe emphysema to match ventilation to perfusion. This process prevents the development of cyanosis. The lungs are usually enlarged; therefore the total lung capacity and residual volume increase.
Pulmonary emphysema results in hyperinflation of the lungs and concomitant changes in the configuration of the thoracic cavity.
Lung Overinflation or Hyperinflation is a critically important component part of Emphysema, and particularly so with Emphysema. In fact, recent evidence suggests that correction of Hyperinflation appears to be more important in the relief of dyspnea, than is the correction of airway obstruction with broncodilator therapy. Said another way, if you use so-called Rescue Drugs such as Metered Dose Inhalers to relieve dyspnea caused by airway bronchospasm, the majority of your dyspnea relief comes not from the bronchospasm relief, but rather as a result of relieved bronchospasm now permitting correction of hyperinflation.
This section will tell you how to obtain further dyspnea relief, after you have used your bronchodilator medications. Bronchodilator medications are very important, but they are only the first step to obtain maximal dyspnea relief.
There are two general types of overinflation. The first is so-called "Anatomic Hyperinflation" seen in Emphysema, where there is actual destruction of alveolar lung tissue, to create enlarged cystic overdistended air spaces.
The second general type of overinflation is so-called "Physiologic Hyperinflation" seen in both COPD and Emphysema. The underlying problem here is the airway obstruction common to both conditions. With increased airway obstruction causing increased resistance to air flow, the lung may not have enough time to empty before the next inhaled breath.
Remember, on breathing in, all the structures in the lung, including the airways, get larger, and therefore air moves into the lung relatively easier on inspiration. Conversely, on breathing out, everything in the lungs, including the airways, gets smaller. Therefore, it is always relatively more difficult to to get air out of the lung on expiration. As a result, some air is trapped in the lung, causing it to overinflate.
This diagram is from 1955 first edition of The Lung, by Dr. Julius Comroe et al.
The lung is depicted as a single air sack, and the arrow indicates air moving in and out of the lung. The dark wavy line below is the subject breathing in and out.
Figure A shows the normal condition, with air moving out freely, and no lung over inflation.
Figure B shows some airway obstruction, and therefore "Air Trapping" on expiration, and with over inflation developing. Note the breathing tracing moving upward.
Figure C shows even more airway obstruction, and the resulting increased over inflation.
The problem here is not enough time for the lung to empty on expiration. The so-called Time Constant required for lung emptying has been exceeded. Note carefully, the faster you breathe, the worse this problem will become. The older term "Physiologic Hyperinflation" is now evolving into the name "Dynamic Hyperinflation," and more recently has generally been used as a phenomenon related to patient exertion. However, this is not entirely correct, as it is now clear that this type of hyperinflation is commonly present to some degree, even with mild to moderate airway obstructive disease, even while patients are at rest. Dynamic Hyperinflation therefore is of two general types, "Resting Dynamic Hyperinflation" and "Active Dynamic Hyperinflation." The importance of this surprising recent observation that Dynamic Hyperinflation is frequently present at rest in mild to moderate airway obstructive disease is not that it is causing significant dyspnea at rest. In fact, it generally is not of significance. However, it is clear that these generally asymptomatic patients are indeed vulnerable to further exacerbation of their Dynamic Hyperinflation should they increase their breathing rate for exertion or whatever reason, and therefore have an exaggerated dyspnea response. Clearly, this problem can no longer be considered as a significant factor only in severe COPD.
THE FUNCTIONAL ABNORMALITIES IN EMPHYSEMA WITH BREATHING PATTERNS Although emphysema (i.e. destruction of alveolar walls) leads to decreased expiratory flow rates, the pathophysiology is different from the situation in pure airway disease. The primary problem in emphysema is loss of elastic recoil (i.e., loss of the lung natural tendency to resist expansion). One consequence of decreased elastic recoil is a decreased driving pressure that expels air from the alveoli during expiration. A simple analogy is a balloon filled with air, in which the elastic recoil is the “stiffness” of the balloon. When a given volume of air inside an unsealed balloon, a stiffer balloon will expel air more rapidly than will a less stiff balloon. An emphysematous lung is like, a less stiff balloon: a smaller than normal force drives air out of the lungs during expiration. Loss of driving pressure is not the only consequence of emphysema. There is also an indirect effect on the collapsibility of airways. Normally outward traction is exerted on the walls of airways by supporting structure of tissue from the lung parenchyma. When the alveolar tissue is disrupted, as in emphysema, the supporting structure for the airways is diminished, and less radial traction is exerted to prevent airway collapse during a forced expiration, the strongly positive pleural pressure promotes collapse. Airways lacking an adequate supporting structure are more likely to collapse (and have diminished flow rates and air trapping) than are normally supported airways. The decrease in elastic recoil in emphysema also alters the compliance curve of the lung and the measured of lung volumes. The compliance curve relates transpulmonary pressure and the associated volume of gas within the lung. Because an emphysematous lung has less elastic recoil (i.e. is loss stiff), it resist expansion loss than does its normal counterpart. Therefore, the compliance curve is shifted upward and to the left, and the lung has more volume at any particular transpulmonary pressure. TLC is increased because loss elastic recoil results in a smaller force opposing the action of the inspiratory musculature. FRC is also increase because the balance between the outward recoil of the chest wall and they inward recoil of the lung is shifted in favor of the chest wall. As in bronchitis, RV is substantially increased in emphysema because poorly supported airways are more susceptible to closure during a maximal expiration. Compliance curve of lung in emphysema compared with that of normal lung. In addition to shift to curve upward and to left. Total lung capacity in emphysema(point B on volume axis) is greater than total ling capacity(point A). in pure chronic bronchitis without emphysema, the compliance curve is normal. Patterns of pulmonary function test in Emphysema
PROGNOSIS
If you catch emphysema in the early stages, it is possible to reverse the effects, particularly if the patient is a smoker who quits. However it is difficult to diagnose emphysema early, since its symptoms mimic those of acute illnesses such as bronchitis. If it remains undiscovered until the middle or end stages, there is no way to reverse the damage done. There are treatments which can alleviate symptoms and increase the quality of life for the patient. These treatments may extend the life expectancy for the patient, but the end result for most cases that are not caught early is fatal.
