Rehabilitation Systems

Chronic Obstructive Pulmonary Disease (COPD)

Pulmonary diseases are becoming more important causes of morbidity and mortality in the modern world, with COPD being the most common and a major cause of lung-related death and disability.
In the United States, COPD accounted for 119,000 deaths in 2000, ranking it the fourth leading cause of death and the only major disease among the top 10 that continues to increase. Mortality data tend to underestimate the impact of COPD because it is more likely to be listed as contributory rather than the underlying cause of death, and is often not listed at all. Between 1980 and 2000, death rates for COPD increased 282% for women compared to 13% for men. Also in 2000, the number of women dying from COPD exceeded the number of men.
JAMA. 2005; 294 (10) : 1255-1259

Chronic obstructive pulmonary disease (COPD) is a lung disease characterized by chronic obstruction of lung airflow that interferes with normal breathing and is not fully reversible. The more familiar terms 'chronic bronchitis' and 'emphysema' are no longer used, but are now included within the COPD diagnosis. COPD is not simply a "smoker's cough" but an under-diagnosed, life-threatening lung disease.

A COPD diagnosis should be considered in any patient who has symptoms of cough, sputum production, or dyspnea (difficult or labored breathing), and/or a history of exposure to risk factors for the disease. Where spirometry is unavailable, the diagnosis of COPD should be made using all available tools. Clinical symptoms and signs, such as abnormal shortness of breath and increased forced expiratory time, can be used to help with the diagnosis. A low peak flow is consistent with COPD, but may not be specific to COPD because it can be caused by other lung diseases and by poor performance during testing. Chronic cough and sputum production often precede the development of airflow limitation by many years; although not all individuals with cough and sputum production go on to develop COPD.

COPD is a progressive disease that makes it hard to breathe. COPD can cause coughing that produces large amounts of mucus, wheezing, shortness of breath, chest tightness, and other symptoms. Cigarette smoking is the leading cause of COPD. Most people who have COPD smoke or used to smoke. Long-term exposure to other lung irritants, such as air pollution, chemical fumes, or dust, also may contribute to COPD.

The air that you breathe goes down your windpipe into tubes in your lungs called bronchial tubes, or airways. The airways are shaped like an upside-down tree with many branches. At the end of the branches are tiny air sacs called alveoli. The airways and air sacs are elastic. When you breathe in, each air sac fills up with air like a small balloon. When you breathe out, the air sac deflates and the air goes out.

In COPD, less air flows in and out of the airways because of one or more of the following:
The airways and air sacs lose their elastic quality.
The walls between many of the air sacs are destroyed.
The walls of the airways become thick and inflamed (swollen).
The airways make more mucus than usual, which tends to clog the airways.
Healthy Alveoli and Damaged Alveoli

In the United States, the term "COPD" includes two main conditions—
emphysema and chronic bronchitis. In emphysema, the walls between many of the air sacs are damaged, causing them to lose their shape and become floppy with air trapping. This damage also can destroy the walls of the air sacs, leading to fewer and larger air sacs instead of many tiny ones, hence the barrel chest patient presentation.

In chronic obstructive bronchitis, the lining of the airways is constantly irritated and inflamed. This causes the lining to thicken. Lots of thick mucus forms in the airways, making it hard to breathe. Most people who have COPD have both emphysema and chronic obstructive bronchitis. Thus, the general term "COPD" is more accurate.

COPD develops slowly. Symptoms often worsen over time and can limit one‘s ability to do routine activities. Severe COPD may prevent one from doing even basic activities like walking, cooking, or taking care of self. Most of the time, COPD is diagnosed in middle-age to older-age. The disease isn't passed from person to person—one can't catch it from someone else. COPD has no cure, and doctors don't know how to reverse the damage to the airways and lungs. However, treatments and lifestyle changes can help one feel better, stay more active, and slow the progress of the disease through disease management standards of care.

Alpha-1 antitrypsin deficiency, or A1A deficiency, is a condition that raises your risk for certain types of lung disease, especially if you smoke. A1A deficiency is an inherited condition; it's passed in the genes from parents to children. Some people who have severe A1A deficiency develop emphysema, often when they're only in their forties or fifties. Emphysema is a serious lung disease in which damage to the airways makes it hard to breathe.

A smaller number of people who have A1A deficiency have cirrhosis and other serious liver diseases. Cirrhosis is a disease in which the liver is scarred. This prevents the liver from working properly. In people who have A1A deficiency, cirrhosis and other liver diseases usually occur in infancy and early childhood. A very small number of people who have A1T deficiency have a rare type of skin disease called necrotizing panniculitis. This skin disease can cause painful lumps under or on the surface of the skin.

