PR as a crucial part in NM disorders

1. Pulmonary Rehabilitation
for Neuromuscular
Disorders
By
Riham Hazem Raafat
Ass. Prof. of Chest Diseases
Ainshams University

2. NM
Disorders

4. Pathophysiology of NM RF

5. Respiratory Dysfunction

6. DISEASE
IMPAIRMENT
DISABILITY
HANDICAP
TREATMENT
REHABILITATION
REHABILITATION
Pulmonary Rehabilitation

7. • Evidence-based, multidisciplinary, and comprehensive
intervention for patients with chronic respiratory
diseases 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, reduce health care costs through
stabilizing or reversing systemic manifestations of the
disease, and increase activities & QOL
ATS – ERS Definition

8. Psychologic
al support
Nutritional
advice
Breathing
Retraining
Education
General
exercise
training
Outcome
Assessment

9. Benefits of Pulmonary Rehabilitation
Pulmonary rehabilitation does not reverse nor have any direct effect
on the primary respiratory pathophysiology, yet it has proven to
improve the following:

10.  Control of symptoms of cough and fatigue:
 Real time eval.: MRC breathlessness & Borg dyspnea scale
 Recall of symptoms
 Performance evaluation: Ability to do ADL
 Directly observed or self reported + PFTs, ABG or Oximetry
 Exercise tolerance:
 6 minutes walking test
 Cardiopulmonary exercise testing
 Quality of life: (specific or non-specific)
 Chronic respiratory disease questionnaire & SGRQs
 SF- 36
 Assessment of respiratory and peripheral muscle strength
 Nutritional assessment
 History, Laboratory, Anthropometric, Calorimetry (D&ID)
Baseline Evaluation

11. Evaluation of Respiratory Muscle Affection
1. Confirm respiratory muscle weakness (respiratory
muscle assessment): S&S, U/S, PFT, Pimax, Pemax, EMG
2. Assess cough (PeMax < 60 cm H2O)
3. Assess need for ventilatory support (20-30-40 rule)
Degree of respiratory muscle weakness cannot be predicted
by the degree of peripheral muscle weakness.
--> tests of respiratory muscle function are essential to
identify the patient who is at risk for respiratory failure.

14. Need for Ventilatory Support is warranted if
measured values fall below the “20/30/40 rule” :
- VC falls below 20 mL/kg,
- MIP above −30 cm H2O, or
- MEP below 40 cm H2O.

15. Begin therapies early, when you
notice the first signs of a weakened cough.
DO NOT WAIT until the cough
is noticeably weak (or non-existent).

16. •Breathing Strategies
•Normal Lung Function and
Pathophysiology of Lung Disease
•Proper Use of Medications, including Oxygen
•Bronchial Hygiene Techniques
•Benefits of Exercise and Maintaining Physical Activities
•Energy Conservation & Work Simplification Techniques
•Eating Right
1- Education

17. • Irritant Avoidance, including Smoking Cessation
• Prevention and Early Treatment of Respiratory
Exacerbations
• Indications for Calling the Health Care Provider
• Coping with Disease and End-of-Life Planning
• Anxiety and Panic Control, including Relaxation
Techniques and Stress Management

18. Components of exercise training:
• Lower extremity exercises
• Arm exercises
• Ventilatory muscle training
Types of exercise:
• Endurance or aerobic
• Strength or resistance
2- Exercise training

19. Designing exercise programs Considerations:
1.Individual differences
2.Specificity
3.Overload
4.Progression
5.Reversibility

20. • Walking
• Treadmill
• Stationary bicycle
• Stair climbing
• Sit & Stand
• Water based exercises
Lower extremity exercise

21. • Arm cycle ergometer
• Unsupported arm lifting
• Lifting weights
Upper extremity exercise

22. Strengthening exercises:
When strength exercise was added to standard exercise
protocol; led to:
greater increase in muscle strength and muscle mass
(FFM)  increased mid-thigh circumference

23. 30 breaths twice daily
Intensity: 50% of Pimax
Increase 5% load/wk
Extent: for 8 wks
Ventilatory muscle training

25. Bronchial Hygiene Techniques (cough assistance)

26. Cough Assist/ MIE

28. • Pursed Lip Breathing – shifts breathing pattern and
inhibits dynamic airway collapse. (2s inh, 4s exh)
• Posture techniques – forward leaning reduces
respiratory effort, elevating depressed diaphragm by
shifting abdominal contents.
• Diaphragm Breathing – Some patients with extreme
air trapping and hyperinflation have increased WOB
with this technique
• Postural Draining – valuable in patients who produce
more than 30cc/24 hours - Coughing techniques
Chest Physical Therapy & Breathing Retraining

