1. Respiratory Diseases Pathophysiology and Medical Treatments

2. Respiratory System Lungs - airways - alveoli - blood vessels - defense system Respiratory pump - Central controller - spinal cord - motor nerves - muscles

3. Respiratory Diseases Lungs - airways-asthma - alveoli-COPD, pulmonary fibrosis - blood vessels-pulmonary hypertension - defense system- inadequate cough, aspiration, immune dysfunction Respiratory pump - Central controller-central alveolar hypoventilation - spinal cord- SCI - motor nerves- ALS - muscles- muscular dystrophy

4. Discussion Topics Lung diseases - Asthma - COPD - Pulmonary fibrosis - Lung Transplant - Pneumonia Respiratory Pump Diseases - Muscular Dystrophy - Spinal Cord Injury

5. Asthma Defined as reversible obstruction or narrowing of the airways - between episodes patients feel normal and have normal pulmonary function tests If you were to see the asthmatic airway under the microscope you would see: - narrowed, edematous airways - inflammation in the airway walls - excess mucous secretion and plugging

6. Asthmatic Airway

7. Asthma Allergy Related (extrinsic) - Immunoglobulin E (IgE) - Pollens, and animal danders, etc. - Seasonal - Younger individuals Unrelated to allergy (intrinsic) - Aspirin sensitivity - Not seasonal

8. Asthma- Symptoms Shortness of breath (dyspnea) Wheezing Chest tightness “Feeling of suffocating” Cough Exercise induced

9. Asthma- Physical Findings Rapid breathing (tachypnea) Perspiring Using “accessory” muscles of respiration - sternoclydomastoid, platysma, pectoralis major and minor cyanosis tachycardia pulsus paradoxus

10. Asthma- Acute Treatment Bronchodilation (opening the airways) - inhaled B-agonists (B 2 receptors bronchodilate) albuterol, salmeterol, pirbuterol, bronkosol - parenteral B-agonists epinephrine, terbutaline, isoproterenol - inhaled anticholinergics (cholinergic receptors constrict) ipatroprium bromide, glycopyrrolate - Theophylline

11. Asthma Treatment-Acute Anti-inflammatory - parenteral steroids Artificial ventilation - Noninvasive-facemask - Invasive-endotracheal tube High risk

12. Asthma - ChronicTreatment anti-inflammatories are key to prevent exacerbations - inhaled steroids at high dose triamcinalone, budesonide, fluticasone, beclomethasone - mast cell stabilizing drugs nedocromil, cromolyn B-agonists and anticholinergics as needed Leukotriene inhibitors - zafirleukast (zyflo) - Montelukast (singulair_ “Stepped care” - Gradual addition of medications

13. Chronic Obstructive Pulmonary Disease (COPD) Is a general term for patients with chronic airflow obstruction that may be due a number of causes - emphysema - chronic bronchitis - chronic severe asthma > 90% of cases are due to smoking Lungs are obstructed and overinflated

14. Physiologic Derangements in COPD Destruction of Alveolar Tissue Loss of lung elastic recoil Airway obstruction

15. Chronic Obstructive Pulmonary Disease (COPD) Functional consequences of airway disease and chronic lung injury - Obstruction to airflow - Hyperinflation of the chest - Improper respiratory muscle function - Increase work of breathing

16. COPD- Symptoms gradually progressive shortness of breath (over years) - may end up disabled with dyspnea at rest - may require oxygen cough frequently productive of sputum leg swelling anxiety

17. COPD- Physical Signs Barrel chest Tachypnea “Pursed-lip” breathing Use of accessory muscles Diaphragm dysfunction - Hoover sign - lack of outward movement of abdomen Reduced and prolonged expiratory airflow

19. COPD X-ray

20. COPD- Treatment B-agonists Anticholinergics Theophylline Steroids - only 20 % of patients are steroid responsive

21. COPD Treatment Pulmonary Rehabilitation Lung Transplant Lung Volume Reduction Surgery (LVRS)

22. Pulmonary Rehabilitation Exercise

23. Pulmonary Rehabilitation Breathing Re-training

24. Pulmonary Rehabilitation Teaching Biology of disease Medications Oxygen Travel Minimizing energy expenditure Interpersonal relationships

25. Break

26. COPD-Surgical interventions Lung volume reduction surgery (LVRS) Lung transplantation

27. LVRS Hypothesis: Hyperinflation of the lungs in COPD is the primary cause of dyspnea. Reducing the sized of the lungs will reduce dyspnea and increase expiratory airflow Procedure: Sternotomy with resection of 25 to 30% of each lung

28. Lung Volume Reduction Surgery

29. Lung Transplantation For very advanced disease Age < 65 years No other major medical problems Post transplant immunosupression - 15-20 medications

30. Pulmonary Fibrosis Scarring of the lung tissue due to inflammation Lungs become too small- “restricted” Due to a wide range of causes: - drugs - toxic exposures - rheumatologic diseases - idiopathic- “IPF”

31. Interstitial Lung Disease

32. Pulmonary Fibrosis- Symptoms Dyspnea Exercise intolerance Cough Symptoms associated with systemic disease

33. Pulmonary Fibrosis- Exam Findings Rapid, shallow breathing clubbing of the fingers “velcro” rales or crackles in the lungs cyanosis findings associated with systemic disease

34. Pulmonary Fibrosis- Treatment Steroids Cytotoxic agents - imuran - cyclophosphamide Lung Transplant

35. Pneumonia Common pulmonary disease Usually there is an associated host defense problem - aspiration - foreign body - immune suppression recent viral illness More global immune problem - Ciliary problem smoking Cystic Fibrosis

36. Pneumonia Xray

37. Pneumonia- Symptoms and Physical Findings Cough Chest pain Fever, chills Dyspnea Evidence of consolidation on lung exam - “bronchial breath sounds” - egophony - dullness to percussion

38. Pneumonia- Treatment One or more antibiotics Choice will depend on patients age, immune status, seriousness of clinical condition Sputum sample with Gram’s stain can be helpful

39. Spinal Cord Injury Level of spinal cord injury is critical C2 or above clearly ventilator dependent C3-C5- likely ventilator dependent at least partially C5 and below usually ventilator independent but cough and secretion clearance is a problem Lung volumes appear “restricted” Cough and expiratory flow always an issue

40. Spinal Cord Injury- Respiratory Treatment Will depend entirely on level of injury Maintaining adequate ventilation is of utmost importance, almost all patients will initially be on a mechanical ventilator Clearance of secretions and prevention of pneumonia is also of critical importance - The leading cause of death in the first year following injury is pneumonia Techniques of Secretion Management - Chest physical therapy, assisted cough - Tracheal suctioning - In-exsufflator

41. Spinal Cord Injury- Respiratory Treatment Some patients may need only partial ventilation at night Non-invasive ventilation may be an option - No tracheostomy - Less complications

42. Muscular Dystophy Many varieties - Frequently genetic Muscle and not nerves are affected Progressive loss of function over years Primary cause of death is pneumonia Currently no medical treatment - Future: ? Gene therapy

43. Muscular Dystrophy Often associated with scoliosis Patients will be short of breath Patients will often breath less well at night and have associated sleep apnea Treatment will be aimed at relieving symptoms and prolonging life Noninvasive ventilation is a definite option

44. Mouthpiece Ventilation- ”SIP”

45. Nocturnal Ventilation

46. Cough-Assist Device

47. Noninvasive Ventilation