RESTRICTIVE PULMONARY DISORDERS: AN OVERVIEW

Transcription

1 by Aimee Staggenborg RRT, MA, BA, RCP RC Educational Consulting Services, Inc Van Buren Blvd, Suite B, Riverside, CA (800) 441-LUNG / (877) 367-NURS

2 BEHAVIORAL OBJECTIVES UPON COMPLETION OF THE READING MATERIAL, THE PRACTITIONER WILL BE ABLE TO: 1. Gain new knowledge about restrictive pulmonary diseases and disorders. 2. Identify the physiologic differences of restrictive lung diseases from those with an obstruction component. 3. Learn to identify the unique signs and symptoms of restrictive diseases. 4. Assess early risk factors that could contribute to development of an occupational lung disease. 5. Make recommendations for testing for restrictive disorders with presentation of acute or chronic symptoms. 6. Learn how restrictive disorders are diagnosed. 7. Know about the course of treatment and medical care for lung diseases, and how they differ from treatment of obstructive lung diseases. 8. Know how to select which treatment and medical care is appropriate for each disease process. 9. Understand about the long term prognosis that comes with restrictive lung diseases. 10. How special considerations can affect managing care of patients that suffer from restrictive disorders. COPYRIGHT 2009 BY RC EDUCATIONAL CONSULTING SERVICES, INC. AUTHORED (2009) BY AIMEE STAGGENBORG, RRT, MA, BA, RCP ALL RIGHTS RESERVED This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 2

3 This course is for reference and education only. Every effort is made to ensure that the Clinical principles, procedures and practices are based on current knowledge and state of the art information from acknowledged authorities, text and journals. This information is not intended as a substitution for diagnosis or treatment given in consultation with a qualified health care professional. This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 3

4 TABLE OF CONTENTS INTRODUCTION... 8 FREQUENCY... 8 UNITED STATES... 8 INTERNATIONAL... 9 MORTALITY / MORBIDITY... 9 PREVALENCE BY POPULATION... 9 ALVEOLAR CAPILLARY MEMBRANE... 9 BACKGROUND CHARACTERISTICS OF RESTRICTIVE DISORDERS PATHOPHYSIOLOGY OF RESTRICTIVE DISEASES PATIENT HISTORY CLINICAL CAUSES INTRINSIC LUNG DISORDERS (LUNG PARENCHYMA DISEASE) FIBROTIC DISORDERS (INTERSTITIAL LLUNG DISEASES) PULMONARY FIBROSIS SACRCOIDOSIS ACUTE LUNG INJURY PLEURISY LUNG CANCER ASBESTOSIS PNEUMONIA RESPIRATORY DISTRESS SYNDROMES This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 4

5 ARDS SARS IRDS EXTRINSIC LUNG DISORDERS (EXTRAPARENCHYMAL DISEASES) NEUROLOGICAL DISORDERS SPINAL CORD INJURY CEREBRAL PALSY (CP) CENTRAL SLEEP APNEA (CSA) MECHANICAL DISORDERS ASCENDING LATERAL SCLEROSIS (ALS) MYASTHENIA GRAVIS (MG) GUILLIAN BARRE MUSCULAR DYSTROPHY (MD) SCOLIOSIS / KYPHOSIS / KYPHOSCOLIOSIS PHYSICAL MANIFESTATIONS INTRINSIC DISORDERS EXTRINSIC DISORDERS CLINICAL WORK UP LAB STUDIES INTRINSIC LUNG DISEASES EXTRINSIC DISORDERS IMAGING STUDIES This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 5

6 (X-RAY) CHEST RADIOGRAPHY FOR INTRINSIC LUNG DISORDERS (CT SCAN) COMPUTED TOMOGRAPHY OF THE CHEST TESTS FOR EXTRINISIC DISORDERS (PFT) PULMONARY FUNCTION TESTS PROCEDURES BRONCHOSCOPY BRONCHOALVEOLAR LAVAGE (BAL) LUNG BIOPSY SURGICAL LUNG BIOPSY THORACENTESIS PROCEDURE HISTOLOGICAL FINDINGS MEDICAL CARE AND TREATMENT INTRINSIC DISORDERS EXTRINSIC DISORDERS SURGICAL PROCEDURES GOALS OF TREATMENT CONSULTATION CONSIDERATIONS DETERRENCE / PREVENTION COMPLICATIONS PROGNOSIS MEDICAL / LEGAL PITFALLS SPECIAL CONCERNS This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 6

7 CLINICAL SCENARIO S CLINICAL SCENARIO DISCUSSIONS SUMMARY SUGGESTED READING AND REFERENCES This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 7