The possible outcome of a person with emphysema show a rapid decline in lung function while others show a more gradual decline. They are usually determined by how the lungs are able to get oxygen in and carbon dioxide out of the body. When the lungs deteriorate to such a point that they cannot function well, symptoms become more obvious and we commonly say the disease is approaching “end stage”.
Dyspnea is a cardinal symptom of emphysema, is a major of disability and anxiety associated with the disease. Typical emphysematous patients describe their dyspnea as a sense of increased effort to breathe, heaviness, air hunger or grasping. The destruction of lung tissue seen with emphysema eventually results in dysfunction of virtually all the functions of the lung, as well as that of the respiratory muscles and chest wall. By losing elasticity, the lungs become more difficult to ventilate. The lungs rapidly become progressively more impaired and air becomes trapped within the lung. This is why emphysema is termed an “obstructive” lung disease. This impairment increases the work of breathing. Patients first experience symptoms with exercise but as the disease progresses shortness of breath can occur with all activities and even at rest.
In addition, as the lung loses its elasticity, the chest wall and ribs begin to expand and become overinflated. This results in a flattening of the major inspiratory muscle, the diaphragm, and causes the muscle to work less efficiently. This further adds to the increased work of breathing and diminishes the ability of the diaphragm to cope with even a normal workload. As a result, shortness of breath worsens.
The main function of the lungs is to add oxygen and remove carbon dioxide from the blood. As emphysema progresses, patients may have an inadequate amount of oxygen in the blood. As the disease worsens the amount of carbon dioxide in the blood increases. If the oxygen in the blood is not supplemented, the entire body suffers, including the heart and the brain.
The patient with emphysema becomes “out of shape” (deconditioned) because the disease does not allow sufficient exercise to stay in shape.
Anorexia which lead to malnutrition. Anorexia is the medical term for "loss of appetite." It is not, however, to be confused with the term, "anorexia nervosa," which is a serious mental condition not associated with COPD. Progressive weight loss in emphysema occurs because of a number of factors: Fatigue and shortness of breath which can interfere with meal preparation and consumption. Increased phlegm which can alter the taste of food. Flattening of the diaphragm (breathing muscle) which presses on the stomach causing a premature feeling of fullness. Depression over having a chronic illness. Side effects of medications such as nausea and indigestion.
The main symptom of an emphysema exacerbation is increasing shortness of breath.
Exacerbations of emphysema can be serious, even life threatening. They can occur when a person has Chronic Obstructive Pulmonary Disease (COPD), including emphysema, and his or her symptoms get worse very quickly. Exacerbations of emphysema can be triggered by infections such as colds or the flu. Being around lung irritants such as air pollution, dusts, fumes or smoke can also trigger an exacerbation. Other medical conditions, such as heart problems or infection elsewhere in the body, can make breathing problems worse. You may cough up more mucus than usual, or mucus that is a different color than usual. Exacerbations of emphysema need to be treated as soon as possible. You may also cough up more than usual or mucus that is a different color than normal or increasing trouble breathing that would be a sign of an infection and a decrease exercise tolerance due to shortness of breath during the exercise may be seen in
emphysema.
Possible complications of emphysema include cor pulmonale. Cor pulmonale is defined as right heart failure secondary to lung disease. Cor pulmonale results from the effects of chronic hypoxemia on the pulmonary vasculature. Hypoxemia causes pulmonary vasoconstriction that increases the right ventricles afterload. Pumping against the high pulmonary vascular pressures increases right muscle work, which eventually causes right sided heart failure. Usually patients with emphysema experience a progressive decline in exercise ability and become short of breath even at rest.
Pneumothorax is a possible complication of emphysema. As emphysema progresses and more alveoli are destroyed the air escapes from the lungs and collects outside the lungs in the thorax. This happens in patients suffering emphysema since the walls of the alveoli are thinner and prone to rupture and finally in this point the body lends to produce excessive number of RBC to help the oxygen saturation. This state is called polycythemia, and leads to increase risk of blood clotting in the lungs called pulmonary embolism.
Secondary Polycythemia is abnormally increase amount of RBC causing sluggish blood flow and an increased risk of clotting.
Large holes in the lungs (giant bullae). Some people with emphysema develop empty spaces in the lungs called bullae. Giant bullae can be as large as half the lung. In addition to reducing the amount of space available for the lung to expand, giant bullae can become infected and are more prone to causing a collapsed lung (pneumothorax).
Hyperventilation leading to pulmonary hypertension. As more of the lung is destroyed and the lung is destroyed and the lung cannot maintain oxygen concentration in the blood stream, the body compensates by gradually increasing the breath rate. Hyperventilation cannot maintain adequate oxygen levels, and the arteries in the lungs begin to constrict and narrow. The heart has to work harder to push blood into the narrow blood vessels, causing the blood pressure in the lung arteries to increase (pulmonary hypertension).
PHYSICAL CONDITION If emphysema is detected before causing symptoms, there may be some chance of reversing it, although permanent changes in the alveoli usually occur, even in young smokers. Patients with the inherited form of early-onset emphysema are at risk for early death, unless the disease is treated and its progression halted or slowed. Emphysema patients who experience severe involuntary weight loss (which indicates muscle wasting) have a poorer outlook, regardless of their lung function.
Patients with emphysema are often described as “pink puffers”. This description is secondary to the increase in PaO2, which is found on an arterial blood gas determination to be greater than 65mm Hg, and the normal (40 mm Hg) or slightly decreased PaCO2. Emphysematous lung destruction leads to a loss of pulmonary capillaries as a result of destruction of the alveolar walls. The ensuing loss of capillary bed area causes the loss of diffusion capacity, although PaO2 is found to be only mildly depressed, resulting in minimal pulmonary vasoconstriction.
Pink puffers are tachypneic (have an increased breathing rate) have mild hypoxemia, and are hypocapnic (Less than the normal level of carbon dioxide in the blood) or normocapnic (having the normal amount of carbon dioxide in arterial blood).
