Pulmonary Conditions Medical and Psychosocial Aspects of Disability RCS 6080 Description & Definitions Chronic Obstructive Pulmonary Disease (COPD) is characterized by decreased expiratory airflow Reduction in expiratory airflow has 2 causes: • Decreased expiratory air flow pressure (decrease in driving pressure) • Increased resistance to expiratory air flow (resulting from narrowing of airways) Descriptions/Definitions Emphysema & chronic bronchitis are often considered together under the term COPD because most people with one of these conditions has the other. Thus most people with COPD with have both airway & alveolar disease. COPD affects as many as 30,000,000 Americans COPD is the 5th leading cause of death Etiology, Pathophysiology & Clinical Features Several factors are involved in the pathogenesis of COPD, but smoking is the most important • Other factors include occupational exposure to dust, fumes & air pollution. Aside from these factors, the development and progression of COPD is largely related to genetic disposition. Emphysema Emphysema is an enlargement of air spaces caused by destruction of alveolar walls. Air spaces greater than one cm are bullae. This photo shows apical bullous disease with relatively little involvement of the rest of the lung. Emphysema Emphysema can be a result of obstruction caused by chronic bronchitis. It occurs when there is back pressure on the alveoli. This increased pressure tends over time to make their walls break down, and instead of having lots of tiny air sacs functioning well, you end up with large cavities consisting of alveoli that have coalesced, and which do not adequately perform the task of gas transfer. Chronic Bronchitis The lungs essentially comprise lots of tubes and tubules (called bronchi and bronchioles) of gradually diminishing size, which end in little collections of air sacs called alveoli. It is across the walls of the alveoli that the gases are exchanged, oxygen being taken into the bloodstream and carbon dioxide passing into the alveoli to be exhaled. Chronic bronchitis is an inflammation or irritation of the airways in the lungs which is associated with: Scarring or fibrosis of the walls of the bronchioles making them less pliable Thickening of their lining causing narrowing of the airway Production of excessive quantities of thick mucus which further plugs the tubules and compromises breathing Categories of COPD Type A - “Pink Puffer” • Considered to have predominantly emphysema Type B - “Blue Bloater” • Considered to have predominantly chronic bronchitis. Functional Disabilities Earliest manifestations of COPD may be relatively mild, but as time goes on, dyspnea becomes the most limiting factor • Years may pass before the degree of dyspnea is severe enough to limit routine ADLs such as walking. • As time progresses, activities such as dressing, bathing, speech and even eating cannot be accomplished without severe shortness of breath Functional Disabilities Until the disease is extremely advanced, sedentary activities may be accomplished without much difficulty. Driving may be possible, but walking - even limited distances - may not be feasible, particularly is there is an incline or stairs. Functional Disabilities Assessment of a given person’s functional capabilities may be difficult to determine based solely on pulmonary function studies and blood gases. Depression, fear, & anxiety are potent factors that may further exacerbate the person’s physical limitations. Preparation of sedentary occupation is useful even when COPD is mild because the rate of progression is variable. Treatment of COPD Many people with COPD might be able to have some reversibility – through proper medical management. Adequate fluid intake and use of expectorants are needed to clear the respiratory tract of secretions. Oxygen therapy Treatment of COPD Chest physical therapy and pulmonary rehabilitation programs are useful to: • Learn how to expel mucus from respiratory tract • Learn breathing exercises and relaxation techniques (useful in ADLs) • Do exercise reconditioning that can help increase endurance & improve work capacity Psychological Implications Counseling often helps the person deal with the anxiety/stress associated with diseases that can cause shortness of breath & limitations of activity. • Learning to deal effectively with problems & make satisfactory lifestyle can reduce feelings of desperation. Vocational implications People may have to change employment goals The American Thoracic Society: • Mild impairment – usually not correlated with reduced ability to perform most jobs. • Moderate impairment – correlates with a decreased ability to meet the demands of many jobs • Severe impairment – pulmonary function is so impaired that a person cannot meet the demands of most occupations. Asthma Asthma is considered an inflammatory disease of the airways • Reversible airway obstruction • Bronchial hyper-reactivity Frequency, duration, & severity of asthma attacks varies from person to person. Asthma attacks are characterized by shortness of breath & wheezing. Asthma Mainly a bronchial disease, asthma is characterized by features in the following 3 images: Mural inflammation (eosinophils, mast cells, lymphocytes) Wall thickening by edema, hyperemia, fibrosis Smooth muscle thickening (arrow) Mucous plugs Epithelial slough Pathology of Asthma Normal Lungs Asthma Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Created and funded by NIH/NHLBI Asthma Prevalence* by Age United States: 1980–1996 8 Under 18 Total 6 5 4 18+ 3 2 1 19 96 19 94 19 92 19 90 19 88 19 86 19 84 19 82 0 19 80 Prevalence (%) 7 Source: National Health Interview Survey * 12-month prevalence Year Asthma Prevalence* by Sex United States: 1982–1996 Female 8 Total 6 5 Male 4 3 2 1 19 96 19 94 19 92 19 90 19 88 19 86 19 84 19 82 0 19 80 Prevalence (%) 7 Source: National Health Interview Survey * 12-month prevalence Year Asthma Prevalence* by Race United States: 1982–1996 8 Black 6 5 White 4 3 2 1 Source: National Health Interview Survey * 12-month prevalence 19 96 19 94 19 92 19 90 19 88 19 86 19 84 19 82 0 19 80 Prevalence (%) 7 Year Asthma Prevalence* by Race 9 8 7 6 5 4 3 2 1 0 Black, 5-34 Source: National Health Interview Survey * 12-month prevalence 19 96 19 94 19 92 19 90 19 88 19 86 19 84 19 82 White, 5-34 19 80 Prevalence (%) Ages 5-34, United States: 1980–1996 Year Age-Adjusted* Asthma Mortality Rates by Sex, United States: 1979–1998 Female Total 25 Male 20 15 10 5 19 97 19 95 19 93 19 91 19 89 19 87 19 85 19 83 19 81 0 19 79 Rate per million 30 Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Age-adjusted to 2000 U.S. population Year Age-Adjusted* Asthma Mortality Rates by Race, United States: 1979–1998 60 50 Rate per million 40 30 Black Other 20 10 White 0 Year Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Age-adjusted to 2000 U.S. population Asthma Mortality Rates by Race Ages 5-34, United States: 1979–1998 20 Rate per million Black 15 10 White Other* 5 Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Unreliable (< 20 deaths) 1979–1995 97 19 95 19 93 19 91 19 89 19 87 19 85 19 83 19 81 19 19 79 0 Year Costs of Asthma United States, 1980–1998 Projection for the Year 2000 15 Estimated costs in billions of dollars 10 5 0 1980* 1985* 1990** 1994** 1998** Year Source: * Weiss, et al. 1992 ** Weiss, et al. 2001 2000 Risk Factors for Development of Asthma Genetic characteristics Environmental exposures Contributing factors Risk Factors for Development of Asthma: Genetic Characteristics Atopy The body’s predisposition to develop an antibody called immunoglobulin E (IgE) in response to exposure to environmental allergens Can be measured in the blood Clearing the Air Categories for Associations of Various Elements Sufficient evidence of a causal relationship Sufficient evidence of an association Limited or suggested evidence of an association Inadequate or insufficient evidence to determine whether an association exists Limited or suggestive evidence of no association Clearing the Air Indoor Air Exposures and Asthma Development Chemical Agents Biological Agents Sufficient evidence of a causal relationship • House dust mite Sufficient evidence of an association • None found • None found • Cockroach (in preschool-aged children) Respiratory syncytial virus (RSV) Sufficient evidence of an association • Environmental tobacco smoke (in preschoolaged children) Limited or suggestive evidence of an association • Sufficient evidence of a causal relationship Limited or suggestive evidence of an association • None found Clearing the Air Indoor Air Exposures and Asthma Exacerbation Biological Agents Sufficient evidence of a causal relationship • • • Dog Fungi/Molds Rhinovirus Limited or Suggestive Evidence of an Association • • • Cat Cockroach House dust mite Sufficient evidence of an association • • • Chemical Agents Domestic birds Chlamydia and Mycoplasma pneumoniae RSV Sufficient evidence of a causal relationship • Environmental tobacco smoke (in preschool-aged children) Sufficient