29. Acapella

30. Vibratory PEP

31. Pursed Lip Diaphragmatic
Breathing

32. Why intervene?
•High prevalence and association with morbidity and
mortality
•Higher caloric requirements from exercise training in
pulmonary rehabilitation, which may further aggravate
these abnormalities (without supplementation)
•Enhanced benefits, which will result from structured
exercise training.
Add?
- Thicker fluids and Good hydration
- Puréed or less solid food
For those having bulbar affection and risk of aspiration
3- Nutritional Interventions

33. Should be considered if :
•BMI less than 21 kg/m2
•Involuntary weight loss of >10% during the last 6 months
or more than 5% in the past month (can’t depend in edema)
•Depletion in FFM or lean body mass (make QoL worse and
less tolerant to exercise even when normal weight)
Nutritional Supplementation

34. INTERVENTION WEIGHT
GAIN
FFM GAIN EXERCISE
CAPACITY
CALORIC SUPP. + - -
CALORIC
SUPPLEMENTATION +
EXERCISE TRAINING
++ + +
STRENGTH EXERCISE - + -

35. • Screening for anxiety and depression should be part of
the initial assessment.
• Mild or moderate levels of anxiety or depression
related to the disease process may improve with
pulmonary rehabilitation
• Patients with significant psychiatric disease should be
referred for appropriate professional care (ATS/ERS
STATEMENT)
4- Psychological considerations

36. 5- Outcome Assessment
Providing patients with an opportunity to give
feedback about the program is a useful measure
of quality control.
Patient feedback also allows coordinators to
evaluate the components of pulmonary
rehabilitation that patients find most useful.
The questionnaire should also provide patients
with a variety of answering options
Exercise capacity measurement

37.  Control of symptoms of cough and fatigue:
 Real time eval.: MRC breathlessness & Borg dyspnea scale
 Recall of symptoms
 Performance evaluation: Ability to do ADL
 Directly observed or self reported
 Exercise tolerance:
 6 minute walking test
 Cardiopulmonary exercise testing
 Quality of life:
 Chronic respiratory disease questionnaire
 St Georges’s respiratory questionnaire
 SF- 36
 Assessment of respiratory and peripheral muscle strength
 Nutritional assessment
Outcome Evaluation

38. • Current guidelines does not comment on maintenance & repeat
rehabilitation
• Yearly repeat rehabilitation program had shown: Short term
benefits in the form of less frequent exacerbations
• But no long term physiological effects on exercise tolerance,
dyspnea & HRQL but in 6Ms begin loss of benefits
6- Maintenance rehabilitation &
Repeat rehabilitation program

40. For people with neuromuscular disorders
who are experiencing respiratory difficulty, however,
oxygen therapy must be used with great caution.
Mechanical ventilation (preferably non-invasive)
should be the initial therapy for people with
neuromuscular disorders who experience
hypoxemia.

41. NPPV will be unsuccessful when any of the
following exist:
1.Bulbar dysfunction
2.Upper airway obstruction
3.Retention of respiratory secretions
4.Poor cooperation
5.Inadequate cough.
Mechanical Ventilation

42. Invasive MV
Should be initiated early for the following reasons:
•Intubation itself has risks that are best managed in a
controlled setting dysautonomia increases the risk of
severe hypotension and bradycardia .
•decrease the risk of early-onset pneumonia.

43. Predictors of successful Extubation
• (MIP) more negative than -50 cm H2O
• Improvement VC by > 4 mL/kg from
preintubation to preextubation.
Predictors of failed extubation
• Dysautonomia
• Advanced age
• Pulmonary complications
(pneumonia, atelectasis) were
associated with extubation failure

44. Tracheostomy
1. Difficulty clearing their secretions.
2. Intermittent long-term mechanical ventilation, but in
whom NPPV is contraindicated (e.g, severe bulbar
dysfunction).
3. Intermittent long-term NPPV is no longer sufficient.
4. Fail to wean from invasive MV

45. General Care
•Correct electrolyte abnormalities
•Avoid neuromuscular blocking agents, aminoglycosides,
and glucocorticoids.
•Nutritional status should be maintained
•Prophylaxis for DVT and stress gastric ulcers should be
provided when indicated.
•The head of the bed should be elevated by 45º to
decrease the risk for ventilator-associated pneumonia .

46. Decisions regarding long-term support
Progressive neuromuscular diseases of childhood, such
as spinal muscular atrophy and Duchenne muscular
dystrophy present different challenges, as parents are
often the main decision makers.
[long-term ventilatory support is more common and
often associated with good quality of life].

47. Other Ways of Care
- Treat respiratory infections as early as possible
- Guide against aspiration
- Prevent GERD
- Well control of co-morbid diseases
- Diaphragmatic pacing in severe weakness