The Role of the Respiratory Therapist in the Treatment of the PH Patient Gerilynn L. Connors, RRT, BS, FAARC, FAACVP

1. The Role of the Respiratory Therapist in the Treatment of the PH PatientGerilynn L. Connors, RRT, BS, FAARC, FAACVPRClinical Manager, Pulmonary RehabilitationInova Fairfax HospitalFalls Church, VAgerilynn.connors@inova.org

2. The Role of the Respiratory Therapist From ICU to Home Care

3. OBJECTIVES: state how the Pulmonary Diagnostic Laboratory test patient�s lung function, exercise capacity and determines what oxygen a patient may need while flying Understand how Pulmonary Rehabilitation can be an adjunct treatment for the PH patient from the inpatient setting to the outpatient setting Know the important role the ICU Respiratory Therapist provides for heart failure patients beyond Nitric Oxide (NO) to Inhaled Epoprostenol Understand the respiratory home care needs of the PH patient from oxygen systems, delivery devices to CPAP Understand how the Respiratory Therapist can be a vital team member in the Pulmonary Hypertension Clinic

4. Medical Direction in Respiratory Care The strength of a Respiratory Care Department, Pulmonary Diagnostic Laboratory and Pulmonary Rehabilitation Program is measured not only by the Respiratory Therapist and Managers who work in these departments but the MEDICAL DIRECTORS who provide guidance, support and evidenced based direction.

5. Pulmonary Diagnostic Laboratory Pulmonary Function Test Pre/post spirometry Lung Volume Diffusion Exercise capacity Test 6 Minute Walk Test Pulmonary Exercise Stress Test Arterial Blood Gas Oxygen Test for High Altitude (air flight, travel)

6. Air Travel for the Patient Requiring Oxygen � the Pulmonary Diagnostic Laboratory Hypoxia-Altitude Simulation Test (HAST) Patient breaths 15.1% oxygen simulating aircraft conditions Determine what patients will develop severe hypoxemia during air travel Able to identify patients at risk of flight-related complications requiring supplemental oxygen during air travel Titration of oxygen during test to determine oxygen l/m in aircraft

7. Calculation for Estimate of In-Flight PaO2 Predicted PaO2 at altitude = 22.8 � 2.74x + 0.68y A regression equation derived from HAST Used in normocapnic chronic airway obstruction X is anticipated cabin altitude in thousands of feet Y is resting PaO2 in mmHG at ground level, on room air Formula provides only a prediction of anticipated PaO2 HAST is able to assess the cardiovascular and symptomatic response plus determine supplemental oxygen need

8. Pulmonary Rehabilitation from the Inpatient to Outpatient Setting Pulmonary Rehabilitation is an adjunct treatment for the PH patient Pulmonary Rehabilitation assess and treat may be appropriate for the PH Inpatient New PH diagnosis New medication program Patients who begin IV PAH medications must be monitored closely when beginning exercise due to hypotension Exacerbation of PH Need to assess exercise function and provide advise for oxygen delivery system and liter flow of home oxygen therapy Pre/Post lung transplant

9. Pulmonary Rehabilitation Definition ATS/ERS 2006 �Pulmonary rehabilitation is evidence-based, multi-disciplinary, and comprehensive intervention for patients with chronic respiratory disease who are symptomatic and often have decreased daily life activities. Integrated into the individualized treatment of the patient, pulmonary rehabilitation is designed to reduce symptoms, optimize functional status, increase participation and reduce health care costs through stabilizing or reversing systemic manifestations of the disease.� This definition applies to the pulmonary hypertension patient with the ultimate goal of optimizing their quality of life through assessment, education and therapeutic exercise. The PH patient�s success in PR starts with a strong partnership between the referring PH Clinic and the local pulmonary rehabilitation program.

