1. Respiratory disorders À la RNOH

2. Obstructive airways disease Restrictive lung disease Infections Tumours

3. Restrictive lung disease Reduced Total lung capacity Vital capacity Functional residual capacity Preserved Airways resistance

4. Involvement of Nerve supply Muscles Chest wall Lung parenchyma Polio Guillain Barre Cerebral palsy Spinal cord injury Muscular dystrophies SMA Scoliosis Kyphosis Obesity I.P.F. Connective tissue dis. C.F.

5. Natural history Gradual decrease in VC, FRC Worsening pathology decrease in FRC, Atelectasis  work of breathing Nocturnal hypoventilation Daytime hypoventilation Recurrent chest infections Death

6. Nocturnal hypoventilation  airways resistance  Intercostal tone  Tidal volume  Resp. rate REM sleep Morning headaches Restless sleep Daytime sleepiness Enuresis Concentration/ memory difficulties  schooling problems

7. Restrictive respiratory disorders Symptoms Nothing Dyspnoea on exertion Poor cough Sx Sleep disordered breathing Dyspnoea at rest Muscular dystrophies Cerebral palsy Scoliosis Spinal cord injury Signs Underlying disease Respiratory rate Auscultation…Quiet Retained secretions Cyanosis Clubbing

8. Investigations Bloods. Polycythaemia Blood gas Hypoxia Hypercarbia Chronic respiratory acidosis Early morning sample Chest Xray

11. Lung function tests Sleep studies Oximetry Transcutaneous Co2 & O2

16. Lung function tests Peak cough flow > 270 L/min

17. Restrictive lung disease Two major problems I can’t breath I can’t or won’t cough

19. Management…breathing Ventilate Invasively Non invasively

20. Maintain range of chest wall movement Frog breathing Inspiratory muscle training Upper limb training Management …….Breathing Long term strategies

21. Long term Ventilation When ? Symptomatic Nocturnal CO2 > 10 Kpa 5% study <88% How ? Non invasively Invasively Few hours per night/ all night During day During exacerbations

22. Sputum

23. Sputum Management Assisted coughs Re-Intubation Tracheostomy

24. Emerson Cough assist Device In-Exsufflator

25. Guidelines for use at RNOH