8 INTRODUCTION Restrictive pulmonary diseases represent only half of the spectrum in the lung disorder equation. They may range in severity and complexity and in the health care and medical treatments needed. The ultimate goals for management of the patient with any pulmonary disease are control of illness related symptoms, improved quality of life, and extension of life expectancy. All lung diseases have a common ground, the patient who lives with the disease. The only thing these patients value is the medical care that can improve his/her life. So extensive knowledge on restrictive diseases for RCP s is necessary to provide the patient with the valuable healthcare treatments they seek. This educational course will provide and overview of many common restrictive lung diseases. Provided in the following sections is information on the signs, symptoms and diagnostic testing related to treating lung disorders that impair the ability to expand the lungs. These types of disorders may be chronic conditions or may exacerbate acutely with caused by a lung infection. Ongoing medical care is great to treat the immediate causes of respiratory diseases, but the most important information RCP s should provide to their patients is education about the patient s disease, how to self treat, how to manage the illness, and how to prevent recurrent exacerbations (if preventable by disease process), and the importance of compliance with MD ordered therapy. FREQUENCY United States F or intrinsic lung diseases, studies cite an overall prevalence of 3-6 cases per 100,000 persons, with a prevalence of idiopathic pulmonary fibrosis (IPF) of cases per 100,000 persons. The prevalence for adults aged years is 2.7 cases per 100,000 persons. Prevalence exceeded 175 cases per 100,000 persons among patients older than 75 years. Exposure to dust, metals, organic solvents, and agricultural employment is associated with increased risk. In North America, the prevalence of sarcoidosis is cases per 100,000 persons. The incidence of chronic interstitial lung diseases in persons with collagen vascular diseases is variable, but it is increasing for most diseases. Kyphoscoliosis is a common extrinsic disorder. It is associated with an incidence of mild deformities amounting to 1 case per 1000 persons, with severe deformity occurring in 1 case per 10,000 persons. Other nonmuscular and neuromuscular disorders are rare, but their incidence and prevalence are not well known. (Sharma, 2006) This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 8

9 International In Sweden, the prevalence rate for sarcoidosis is 64 cases per 100,000 persons. In Japan, the prevalence rate of sarcoidosis is cases per 100,000 persons. The prevalence of sarcoidosis is difficult to determine, and tuberculosis is common. The worldwide prevalence of fibrotic lung diseases is difficult to determine because studies have not been performed. (Sharma, 2006) Mortality / Morbidity The median survival time for patients with Idiopathic Pulmonary Fibrosis (IPF) is less than 3 years. Factors that predict poor outcome include older age, male gender, severe dyspnea, history of cigarette smoking, severe loss of lung function, appearance and severity of fibrosis on radiologic studies, lack of response to therapy, and prominent fibroblastic foci on histopathology evaluation. Those patients with neuromuscular disorders often succumb to respiratory distress, then respiratory failure, coupled with lung infections from impaired cough. The rate of mortality is proportional to availability of care for treatment, family support, compliance to treatment routine and ongoing exposure to environmental irritants. Restrictive disorders are not reversible, but somewhat manageable with medical care. Prevalence by Population Age prevalence although a familial variant of IPF exists, a genetic predisposition is not documented. US prevalence of sarcoidosis is estimated to be times higher among African Americans compared to white Americans. Race Lymphangioleiomyomatosis (LAM) and lung involvement in tuberous sclerosis occur exclusively in premenopausal women. Men are more likely to have pneumoconiosis because of occupational exposure, IPF, and collagen vascular diseases (rheumatoid lung). Worldwide, sarcoidosis is slightly more common in women. Sex IPF is rare in children. Some intrinsic lung diseases present in patients aged years. These include sarcoidosis, collagen vascular-associated diseases, and histiocytosis X. Most patients with IPF are older than 50 years. ALVEOLAR CAPILLARY MEMBRANE The alveolar capillary membrane is the thin layer of tissue between the alveolar sacs and the blood in the pulmonary capillaries by which oxygen and carbon dioxide diffuses. Many restrictive disorders are affected by low levels of pulmonary surfactant in the lungs. Surfactant is a protein based substance in the lungs that coats the surface area of the lung sacs and helps to reduce surface tension. Surfactant functions to increase pulmonary lung compliance This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 9

10 and prevent lung collapse and the end of expiration. Low levels of lung surfactant decreases the diffusing ability for gas exchange across the alveolar capillary membrane. Acute Infant respiratory syndrome occurs with inadequate surfactant levels, affecting the lungs ability to expand and ventilate. Background Restrictive pulmonary disorders are classified by decreased lung capacity, either because of an alteration in lung wall lining or a disease of the internal wall of the lung, or neuromuscular mechanism. In physiology terms, restrictive lung diseases are identified by reduction in total lung capacity (TLC), vital capacity, or resting lung volume. Associated characteristics are conversed airflow and regular airway resistance, which are calculated as the functional residual capacity (FRC). If caused by parenchymal lung disease, restrictive lung disorders are accompanied by decreased gas exchange, which may be clear clinically by desaturation following exertion. The many disorders that cause reduction or restriction of lung volumes may be divided into 2 groups based on anatomical structures. The first is intrinsic lung disorders or disorders of the lung lining. The diseases cause swelling or scarring of the lung tissue (interstitial lung disease) or cause influx of the air spaces with secretions and waste. (These diseases can be characterized according to etiological factors. They include: idiopathic fibrotic diseases, connective tissue diseases, drug-induced lung disease, and primary diseases of the lungs, including sarcoidosis). (Sharma, 2006) The second is extrinsic disorders or extraparenchymal diseases. The chest wall, pleura, and respiratory muscles are the components of the respiratory pump, and they need to function normally for effective ventilation. Diseases of these structures result in lung restriction, impaired ventilatory function, and respiratory failure (eg, nonmuscular diseases of the chest wall, neuromuscular disorders). (Sharma, 2006) CHARACTERISTICS OF RESTRICTIVE DISORDERS Loss of lung tissue comes from exposure to environmental irritants, connective tissue diseases; interstitial lung diseases that destroy fragile pulmonary tissue. Fibrotic scar tissues form in the space. Fibrotic tissue does not have the ability to exchange gas. This type of damage is not reversible. Decrease of lungs ability to expand is affected by the actual available space in the lungs to expand. In the case of extrinsic disorders the area for expansion is restricted by physical limitations in the lung. If the expansion mechanism in the lung is impaired the capacity of the lungs is reduced. This leads in hypoventilation for inadequate ventilation This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 10