Pink Puffer
The pulmonary ventilation in is not uniform in Emphysema. The destruction of the alveolar septa with their capillaries results in diversion of blood to the intact alveoli which consequently get excessively perfused. On the other hand, the large alveoli formed by the breaking down of alveolar septa are overinflated as they have higher compliance, and the smaller, intact alveoli remain under ventilated. Thus emphysema, the normal alveoli are over perfused and under ventilated. This ventilation-perfusion mismatch results in greater reduction in the diffusion capacity than would have resulted from a reduction of alveolar surface area alone.
The chest becomes barrel-shaped and fixed in inspiration, with widening of the intercostal spaces. There may also be indrawing of the lower intercostal spaces and supraventricular fossa on inspiration. This is associated with the difficulty of ventilating stiff lungs through narrowed airways. The ribs are elevated by the accessory muscles of resoiration and there is loss of thoracic mobility.
Illustration shows a normal chested man and a barrel chested
There may be poor posture to a person with emphysema. There may be thoracic kyphosis plus elevated shoulder girdles.
Poor Posture
Flattening of the diaphragm also tends to impair the normal respiratory mechanisms of breathing because the flattened diaphragm functions of a mechanical disadvantage. As discussed loss of lung elastic recoil also results in restricted airflow because it facilitates compression of the airways during expiration. In severe cases, airflow may be limited even when the individual is breathing at rest, causing an individual to appear to be short of breath even when not engaged in any physical activity.
Cyanosis or bluish discoloration of the skin is due to the decrease oxygenation of the blood. The decrease however cannot be mild; there must be at least 5g of reduced hemoglobin per 100 mL of blood if cyanosis is to appear. It should be understood that cyanosis will appear with less severe anoxia in polycythemia than it will in anemia. Decreased oxygenation of the blood in emphysema may result from obstruction to the intake oxygen(i.e. emphysema) from the decreased absorption, as in conditions with alveolar capillary block; or from a ventilation-perfusion defect. Decreased oxygenation of the blood may also result from decreased perfusion of the lung with the blood in shock, pulmonary hemangiomas and Congenital heart disease. The hemoglobin may be unable to latch onto the oxygen in carbon monoxide poisoning and metheglobinemia, but the cyanotic hue to the face in cold weather, but the arterial oxygen saturation is not necessarily decreased.
Cyanosis
Severe chronic (long-term) lung diseases, including emphysema and chronic bronchitis, increase pressure in the blood vessels that lead from the heart to the lungs. This pressure backs up in the heart and the higher pressure causes swelling in the legs and feet.
Pedal Edema
Jugular vein distension certainly would be most suggestive of congestive heart failure, but other causes of jugular vein distension include superior vena cava syndrome due to a mediastinal mass such as carcinoma of the lung and constrictive pericarditis. Right heart side failure secondary to pulmonary emphysema can cause jugular vein distension.
PERSONALITY CHARACTERISTICS
Mood disorders such as depression and anxiety are common in patients with emphysema and have been associated with higher risk for hospitalizations, longer stays in the hospital, and poorer survival. Some estimates place the incidence of depression and anxiety at up to 44% of patients. Breathlessness or the fear of breathlessness can often lead to feelings of anxiety and depression. Anxiety was more common in women while depression was similar between genders. Fatigue leads to a lower level of oxygen in the blood, which in turn causes people to feel tired easily. Impotence are stress in a man's life or difficulties in his sexual relationships because of some drugs like Diuretics, tricyclic antidepressants, H2 blockers, beta-blockers being taken by the patients with emphysema. Other personality characteristics of emphysema include confusion, dizziness, and stress. Health care providers should be vigilant in monitoring the patients for mood disorders since these disorders are treatable and can play an important role in the quality of life especially for patients with severe emphysema.
PULMONARY REHABILITATION OF EMPHYSEMA
Pulmonary rehabilitation and exercise may increase your endurance. It is extremely important when doing pulmonary rehabilitation that you exercise in a monitored safe atmosphere where your oxygen status can be monitored.
Chest X-rays in advance disease may show a flattened diaphragm, reduced vascular markings at lung periphery, overrearation of the lungs, a vertical heartt, enlarged anteroposterior chest diameter, and a large retrosternal air space. CT scans may detect the disease earlier than other tests. 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. Pulmonary function test indicate increased TLC with decreased VC and a decrease in maximal expiratory flow rates, such as FEV1/FVC. RV is also increased, reflecting the larger TLC with decreased VC.
ABG analysis usually reveals reduced partial pressure of arterial oxygen and a normal partial pressure of arterial carbon dioxide until late in the disease process.
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.
Electrocardiography may show tall, symmetrical P waves in leads II, III, and aVF, a vertical QRS axis and signs of right ventricular hypertrophy are seen in late disease process.
Complete blood count usually reveals an increased hemoglobin level late in the disease when the patient has persistent, severe hypoxia.
Oxygen therapy is a key component of hospital treatment of an exacerbation. Supplemental oxygen should be titrated to improve the patient’s hypoxemia. Once oxygen is started arterial blood gases should be checked 30-60 minutes later to ensure satisfactory oxygenation without carbon dioxide retention or acidosis. Oxygen therapy constitutes the cornerstone of treatment in emphysema. Prolonged use of oxygen for 15 hours per day increases the life expectancies of patients experiencing chronic respiratory failure.13 For patients who have a PaO2 of 55 mm Hg or less (or a pulse-oximetry result of 88% or less), supplemental oxygen is indicated. The administration of oxygen to these patients generally improves gas exchange, decreases the work of the heart, reduces pulmonary vascular resistance, and improves the ability to perform activities of daily living. Oxygen is usually administered via standard nasal cannula or some type of oxygen-conserving device. Low flow oxygen therapy based on arterial blood gas results is often administered to treat hypoxemia.
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.
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.
Lung Volume Reduction Surgery is a surgical procedure during which the surgeon removes about 25% of the disease upper lobe of the lung so that the remaining healthier areas can function better. This procedure is usually reserved for patients with emphysema and has been shown to improve breathing, lung capacity, and quality of life.
