[PPT] - get on top of them !"#$!%&%'#()"*' (*%+,-. PowerPoint Presentation

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Breathing problems and how to get on top of them

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Clinical assessment

REM related sleep disordered breathing FVC < 60% pred NREM and REM sleep disordered breathing FVC < 40% pred Daytime ventilatory failure FVC < 20% pred Chest infections Ineffective cough Cough peak flow <270l/min Inspiratory, expiratory, bulbar muscle weakness Intervention

Physical examination, pulmonary function, cough peak flow, resp muscle strength Chest radiology, sleep study. Swallow function Intervention: cough assistance Non-invasive ventilation, combination with cough assist, PEG/PEJ, T- IPPV

Swallowing dysfunction Consensus Statement J Child Neurol 2007 Wang et al Standardisation of respiratory surveillance

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Lung function tests

Inspiratory vital capacity (IVC) Forced vital capacity (FVC) Lying and upright FVC

Respiratory muscle strength

Sniff nasal inspiratory pressure (SNIP) Maximum inspiratory pressure (MIP) Maximum expiratory pressure (MEP) Peak cough flow (PCF)

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Sleep Studies

Pulse oximetry (SpO2) Transcutaneous carbon dioxide (TcCO2) Polygraphy or NPSG

Daytime arterial blood gas tensions

Transcutaneous PCO2 Capillary gases End-tidal CO2

Subjective symptom score

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Simple easy test to perform can use a mouthpiece or mask No threshold values in children

PCF > 160L/min is sufficient to eliminate airway debris and secretions

Bach & Saporito, 1996, Chest

Normal adult PCF = 360-840L/min

Leiner et al, 1963 Am Rev Respir Dis

If PCF does not exceed 270-300L/min when you are well it can decrease to a critical level

Bach et al., 1997, Chest

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Predictors of severe chest infections in children with NMD

History of severe chest infection

yes N=22 no N=24 p

IVC (L) IVC (% pred) FEV1 (L/s) Peak cough flow (L/min) Peak inspiratory pressure (cmH2O) Peak expiratory pressure (cmH2O) 0.64 0.42 28 13 0.53 0.36 116 62 28 11 24 11 1.44 0.65 47 24 1.21 0.55 211 74 36 21 30 03 <0.00001 <0.005 <0.0001 <0.0005 NS NS

Dohna-Schwake et al. Neuromuscular Dis 2006;16:325

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Symptoms and signs of nocturnal hypoventilation

Poor quality, restless sleep Need for frequent turning Snuffly or laboured breathing Sweaty at night Irritable/tired in morning or morning headaches Poor appetite for breakfast Concentration problems at school, college or work

particularly in the morning

Increased breathlessness Cyanosis on eating and transfers Recurrent chest infections

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Mellies et al,. 2004. Neuromuscular Disorders

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Group 1 Randomised to control No NIV n = 12 Group 2 Randomised to NIV n=14 Daytime normocapnia n = 26 Group 3 Elective NIV n=19 Daytime hypercapnia n = 19 Nocturnal hypoventilation TcCO2 > 6.5 kPa n = 48 Total number of patients screened

Ward, Chatwin, Heather & Simonds Thorax 2005; 60:1019-24

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% Time TcCO2 > 6.5 kPa

Months

6 12 18 24

Percent time TcCO2 > 6.5 kPa:

100
80
60
40
20

20 Group 1 Control Group 2 NIV

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p=0.031 p=0.049

Significant reduction in time TcCO2 > 6.5 kPa and mean Sao2 in NIV group Ward et al Thorax 2005;60:1019-24

Mean nocturnal SaO2

Months

6 12 18 24

SaO2 %

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1 2 3 4 5 Group 1 Control Group 2 NIV p=0.005 p=0.024

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Cargiver Strain Scale - Total Score

Time

Baseline 6 Months 12 Months

Score (0-56)

10 20 30 40 50

Children aged 10 mths to 14 years with SMA Type 1.8-2.4

Chatwin M, Simonds AK ATS 2006

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VAS Results: Comfort - Children, NMD

Cough Physiotherapist NIV Exsufflation In-Exsufflation 1 2 3 4 5 6 7 8 9 10

Intervention

Mean Score (cm)

Most uncomfortable Most comfortable

Chatwin et al ERJ 2003:21:502

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Any questions??

A.Simonds@rbht.nhs.uk

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