11 with V/Q mismatch and hypoxia. It becomes difficult to properly oxygenate the tissues of the body if the ability to ventilate the lungs is adversely affected. Decrease of lungs ability to transfer oxygen or carbon dioxide in and out of the blood when lung damage exists. Reduction of the available surface area for gas diffusion is uneven when fibrotic scar tissue present. Dyspnea is indicative of restrictive disorders as the body tries to compensate for shallow breaths by increasing the respiratory rate causing tachypnea or hyperventilation. Pathophysiology of Restrictive Diseases Restrictive Lung Disorders (RDL) are diseases and disorders that impair both lung expansion and lung compliance (RLD) diseases and disorders cause the lungs to become stiff and increase inability to ventilate the lungs. Without proper ventilation of the lungs become less able to get rid carbon dioxide. The by products of metabolism are retained in the lung s tiny air sacs. This change manifests itself in a reduced Total Lung Capacity, Inspiratory Capacity and Vital Capacity. As the elasticity of the lungs become weaker the patient may begin to suffer from hypoventilation, hypoxia, and inability to clear lung secretions with ineffective coughing. Restrictive lung disease is a chronic disorder that causes a decrease in the ability to expand the lung (breathe in) and sometimes makes it harder to get enough oxygen to meet the body's needs. The most common restrictive pulmonary diseases are: Interstitial pulmonary fibrosis/interstitial lung disease (including Sarcoidosis-granulomatous disorder) extra pulmonary restrictive lung disease (including scoliosis). Interstitial lung disease is a disease of the lung parenchyma and connective tissue. The lung parenchyma is the covering of the lungs. The connective tissue is the tissue that holds the air sacs together. Interstitial lung disease happens when the lung parenchyma is damaged in some way and inflammation occurs. Changes in the parenchyma and connective tissue happen when the inflammation is chronic. Once significant amount of damage to the air sacs develop, the disease is not reversible. In advanced disease, air sac dilation occurs, resulting in impaired blood flow in the lungs. This reduced blood flow can reduce the amount of oxygen available for your body to use and increase shortness of breath. There are more than 100 different interstitial lung diseases. In about 70 percent of patients the cause of the disease remains unknown. Known causes are occupational and environmental inhaled irritants (dust, gases, fumes, fiber glass, asbestosis, or aerosols), radiation, poisons and drugs. Sarcoidosis is one type of interstitial lung disease. Sarcoidosis is a multi-system disorder which commonly affects the lungs, skin and eyes. During chronic inflammation, normally elastic tissue stiffens, decreasing the flexibility of the lung tissue. Affected tissue in the lung looks like a honeycomb. The cause of sarcoidosis is unknown. In contrast to OPD, RLD values for Tidal Volume, Expiratory Reserve Volume, Functional Residual Capacity and Respiratory Volume are unchanged. The FEV1 and FCV for a patient with RLD will be decreased however FEV1/FVC ratio will be normal or increased for a RLD patient. This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 11

12 Patient History Initial or ongoing diagnosis is affected by information revealed in the patient past medical history. Each patient should receive an initial assessment consisting of a complete history, including onset of symptoms, past environmental exposure, habits, recent travels, occupation history, hobbies, and HIV risk factors is key for determination of etiologic cause. Duration of symptoms for diagnosis of restrictive disorders factor in the treatment and plan of care. Acute disorders with symptoms that may last from a few days to a few weeks, pneumonia, pneumonitis or alveolar hemorrhage have a sudden onset and require immediate medical intervention. Chronic disorders occur frequently with numerous exacerbations over several months to several years. Treatment of chronic disorders focus on reduction of symptoms, hospitalizations, improvements in ADL s (activities of daily living), and improved overall quality of life. Smoking history is important when assessing signs and symptoms of pulmonary disease. History of smoking has been shown it contribute to restrictive pulmonary diseases. Impairment of the respiratory cilliary function affects the lungs ability to deep cough and expels irritants and foreign particulate for the lungs, resulting in concurrent lung infections. Histiocytosis X, desquamative interstitial pneumonitis, IPF, and respiratory bronchiolitis occur with increased frequency among persons who smoke or those who previously smoked. Assessment of past exposure to smoking is necessary to determine contributing factors for current clinical presentation. Prior medication use. An increasing number of drugs are recognized to induce distinctive patterns of infiltrative lung disease (ILD), ranging from benign infiltrates to life-threatening adult respiratory distress syndromes. Drug-induced respiratory disease from prior drug use warrants a detailed past history of medication use as a tool to rule out or confirm origination of a pulmonary disease. Syndromes of drug induced may cause mild effects or major life threatening respiratory distress and impending respiratory failure, pulmonary diseases may include: asthma, bronchiolitis obliterans organizing pneumonia (BOOP) hypersensitivity infiltrate, interstitial pneumonitis or fibrosis, noncardiogenic pulmonary edema, pleural effusions, pulmonary infiltrates with eosinophilia or pulmonary vascular disease. Considerations to explore when assessing past usages of drugs and medications can give a blue print for where to start questions for the patient to help arrive at preliminary conclusions. Some common drugs that can cause pulmonary disease may include: anticonvulsant, antipsychotic, antidepressants (i.e.: fluoxetine, phenothiazines phenytoin), anti-inflammatory, (i.e.: aspirin, gold, methotrexate), antimetabolic, (i.e.: cytarabin fludarabine), biologic response modifiers, (i.e.: Interferon, Interleukin-2), Cardiovascular, (i.e.: beta-blockers, anticoagulants), chemotherapeutic and immunosuppressive agents, intravenous substances, (i.e.: blood products), illicit drugs, (i.e.: cocaine, heroin, methadone, methylphenidate narcotic and sedative drugs). Misc. agents, (i.e.: appetite suppressants, mineral oil, silicone, and radiation therapy). This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 12