The overall management of stable emphysematous patient is based on the following: 1.) the proper diagnosis and assessment of severity of the disease. 2.) Reduction of risk factors; 3.) Relief of symptoms; and 4.) Improvement in in exercise capacity, health status and patients quality of life.
(CHRONIC OBSTRUCTIVE
PULMONARY DISEASE)
EMPHYSEMA -is a condition of the lung characterized by abnormal, permanent enlargement of the airspaces distal to the terminal bronchiole, accompanied by destruction of their walls. This over-inflation results from a breakdown of the walls of the alveoli, which causes a decrease in respiratory function and breathlessness. In emphysema, the lungs loose elasticity and are unable to fully expand and contract. This occurs because the air sacs cannot completely deflate, thus unable to fill with fresh air for adequate ventilation. Emphysema in children is usually caused by congenital abnormalities of the lung and alpha-1 antitrypsin deficiency.In emphysema, the patient can breathe in but breathing out is difficult and inefficient. The seriousness of emphysema varies greatly. Some persons with emphysema never reach a stage of incapacity and go through life with relatively little …show more content…
inconvenience, while in others, emphysema worsens until final degeneration of the ability to breath occurs.
PREVALENCE OF EMPHYSEMA
-prevalence generally refers to the number of patients suffering with a condition at any particular time, while incidence rate refers to newly diagnosed cases or annual rates. In the US, around 2 million persons are managing emphysema or about 17 per 1000 population or 1 in 136, with a lifetime incidence risk of 1.4%. Around the world, China and India lead in emphysema prevalence with nearly 10 million and 8 million cases respectively; nations with approximately 1 million emphysema patients include Pakistan, Indonesia, Brazil, Russia, Japan, and Bangladesh; nations with emphysema prevalence rates of approximately 1/2 million include Germany, Italy, UK, Iran, Thailand, Egypt, Vietnam, and the Philippines.
ECONOMIC BURDEN OF EMPHYSEMA
Emphysema affect approximately 1.9 million Americans and is one of the fastest growing causes of morbidity and mortality in the USA. Worldwide, the social burden of chronic obstructive pulmonary disease (COPD), in terms of days lost to disability, is expected to increase from twelfth to fifth among all chronic diseases from 1990–2020. Given the prevalence of this disease and the duration of illness for those affected, medical expenditures for treating COPD and the indirect costs of morbidity can represent a substantial economic and social burden for societies and for public and private payers. More importantly, because emphysema is highly prevalent, new treatments that are widely adopted for this condition, even if inexpensive at the individual patient level, can have a tremendous impact on the overall economic burden of the disease. In today’s cost-conscious environment, evaluating the economic impact of new therapies has become nearly as important as understanding their clinical impact. As healthcare costs continue to escalate, more emphasis is being placed on understanding the economic implications associated with disease processes and their treatments. Unfortunately, very little economic information concerning COPD is available, particularly outside of a few developed Western nations. Given the rising prevalence of COPD worldwide, it is urgently necessary to understand its economic burden and to provide more robust evaluations of healthcare interventions designed to reduce its incidence and impact. Studies designed for making decisions and policy must apply robust methods and report results in a standardized fashion.
FACTORS THAT INFLUENCE DISEASE DEVELOPMENT AND PROGRESSION
Smoking. The primary risk factor for the development of emphysema is tobacco abuse. Cigarette smoke contributes to this disease process in 2 ways. It destroys lung tissue, which is the cause of the obstruction, and it causes inflammation and irritation of airways that can cause the disease to get worse. It is not possible to predict in any individual smoker who will develop emphysema, how long it will take, or how many cigarettes one must smoke. In fact, most smokers do not develop symptomatic emphysema, suggesting that most people have sufficient reserve capacity in the lung to get by despite any damage.
Tobacco smoking contributes to emphysema by:
1. Recruiting neutrophils into the lung by factors from smoke-activated alveolar macrophages.
2. Activating alveolar macrophages release elastase which injures elastic tissue of the lung.
3. Alveolar macrophage also stimulates the release of elastase from neutrophils.
4. Inactivation of alpha-1-antitrypsin by oxidants in tobacco smoke or free radicals released by activated neutrophils.
Age. Although the lung damage that occurs in emphysema develops gradually, most people with tobacco-related emphysema begin to experience symptoms of the disease between the ages of 40 and 60. Older age is a risk factor for emphysema. Lung function normally declines with age. Therefore, it stands to reason that the older the person, the more likely they will have enough lung tissue destruction to produce emphysema. In a few individuals, genetic factors such as a reduced level or activity of protective enzymes in the lung (such as occurs in alpha1-antiprotease deficiency) may result in emphysema even in the absence of smoking, but this is much rarer.
Exposure to secondhand smoke. Secondhand smoke, also known as passive or environmental tobacco smoke, is smoke that you inadvertently inhale from someone else's cigarette, pipe or cigar. Being around secondhand smoke increases your risk of emphysema.
Occupational exposure to fumes or dust. If you breathe fumes from certain chemicals or dust from grain, cotton, wood or mining products, you're more likely to develop emphysema. This risk is even greater if you smoke.
Exposure to indoor and outdoor pollution. Breathing indoor pollutants, such as fumes from heating fuel, as well as outdoor pollutants — car exhaust, for instance — increases your risk of emphysema. Air pollution acts in a similar manner to cigarette smoke. The pollutants cause inflammation in the airways, leading to lung tissue destruction. Abnormal airway reactivity, such as bronchial asthma, has been shown to be a risk factor for the development of emphysema. Men are more likely to develop emphysema than women. The exact reason for this is unknown, but differences between male and female hormones are suspected.
Genes. Alpha–1 antitrypsin (AAT), also called alpha-1 protease inhibitor, is an enzyme (protein) that helps to protect lung tissue and allow the alveoli to function properly. People who have a genetic (hereditary) deficiency of alpha-1 antitrypsin are at increased risk for developing alpha-1 antitrypsin deficiency emphysema or familial emphysema.
