Respiratory Diseases

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 (B2 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 RehabilitationExercise

23. Pulmonary RehabilitationBreathing Re-training

24. Pulmonary RehabilitationTeaching • 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