13 Family history has not been shown to hide restrictive pulmonary diseases in primary or recessive DNA genes, however a familiar link has been found to exist in (IPF) interstitial pulmonary fibrosis, sarcoidosis, and LAM. Occupational exposure. A detail patient history of employment and know exposures to toxins, is necessary for assessment of pulmonary diseases. Discussion of exposure to inhaled irritants could involve education about risk factors and history. Occupational lung disease is the number one work-related illness in the United States based on the frequency, severity, and preventability of diseases. These illnesses are usually caused by extended exposure to irritating or toxic substances that may cause acute or chronic respiratory ailments, although severe single exposures can cause chronic lung disease as well. Known occupational related disorders: Occupational lung cancer, asbestosis, mesothelioma, byssinosis, black lung disease, Silicosis, Hypersensitivity pneumonitis, WTC lung disease. (Further examination of diseases is discussed later in this course). Environmental exposure is similar to those occurring during occupational exposure however, environmental exposure may occur in a less controlled situation, such as in the home or in the outdoors. The patient assessment for this type of exposure is the same as occupational. Some environmental considerations: Stachybotrys chartarum, and Aspergillus. Symptoms of intrinsic diseases. Progressive proportional dyspnea is the primary symptom. Assessing the severity of dyspnea is useful as a method to determine the advancement of the disease and to follow its course. A dry cough is common and may be a tell-tell sign. A productive cough is a common sign in most patients with diffuse parenchyma lung disorders. Hemoptysis or grossly bloody sputum occurs in patients with diffuse alveolar hemorrhage syndromes and vasculitis. Wheezing is an uncommon manifestation but can occur in patients with lymphangitic carcinomatosis, chronic eosinophilic pneumonia, and respiratory bronchiolitis. Chest pain is uncommon in most instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid arthritis, systemic lupus erythematosus, and some drug-induced disorders. Symptoms of extrinsic diseases. Nonmuscular diseases of the chest wall affect patients with scoliosis or kyphosis or combination of both. Patients under 35 years of age may be asymptomatic, with higher risk middle-aged patients developing shortness of breath, decreased activity tolerance, and recurrent respiratory infections. The cause of respiratory failure is often variable between several function and is secondary to spinal deformity, muscle weakness, disordered ventilatory control, sleep disordered breathing, and airway disease. Neuromuscular disorders occur as the respiratory muscle weakness progresses. Patients develop dyspnea upon exertion, followed by dyspnea at rest, and their condition ultimately advances to respiratory failure. Patients with neuromuscular diseases develop significant respiratory muscle weakness and may demonstrate fatigue, dyspnea, impaired control of secretions, and recurrent lower respiratory tract infections. This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 13

14 Acute and chronic respiratory failure, pulmonary hypertension, and cor pulmonale eventually ensue. Disorders include: ALS, MD, and MS. CLINICAL CAUSES Intrinsic Lung Disorders (Lung Parenchyma Disease) Fibrotic Disorders (Interstitial Lung Diseases) I nterstitial Lung Disease (ILD) is a general term that includes a variety of chronic lung disorders. When a person has ILD, the lung is affected in three ways. First, the lung tissue is damaged in some known or unknown way. Second, the walls of the air sacs in the lung become inflamed. Finally, scarring (or fibrosis) begins in the interstitium (or tissue between the air sacs), and the lung becomes stiff. The tissue between the air sacs of the lungs is called the interstitium. Interstitial lung disease is named after this tissue because this is the tissue affected by fibrosis (scarring). Interstitial lung disease is sometimes also known as interstitial pulmonary fibrosis. The terms interstitial lung disease, pulmonary fibrosis and interstitial pulmonary fibrosis are often used to describe the same condition. The course of these diseases is unpredictable. If they progress, the lung tissue thickens and becomes stiff. The work of breathing then becomes more difficult and demanding. Some of the diseases improve with medication if treated when inflammation occurs. Some people may need oxygen therapy as part of their treatment. Pulmonary Fibrosis Pulmonary fibrosis is generally idiopathic when diagnosed. Pulmonary Fibrosis involves scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue. When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue s ability to transfer oxygen into the bloodstream. Traditional theories have postulated that it might be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a growing body of evidence which points to a genetic predisposition. A mutation in the SP-C protein has been found to exist in families with a history of Pulmonary Fibrosis. The most current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While the exact cause remains unknown, associations have been made with the following: Inhaled environmental and occupational pollutants; cigarette smoking; diseases such as Scleroderma; Rheumatoid Arthritis; Lupus and Sarcoidosis; Certain medications; therapeutic radiation. Clinical presentation: Shortness of breath, particularly with exertion; chronic dry, hacking cough; fatigue and weakness; discomfort in the chest; loss of appetite; rapid weight loss. Sacrcoidosis An immune system disorder characterized by non-caseating granuloma (small inflammatory nodules) that most commonly arises in young adults. The cause of the disease is still unknown. Virtually any organ can be affected, however granulomas most often appear in the lungs or the This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 14