In this condition, damage to the structure and elasticity of the air sacs occurs unchecked. It is important that people who have this deficiency never smoke. AAT is manufactured in the liver, and patients who have this deficiency also may experience liver damage (e.g., cirrhosis, hepatitis).
PATHOLOGY, PATHOGENESIS, PATHOPHYSIOLOGY
PATHOLOGY
It is classified according to anatomical distribution of the lesion within the acinus.
Types of emphysema:
1.Centriacinar (centrilobular) emphysema: This is the commonest form of emphysema and is usually associated with clinical symptoms. It involves the cluster of terminal bronchioles near the end of bronchiolar tree, more exactly the proximal part of the acinus.
It is characterized by:
i) There is destruction and enlargement of central or proximal parts of respiratory unit, formed by respiratory bronchioles, whereas distal alveoli are spared. ii) The lesions are more common and severe in the upper lobes and superior segment of lower lobe. iii) Severe lesions are seen primarily in male smokers, often in association with chronic bronchitis. A. Cut surface from a lung with centriacinar emphysema showing holes in the center of lobules surrounded by relatively normal parenchyma. The severity varies among lobules. B. Microscopic section showing that the airspace enlargement in centriacinar emphysema is most marked adjacent to the abnormal respiratory bronchiole, corresponding to the center of the lobule. Also, some of the alveolar walls of the abnormal airspaces are thickened and fibrotic (H&E × 16).
2.Panacinar(panlobular)emphysema:
This is characterized by:
i) Initial involvement of alveolus and alveolar duct which extends to respiratory bronchioles. ii) Uniform destruction and enlargement of the acinus from the respiratory bronchiole to the terminal blind alveoli. iii) Mainly seen in lower basal zones. iv) Strong association with alpha-1-antitrypsin deficiency. C . Cut surface of a lung slice showing how the entire lobule is uniformly affected in panacinar emphysema. D. Microscopic section demonstrating that in panacinar emphysema, the airspaces adjacent to the lobular septa are enlarged to the same degree as those in the center of the lobule (H&E × 16).
3.Paraseptal(distal)emphysema: It involves mostly the distal acinus, proximal portion of the acinus is normal. It is found near the pleura along the lobular septa and at the margin of the lobules. It also occurs adjacent to fibrosis, scars or atelectasis and usually more severe in the upper half of the lungs. They show multiple, continuous, enlarged air spaces from less than 0.5 mm to more than 2.0 cm in diameter, often forming cyst-like structures. This type probably underlies many of the cases of spontaneous pneumothorax in young adults.
4. Irregular emphysema -is the irregular involvement of the acinus and is associated with scarring. It is usually asymptomatic and is the commonest autopsy finding.
Other emphysemas are:
i) Bullous emphysema: These are blebs or bullae larger than 1 cm in diameter and mostly subpleural near the apex. They are the localized form of one of the four types of emphysema. Sometimes they are seen in relation to tuberculous scarring.
ii) Interstitial emphysema: This is characterized by entry of air into the connective tissue of lung, mediastinum or subcutaneous tissue. Alveolar tears allow the entry of air into the connective tissues. Progressive accumulation of air in the connective tissue may cause marked swelling of the head and neck, chest wall with cracking crepitation all over the chest. In most instances such air is promptly reabsorbed as soon as the point of entrance is sealed.
Common causes of interstitial emphysema:
Lung penetrating chest injury.
Fracture rib that punctures the lung substance.
Patients being artificially ventilated.
Individuals who suddenly inhale irritant gases.
Combination of coughing with bronchiolar obstruction, which increase alveolar-sac pressure.
Children with whooping cough and airway obstruction by blood clots, foreign body etc.
iii) Compensatory emphysema: This is the dilation of alveoli not due to destruction of septal walls but compensatory expansion of the residual lung parenchyma after surgical removal of a diseased lung or lobe. iv) Senile emphysema - is the over- distended, voluminous lungs found in the aged. This is due to age-related changes in the lung, i.e. larger alveolar ducts and smaller alveoli without loss of elastic tissue or destruction of lung substance.
PATHOGENESIS
Protease-antiprotease hypothesis holds that destruction of alveolar walls in emphysema is due to an imbalance between proteases and their inhibitors in the lung.
This is evidenced from:
i) Hereditary deficiency of the major protease inhibitor, alpha-1-antitrypsin, invariably develop emphysema. ii) Pulmonary instillation of proteolytic enzyme, and neutrophil elastase, results in emphysema in experimental animals.
PATHOPHYSIOLOGY
In emphysema, recurrent inflammation is associated with the release of proteolytic enzymes from the lung cells. This causes irreversible enlargement of the airspaces distal to the terminal bronchioles. Enlargement of the airspaces destroys the alveolar walls, which results in a breakdown of elasticity and loss of fibrous and muscle tissue, thus making the lungs more compliant.
In patients with emphysema, recurrent pulmonary inflammation damages and eventually destroys the alveolar walls creating large spaces. The alveolar septa are initially destroyed, eliminating a portion of the capillary bed and increasing air volume in the acinus. This breakdown leaves the alveoli unable to recoil normally after expanding and results in bronchiolar collapse on expiration. The damage or destroyed alveolar walls can’t support the airways to keep them open. The amount of the air that can be expired passively is diminished, thus trapping air in the lungs and leading to over distension.
Hyperinflation of the alveoli produces bullae (air spaces) adjacent to the pleura (blebs). Septal destruction also decreases airway calibration. Part of each inspiration is trapped because of increased residual volume and decreased calibration. Septal destruction may affect only the respiratory bronchioles and alveolar ducts, leaving alveolar sacs intact (centriacinar emphysema), or it can involve the entire acinus (panacinar emphysema), producing damage that’s more random and involves in the lower lobes of the lungs.
Associated pulmonary capillary destruction usually allows a patient with severe emphysema to match ventilation to perfusion. This process prevents the development of cyanosis. The lungs are usually enlarged; therefore the total lung capacity and residual volume increase.
Pulmonary emphysema results in hyperinflation of the lungs and concomitant changes in the configuration of the thoracic cavity.