Alpha-1 antitrypsin, is a protein made in the liver. Normally, the protein goes into the bloodstream and helps protect the body's organs from the harmful effects of other proteins. One of the main organs it protects is the lungs. A1A deficiency occurs when the A1A proteins made in the liver aren't the right shape. They get stuck inside liver cells and can't get into the bloodstream. Because not enough A1A protein travels to the lungs to protect them, the risk of lung disease increases. Also, because too many A1A proteins are stuck in the liver, liver disease can develop.

Persistent asthma is a chronic inflammatory pulmonary disorder that is characterized by reversible obstruction of the airways.

The underlying cause of the increasing prevalence of asthma is unknown. However, the airway inflammation that is noted in asthma is due to an immune-mediated process in which inflammatory cells and inflammatory mediators enter airway tissues to cause disease. Many cell-mediated immunologic factors participate in the inflammatory process of asthma. The most important inflammatory cells involved are eosinophils, mast cells, and T lymphocytes.

Important aspects that define asthma include airway hyperresponsiveness and bronchoconstriction. Airway hyperresponsiveness refers to an increased tendency of the asthmatic airway to react to a variety of stimuli that would not cause a response in a normal airway. These asthma triggers can cause an asthma attack in an inflamed airway. Bronchoconstriction refers to a narrowing of the airways that causes obstruction of airflow (sometimes termed airflow limitation). The bronchoconstriction of asthma is unique because it is at least partly reversible, either spontaneously or with treatment.

When inflamed airways respond to an asthma trigger through bronchoconstriction, the characteristic symptoms of asthma appear namely wheezing, cough, and chest tightness or dyspnea. Wheezing is due to airflow limitation, causing a high-pitched whistling sound, which is usually heard on expiration, but it may also be heard on inspiration. Cough probably results from stimulation of sensory nerves in the airways by inflammatory mediators that are released by various inflammatory cells involved in asthma. Chest tightness or dyspnea is the sensation associated with the increased work needed to breathe when the airways are constricted that patients often feel.

Bronchiolitis Obliterans Organizing Pneumonia.
BOOP is inflammation in the lungs. Bronchiolitis is inflammation of the very small airways called the bronchioles, less than one millimeter in diameter. There are over 200,000 of these airways.
Obliterans means that the inflammation in these small airways completely fills the bronchioles and obliterates the opening of the airway with inflammation. Organizing is a term used to describe the pattern of the inflammation cells. Pneumonia means that the lung is filled with inflammation, where the blood takes up oxygen. The rounded structures where this occurs are called alveoli. There are millions of them. If unfolded, they create an area the size of a tennis court.

Dr. Epler first reported BOOP in 1985. Dr. Epler reported about 50 individuals who developed a flu-like illness, "crackles" in the lungs, and lung function tests that showed a decreased lung capacity and a decreased diffusion of oxygen into the blood. The chest x-ray showed "patchy shadows" in both lungs.
Dr. Epler named this illness bronchiolitis obliterans organizing pneumonia (BOOP) because this is the pattern seen by the pathologists - doctors who examine lung tissue material. Some colleagues refer to it as Epler's pneumonia. Most of the time, about 80% of the time, the cause of BOOP is not known and referred to as idiopathic BOOP.

BOOP may be caused by an infection such as a virus or unusual bacteria can cause BOOP. There has been a report of BOOP caused by the malaria parasite. BOOP may be caused by medications. These include some anti-cancer medications - rituximab therapy for non-Hodgkin's lymphoma.
Conditions and circumstances that have been shown to be associated with BOOP include:
 A heart-lung, lung or bone marrow transplant
 infection by a virus or unusual bacteria
 Anti-cancer medications such as chemo therapy
 Long term use of antibiotics
 Illicit use of cocaine
 Some connective tissue disorders such as lupus and rheumatoid arthritis
 Exposure to toxins and fumes, especially NO2 and old textile dye processes
There are miscellaneous causes or associated disorders. Almost every month, there are new reports. For example, BOOP occurs after radiation therapy for breast cancer. BOOP is associated with lymphoma or other cancers. BOOP has occurred in textile workers exposed to an unusual type of textile dye. Remarkably, a high dose of L-Tryptophan, a popular supplement among health enthusiasts for use in sleep and pain disorders, can cause BOOP. There has been a report of BOOP from exposure to a high concentration of mold dust.

