The Ventilator D epend e nt P atient : Pulmonary R ehabilitation at H ome

1. The Ventilator Dependent Patient: Pulmonary Rehabilitation at Home Nicholas S.Hill MD Division of Pulmonary, Critical Care and Sleep Medicine Tufts Medical Center

2. Disclosures • Research Grants • Respironics • Breathe Technologies • Versamed

3. Definition: Pulmonary Rehab “ a multidisciplinary program of care for patients with chronic respiratory impairment that is individually tailored and designed to optimize physical and social performance and autonomy” ATS Statement Holistic: Consider all aspects of patient’s life

4. Pulm Rehab Applied to Vent Dependent Patients • Optimize pulmonary status • Ventilator mode, settings • Target gas exchange • Secretion management • Optimize physical status • Mobility • Physical therapy • Optimize environment • Caregivers • Finances • Psychological

5. Ventilator Modes/Settings • “Body Ventilators” • Negative pressure • Abdominal displacement ventilators • Positive pressure ventilators • Noninvasive • Invasive

6. Noninvasive Ventilation – Early 1980s Hill NS. Clinical Applications of Body Ventilators Chest ‘86

9. LP-4 Portable Volume Ventilator

10. Negative vs Positive Pressure Noninvasive Ventilation • Negative pressure vent exacerbates or induces OSA – positive pressure ameliorates it. Hill et al, Chest 1991 • Body ventilators • less portable • More difficult and time consuming to apply • More expensive

11. Positive Pressure Noninvasive vs Invasive (tracheostomy) Ventilation • Invasive ventilation • More technically demanding and costly to administer • Need for secretion management, suctioning • Caregiver burnout • May preclude home management • More airway complications, pulmonary infections • But more secure and sleep better • Bach et al, Chest ‘93

12. Epidemiology of HMV in Europe NMD RTD COPD Loyd-Owen et al, ERJ 2005: 25:1025

13. Epidemiology of HMV in Europe Trache NMD VOL Oronasal RTD Nasal Pressure Vents COPD Loyd-Owen et al, ERJ 2005: 25:1025

14. Epidemiology of HMV in Europe NMD VOL RTD Pressure Vents COPD Loyd-Owen et al, ERJ 2005: 25:1025

15. Epidemiology of NIV in Europe NMD VOL RTD Pressure Vents COPD Janssens et al, Chest 2003: 123:67

16. Optimal Management of NPPV • Select patients with intact upper airway function, cooperative, motivated • Not too rapidly progressive (Muscular dystrophy, post-polio, not Guillain Barre) • Optimally fit mask • Start when nocturnally hypoventilating • Gradually increase pressure IPAP vs EPAP, aiming for ∆ of 8-12 cm H2O

17. Rehabilitation Program Depends on Indication for NPPV • Neuromuscular Disease • Amyotrophic Lateral Sclerosis • Muscular Dystrophies • Post-Polio • wheelchair, communication devices, speech swallowing • Obesity Hypoventilation • Reconditioning, weight loss, ? Bariatric Surg • COPD • Medical therapy, reconditioning, O2

18. When to Start NPPV in NMD? 4.65 hrs use/night Overall SF-36 better in Group 2 Reduce frequency of acute hypercapnic crises (trend) Ward et al, Thorax 2005; 60:1019

19. RCT for NIV in ALS • 41 pts with orthopnea/ FVC < 60% or  PaCO2 • 205 day prolongation of survival with  QOL • Survival not  in bulbar, but QOL, sleep-related symptoms improved • Bourke SC, Lancet Neurol 2006;5:140-7 • Predictors • survival: NIV tol, BMI, Bulbar • NIV tol: Orthopnea- yes, bulbar - no Bourke SC et al. Lancet Neurol 2006; 5: 140-147

21. Swift and Ultra Mirage Masks

22. Indications for Invasive (Trachestomy) Management • Failure of NPPV • Inability to protect airway • Copious secretions • Persistent aspiration • Very weak cough • Repeated pneumonias • Continuous NPPV (optional) • Assumes ethical aspects have been discussed and agreed upon

23. Optimization of Invasive Home Mechanical Ventilation • Deal with ethical issues early • Both patient and family • Select appropriate patient • Motivated, management skills, self-care • Family! • Simplify - nocturnal only? • Permit speech and eating • Avoid excessive cuff pressures/volumes • Well-trained caregivers– emergencies • Low threshold for antibiotics

25. CT of Neck Showing marked tracheomegaly and...

26. Tracheo-Esophageal Fistula

28. Optimize Independence, Mobility • Self suctioning • Portability • Ventilators mounted on wheelchairs • Battery backup

31. Importance of Mobility

34. Secretion Management • Extremely important in neuromuscular diseases with impaired cough • Manually assisted coughing • Mechanical In-exsufflator • VEST not so important unless thick secretions (bronchiectasis, cystic fibrosis – also postural drainage, flutter valve)

36. Cough Assist • Delivers deep insufflations (+30-40 X 2 sec ) followed immediately by deep exsufflations (-30-40). Pressures and delivery times are independently adjustable. • Simulates the physiologic mechanism of cough.

39. Caregivers • Family usually major source • Personal care attendants - Boon and bane • Good ones worth their weight in gold • Source of enormous stress • Insurance constantly trying to cut hours • Business skills helpful • Burnout major risk - ? Respite

40. Parents – Lifelong commitment

43. Rehab of Home Mechanical Ventilated patients • Seeks to optimize pulmonary status by choosing the appropriate ventilator and settings at the appropriate time • Deals effectively with secretion management • Aids patient in living full life, taking advantage of capabilities like speech and eating • Enhances independence and mobility • Considers psychosocial aspects of life: • Family interactions and remaining at home • Preserving Fun, travel • Considering Religious values and early discussions of ethical aspects of management