Lung Overinflation or Hyperinflation is a critically important component part of Emphysema, and particularly so with Emphysema. In fact, recent evidence suggests that correction of Hyperinflation appears to be more important in the relief of dyspnea, than is the correction of airway obstruction with broncodilator therapy. Said another way, if you use so-called Rescue Drugs such as Metered Dose Inhalers to relieve dyspnea caused by airway bronchospasm, the majority of your dyspnea relief comes not from the bronchospasm relief, but rather as a result of relieved bronchospasm now permitting correction of hyperinflation.
This section will tell you how to obtain further dyspnea relief, after you have used your bronchodilator medications. Bronchodilator medications are very important, but they are only the first step to obtain maximal dyspnea relief.
There are two general types of overinflation. The first is so-called "Anatomic Hyperinflation" seen in Emphysema, where there is actual destruction of alveolar lung tissue, to create enlarged cystic overdistended air spaces.
The second general type of overinflation is so-called "Physiologic Hyperinflation" seen in both COPD and Emphysema. The underlying problem here is the airway obstruction common to both conditions. With increased airway obstruction causing increased resistance to air flow, the lung may not have enough time to empty before the next inhaled breath.
Remember, on breathing in, all the structures in the lung, including the airways, get larger, and therefore air moves into the lung relatively easier on inspiration. Conversely, on breathing out, everything in the lungs, including the airways, gets smaller. Therefore, it is always relatively more difficult to to get air out of the lung on expiration. As a result, some air is trapped in the lung, causing it to overinflate.
This diagram is from 1955 first edition of The Lung, by Dr. Julius Comroe et al.
The lung is depicted as a single air sack, and the arrow indicates air moving in and out of the lung. The dark wavy line below is the subject breathing in and out.
Figure A shows the normal condition, with air moving out freely, and no lung over inflation.
Figure B shows some airway obstruction, and therefore "Air Trapping" on expiration, and with over inflation developing. Note the breathing tracing moving upward.
Figure C shows even more airway obstruction, and the resulting increased over inflation.
The problem here is not enough time for the lung to empty on expiration. The so-called Time Constant required for lung emptying has been exceeded. Note carefully, the faster you breathe, the worse this problem will become. The older term "Physiologic Hyperinflation" is now evolving into the name "Dynamic Hyperinflation," and more recently has generally been used as a phenomenon related to patient exertion. However, this is not entirely correct, as it is now clear that this type of hyperinflation is commonly present to some degree, even with mild to moderate airway obstructive disease, even while patients are at rest. Dynamic Hyperinflation therefore is of two general types, "Resting Dynamic Hyperinflation" and "Active Dynamic Hyperinflation." The importance of this surprising recent observation that Dynamic Hyperinflation is frequently present at rest in mild to moderate airway obstructive disease is not that it is causing significant dyspnea at rest. In fact, it generally is not of significance. However, it is clear that these generally asymptomatic patients are indeed vulnerable to further exacerbation of their Dynamic Hyperinflation should they increase their breathing rate for exertion or whatever reason, and therefore have an exaggerated dyspnea response. Clearly, this problem can no longer be considered as a significant factor only in severe COPD.
THE FUNCTIONAL ABNORMALITIES IN EMPHYSEMA WITH BREATHING PATTERNS Although emphysema (i.e. destruction of alveolar walls) leads to decreased expiratory flow rates, the pathophysiology is different from the situation in pure airway disease. The primary problem in emphysema is loss of elastic recoil (i.e., loss of the lung natural tendency to resist expansion). One consequence of decreased elastic recoil is a decreased driving pressure that expels air from the alveoli during expiration. A simple analogy is a balloon filled with air, in which the elastic recoil is the “stiffness” of the balloon. When a given volume of air inside an unsealed balloon, a stiffer balloon will expel air more rapidly than will a less stiff balloon. An emphysematous lung is like, a less stiff balloon: a smaller than normal force drives air out of the lungs during expiration. Loss of driving pressure is not the only consequence of emphysema. There is also an indirect effect on the collapsibility of airways. Normally outward traction is exerted on the walls of airways by supporting structure of tissue from the lung parenchyma. When the alveolar tissue is disrupted, as in emphysema, the supporting structure for the airways is diminished, and less radial traction is exerted to prevent airway collapse during a forced expiration, the strongly positive pleural pressure promotes collapse. Airways lacking an adequate supporting structure are more likely to collapse (and have diminished flow rates and air trapping) than are normally supported airways. The decrease in elastic recoil in emphysema also alters the compliance curve of the lung and the measured of lung volumes. The compliance curve relates transpulmonary pressure and the associated volume of gas within the lung. Because an emphysematous lung has less elastic recoil (i.e. is loss stiff), it resist expansion loss than does its normal counterpart. Therefore, the compliance curve is shifted upward and to the left, and the lung has more volume at any particular transpulmonary pressure. TLC is increased because loss elastic recoil results in a smaller force opposing the action of the inspiratory musculature. FRC is also increase because the balance between the outward recoil of the chest wall and they inward recoil of the lung is shifted in favor of the chest wall. As in bronchitis, RV is substantially increased in emphysema because poorly supported airways are more susceptible to closure during a maximal expiration. Compliance curve of lung in emphysema compared with that of normal lung. In addition to shift to curve upward and to left. Total lung capacity in emphysema(point B on volume axis) is greater than total ling capacity(point A). in pure chronic bronchitis without emphysema, the compliance curve is normal. Patterns of pulmonary function test in Emphysema
PROGNOSIS
If you catch emphysema in the early stages, it is possible to reverse the effects, particularly if the patient is a smoker who quits. However it is difficult to diagnose emphysema early, since its symptoms mimic those of acute illnesses such as bronchitis. If it remains undiscovered until the middle or end stages, there is no way to reverse the damage done. There are treatments which can alleviate symptoms and increase the quality of life for the patient. These treatments may extend the life expectancy for the patient, but the end result for most cases that are not caught early is fatal.