BOOP may also be secondary to an underlying lung disease. For example, BOOP may be the inflammation associated with idiopathic pulmonary fibrosis (IPF), which is also called usual interstitial pneumonia (UIP). This is a progressive scarring lung disease.
Anyone, anywhere in the world can get BOOP. It occurs in both men and women equally. It usually develops between the ages of 40 years and 60 years. It can occur in children and in patients over 80 years old. BOOP may occur at any time of the year.

Bronchiectasis is a condition in which damage to the airways causes them to widen and become flabby and scarred. The airways are tubes that carry air in and out of your lungs. Bronchiectasis usually is the result of an infection or other condition that injures the walls of your airways or prevents the airways from clearing mucus. Mucus is a slimy substance that the airways produce to help remove inhaled dust, bacteria, and other small particles.

In bronchiectasis, your airways slowly lose their ability to clear out mucus. When mucus can't be cleared, it builds up and creates an environment in which bacteria can grow. This leads to repeated, serious lung infections. Each infection causes more damage to your airways. Over time, the airways lose their ability to move air in and out. This can prevent enough oxygen from reaching your vital organs. Bronchiectasis can lead to serious health problems, such as respiratory failure, atelectasis and heart failure.

Bronchiectasis can affect just one section of one of your lungs or many sections of both lungs. The initial lung damage that leads to bronchiectasis often begins in childhood. However, symptoms may not appear until months or even years after you start having repeated lung infections.

In the United States, common childhood infections, such as whooping cough and measles, used to cause many cases of bronchiectasis. However, these causes are now less common due to the use of vaccines and antibiotics. Now, in the United States, bronchiectasis usually is due to an underlying medical condition that injures the airway walls or prevents the airways from clearing mucus. Examples of such conditions include cystic fibrosis and primary ciliary dyskinesia.

Cystic fibrosis, or CF, is an inherited disease of your secretory glands, including the glands that make mucus and sweat. People who have CF inherit two faulty CF genes—one from each parent. The parents likely don't have the disease themselves. CF mostly affects the lungs, pancreas, liver, intestines, sinuses, and sex organs.

Mucus is a substance made by the lining of some body tissues. Normally, mucus is a slippery, watery substance. It keeps the linings of certain organs moist and prevents them from drying out or getting infected. However, if one has CF, the mucus becomes thick and sticky. The mucus builds up in one‘s lungs and blocks the airways. The buildup of mucus makes it easy for bacteria to grow. This leads to repeated, serious lung infections. Over time, these infections can severely damage the lungs. The thick, sticky mucus also can block tubes, or ducts, in the pancreas. As a result, the digestive enzymes that the pancreas makes can't reach the small intestine.

These enzymes help break down the food that one eats. Without them, the intestines can't fully absorb fats and proteins. This can cause vitamin deficiency and malnutrition because nutrients leave the body unused. It also can cause bulky stools, intestinal gas, a swollen belly from severe constipation, and pain or discomfort. CF also causes the sweat to become very salty. As a result, the body loses large amounts of salt when one sweats. This can upset the balance of minerals in the blood and cause a number of health problems. Examples include dehydration, increased heart rate, tiredness, weakness, decreased blood pressure, heat stroke, and, rarely, death. If one has CF, they are also at increased risk for diabetes or osteoporosis. CF also causes infertility in men, and it can make it harder for women to get pregnant.

The symptoms and severity of CF vary from person to person. Some people who have CF have serious lung and digestive problems. Other people have more mild disease that doesn't show up until they're adolescents or adults. The symptoms and severity of CF also vary over time. Sometimes, one will have few symptoms. Other times, the symptoms may become more severe. As the disease gets worse, one will have more severe symptoms more often.

Lung function often starts to decline in early childhood in people who have CF. Over time, permanent damage to the lungs can cause severe breathing problems. Respiratory failure is the most common cause of death in people who have CF.

As treatments for CF continue to improve, so does life expectancy for those who have the disease. Today, some people who have CF are living into their forties, fifties, or older. Early treatment for CF can improve both your quality of life and lifespan. Such early treatment includes nutritional and respiratory therapies, medicines, exercise rehabilitation, and deliberate physical activity plans.

Pulmonary fibrosis is a condition in which tissue deep in the lungs becomes thick and stiff, or scarred, over time. The development of the scarred tissue is called fibrosis. As the lung tissue becomes thicker, the lungs lose their ability to move oxygen into the bloodstream. As a result, one‘s brain and other organs don't get the oxygen they need. In some cases, doctors can find out what is causing the fibrosis. But in most cases, they can't find a cause. They call these cases idiopathic pulmonary fibrosis (IPF).

