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Standardisation of respiratory surveillance Intervention Clinical assessment Physical examination, pulmonary function, cough peak flow, resp muscle Inspiratory, expiratory, bulbar muscle weakness strength Chest radiology, sleep study. Ineffective cough REM related sleep Swallow function disordered breathing Intervention: cough Cough peak assistance flow <270l/min FVC < 60% pred Swallowing dysfunction NREM and REM sleep Non-invasive Chest infections disordered breathing ventilation, combination FVC < 40% pred with cough assist, PEG/PEJ, T- Daytime ventilatory failure IPPV FVC < 20% pred Consensus Statement J Child Neurol 2007 Wang et al

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G*164.#&>%H*$746'%F'#)*&')%I � 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)

G*164.#&>%H*$746'%F'#)*&')%J � 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

F'#)*&'6'.$)% � G'#8%74*/"%<140%@GB;A Simple easy test to perform can use a mouthpiece or mask No threshold values in children 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 PCF > 160L/min is sufficient to eliminate airway debris and secretions Bach & Saporito, 1996, Chest

Predictors of severe chest infections in children with NMD History of severe yes no p N=22 N=24 chest infection IVC (L) 0.64 � 0.42 1.44 � 0.65 <0.00001 IVC (% pred) 28 � 13 47 � 24 <0.005 FEV1 (L/s) 0.53 � 0.36 1.21 � 0.55 <0.0001 Peak cough flow (L/min) 116 � 62 211 � 74 <0.0005 Peak inspiratory pressure (cmH2O) 28 � 11 36 � 21 NS Peak expiratory pressure (cmH2O) 24 � 11 30 � 03 NS Dohna-Schwake et al. Neuromuscular Dis 2006;16:325

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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=#.946()'9%74.$&411'9%$&(#1%4<%LRS%(.%.47$*&.#1% ">-43'.$(1#$(4.%(.%74./'.($#1%.'*&46*)7*14E)8'1'$#1% 9()'#)'W%$&(#1%9')(/. n = 48 Total number of patients screened Nocturnal hypoventilation TcCO2 > 6.5 kPa Daytime normocapnia Daytime hypercapnia n = 26 n = 19 Group 1 Group 2 Group 3 Randomised to control Randomised to NIV Elective NIV No NIV n=19 n = 12 n=14 Ward, Chatwin, Heather & Simonds Thorax 2005; 60:1019-24

F'#.%7"#./')%<&46%5#)'1(.'%(.%.47$*&.#1% GBHJ%#.9%?#4J%(.%74.$&41%#.9%LRS%/&4*-) % Time TcCO2 > 6.5 kPa Mean nocturnal SaO2 20 5 4 0 Percent time TcCO2 > 6.5 kPa: 3 -20 2 SaO2 % -40 ! 1 -60 0 -1 -80 p=0.031 p=0.049 -2 -100 p=0.005 p=0.024 0 6 12 18 24 -3 Months 0 6 12 18 24 Group 1 Control Months Group 2 NIV Group 1 Control Group 2 NIV Significant reduction in time TcCO2 > 6.5 kPa and mean Sao2 in NIV group Ward et al Thorax 2005;60:1019-24

G&'3'.$#$(3'%*)'%4<%"46'%LRS%$4%&'9*7'%"4)-($#1%#96())(4.)%(.%7"(19&'.% 0($"%N>-'%R%UJ%?FV%W%% !"#$%&'$#()*#"&+(),-#$) Cargiver Strain Scale - Total Score 50 40 Score (0-56) 30 20 Chatwin M, Simonds AK 10 ATS 2006 0 Baseline 6 Months 12 Months Time Children aged 10 mths to 14 years with SMA Type 1.8-2.4

N41'&#.7'%4<%6'7"#.(7#1%(.E'C)*<<1#$(4. Most VAS Results: Comfort - Children, NMD uncomfortable 10 9 Mean Score (cm ) 8 7 6 5 4 3 2 1 0 Most Cough Physiotherapist NIV Exsufflation In-Exsufflation comfortable Chatwin et al ERJ 2003:21:502 Intervention

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Any questions?? A.Simonds@rbht.nhs.uk