The possible outcome of a person with emphysema show a rapid decline in lung function while others show a more gradual decline. They are usually determined by how the lungs are able to get oxygen in and carbon dioxide out of the body. When the lungs deteriorate to such a point that they cannot function well, symptoms become more obvious and we commonly say the disease is approaching “end stage”.
Dyspnea is a cardinal symptom of emphysema, is a major of disability and anxiety associated with the disease. Typical emphysematous patients describe their dyspnea as a sense of increased effort to breathe, heaviness, air hunger or grasping. The destruction of lung tissue seen with emphysema eventually results in dysfunction of virtually all the functions of the lung, as well as that of the respiratory muscles and chest wall. By losing elasticity, the lungs become more difficult to ventilate. The lungs rapidly become progressively more impaired and air becomes trapped within the lung. This is why emphysema is termed an “obstructive” lung disease. This impairment increases the work of breathing. Patients first experience symptoms with exercise but as the disease progresses shortness of breath can occur with all activities and even at rest.
In addition, as the lung loses its elasticity, the chest wall and ribs begin to expand and become overinflated. This results in a flattening of the major inspiratory muscle, the diaphragm, and causes the muscle to work less efficiently. This further adds to the increased work of breathing and diminishes the ability of the diaphragm to cope with even a normal workload. As a result, shortness of breath worsens.
The main function of the lungs is to add oxygen and remove carbon dioxide from the blood. As emphysema progresses, patients may have an inadequate amount of oxygen in the blood. As the disease worsens the amount of carbon dioxide in the blood increases. If the oxygen in the blood is not supplemented, the entire body suffers, including the heart and the brain.
The patient with emphysema becomes “out of shape” (deconditioned) because the disease does not allow sufficient exercise to stay in shape.
Anorexia which lead to malnutrition. Anorexia is the medical term for "loss of appetite." It is not, however, to be confused with the term, "anorexia nervosa," which is a serious mental condition not associated with COPD. Progressive weight loss in emphysema occurs because of a number of factors: Fatigue and shortness of breath which can interfere with meal preparation and consumption. Increased phlegm which can alter the taste of food. Flattening of the diaphragm (breathing muscle) which presses on the stomach causing a premature feeling of fullness. Depression over having a chronic illness. Side effects of medications such as nausea and indigestion.
The main symptom of an emphysema exacerbation is increasing shortness of breath.
Exacerbations of emphysema can be serious, even life threatening. They can occur when a person has Chronic Obstructive Pulmonary Disease (COPD), including emphysema, and his or her symptoms get worse very quickly. Exacerbations of emphysema can be triggered by infections such as colds or the flu. Being around lung irritants such as air pollution, dusts, fumes or smoke can also trigger an exacerbation. Other medical conditions, such as heart problems or infection elsewhere in the body, can make breathing problems worse. You may cough up more mucus than usual, or mucus that is a different color than usual. Exacerbations of emphysema need to be treated as soon as possible. You may also cough up more than usual or mucus that is a different color than normal or increasing trouble breathing that would be a sign of an infection and a decrease exercise tolerance due to shortness of breath during the exercise may be seen in
emphysema.
Possible complications of emphysema include cor pulmonale. Cor pulmonale is defined as right heart failure secondary to lung disease. Cor pulmonale results from the effects of chronic hypoxemia on the pulmonary vasculature. Hypoxemia causes pulmonary vasoconstriction that increases the right ventricles afterload. Pumping against the high pulmonary vascular pressures increases right muscle work, which eventually causes right sided heart failure. Usually patients with emphysema experience a progressive decline in exercise ability and become short of breath even at rest.
Pneumothorax is a possible complication of emphysema. As emphysema progresses and more alveoli are destroyed the air escapes from the lungs and collects outside the lungs in the thorax. This happens in patients suffering emphysema since the walls of the alveoli are thinner and prone to rupture and finally in this point the body lends to produce excessive number of RBC to help the oxygen saturation. This state is called polycythemia, and leads to increase risk of blood clotting in the lungs called pulmonary embolism.
Secondary Polycythemia is abnormally increase amount of RBC causing sluggish blood flow and an increased risk of clotting.
Large holes in the lungs (giant bullae). Some people with emphysema develop empty spaces in the lungs called bullae. Giant bullae can be as large as half the lung. In addition to reducing the amount of space available for the lung to expand, giant bullae can become infected and are more prone to causing a collapsed lung (pneumothorax).
Hyperventilation leading to pulmonary hypertension. As more of the lung is destroyed and the lung is destroyed and the lung cannot maintain oxygen concentration in the blood stream, the body compensates by gradually increasing the breath rate. Hyperventilation cannot maintain adequate oxygen levels, and the arteries in the lungs begin to constrict and narrow. The heart has to work harder to push blood into the narrow blood vessels, causing the blood pressure in the lung arteries to increase (pulmonary hypertension).
PHYSICAL CONDITION If emphysema is detected before causing symptoms, there may be some chance of reversing it, although permanent changes in the alveoli usually occur, even in young smokers. Patients with the inherited form of early-onset emphysema are at risk for early death, unless the disease is treated and its progression halted or slowed. Emphysema patients who experience severe involuntary weight loss (which indicates muscle wasting) have a poorer outlook, regardless of their lung function.
Patients with emphysema are often described as “pink puffers”. This description is secondary to the increase in PaO2, which is found on an arterial blood gas determination to be greater than 65mm Hg, and the normal (40 mm Hg) or slightly decreased PaCO2. Emphysematous lung destruction leads to a loss of pulmonary capillaries as a result of destruction of the alveolar walls. The ensuing loss of capillary bed area causes the loss of diffusion capacity, although PaO2 is found to be only mildly depressed, resulting in minimal pulmonary vasoconstriction.
Pink puffers are tachypneic (have an increased breathing rate) have mild hypoxemia, and are hypocapnic (Less than the normal level of carbon dioxide in the blood) or normocapnic (having the normal amount of carbon dioxide in arterial blood).
