PULMONARY REHABILITATION IN RESTRICTIVE PULMONARY DISEASES

1. PULMONARY REHABILITATION IN RESTRICTIVE PULMONARY DISEASES Doç. Dr. Pınar Ergün Atatürk Göğüs Hastalıkları ve Göğüs Cerrahisi Eğitim Araştırma Hastanesi

2. Presentation plan Rationals ? • Interstitial Lung Diseases • Chest Wall Disorders • Neuromuscular Disorders • Obesity- related Disorders Disease specific approaches ?

3. Key Considerations Patient selection Multidisciplinary team Special equipments Individual goals

4. Rationals for Pulmonary Rehabilitation ? • Interstitial lung disease • Chest wall disoders • Neuromuscular disorders • Obesity related respiratory disorders

5. Restrictive Pulmonary Diseases Muscle disfunction Nutrition Skelatel Cardiovascular Psychosocial Exercise intolerance Symptoms Disability + Impaired Quality of Life

6. The Rationale for PR (II) • Complex treatment interventions often required • Immunosuppressive medications • CPAP/BIPAP- acclimatization, training, coaching, reassurance • Transplantation • Mechanical ventilation/end of life decisions • Limited time for patient education and training with self-management strategies in the routine clinical setting

8. PulmonaryRehabilitationisEffectivefortheNon-COPD Patients ! Rationale-III

9. PULMONARY REHABILITATION INTERSTITIAL LUNG DISEASES • IPF • Sarcoidosis • Ocupational lung diseases • Hypersensitivity pneumonia • Drug induced • Collagen vascular diseases • ARDS • BOOP

10. Pulmonary fibrosis Airway-alveolar destruction Capillary destruction Elastic recoil  Vd/Vt  Hypoxemia PVR  Breathing work load  Left vent pre load  Vent. funct  Vent. demand  CO demand  Cardiac funct  Ventilatory impairment Circulatory impairment Exrcise intolerance Wasserman K, Principles of exercise testing, 1999

11. Pulmonary fibrosis Airway-alveolar destruction Capillary destruction Weight loss Reduction in muscle mass Steroid myopaty Deconditioning Elastic recoil  Vd/Vt  Hipoksemi PVR  Breathing work load  Left vent pre load  Vent. funct  Vent. demend  CO demend  Cardiac funct  Ventilatuvar impairment Circulatuarimpairment Exrcise intolerance Wasserman K, Principles of exercise testing, 1999

12. Improvement in muscle function leads to increase in exercise tolerance Chest 2005;127:2028-33

14. Outcome Assessments in Pulmonary Rehabilitation 35 interstitial lung disease 11 Skelatal abnormality ∙ IPF ∙ Pneomoplasty ∙Sarcoidosis∙ Kyphoscolyosiz ∙Sjögren ∙Ankilozan spondilitis ∙Scleroderma ∙SLE ∙RA ∙Radiation fibrosis 8 Week PR program J Cardiopulm Rehabilitation 2006

15. Outcome assessment in pulmonary rehabilitation Exercise endurance↑ HRQL↑ Hospital addmitions ↓ J Cardiopulm Rehabilitation 2006

16. Disease specific programs !

17. PULMONARY REHABILITATION • CHEST WALL DISORDERS • NEUROMUSCULAR DISEASE

18. RATIONALS ?

19. Reduced thoracic wall compliance Positional mechanical disadvantage Respiratory muscle weakness? Restrictive pulmonary function impairment Alveolar hypoventilation + V/Q mismatch HYPOXEMIA + HYPERCAPNIA

20. Reduced thoracic wall compliance Positional mechanical disadvantage Respiratory muscle weakness ? Restrictive pulmonary function impairment Alveolar hipoventilation + V/Q mismatch SYMPTOMS Rapid shallow breathing Dyspnea Peripheral muscle weakness NIPPV RMT Reduction in exercise tolerance HYPOXEMIA + HYPERCAPNIA

21. VO2 peak ↑(13.6±2.8, 14.8 ± 2.8) • 6 MWD↑ (399±62, 467±65) Internal medicine 2006

22. MRC ADL 6 DYT Pulmonary rehabilitation is effective in symptomatic patients with Post TB 6DYT Chest 2003;123:1988-95

23. Respiratory muscle weakness Peripheral weakness Anatomical changes Reduced tidal volumes Reduced lung compliance Ineffective cough General fatigue Decreased mobility Positioning limitations Possible impaired swallowing and increased risk of aspiration Problems faced Faced Problems

24. AIMS PR • Reduce work of breathing Increase tidal volumes and maintain lung compliance • Improve secretion clearance and effectiveness of cough • Maximise independence • Optimise quality of life NIMV Breathing exercise

25. Breathing work rate increase induce reductions in VO2 demand for peripheral muscles • Reduction in work load of respiratory muscles serve for peripheral muscles BORG Harms et al. J Appl Physiol 1997; 1573-1583 Bianchi L, et al, ERJ, 1998 Time

26. Short-Term Effect of Nasal Intermittent Positive-Pressure Ventilation in Patients with Restrictive Thoracic DiseasePınar Ergün, Gülümser Aydın, Ülkü Yılmaz Turay, Yurdanur Erdoğan, Atalay Çağlar, Çiğdem Biber Respiration 2002;69:303-308 ATS dispnea score: 2.5 ± 0.9’-1.6 ± 0.4 (p < 0.01) 6MWD 320.66 ± 93.56 to 382.41 ± 121.20 m (p < 0.05)