IPF is a serious condition. About 200,000 Americans have it. About 50,000 new cases are diagnosed each year. IPF mostly affects people who are 50 to 75 years of age. IPF varies from person to person. In some people, the lung tissue quickly becomes thick and stiff. In others, the process is much slower. In some people, the condition stays the same for years. IPF has no cure yet. Many people live only about 3 to 5 years after diagnosis. The most common cause of death related to IPF is respiratory failure. Other causes include:
 Pulmonary hypertension
 Heart failure
 Pulmonary embolism
 Pneumonia
 Lung Cancer

Some people may be more likely to develop IPF because of their genes (the basic units of heredity). If more than one member of your family has IPF, the condition is called familial idiopathic pulmonary fibrosis. Today, scientists are beginning to understand more about what causes IPF, and they can diagnose it more quickly. They also are studying several medicines that may slow the progress of the disease. These efforts should improve the lifespan and quality of life for people who have IPF.
In IPF, lung tissue becomes scarred. The scarring typically starts at the edges of the lungs and progresses towards the center of the lungs, making it more and more difficult for a person to breathe. Unfortunately, IPF is a disabling disease that can be fatal.

Occupational lung disease Occupational lung diseases are a branch of occupational diseases concerned primarily with work related exposures to harmful substances, be they dusts or gases, and the subsequent pulmonary disorders that may occur as a result. Substances known to cause lung disease include coal dust, asbestos, silicon and barium. An occupational disease is any chronic ailment that occurs as a result of work or occupational activity. An occupational disease is typically identified when it is shown that it is more prevalent in a given body of workers than in the general population, or in other worker populations. Occupational hazards that are of a traumatic nature (such as falls by roofers) are not considered to be occupational diseases.

U.S. Department of Labor's Mine Safety and Health Administration (MSHA)
Mandated plans for reducing Black Lung Disease. 12-03-09.

Sarcoidosis is a disease of unknown cause that leads to inflammation. It can affect various organs in the body. Normally, your immune system defends your body against foreign or harmful substances. For example, it sends special cells to protect organs that are in danger.
These cells release chemicals that recruit other cells to isolate and destroy the harmful substance. Inflammation occurs during this process. Once the harmful substance is destroyed, the cells and the inflammation dissipate. In people who have sarcoidosis, the inflammation doesn't go away. Instead, some of the immune system cells cluster to form lumps called granulomas in various organs in your body.

Sarcoidosis can affect any organ in your body. However, it's more likely to occur in some organs than in others. The disease usually starts in the lungs, skin, and/or lymph nodes (especially the lymph nodes in your chest). The disease also often affects the eyes and the liver. Although less common, sarcoidosis can affect the heart and brain, leading to serious complications. If many granulomas form in an organ, they can affect how the organ works. This can cause signs and symptoms. Signs and symptoms vary depending on which organs are affected. Many people who have sarcoidosis have no symptoms or mild symptoms.

Lofgren's syndrome is a classic set of signs and symptoms that is typical in some people who have sarcoidosis. Lofgren's syndrome may cause fever, enlarged lymph nodes, arthritis (usually in the ankles), and/or erythema nodosum. Erythema nodosum is a rash of red or reddish-purple bumps on your ankles and shins. The rash may be warm and tender to the touch.

The outcome of sarcoidosis varies. Many people recover from the disease with few or no long-term problems. More than half of the people who have sarcoidosis have remission within 3 years of diagnosis. Two-thirds of people who have the disease have remission within 10 years of diagnosis. People who have Lofgren's syndrome usually have remission. Relapse 1 or more years after remission occurs in less than 5 percent of patients. Sarcoidosis leads to organ damage in about one-third of the people diagnosed with the disease. Damage may occur over many years and involve more than one organ. Rarely, sarcoidosis can be fatal. Death usually is the result of complications with the lungs, heart, or brain.

The chest wall is a critical component of the respiratory pump. Diseases that alter the structure of the chest wall affect the function of the pump, and may result in respiratory compromise or failure. The components of the chest wall include the bony structures (ribs, spine), respiratory muscles, and nerves connecting the central nervous system with the respiratory muscles.

Chest wall structure and physiology and diseases that affect the bony portion of the chest wall will be reviewed here. Included are discussions of the following conditions:

Ankylosing spondylitis
Congenital deformities
Flail chest
Kyphoscoliosis
Fibrothorax
Obesity and ascites
Chest wall tumors

Normal Structure and Function
The rib cage and vertebral column form the boundaries of the chest wall superiorly and laterally, while the diaphragm constitutes the inferior component. Changes in the intra-abdominal contents or compliance of the abdominal wall alter the mechanical properties of the chest wall through their effects on the motion of the diaphragm. The external boundary of the lower chest wall is the lower border of the rib cage, but internally, this boundary overlaps with the diaphragm, which covers as much as one-half of the rib cage at residual volume (RV). By virtue of this "zone of apposition" between the diaphragm and the ribs, the rib cage is exposed both to abdominal and pleural pressures, such that the normal function of the chest wall depends on the intricate interaction of the two spaces. The mechanics of the chest wall are described by a pressure-volume curve.