Pink Puffer
The pulmonary ventilation in is not uniform in Emphysema. The destruction of the alveolar septa with their capillaries results in diversion of blood to the intact alveoli which consequently get excessively perfused. On the other hand, the large alveoli formed by the breaking down of alveolar septa are overinflated as they have higher compliance, and the smaller, intact alveoli remain under ventilated. Thus emphysema, the normal alveoli are over perfused and under ventilated. This ventilation-perfusion mismatch results in greater reduction in the diffusion capacity than would have resulted from a reduction of alveolar surface area alone.
The chest becomes barrel-shaped and fixed in inspiration, with widening of the intercostal spaces. There may also be indrawing of the lower intercostal spaces and supraventricular fossa on inspiration. This is associated with the difficulty of ventilating stiff lungs through narrowed airways. The ribs are elevated by the accessory muscles of resoiration and there is loss of thoracic mobility.
Illustration shows a normal chested man and a barrel chested
There may be poor posture to a person with emphysema. There may be thoracic kyphosis plus elevated shoulder girdles.
Poor Posture
Flattening of the diaphragm also tends to impair the normal respiratory mechanisms of breathing because the flattened diaphragm functions of a mechanical disadvantage. As discussed loss of lung elastic recoil also results in restricted airflow because it facilitates compression of the airways during expiration. In severe cases, airflow may be limited even when the individual is breathing at rest, causing an individual to appear to be short of breath even when not engaged in any physical activity.
Cyanosis or bluish discoloration of the skin is due to the decrease oxygenation of the blood. The decrease however cannot be mild; there must be at least 5g of reduced hemoglobin per 100 mL of blood if cyanosis is to appear. It should be understood that cyanosis will appear with less severe anoxia in polycythemia than it will in anemia. Decreased oxygenation of the blood in emphysema may result from obstruction to the intake oxygen(i.e. emphysema) from the decreased absorption, as in conditions with alveolar capillary block; or from a ventilation-perfusion defect. Decreased oxygenation of the blood may also result from decreased perfusion of the lung with the blood in shock, pulmonary hemangiomas and Congenital heart disease. The hemoglobin may be unable to latch onto the oxygen in carbon monoxide poisoning and metheglobinemia, but the cyanotic hue to the face in cold weather, but the arterial oxygen saturation is not necessarily decreased.
Cyanosis
Severe chronic (long-term) lung diseases, including emphysema and chronic bronchitis, increase pressure in the blood vessels that lead from the heart to the lungs. This pressure backs up in the heart and the higher pressure causes swelling in the legs and feet.
Pedal Edema
Jugular vein distension certainly would be most suggestive of congestive heart failure, but other causes of jugular vein distension include superior vena cava syndrome due to a mediastinal mass such as carcinoma of the lung and constrictive pericarditis. Right heart side failure secondary to pulmonary emphysema can cause jugular vein distension.
PERSONALITY CHARACTERISTICS
Mood disorders such as depression and anxiety are common in patients with emphysema and have been associated with higher risk for hospitalizations, longer stays in the hospital, and poorer survival. Some estimates place the incidence of depression and anxiety at up to 44% of patients. Breathlessness or the fear of breathlessness can often lead to feelings of anxiety and depression. Anxiety was more common in women while depression was similar between genders. Fatigue leads to a lower level of oxygen in the blood, which in turn causes people to feel tired easily. Impotence are stress in a man's life or difficulties in his sexual relationships because of some drugs like Diuretics, tricyclic antidepressants, H2 blockers, beta-blockers being taken by the patients with emphysema. Other personality characteristics of emphysema include confusion, dizziness, and stress. Health care providers should be vigilant in monitoring the patients for mood disorders since these disorders are treatable and can play an important role in the quality of life especially for patients with severe emphysema.
PULMONARY REHABILITATION OF EMPHYSEMA
Pulmonary rehabilitation and exercise may increase your endurance. It is extremely important when doing pulmonary rehabilitation that you exercise in a monitored safe atmosphere where your oxygen status can be monitored.
Chest X-rays in advance disease may show a flattened diaphragm, reduced vascular markings at lung periphery, overrearation of the lungs, a vertical heartt, enlarged anteroposterior chest diameter, and a large retrosternal air space. CT scans may detect the disease earlier than other tests. 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. Pulmonary function test indicate increased TLC with decreased VC and a decrease in maximal expiratory flow rates, such as FEV1/FVC. RV is also increased, reflecting the larger TLC with decreased VC.
ABG analysis usually reveals reduced partial pressure of arterial oxygen and a normal partial pressure of arterial carbon dioxide until late in the disease process.
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.
Electrocardiography may show tall, symmetrical P waves in leads II, III, and aVF, a vertical QRS axis and signs of right ventricular hypertrophy are seen in late disease process.
Complete blood count usually reveals an increased hemoglobin level late in the disease when the patient has persistent, severe hypoxia.
Oxygen therapy is a key component of hospital treatment of an exacerbation. Supplemental oxygen should be titrated to improve the patient’s hypoxemia. Once oxygen is started arterial blood gases should be checked 30-60 minutes later to ensure satisfactory oxygenation without carbon dioxide retention or acidosis. Oxygen therapy constitutes the cornerstone of treatment in emphysema. Prolonged use of oxygen for 15 hours per day increases the life expectancies of patients experiencing chronic respiratory failure.13 For patients who have a PaO2 of 55 mm Hg or less (or a pulse-oximetry result of 88% or less), supplemental oxygen is indicated. The administration of oxygen to these patients generally improves gas exchange, decreases the work of the heart, reduces pulmonary vascular resistance, and improves the ability to perform activities of daily living. Oxygen is usually administered via standard nasal cannula or some type of oxygen-conserving device. Low flow oxygen therapy based on arterial blood gas results is often administered to treat hypoxemia.
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.
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.
Lung Volume Reduction Surgery is a surgical procedure during which the surgeon removes about 25% of the disease upper lobe of the lung so that the remaining healthier areas can function better. This procedure is usually reserved for patients with emphysema and has been shown to improve breathing, lung capacity, and quality of life.
The overall management of stable emphysematous patient is based on the following: 1.) the proper diagnosis and assessment of severity of the disease. 2.) Reduction of risk factors; 3.) Relief of symptoms; and 4.) Improvement in in exercise capacity, health status and patients quality of life.