15 lymph nodes. Symptoms can occasionally appear suddenly but usually appear gradually. The clinical course varies and ranges from asymptomatic disease to a debilitating chronic condition that may lead to death. Sarcoidosis most often manifests as a restrictive disease of the lungs, causing a decrease in lung volume and decreased compliance (the ability to stretch). The disease typically limits the amount of air drawn into the lungs, but produces higher than normal expiratory flow ratios. The vital capacity (full breath in, to full breath out) is decreased, and most of this air can be blown out in the first second. Sarcoidosis is a systemic disease that can affect any organ. Common symptoms are vague, such as fatigue unchanged by sleep, lack of energy, weight loss, aches and pains, arthralgia, dry eyes, blurry vision, shortness of breath, a dry hacking cough or skin lesions. The cutaneous symptoms vary, and range from rashes and noduli (small bumps) to erythema nodosum or lupus pernio. It is often asymptomatic. Acute Lung Injury Acute lung injury is generally caused by a direct injury to the lungs. These lung injuries cause inflammation to the fragile lung tissue. Inflammatory disorders of the lung are most commonly caused by sepsis, pneumonia, trauma, and/or aspiration following an acute lung injury. Damage to the endothelium and the alveolar epithelium results in the creation of an open interface between the lung and the blood, facilitating the spread of micro-organisms from the lung systemically, stoking up a systemic inflammatory response. Moreover, the injury to epithelial cells handicaps the lung s ability to pump fluid out of airspaces. Fluid filled airspaces, loss of surfactant, microvascular thrombosis and disorganized repair (which leads to fibrosis) reduces resting lung volumes (decreased compliance), increasing ventilation-perfusion mismatch, right to left shunt and the work of breathing. Pleurisy Pleurisy also known as plueritis, inflammation, swelling and irritation of the pleural chest wall lining surrounding the lungs, which can cause painful respiration (also called pleuritic chest pain) and other symptoms. Pleurisy can be generated by a variety of infectious and non-infectious causes. The effects of pleurisy can often be felt long after the condition has gone away. Pleurisy results following other illnesses such as viral or bacterial infections of the lungs, injury or trauma to the lungs may also cause pleurisy. Often deemed idiopathic by doctors, pleurisy may be acute in nature with a sudden onset. Sharp stabbing is felt with inspiration, and may subside between breaths. Deep breathing, coughing, or movement may make the pain feel worse and more intense. Often the pain is localized in a specific location which may be pin-pointed, quick shallow breathing may result from the deep pain. Lung Cancer Lung cancer is a disease of uncontrolled cell growth in tissues of the lung. This growth may lead to metastasis, invasion of adjacent tissue and infiltration beyond the lungs. The vast majority of primary lung cancers are carcinomas of the lung, derived from epithelial cells. Lung cancer is the most common cause of cancer-related death in men and the second most common in woman. This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 15

16 The main causes of lung cancer (and cancer in general) include carcinogens (such as those in tobacco smoke), ionizing radiation, and viral infection. This exposure causes cumulative changes to the DNA in the tissue lining the bronchi of the lungs (the bronchial epithelium). As more tissue becomes damaged, eventually a cancer develops. Asbestosis Asbestosis is a restrictive lung disorder caused by inhaling asbestos fibers. Prolonged exposure over time can cause accumulation of particulate in the lung tissue. Extensive scaring to the lung tissue can diminish the lungs capacity to diffuse gas across the alveolar capillary membrane into the blood stream. Asbestosis is considered an occupational hazard that leads to this disorder. Asbestosis is a chronic inflammatory medical condition affecting the parenchymal tissue of the lungs. It occurs after long-term, heavy exposure to asbestos, e.g. in mining, and is therefore regarded as an occupational lung disease. Sufferers have severe dyspnea (shortness of breath) and are at an increased risk regarding several different types of lung cancer. Asbestosis is a natural mineral product that is resistant to heat and corrosion. It has been used in the past in the building and manufacturing industry. Some of its more common uses were in pipe and duct insulation, fire-retardant materials, brake and clutch linings, cement, and some vinyl floor tiles. The primary symptom of asbestosis is generally the slow onset of shortness of breath on exertion. In severe, advanced cases, this may lead to respiratory failure. Coughing is not usually a typical symptom, unless the patient has other, concomitant respiratory tract diseases. People with extensive occupational exposure to the mining, manufacturing, handling or removal of asbestos are at risk for developing asbestosis sometime in the future. Long tern use of tobacco has shown to increase the risk factors of developing asbestosis symptoms. Pneumonia Pneumonia is an infection of the lung wall lining. There are several types of pneumonia including viral, bacterial, fungi. Bacterial pneumonias tend to be the most serious and, in adults, the most common cause of pneumonia. The most common pneumonia-causing bacterium in adults is Streptococcus pneumoniae (pneumococcus). The route for transmission is inhalation of small droplets containing pneumonia causing organisms. Pneumonia is often a complication of a pre-existing condition/infection and triggered when a patient's defense system is weakened, most often by a simple viral upper respiratory tract infection or a case of influenza, especially in the elderly. Pneumonia affects the lungs in different ways. Lobar pneumonia affects a lobe of the lungs, and bronchial pneumonia can affect patches throughout both lungs. Pneumonia can also be caused by the inhalation of food, liquid, gases or dust. One type of pneumonia caused by fungi is pneumocystis carinii pneumonia (PCP) which primarily affects AIDS patients. Certain diseases, such as tuberculosis, can also predispose someone to pneumonia. People considered at high risk for pneumonia include the elderly, the very young, and those with underlying health problems, such as chronic obstructive pulmonary disease (COPD), diabetes mellitus, congestive heart failure and sickle cell anemia. Patients with diseases that impair the immune system, such as AIDS, or those undergoing cancer therapy or organ transplantation, or patients with other chronic illnesses are particularly vulnerable. Initially symptoms mimic a cold which are then This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 16

