Respiratory Care in Neurodisability & Neuromuscular Disease

1. Respiratory Care in Neurodisability & Neuromuscular Disease

2. Risk factors • Assessment • Posture • Airway Clearance

3. Increase Airway Resistance. Increase Work Of Breathing. Hypoxaemia. Discomfort. Repeated Infections. Atelectasis. Airway Damage. Chronic Pathology. Respiratory Failure. Result of Impaired Clearance

4. 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 Assessment

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. 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: http://thorax.bmj.com/content/vol64/issueSupplI and at: http://www.brit-thoracic.org.uk/physioguide British Thoracic Society Reports, Vol 1, No 1, 2009 ISSN 2040

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

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. 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) Current Consensus