PULMONARY REHABILITATION IN RESTRICTIVE PULMONARY DISEASES

PULMONARY REHABILITATION IN RESTRICTIVE PULMONARY DISEASES

Doç. Dr. Pınar ErgünAtatürk Göğüs Hastalıkları ve

Göğüs Cerrahisi Eğitim AraştırmaHastanesi

Rationals ?

Interstitial Lung Diseases Chest Wall Disorders Neuromuscular Disorders Obesity- related Disorders

Disease specific approaches ?

Presentation planPresentation plan

Key Considerations

Patient selection

Multidisciplinary

team

Special equipments

Individual goals

Rationals for Pulmonary Rehabilitation ?

Rationals for Pulmonary Rehabilitation ?

•Interstitial lung disease•Chest wall disoders•Neuromuscular disorders•Obesity related respiratory disorders

Restrictive Pulmonary Diseases

Restrictive Pulmonary Diseases

Muscle disfunctionNutrition Skelatel Cardiovascular Psychosocial

Exercise intoleranceSymptoms

Disability + Impaired Quality of Life

The Rationale for PR (II)

Complex treatment interventions often requiredo Immunosuppressive medicationso CPAP/BIPAP- acclimatization, training,

coaching, reassuranceo Transplantationo Mechanical ventilation/end of life decisions

Limited time for patient education and training with self-management strategies in the routine clinical setting

Pulmonary Rehabilitation is Effective

for the Non-COPD Patients !

Rationale-III

INTERSTITIAL LUNG DISEASES

IPF Sarcoidosis Ocupational lung diseases Hypersensitivity pneumonia Drug induced Collagen vascular diseases ARDS BOOP

PULMONARY REHABILITATION

PULMONARY REHABILITATION

Pulmonary fibrosisPulmonary fibrosis

Airway-alveolar destruction Capillary destruction

Elastic recoil Vd/Vt Hypoxemia PVR

Breathing workload Left vent pre load

Vent. funct Vent. demand CO demand Cardiac funct

Ventilatory impairment Circulatory impairment

Exrcise intoleranceExrcise intolerance

Wasserman K, Principles of exercise testing, 1999

Pulmonary fibrosisPulmonary fibrosis

Airway-alveolar destruction Capillary destruction

Elastic recoil Vd/Vt Hipoksemi PVR

Breathing workload Left vent pre load

Vent. funct Vent. demend CO demend Cardiac funct

Ventilatuvar impairment Circulatuarimpairment

Exrcise intoleranceExrcise intolerance

Wasserman K, Principles of exercise testing, 1999

Weight lossReduction in muscle mass

Steroid myopatyDeconditioning

Chest 2005;127:2028-33

Improvement in muscle function leads to increase in

exercise tolerance

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

Outcome Assessments in Pulmonary Rehabilitation

Outcome Assessments in Pulmonary Rehabilitation

Outcome assessment in pulmonary rehabilitation

Exercise endurance↑

HRQL↑

Hospital addmitions ↓

J Cardiopulm Rehabilitation 2006

Disease specific programs !

PULMONARY REHABILITATION

PULMONARY REHABILITATION

CHEST WALL DISORDERS NEUROMUSCULAR DISEASE

CHEST WALL DISORDERS NEUROMUSCULAR DISEASE

RATIONALS ?RATIONALS ?

Respiratory muscle weakness?

Reduced thoracic wall compliance

Positional mechanical disadvantage

Restrictive pulmonary function impairment

Restrictive pulmonary function impairment

Alveolar hypoventilation + V/Q mismatchAlveolar hypoventilation + V/Q mismatch

HYPOXEMIA + HYPERCAPNIA

HYPOXEMIA + HYPERCAPNIA

Respiratory muscle weakness ?