17 followed by a high fever (sometimes as high as 104 degrees Fahrenheit), shaking chills, and a cough with sputum production. The sputum is usually discolored and sometimes bloody. People with pneumonia may become short of breath. The only pain fibers in the lung are on the surface of the lung, in the area known as the pleura. Chest pain may develop if the outer pleural aspects of the lung are involved. This pain is usually sharp and worsens when taking a deep breath, known as pleuritic pain. RESPIRATORY DISTRESS SYNDROMES (ARDS) - Adult respiratory distress syndrome is an acute, quick onset lung injury effecting one or both lungs. Generally the injury covering most of the surface area of the lungs. Patient s who suffer from ARDS suffer from severe dyspena, which may require mechanical ventilation to counter respiratory failure. ARDS is not a specific disease; instead it is a type of severe, acute lung dysfunction that is associated with a variety of diseases, such as pneumonia, shock, sepsis (a severe infection in the body) and trauma. ARDS us characterized by inflammation of the lung parenchyma leading to impaired gas exchange with concomitant systemic release of inflammatory mediators causing inflammation, hypoxemia and frequently resulting in multiple organ failure. This condition is life threatening and often lethal. (SARS) - Sudden acute respiratory syndrome is an infectious respiratory disease in humans which is caused by the SARS coronavirus (SARS-CoV). Initial symptoms are flu like and may include: ever, myalgia, lethargy, gastrointestinal symptoms, cough, sore throat and other non-specific symptoms. The only symptom that is common to all patients appears to be a fever above 38 C (100.4 F). Shortness of breath may occur later. Symptoms usually appear 2-10 days following exposure, but up to 13 days have been reported. In most cases symptoms appear within 2-3 days. About 10-20% of cases require mechanical ventilation. (IRDS) - Infant respiratory distress syndrome is a syndrome caused in premature infants by developmental insufficiency of surfactant production and structural immaturity in the lungs. It can also result from a genetic problem with the production of surfactant associated proteins. RDS affects about 1% of newborn infants and is the leading cause of death in preterm infants. The incidence decreases with advancing gestational age, from about 50% in babies born at weeks, to about 25% at weeks. Surfactant deficient preemie lungs have higher surface area tension causing decreased ability to ventilate and oxygenate the lungs. Respiratory distress syndrome begins shortly after birth and is characterized by tachypnea, tachycardia, chest wall retractions, expiratory grunting, nasal flaring and cyanosis during breathing efforts. As the disease progresses, the baby may develop ventilatory failure (rising carbon dioxide concentrations in the blood), and prolonged apneic episodes during the breathing cycle. Early interventions are essential for better recovery outcomes. Initiation of surfactant replacement therapy early on can help minimized potential lung damage. Nasal CPAP has also been shown to deliver a continuous positive airway pressure, which helps to hyper-inflate the lungs with This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 17

18 inspiration, and prevent alveolar collapse upon expiration. Untreated IRDS can be fatal, so early intervention is needed for the best possibility for treatment response and recovery. (Further treatment information is covered later in the course). EXTRINISIC LUNG DISORDERS (EXTRAPARENCHYMAL DISEASES) Neurological Disorders Spinal cord injury presents a unique condition where respiratory complications are common, depending on location of the traumatic injury to spinal column. Injuries to cervical vertebrae affect the nerves impulses that control brain signals to the respiratory muscles, the higher the level of injury, the greater the loss to the respiratory muscle control. The lungs and trachea are no usually affected with an injury, but complications may occur post injury. Respiratory problems may occur when the signals sent from the brain can no longer flow through the spinal cord to control the respiratory muscles. Loss of control of the respiratory muscles can lead to a restrictive component affecting lung expansion. Complete injuries in the thoracic or cervical regions usually result in the permanent loss of respiratory muscle function below the level of injury. However, Injuries in the thoracic area (T1 - T12) of the spinal cord affect the control of the intercostal and abdominal muscles. A lower level of injury, such as a T10, results in the individual losing a small amount of muscle control. With a higher level of injury, such as a T2, individuals will lose most of their inter-costal and abdominal muscle control. Loss of respiratory muscle control often will involve respiratory failure and the inability for the patient to breathe on his/her own. Mechanical ventilation is common for treating muscle failure. This poor ability for lung expansion may result in pneumonia, atelectasis and pulmonary embolism. Complications require adequate health maintenance and protection from these complications are appropriate and necessary as part of the longterm care of he spinal cord-injured individual. Cerebral Palsy (CP) characterized by faulty development or damage to the motor part of the brain that controls body movement and posture. The disorder normally occurs as result before or during birth (anoxic brain injury from asphyxia) or from head trauma or infection in brain within the first months after birth. Spastic CP is the most common type of cerebral palsy. It causes the muscles to be stiff and permanently contracted. Respiratory complications may occur. Children with cerebral palsy do not frequently have problems with their lungs as a direct result of their impairment. However, depending upon the type of cerebral palsy they have, they may have physical (muscle constriction) limitations that significantly add to respiratory problems. Difficulties in swallowing, a weak cough, reflux, and seizure can all cause a child to accidentally breathe in saliva, food, liquid, or stomach contents, these conditions are risks factors for development of lung infections such as pneumonia from impaired ability to clear the upper airway. Cerebral palsy related to head trauma (head injury, shaken baby syndrome) or brain infection (bacterial meningitis, encephalitis) effect the ability to regulate respiration results from damage to the brain stem (respiratory center). This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 18