In the upright position, the resting volume of the chest wall is approximately 75 percent of vital capacity (VC). The rib cage recoils outward when the volume is smaller and recoils inward when the volume is larger. In contrast, the resting volume of the respiratory system as a whole — the functional residual capacity (FRC) — is approximately 35 percent of VC. This measure takes into account the mechanical properties of the chest wall plus the inward recoil of the lung.

Lung cancer is the uncontrolled growth of abnormal cells in one or both lungs. These abnormal cells do not carry out the functions of normal cells and do not develop into healthy lung tissue. As they grow, the abnormal cells can form tumors and impede the function of the lung, which is to provide oxygen to the body via the blood.

There are different types of lung cancer and each type is treated differently.
All cells in the body contain the genetic material called deoxyribonucleic acid (DNA). Every time a mature cell divides into two daughter cells, it replicates its DNA exactly. The daughter cells are clones of the original cell, identical in every way. It is in this way that our bodies continually replenish themselves. Old cells die off and the next generation takes their place.

A cancer begins with an error, or mutation, in a cell's DNA. DNA mutations can be caused by the normal aging process or through environmental factors, such as cigarette smoke or breathing in asbestos. Researchers have found that it takes a series of genetic changes to create a lung cancer cell. Before becoming fully cancerous, cells can be precancerous, which means they have some irregularities (mutations) but still function as lung cells. However, precancerous changes may signify progression toward cancer. When a cell with a genetic mutation divides, it passes along its abnormal genes to the two daughter cells, which then divide into four cells with errors in their DNA and so on. Once a cell has a genetic mutation, it may develop more. With each new mutation, the cell becomes more irregular and may not be as effective in carrying out their function in lung tissue. At a later stage of disease, some cells can migrate away from the main tumor and start growing in other parts of the body. These sites are called metastases.

For lung cancer to develop, suppressor genes (genes that normally serve as the brakes on cellular growth), and oncogenes (genes that encourage the cell to divide), have become mutated. Unlimited growth, in the face of no mechanism to stop or slow the growth, is what fuels the cancer. Other genetic changes occur in cancer as well. Researchers have pinpointed changes to specific genes that contribute to the disease and hope one day to develop specific agents to act on these changes before they can snowball into cancer. Already, some targeted therapies and chemoprevention agents have been developed that work this way.

Primary lung cancer starts in the lungs. The cancer cells are abnormal lung cells. Sometimes, people will have cancer from another part of their body travel, or metastasize, to their lungs. This is called secondary lung cancer, because the lungs are secondary compared to the original, primary, and location of the cancer. Secondary lung cancer is not lung cancer, but rather the type of cancer from its original site, such as breast cancer. Secondary lung cancer will be treated differently than primary lung cancer, because it is a different disease.

There are two main types of lung cancer, non-small cell lung cancer and small cell lung cancer. These names refer to how the cancers look under a microscope to a pathologist.
Most cancers are non-small cell. There are subtypes of non-small cell lung cancer. Because different types of lung cancer are treated differently, oncologists will determine exactly what treatment is best.

NON-SMALL CELL LUNG CANCER (NSCLC)
NSCLC accounts for about 80% of lung cancers. There are different types of NSCLC, including
 Squamous cell carcinoma (also called epidermoid carcinoma). This is the most common type of NSCLC. It forms in the lining of the bronchial tubes and is the most common type of lung cancer in men.
 Adenocarcinoma. This cancer is found in the glands of the lungs that produce mucus. This is the most common type of lung cancer in women and also among people who have not smoked.
 Bronchioalveolar carcinoma. This is a rare subset of adenocarcinoma. It forms near the lungs' air sacs. Recent clinical research has shown that this type of cancer responds more effectively to Targeted therapies -Targeted therapies: These therapies include monoclonal antibodies, anti-angiogenesis drugs, and growth factor inhibitors, are designed to treat only the cancer cells, thus sparing normal cells from damage.
 Large-cell undifferentiated carcinoma. This cancer forms near the surface, or outer edges, of the lungs. It can grow rapidly.

SMALL CELL LUNG CANCER (SCLC)
SCLC accounts for about 20% of all lung cancers. Although the cells are small, they multiply quickly and form large tumors that can spread throughout the body. Smoking is almost always the cause of SCLC.