10. Essential Components of Pulmonary Rehabilitation Assessment Education/Training Therapeutic Exercise Psychosocial Intervention Long Term Adherence** **with Prevention and Outcomes **

11. Assessment Respiratory Therapy Assessment Exercise Assessment (6 min. walk test) Hypoxemia: at rest and with exercise Nutritional Assessment Other Assessments as determined: physical therapy occupational therapy Social/ psychological PAH Specific New York Heart Functional Class/ Symptoms Assessment PA Pressures Diagnostic Classification Expected side effects of medications/ INR (Prothrombin time (PT) and its derived measures of prothrombin ratio (PR) and international normalized ratio (INR) are measures of coagulation.) Patients understanding of medications/ back-up pumps Lower baseline blood pressures Peripheral edema

12. PAH: Signs and Symptoms Symptoms Syncope Palpitations Fatigue Dyspnea on exertion Anginal Chest Pain Hemoptysis Light headedness Signs Prominent Right Ventricular Impulse Accentuated Pulmonic Valve component (P2) Right-Sided third heart sound (S3) Hepatomegaly Peripheral Edema Jugular Vein Distention One of the challenges of treating PH is recognizing and diagnosing it. Typical signs and symptoms are shown here. These signs and symptoms are often subtle and nonspecific, making diagnosis difficult. Patients often initially present with increasing dyspnea on exertion. All of these but P2 are indicators that often only become apparent in later stages of disease progression. The last four signs (S3, hepatomegaly, peripheral edema, JVD) are seen once the patient has progressed to right heart failure. One of the challenges of treating PH is recognizing and diagnosing it. Typical signs and symptoms are shown here. These signs and symptoms are often subtle and nonspecific, making diagnosis difficult. Patients often initially present with increasing dyspnea on exertion. All of these but P2 are indicators that often only become apparent in later stages of disease progression. The last four signs (S3, hepatomegaly, peripheral edema, JVD) are seen once the patient has progressed to right heart failure.

13. Potential Side Effects of PAH Medications Cough is usually worse in the first month of treatment and gets better over time. - baseline systolic BP < 85 mmHg - antihypertensives, vasodilators, anticoagulants Cough (39%) Headache (30%) Flushing (27%) Flu-like syndrome (14%) Nausea (13%) Trismus (12%) Jaw pain (12%) Hypotension (11%) Cough is usually worse in the first month of treatment and gets better over time. - baseline systolic BP < 85 mmHg - antihypertensives, vasodilators, anticoagulants Cough (39%) Headache (30%) Flushing (27%) Flu-like syndrome (14%) Nausea (13%) Trismus (12%) Jaw pain (12%) Hypotension (11%)

14. PAH PR Assessment Cont. PR Assessment to include: WHO Clinical Classification of PAH WHO Functional Classification, Class I-IV Results of Rt. heart catheterization Important to record drug therapy, route given Symptoms: syncope, palpitations, fatigue, chest pain, light headedness, edema, blood pressure Anticoagulation, INR Results of overnight oximetry or formal sleep study Are they a candidate for lung transplant?

15. Education/Patient Training Normal Anatomy and physiology Chronic Lung Disease Description and interpretation of medical tests Breathing Retraining Bronchial Hygiene Medications Oxygen Therapy/Sleep Disorders Activities Of Daily Living Eating Right Preventing Infection Leisure Activities Coping With Chronic disease/ advanced directives

16. PAH Specific Education/Patient Training Identifying and self monitoring of PH symptoms Recognizing symptom limited exercise Know signs of right heart failure Emergency procedures (pumps & lines) Expected reactions to medications Identifying symptoms/ understanding heart cath results Pregnancy risks Avoiding falls for the anti-coagulated patient INR test results & frequency Recognizing �symptom limited exercise� Self monitoring of weight and edema Weight and edema checks Expected reaction to PH meds Need for lifelong medication Patients MUST bring their back up pump at all times Lung transplantation

18. Exercise Testing 6-minute walk test Pulmonary Exercise Stress Test Detect exercise-induced hypoxemia and determine O2 titration Establish a baseline for outcome determination Evaluation of current functional activity level and limitations, ADLS, pain, strength, range of motion, posture, balance, gait, safety, and breathing pattern Evaluation of PAH symptoms, chest pain, shortness of breath, syncope, and fatigue

19. EXERCISE exercise training should be initiated in a supervised setting - PR Patient has Fear of exertion Patient should Never exercise alone Always have back-up pumps and medications as prescribed Know the safety measures for lines/pumps with exercise equipment Avoid exercises that increase intra thoracic pressure or valsalva maneuvers Detect exercise-induced hypoxemia, O2 titration (may require high flow oxygen devices) goal is to keep patients = 90% O2 saturation Determine best home oxygen system, delivery device and flow rate, especially when high flow oxygen required (beyond the nasal cannula) PH PR exercise documentation form to include PH symptoms, vitals plus edema, daily weight Collaborative partnership with PH Clinic and PR a must to communicate concerns, issues, symptoms

20. Flolan� (epoprostenol)  Here is an example of the supplies that may be used in mixing Flolan each day. The number of Flolan vials will depend on the prescribed concentration and the tubing illustrated is changed every three days or on M,W, and F. Here is an example of the supplies that may be used in mixing Flolan each day. The number of Flolan vials will depend on the prescribed concentration and the tubing illustrated is changed every three days or on M,W, and F.