19 Central Sleep Apnea (CSA) results in a lack of effort to breath. Characterized by cheyne-stokes breathing, central apnea can cause a restrictive disorder in the lungs. The cause of central apnea occurs in the respiratory center of the brain involving problems in how the brain controls respiration. CSA may be caused by cervical spine injury. CSA may cause difficulty swallowing, numbness and weakness throughout the body. Poor control of respiratory muscles may translate to a restrictive lung component. Muscle weakness affects the ability to expand the lungs. Consideration for central sleep apnea should be examined when doing a patient history and assessment. Mechanical Disorders Ascending Lateral Sclerosis (ALS) also known as Lou Gehrig's disease is a progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor neurons, the nerve cells in the central nervous system that control voluntary muscle movement. The disorder causes muscle weakness and atrophy throughout the body as both the upper and lower motor neurons degenerate, ceasing to send messages to muscles. Unable to function, the muscles gradually weaken. Progression of atrophy and weakness reaches the diaphragm, which will impair the diaphragm and the ability to expand the lungs. The onset of symptoms usually begins with weakness in the limbs, (arms, legs, hands, and feet) with tingling, twitching, stiffness, and cramping soon following. The progression of the muscle atrophy varies depending on which, and location of damaged motor neurons. The restrictive respiratory component becomes more apparent as degeneration occurs. As the diaphragm and intercostal muscles (rib cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset ALS, this may occur before significant limb weakness is apparent. Non-invasive ventilation like Bi-level positive pressure ventilation is frequently used to support breathing, first at night, and later during the daytime as well. It is recommended that long before BiPAP becomes insufficient, patients must decide whether to have tracheotomy and long term mechanical ventilation. Most patients do not elect this route, and instead choose palliative hospice care at this point. Most people with ALS die of respiratory failure or pneumonia, not the disease itself. Myasthenia Gravis (MG) is a muscular disorder that causes serious muscle weakness is a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. It is an autoimmune disorder, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the post-synaptic neuromuscular junction, inhibiting the stimulative effect of the neurotransmitter acetylcholine. Myasthenia is treated medically with cholinesterase inhibitors or immunosuppressant s, and in selected cases, thymectomy. At cases per million it is one of the less common autoimmune disorders. The disease begins at the top of the body around the head area and progresses downward to the feet. Medical considerations must be addressed with pending muscle weakness to the diaphragm. Impairment of the major muscles of ventilation can lead aspiration, dysphasia, higher risk for pneumonia and respiratory failure. MG is a rare autoimmune disease, in the past, untreated MG carried a mortality rate of 30-70%. In This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 19

20 the modern era, patients with MG have a near-normal life expectancy. Prognosis is good with early diagnosis, intervention, and supportive treatment. Guillian Barre is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral nervous system, usually triggered by an acute infectious process. Acute inflammatory demyelinating polyneuropathy (AIDP), which is frequently severe and usually exhibits as an ascending paralysis (ascends from feet to head) noted by weakness in the feet that spreads to the upper body and arms and the face along with complete loss of deep tendon reflexes. Many patients may require hospitalization for medical care, and management of ventilatory complications that accompany generalized diaphragmatic weakness. With prompt treatment of plasmapheresis followed by immunoglobulin and supportive care, the majority of patients will regain full functional capacity. However, death may occur if severe pulmonary complications and dysautonomia are present. Muscular Dystrophy (MD) is progressive muscular degenerative disease that causes may symptoms from muscle weakness and wasting. The most common is Duchene s (DMD). The onset is generally in child hood. Other dystrophies can be diagnosed anytime with symptom onset. These conditions are inherited, and the different muscular dystrophies follow various inheritance patterns. Onset of symptoms can affect the patient s ability to: walk; balance; limited ranges of motion; notably respiratory difficulties and in some dystrophies affects on the heart from cardiomyopathy or arrhythmias. The diagnosis of muscular dystrophy is based on the results of a muscle biopsy. In some cases, a DNA blood test may be all that is needed. A physical examination and the patient's medical history will help the doctor determine the type of muscular dystrophy. Specific muscle groups are affected by different types of muscular dystrophy. The MD s that effect the main muscles of respiration are of special concern. The diaphragm, inter-costal, and other muscles of ventilation with atrophy may cause inability for the patient to breathe adequately and ventilate the lungs. The severity of the atrophy will warrant treatment s ranging from bi-level non-invasive ventilation, noninvasive mask ventilation via ventilator or invasive ventilation via tracheotomy. Scoliosis / Kyphosis / Kyphoscoliosis. Scoliosis is a medical condition in which a person's spine is curved from side to side, and may also be rotated. It is an abnormal lateral curvature of the spine. On an x-ray, the spine of an individual with a typical scoliosis may look more like an "S" or a "C" than a straight line. Kyphosis is a hump of the upper spine. In general terms, it is a curvature of the upper spine. It can be either the result of bad posture or a structural anomaly in the spine. In the sense of a deformity, it is the pathological curving of the spine, where parts of the spinal column lose some or all of their lordotic profile. This causes a bowing of the back, seen as a slouching posture. Symptoms of Kyphosis, that may be present or not, depending on the type and extent of the deformity, include mild back pain, fatigue, appearance of round back and breathing difficulties Severe cases can cause great discomfort and even lead to death. Kyphoscoilosis is term used to describe a condition that combines both types of spine This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 20

