Respiratory disorders À la RNOH

Obstructive airways disease Restrictive lung disease Infections Tumours

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

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.

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

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

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

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

Lung function tests Sleep studies Oximetry Transcutaneous Co2 & O2

Lung function tests Peak cough flow > 270 L/min

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

Management…breathing Ventilate Invasively Non invasively

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

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

Sputum

Sputum Management Assisted coughs Re-Intubation Tracheostomy

Emerson Cough assist Device In-Exsufflator

Guidelines for use at RNOH