1. Respiratory Care in Neurodisability & Neuromuscular Disease

2. Risk factors
Assessment
Posture
Airway Clearance

3. Result of Impaired Clearance
Increase Airway Resistance.
Increase Work Of Breathing.
Hypoxaemia.
Discomfort.
Repeated Infections.
Atelectasis.
Airway Damage.
Chronic Pathology.
Respiratory Failure.
The effect of poor clearance of mucous can result in the following:

4. Assessment Respiratory assessment Posture Scoliosis
Increased or decreased tone
Swallow
Coordination
Obstruction
Cough – peak cough flow
Secretion management
Pain
Cognitive ability
What are problems and how to solve

5. Musculoskeletal Problems

6. Respiration v posture!

7. Aims of ACT volumes flows Position for
V/Q matching and where air is going
In neurological patients may be easier to breathe in supine

8. Flow and Volume
Children with CP and severe learning difficulties often appear to have diminished sensitivity to cough
May not cough well even during infections
Changing ventilation patterns important in aiding secretion clearance and opening up underventilated lung regions

9. Airway Clearance

10. Volume & Flow
Volume
Flow

11. Airway Clearance Volume Lung Volume – VC  1.5L Bulbar function
Inspiratory muscles – maximum insufflation capacity (MIC) ? More important than VC
Flow
PCF  2.7 l/s (162l/min)to move secs
PCF  6l/s (360L/min)for expectoration
Abdominal/ thoracic expiratory muscles

12. Airway clearance techniques if able Cough assist (also volume)
Breath holds –good bulbar function
Breath stacking – some bf
“frog” breathing- less bf
NIV – 1 breath if no bf
Maximum insufflation Laerdel- 1 breath if no bf
Flow
Manual assisted cough
Airway clearance techniques if able
Cough assist (also volume)

13. Useful tools
Bianchi C, Baiardi P: Cough peak flows: standard values for children and adolescents. Am J Phys Med Rehabil 2008;87:461–467.
The full BTS/ACPRC Guideline is published in Thorax Vol 64 Supplement 1
Available online at:
and at:
British Thoracic Society Reports, Vol 1, No 1, 2009
ISSN 2040

14. www.smn.scot.nhs.uk DUCHENNE MUSCULAR DYSTROPHY SCOTTISH
PHYSIOTHERAPY MANAGEMENT PROFILE
April 2009
Working Party
Marina Di Marco
Lesley Harrison
Anne Keddie
Pierette Melville
Jacky Yirrell

15. Recent Advances in Respiratory Care for Neuromuscular Disease
Anita K. Simonds, MD CHEST / 130 / 6 / DECEMBER, 2006
Developing a breath-stacking system to achieve lung volume recruitment Alison Armstrong British Journal of Nursing, 2009, Vol 18, No 19
Physiologic Benefits of Mechanical Insufflation-Exsufflation in Children With Neuromuscular Diseases Brigitte Fauroux, MD, PhD et al CHEST / 133 / 1 / JANUARY, 2008
Respiratory function assessment and intervention in
neuromuscular disorders Uwe Mellies et al Current Opinion in Neurology 2005, 18:543–547

16. The Respiratory Management of Patients With Duchenne
Muscular Dystrophy: A DMD Care Considerations Working Group Specialty Article David J. Birnkrant, MD et al Pediatric Pulmonology 45:739–748 (2010)
Airway clearance modalities in neuromuscular disease
Jonathan D. Finder Paediatric Respiratory Reviews 11 (2010) 31–34
Predictors of severe chest infections in pediatric neuromuscular disorders C. Dohna-Schwake Neuromuscular Disorders 16 (2006) 325–328
Airway clearance in neuromuscular weakness Leanne Maree Gauld Developmental Medicine and Child Neurology; May 2009; 51, 5

18. Current Consensus Be aware of respiratory function
Airway clearance techniques including augmenting volume & flow
Early respiratory referral
Start NIV early
MIC – breath stacking, frog breathing, Laerdel, NIV etc
Produce effective PCF (manual +/- MI-E)
Manual assist always adds flow
Effective secs clearance + at home (suction, tracheostomy)