evidence of an association • NO2, NOx (high levels) Limited or suggestive evidence of an association • Environmental tobacco smoke (school-aged, older children and adults) • Formaldehyde • Fragrances Medications to Treat Asthma Medications come in a variety of forms. Two major categories of medications are: • Long-term control • Quick relief Medications to Treat Asthma: Long-Term Control Taken daily, over a long period of time Used to reduce inflammation, relax airway muscles, and improve symptoms and pulmonary function • Inhaled corticosteroids • Long-acting beta2-agonists • Leukotriene modifiers Medications to Treat Asthma: Quick-Relief Used in acute asthma episodes Generally they are short-acting beta2agonists Medications to Treat Asthma: How to Use a Spray Inhaler Health-care provider should evaluate inhaler technique at each visit. Source: “What You and Your Family Can Do About Asthma” by the Global Initiative For Asthma Created and funded by NIH/NHLBI Medications to Treat Asthma: Inhalers and Spacers Spacers can help patients who have difficulty with technique and can reduce potential side effects. Inhalers Spacers Medications to Treat Asthma: Nebulizers Uses compressed air machine to deliver medicine as a mist Good for small children or for severe asthma episodes Managing Asthma: Asthma Management Goals Control symptoms Prevent exacerbation Maintain lung function as close to normal as possible Avoid adverse effects from medications Prevent irreversible airway obstruction Prevent asthma mortality Managing Asthma: Asthma Management Plan Develop with a physician Tailor to meet individual needs Educate patients and families on all aspects of the plan Recognizing symptoms Medication benefits and side effects Proper use of inhalers and peak expiratory flow (PEF) meters Managing Asthma: Indications of a Severe Attack Breathless at rest Hunched forward Talking in words rather than sentences Agitated Peak flow rate is less than 60% of normal Resources National Asthma Education and Prevention Program • http://www.nhlbi.nih.gov/about/naepp/index.htm Asthma and Allergy Foundation of America • http://www.aafa.org American Lung Association • http://www.lungusa.org American Academy of Allergy, Asthma, and Immunology • http://www.aaaai.org Resources Allergy and Asthma Network, Mothers of Asthmatics. Inc. • http://www.aanma.org/ American College of Allergy, Asthma, and Immunology • http://allergy.mcg.edu American College of Chest Physicians • http://www.chestnet.org American Thoracic Society • http://www.thoracic.org Cystic Fibrosis CF is a hereditary disease that causes some glands to produce abnormal secretions that results in tissue and organ damage. Lungs and digestive tract appear to be affected the most. Most common inherited disease leading to a shortened life among white people in the US • • • • 1:3,300 white infants 1:15,300 black infants Rare in Asians Found equally in boys and girls Cystic Fibrosis Currently, there is no cure for CF, but there are many promising new treatments in use and even more on the horizon. The median life expectancy for a person with CF is now 32 • thirty years ago, a CF patient was not expected to reach adulthood. Many people even live into their fifties and sixties. Cystic Fibrosis Cystic fibrosis results when a person inherits two defective copies of a particular gene. This gene controls the production of a protein that regulates the transport of chloride and sodium across cell membranes. Worldwide, about 3 of 100 white people carry one defective copy of the gene. About 3 of 10,000 white people inherit two defective copies of the gene; thus, they develop cystic fibrosis. In these people, chloride and sodium transport is disrupted and dehydration and increased stickiness of secretions occur. The key to Cystic Fibrosis is clogging. The affected areas of the body are the airways, liver, pancreas, intestine, and reproductive tract. CF - Symptoms The lungs are normal at birth, but breathing problems can develop at any time afterward. Thick secretions eventually block the small airways, which leads to inflammation and thickening of their walls. As larger airways fill with secretions, areas of the lung collapse and contract (a condition called atelectasis) and the lymph nodes enlarge. All these changes make breathing increasingly difficult and reduce the lungs' ability to transfer oxygen to the blood. Respiratory tract infections occur because of bacterial growth in the bronchial secretions and walls of the airways. CF – Symptoms (cont) The blockage of pancreatic ducts and intestinal glands leads to digestive problems, including poor absorption of fats, proteins, and vitamins. This, in turn, can lead to nutritional deficiencies, and slower than expected growth. Some people may have episodes of intestinal obstruction when abnormal stool contents block the bowel. About 15 to 20% of newborns who have cystic fibrosis have meconium ileus, a serious obstruction of the small intestine CF - Symptoms About half the children with cystic fibrosis are first taken to the doctor because of frequent coughing, wheezing, and respiratory tract infections. Coughing, the most noticeable symptom, is often accompanied by gagging, vomiting, and disturbed sleep. As the disease progresses, the chest becomes barrel-shaped, and insufficient oxygen may make the fingers clubbed and the nail beds bluish. Polyps may form in the nose. The sinuses may fill with thick secretions, leading to chronic or recurrent sinus infections. CF - Symptoms When a child or adult with cystic fibrosis sweats excessively in hot weather or because of a fever, dehydration may result because of the increased loss of salt and water. A parent may notice the formation of salt crystals or even a salty taste on the child's skin. Adolescents often have slowed growth, delayed puberty, and declining physical endurance. As the disease progresses, lung infection becomes a major problem. Recurrent bronchitis and pneumonia gradually destroy the lungs. CF - Complications About 15% of adults with cystic fibrosis develop insulin-dependent diabetes because the scarred pancreas can no longer produce enough insulin. The blockage of bile ducts by thick secretions can lead to inflammation of the liver and eventually to scarring of the liver (cirrhosis) in about 5% of adults with cystic fibrosis CF – Complications (cont) People with cystic fibrosis often have impaired reproductive function. Almost all men have a low sperm count (which makes them sterile) because one of the ducts of the testis (the vas deferens) has developed abnormally and blocks the passage of sperm. In women, cervical secretions are too thick, causing decreased fertility. Otherwise, sexual function is not affected. Women with cystic fibrosis have a higher likelihood of complications during pregnancy (such as developing a lung infection or diabetes), but many women with cystic fibrosis have given birth. Other complications may include arthritis, kidney stones, and inflammation of the blood vessels (vasculitis). CF - Treatment Advances in antibiotic therapy, nutritional support, and chest physiotherapy have markedly increased survival in people with CF Heart-lung transplantation has been applied to people with CF 5-year survival rates has reached more than 50% in some centers People with CF require daily chest physiotherapy to loosen secretions and prevent stagnation and secondary infections Antibiotics are essential in treating infection, often given intravenously Nutritional support can also be given intravenously in people who are malnourished CF – Voc & Psych Implications People with CF have excellent educational success and are typically productive individuals The counselor will have to work with employers to provide the support mechanisms that will allow the person to remain in the workplace This may include the provision of time for chest physiotherapy or antibiotic treatment during the workday The work environment must be reviewed to ensure the absence of irritants that might exacerbate the disease CF – Voc & Psych Implications Supplemental oxygen may be necessary to allow the person the continue to be productive and ambulatory Psychological outcome in people with CF appears to depend on factors such as altered physical appearance, loneliness, and family strife that the person may attribute to his or her illness The counselor can also work with the person's family to improve support at home that will allow the person to increase social and vocational activities References American Thoracic Society (www.thoracic.org) American Lung Association (www.lungusa.org) American Lung Association of Florida (www.lungfla.org) American Association of Cardiovascular and Pulmonary Rehabilitation (www.aacvpr.org) Asthma and Allergy Foundation of America (www.aafa.org) Allergy and Asthma Network (www.aanma.org) COPD-Support, Inc. (www.copd-support.com) Cystic Fibrosis Foundation (www.cff.org) Cystic Fibrosis Research, Inc. (www.cfri.org) Cystic Fibrosis Resources (www.cysticfibrosis.com)