Reduced thoracic wall compliance

Positional mechanical disadvantage

Restrictive pulmonary function impairment

Restrictive pulmonary function impairment

Alveolar hipoventilation + V/Q mismatchAlveolar hipoventilation + V/Q mismatch

HYPOXEMIA + HYPERCAPNIA

HYPOXEMIA + HYPERCAPNIAReduction in exercise toleranceReduction in exercise tolerance

NIPPVRMT

NIPPVRMT

SYMPTOMSRapid shallow breathingDyspnea

Peripheral muscle weakness

Internal medicine 2006

• VO2 peak ↑ (13.6±2.8, 14.8 ± 2.8)

• 6 MWD↑ (399±62, 467±65)

MRC ADL 6 DYT

6DYT

Chest 2003;123:1988-95

Pulmonary rehabilitation is effective in symptomatic

patients with Post TB

Pulmonary rehabilitation is effective in symptomatic

patients with Post TB

Problems faced

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

Faced Problems

• Reduce work of breathingIncrease tidal volumes and maintain lung

compliance

• Improve secretion clearance and effectiveness of cough• Maximise independence• Optimise quality of life

AIMS PR

NIMV

Breathing exercise

•

Harms et al. J Appl Physiol 1997; 1573-1583

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Time Bianchi L, et al, ERJ, 1998

BORG

•Breathing work rate increase induce reductions in VO2 demand for peripheral muscles•Reduction in work load of respiratory muscles serve for peripheral muscles

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)

Respiratory Muscle TrainingRespiratory Muscle Training

SkoliosisCyphosisCyphoskoliosis

Changes in AirwayResistance (?)

Physiopathology

Changes in muscle length

Changes in Compliance

Increased DemandsNutrients and O2

Nutrients and O2 Delivery

can be compromised

Changes in Chest Wall Changes in Chest Wall

Chest Wall Deformities & RM Training

Few studies

RM training appears to improve - Lung function, dyspnea & exercise toleranceEffects on the disease time-course: unknown

Few studies

RM training appears to improve - Lung function, dyspnea & exercise toleranceEffects on the disease time-course: unknown

RestTraining

Respiratory Muscle TrainingRespiratory Muscle Training

Amyotrophic Lateral SclerosisMultiple SclerosisSpine Cord injuryMiasthenia GravisDuchenne’s Muscular DystrophyPost-polio SyndromeGuillain-Barré SyndromeMyotonic Dystrophy (Steinert’s Disease)

Changes in AirwayResistance (?)

Physiopathology

Inefficiency of Muscles

Changes in Compliance

Increased DemandsNutrients and O2

Nutrients and O2 Delivery

can be compromised

Changes inThorax Geometry

RESPIRATORY MUSCLE RESPIRATORY MUSCLE TRAININGTRAININGRESPIRATORY MUSCLE RESPIRATORY MUSCLE TRAININGTRAINING

Neuromuscular Diseases & RM Training

Few studies • Education, coordination• Insuflation (active and pasive)• RM training: controversial • - Early, Mild disease, Slow evolution• - Balance with rest

RestTraining

RESPIRATORY MUSCLE TRAINING

RESPIRATORY MUSCLE TRAINING

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

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

Patient AssessmentExerciseEducationPsychosocial/Nutritional

interventionOutcomes measurement

Core Components of PR for the “Non-COPD” Patient

Core Components of PR for the “Non-COPD” Patient

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

Special Considerations in Patient Assessment

Patient AssessmentExercise trainingEducationPsychosocial/Nutritional

interventionOutcomes measurement

Core Components of PR for the “Non-COPD” Patient

Core Components of PR for the “Non-COPD” Patient

DISEASE SPECIFIC CONSEDERATOIN IN EXERCISE TRAINING (I)

Interstitial lung disease;

Pacing and energy conservation techniques

Slow and deep breathing Adequete oxygenation

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)

Obesity related respiratory diseases

Strength and endurans exercise training/ Upper and Lower extremity Consider low-impact water-based exerciseBariatric equipment may be needed

Cardiac and musculo skeletal intervention before enrollment

Disease-specific Considerations in Exercise Training

(III)

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

o Test O2 saturation using patients’ own portable system

EXERCISE TRAINING

Patient AssessmentExerciseEducationPsychosocial/Nutritional

interventionOutcomes measurement

Core Components of PR for the “Non-COPD” Patient

Core Components of PR for the “Non-COPD” Patient

EDUCATION: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

DISEASE SPECIFIC PROGRAM MANAGEMENT

DISEASE SPECIFIC PROGRAM MANAGEMENT

Ensure patient safety,individual goals Individual education sessions may be

needed Written and video educational materials

Assess outcomes acording to goalsIndividual, disease specific

approaches is necessaryIndividual, disease specific approaches is necessary

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

AGHH Pulmonary rehabilitation unit documents; 2005- 2007

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