27. Respiratory Muscle Training Changes in Airway Resistance (?) Changes in Chest Wall Changes in Compliance Changes in muscle length Increased Demands Nutrients and O2 Nutrients and O2 Delivery can be compromised Physiopathology Skoliosis Cyphosis Cyphoskoliosis

28. Respiratory Muscle Training Chest Wall Deformities & RM Training Rest Training Few studies RM training appears to improve - Lung function, dyspnea & exercise tolerance Effects on the disease time-course: unknown

29. RESPIRATORY MUSCLE TRAINING Changes in Airway Resistance (?) Changes in Thorax Geometry Changes in Compliance Inefficiency of Muscles Increased Demands Nutrients and O2 Amyotrophic Lateral Sclerosis Multiple Sclerosis Spine Cord injury Miasthenia Gravis Duchenne’s Muscular Dystrophy Post-polio Syndrome Guillain-Barré Syndrome Myotonic Dystrophy (Steinert’s Disease) Nutrients and O2 Delivery can be compromised Physiopathology

30. RESPIRATORY MUSCLE TRAINING Neuromuscular Diseases & RM Training Rest Training • Few studies • Education, coordination • Insuflation (active and pasive) • RM training: controversial • - Early, Mild disease, Slow evolution • - Balance with rest

31. Results: Intermittant-RMT is effective in generalized MG patients in improving respiratory muscle force and patern,endurance. Chest 2005;128:1524-30 Chest 2001;120:765-69

32. PULMONARY REHABILITATION; is indicated in ALL patients with chronic respiratory diseases who is sypmtomatic and whose health related quality of life is impaired

33. Core Components of PR for the “Non-COPD” Patient • Patient Assessment • Exercise • Education • Psychosocial/Nutritional intervention • Outcomes measurement

34. Special Considerations in Patient Assessment • CPET: -identify exercise factors contributing to symptoms and limitation -formulate the exercise prescription -Identify pulmonary vascular or cardiac limitations and/or need for supplemental O2 -Incremental exercise to high intensity NOT recommended for known severe pulmonary HTN or degenerative neuromuscular disease • Asses candidacy for inpatient PR

35. Core Components of PR for the “Non-COPD” Patient • Patient Assessment • Exercise training • Education • Psychosocial/Nutritional intervention • Outcomes measurement

36. Interstitial lung disease; Pacing and energy conservation techniques Slow and deep breathing Adequete oxygenation DISEASE SPECIFIC CONSEDERATOIN IN EXERCISE TRAINING (I)

37. Neuromuscular and Chest wall disease Maintain conditioning, avoid fatigue Consultation with neurologist/physiatrist Low intensity aerobic/water-based exercise Shorter, more frequent exercise sessions Emphasis on optimizing functional status Acclimatization to non-invasive ventilation Disease-specific Considerations in Exercise Training (II)

38. Disease-specific Considerations in Exercise Training (III) • Obesity related respiratory diseases • Strength and endurans exercise training/ Upper and Lower extremity • Consider low-impact water-based exercise • Bariatric equipment may be needed • Cardiac and musculo skeletal intervention before enrollment

39. EXERCISE TRAINING Aerobic and Strength Training • No evidence-based guidelines for exercise prescription or training • Mode, intensity and duration individualized to patient ability and need using resources available • Age- and interest- specific modes oxygen saturation > 90% • Maintain Meet metabolic needs, avoid anaerobic metabolism and exercise-induced increased PA pressure • Test O2 saturation using patients’ own portable system

40. Core Components of PR for the “Non-COPD” Patient • Patient Assessment • Exercise • Education • Psychosocial/Nutritional intervention • Outcomes measurement

41. EDUCATION: • PR program staff: Knowledge of physiologic basis of exercise/functional impairment, symptoms and available therapies for different disorders • PATIENT and FAMILY • Nature and expected course of disease • Physiologic basis of symptoms an exercise limitations • Rational for and proper use of supplemental oxygen • Pulmonary drainage technigues • Nutrition • Recognition of symptoms and sings of secondary infection • Prevention strategies • Coping techniques for assistance in managing anxiety and depression • Training options for and outcomes of mechanical ventilation

42. DISEASE SPECIFIC PROGRAM MANAGEMENT • Ensure patient safety,individual goals • Individual education sessions may be needed • Written and video educational materials • Assess outcomes acording to goals Individual, disease specific approaches is necessary

43. AGHH Pulmonary rehabilitation unit documents; 2005- 2007 • 2days in a week / 8 week • Patient number completed the program ;71 • 49 COPD: (Stage I)=5 • ( Stage II)=10 • (Stage III)= 18 • (Stage IV)= 16 • 3 Persistant Asthma • 7 Bronchiectasis • 4 Kyphoscolyosis + restrictive LD • 1 Pneumoconiosis • 1 IPF • 1 Sarcoidosis

44. PR Outcomes/DISPNEA

45. PR Outcomes/ EXERCISE TOLERANCE Endurans/Dakika

46. PR Outcomes/ HRQL

47. PR Outcomes/ HRQL