21. Avoid activities that increase intra-thoracic pressure or valsalva effort

23. PSYCHOSOCIAL INTERVENTION Quality of life testing (CAMPHOR) Loss of job or income, disability Family dynamics Pregnancy issues Impact of severe lung disease at relatively young age Genetic testing Lack of visible signs of illness Possible lung transplant evaluation

24. LONG TERM ADHERENCE Schedule and keep PH Clinic appts Medications necessary for life Attend PH support groups Treatment of PH resulting in prolongation of life and increased functional capacity Exercise with a partner or in a supervised setting Be connected with the National PH Association

25. Pulmonary Rehabilitation: PR is not just exercise or education but must have the essential components Typical PR program may meet three times a week, over an 8-12 week period of time, have approximately 10-15 hours of education and 30 hours of therapeutic exercise. The commitment by the PH patient is great but so are the benefits. The success of the PR program is also measured by the strength of the PR�s Medical Director who guides the multi-disciplinary team in evidence-based practice. The PR goals for the PH patient are not that different from the goals of PH medical management: improve cardiovascular endurance, increase exercise performance, enhance ability to perform Activities of Daily Living (ADL), improve quality of life, reduce hospitalizations, decrease symptoms, especially dyspnea through breathing retraining and ensuring adequate oxygenation at rest and with activity.

26. Positive Outcomes from Pulmonary Rehabilitation Patient will have a better understanding of how PH affects their lungs, oxygen and exercise Understand lung symptoms and decrease shortness of breath through breathing retraining and ensuring adequate oxygenation at rest and with activity Increase exercise performance that translates into improvements in activities of daily living Improve cardiovascular endurance through a safe and supervised exercise program Improve quality of life through education and therapeutic exercise Exercise in a facility that allows the patient to feel secure and safe because of the skill set of the pulmonary rehabilitation respiratory therapist working with them PR team communicates with referring MD and the PH clinic on patient�s progress in PR

27. How to Locate a Pulmonary Rehabilitation Program  American Association of Respiratory Care (AARC) http://www.yourlunghealth.org/finding_care/qrc/pulm_care/index.cfm American Association of Cardiovascular and Pulmonary Rehabilitation, (AACVPR) http://www.aacvpr.org/Resources/SearchableCertifiedProgramDirectory/tabid/113/Default.aspx

28. Respiratory Home Care Needs of the PH Patient Oxygen Systems Oxygen Delivery Devices CPAP or Bi-Level Positive Airway Pressure to treat Sleep Apnea

29. Oxygen Systems: Compressed gas Liquid oxygen Oxygen concentrator

30. Oxygen Delivery Devices Delivery Device Description Liter Flow Nasal Cannula Delivers approx. 44% 1-6 l/m O2 depending on liter flow, patients respiratory rate, etc. Oxymizer Pendant Higher FiO2 achieved or Mustache 1-12 l/m http://www.chadtherapeutics.com/usa/Disposable-Conservers/Oxymizer.html High Flow Cannula High flow without a face mask. (various manufacturers) Patient can eat, drink etc. 6-15 l/m Oxymask provide greater FiO2 at lower flows 1 - flush l/m http://www.southmedic.com/products/oxymask-adult.php

31. Respiratory Therapy in the ICU Know the important role the ICU Respiratory Therapist provides for heart failure patients beyond Nitric Oxide (NO) to Inhaled Epoprostenol (iEPO) Ventilated patients can be challenging to liberate (wean) off mechanical ventilation and the ICU Respiratory Therapist is a vital member of the ICU team