21 curvature; spine curvatures present distortion to the thoracic and constriction of surface area for lung expansion. PHYSICAL MANIFESTATIONS Intrinsic Disorders The physical examination in patients with intrinsic lung disorders may yield distinguishing physical findings. Those with chest wall disorders show obvious massive obesity and an abnormal configuration of the thoracic cage (kyphoscoliosis, ankylosing spondylitis). Velcro crackles are common in most patients with interstitial lung disorders. Inspiratory squeaks or scattered, late, inspiratory high-pitched rhonchi are frequently heard in patients with bronchiolitis. Cyanosis at rest is uncommon in persons with interstitial lung diseases, and this is usually a late manifestation of advanced disease. Digital clubbing is common in those with IPF and is rare in others (eg, those with sarcoidosis or hypersensitivity pneumonitis). Extra pulmonary findings, including erythema nodosum, suggest sarcoidosis. A maculopapular rash can occur in those with connective tissue diseases, or it may be drug-induced. Cor pulmonale occurs in the late stages of pulmonary fibrosis or advanced kyphoscoliosis. Pulmonary hypertension and cor pulmonale become evident when signs include a loud P2, right-sided precordial lift, and right-sided gallop. Extrinsic Disorders Nonmuscular diseases of the chest wall, in which kyphosis/scoliosis can be idiopathic or secondary, may cause restrictive lung disease. The most common cause of secondary kyphoscoliosis is neuromuscular disease (eg, polio, muscular dystrophy). Fibrothorax, massive pleural effusion, morbid obesity, ankylosing spondylitis, and thoracoplasty are other causes. Neuromuscular diseases manifest as respiratory muscle weakness and are due to myopathy or myositis, quadriplegia, or phrenic neuropathy from infectious or metabolic causes. CLINICAL WORK UP Lab Studies Intrinsic Lung Diseases Routine laboratory evaluations often fail to reveal positive findings. However, anemia can indicate vasculitis, polycythemia can indicate hypoxemia in advanced disease, and leukocytosis can suggest acute hypersensitivity pneumonitis. The decision to perform additional tests should be directed by the findings of the clinical assessment. Antinuclear antibodies and rheumatoid factor should be measured to screen for collagen vascular disorders, creatine kinase for polymyositis, antineutrophilic cytoplasmic This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 21

22 antibodies for vasculitis, and antiglomerular basement membrane antibody for Good Pasture Syndrome. The presence of precipitating antibodies to an antigen may help in diagnosing hypersensitivity pneumonitis. Serum angiotensin-converting enzyme levels are often elevated in patients with sarcoidosis, but this finding has poor specificity. Extrinsic Disorders An elevated creatine kinase level may indicate myositis, which may cause muscle weakness and restrictive lung disease. Imaging Studies (X-ray) Chest Radiography For Intrinsic Lung Disorders The diagnosis of an interstitial lung disorder is often initially based on abnormal chest radiograph findings, which can be normal in as many as 10% of patients. All previous chest films should be reviewed. The most common radiographic abnormality is a reticular pattern. Nodular, reticulonodular, or mixed patterns, such as alveolar filling (i.e., ground-glass appearance), and increased interstitial markings are not unusual, however these are not predictive of a specific pathological picture. Air-space opacities suggest pulmonary hemorrhage, eosinophilic pneumonia, and BOOP. Upper-zone predominance on chest radiographs is observed in patients with sarcoidosis, histiocytosis X, chronic hypersensitivity pneumonitis, pneumoconiosis, or ankylosing spondylitis. Lower-zone predominance is seen in patients with IPF, asbestosis, or collagen vascular diseases. The finding of honeycombing correlates with advanced fibrosis and indicates a poor prognosis. Bilateral hilar lymphadenopathy, with or without mediastinal adenopathy, suggests sarcoidosis. (CT Scan) Computed Tomography of the Chest High-resolution computed tomography of the chest can be helpful, but the accuracy of the findings for helping determine a specific etiology is inconsistent. Bibasilar peripheral lung zone involvement is seen in patients with IPF, asbestosis, connective tissue disease, or eosinophilic pneumonia. This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law. 22