Pulmonary Conditions

1. Pulmonary Conditions Medical and Psychosocial Aspects of Disability RCS 6080

2. 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)

3. 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

4. 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.

6. 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.

7. 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.

8. 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

9. Categories of COPD • Type A - “Pink Puffer” • Considered to have predominantly emphysema • Type B - “Blue Bloater” • Considered to have predominantly chronic bronchitis.

10. 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

11. 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.

12. 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.

13. 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

14. 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

15. 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.

16. 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.

17. 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.

18. 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

20. 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

21. Asthma Prevalence* by AgeUnited States: 1980–1996 Under 18 Total 18+ Source: National Health Interview Survey * 12-month prevalence

22. Asthma Prevalence* by SexUnited States: 1982–1996 Female Total Male Source: National Health Interview Survey * 12-month prevalence

23. Asthma Prevalence* by RaceUnited States: 1982–1996 Black White Source: National Health Interview Survey * 12-month prevalence

24. Asthma Prevalence* by RaceAges 5-34, United States: 1980–1996 Black, 5-34 White, 5-34 Source: National Health Interview Survey * 12-month prevalence

25. Age-Adjusted* Asthma Mortality Ratesby Sex, United States: 1979–1998 Female Total Male Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Age-adjusted to 2000 U.S. population

26. Age-Adjusted* Asthma Mortality Ratesby Race, United States: 1979–1998 60 50 Black 40 Other 30 20 Rate per million White 10 0 Year 1979 1981 1983 1985 1987 1989 1991 1993 1995 1997 Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Age-adjusted to 2000 U.S. population

27. Asthma Mortality Rates by RaceAges 5-34, United States: 1979–1998 Black White Other* Source: Underlying Cause of Death dataset by the National Center for Health Statistics * Unreliable (< 20 deaths) 1979–1995

28. Costs of AsthmaUnited States, 1980–1998Projection for the Year 2000 Source: * Weiss, et al. 1992 ** Weiss, et al. 2001

29. Risk Factors for Development of Asthma • Genetic characteristics • Environmental exposures • Contributing factors

30. 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

31. Clearing the AirCategories 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

32. Biological Agents Sufficient evidence of a causal relationship House dust mite Sufficient evidence of an association None found Limited or suggestive evidence of an association Cockroach (in preschool-aged children) Respiratory syncytial virus (RSV) Chemical Agents Sufficient evidence of a causal relationship None found Sufficient evidence of an association Environmental tobacco smoke (in preschool-aged children) Limited or suggestive evidence of an association None found Clearing the AirIndoor Air Exposures and Asthma Development

33. Biological Agents Sufficient evidence of a causal relationship Cat Cockroach House dust mite Sufficient evidence of an association Dog Fungi/Molds Rhinovirus Limited or Suggestive Evidence of an Association Domestic birds Chlamydia and Mycoplasma pneumoniae RSV Chemical Agents 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 Clearing the AirIndoor Air Exposures and Asthma Exacerbation

34. Medications to Treat Asthma • Medications come in a variety of forms. • Two major categories of medications are: • Long-term control • Quick relief

35. 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

36. Medications to Treat Asthma:Quick-Relief • Used in acute asthma episodes • Generally they are short-acting beta2-agonists

37. 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

38. Medications to Treat Asthma:Inhalers and Spacers Spacers can help patients who have difficulty with technique and can reduce potential side effects. Inhalers Spacers

39. Medications to Treat Asthma:Nebulizers • Uses compressed air machine to deliver medicine as a mist • Good for small children or for severe asthma episodes

40. 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

41. 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

42. 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

43. 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

44. 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

45. 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

46. 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.

47. 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.

48. The key to Cystic Fibrosis is clogging. The affected areas of the body are the airways, liver, pancreas, intestine, and reproductive tract.

49. 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.

50. 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