32. Inhaled Nitric Oxide (iNO) Objective: Decrease pulmonary artery pressure (PAP) Decrease pulmonary vascular resistance (PVR) Improve oxygenation Patient Populations: adults and children Indications: respiratory failure with mechanical ventilation, secondary to diffuse parenchyma lung disease, severe respiratory disease requiring FiO2 >70%, oxygenation index X Mean Airway Pressure of >10, patients with congenital or acquired heart disease with anatomic and/or physiologic abnormalities associated with pulmonary artery hypertension or pulmonary vascular changes, lung and cardiac transplant, LVAD Benchmarking and Evidenced Based Data Cost: Expensive

33. Going Beyond Inhaled Nitric Oxide (iNO) ��������..Inhaled Epoprostenol (iEPO) Objective: treat pulmonary hypertension and right ventricular failure as confirmed by rt. heart cath., echo, or direct visual inspection during cardiac surgery Treat severe hypoxemia (PaO2/FiO2 ration < 200) unresponsive to standard therapy in patients with ARDS Patient Populations: adults and children, Indications: lung, heart transplant, LVAD, ARDS Inhaled Epoprostenol (iEPO) Comparable to the effect of iNO, clinical & hemodynamic response good Lack of toxic reactions Easy administration Cost effective alternative Benchmarking and Evidenced Based Data

34. The Respiratory Therapist and the Pulmonary Hypertension Clinic Role the Respiratory Therapist has is dependent on the facility and program needs as directed by the PH Medical Director and Manager Assessment and Education of the PH Patient clinic evaluation To include H & P physical exam medication review Diagnostic testing: 6 MWT and spirometry test Education of the PH patient on specific topics

35. References CJ Dine, ME Kreider. Hypoxia Altitude Simulation Test. Chest. 2008;133;1002-1005. Aina Akero, MD, Anne Edvardsen, Carl Christensen, et.al., COPD & Air Travel. Oxygen Equipment and Preflight titration of supplemental oxygen. Trial registry: Clinical Trials.gove; No.: Identifier NCT01019538; URL: clinicaltrials.gov. Chest Journal de Man FS, Handoko ML, Groepenhoff H, et. al., Effects of exercise training in patients with idiopathic pulmonary arterial hypertension. Eur Respir J 2009; 34: 669-675. Shapiro S, Traiger GL, Exercise and Pulmonary Hypertension, Chapter 32, pg 518- 528 in Hodgkin JE, Celli BR, Connors GL. Editors. Pulmonary Rehabilitation: Guidelines to Success, 4th Edition, Mosby Elsevier, 2009.

36. References Cont. Mereles D, Ehlken N, Kreuscher S et al. Exercise and respiratory training improve exercise capacity and quality of life in patients with severe chronic pulmonary hypertension. Circulation 2006 October 3;114(14):1482-9. Adamali H, Gaine SP, Rubin LJ. Medical treatment of pulmonary arterial hypertension. Semin Respir Crit Care Med 2009;30:484-492. Dose-Response to Inhaled Aerosolized Prostacyclin for Hypoxemia Due to ARDS Chest March 2000 117:819; 10.1378/chest.117.3.819 Suhail Raoof, Keith Goulet, et.al., Severe Hypoxemic Respiratory Failure: Part 2�Nonventilatory Strategies Chest June 2010 137:1437; 10.1378/chest.09-2416

37. References Cont. Kieter Wlamrath, Thomas Schneider, et. al., Direct Comparison of Inhaled Nitric Oxide & Aerosolized Prostacycline in Acute Respiratory Distress Syndrome. Am J Respir Crit Care Med 1996;153:991-6. Charl J. De Wet, David Afflect, et. al., Inhaled prostacycline is safe, effective, and affordable in patients with pulmonary hypertension, right heart dysfunction, and refractory hypoxemia after cardiothoracic surgery. J. Thorac. Cardiovasc. Surg., December 1, 2004; 128(6):949-950.

38. SUMMARY���������������... Respiratory Therapist have a critical role in optimizing the treatment and quality of life for the PH patient from the ICU to Pulmonary Rehabilitation to Pulmonary Diagnostics to Home Care, to the PH Clinic setting through collaboration with the Pulmonary Hypertension Specialist.

39. THANK YOU!!!!!!! Gerilynn L. Connors, RRT, BS, FAARC, FAACVPRClinical Manager, Pulmonary RehabilitationInova Fairfax HospitalFalls Church, VAgerilynn.connors@inova.org