Thorax. Guidelines for home oxygen in children. thorax.bmj.com. British Thoracic Society Home Oxygen Guideline Development Group (Paediatric Section)

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1 thorx64_s2over.qxd 7/7/ :53 PM Page 1 August 2009 Vol 64 Supplement II Thorax AN INTERNATIONAL JOURNAL OF RESPIRATORY MEDICINE Guidelines for home oxygen in hildren British Thorai Soiety Home Oxygen Guideline Development Group (Paediatri Setion) thorax.bmj.om

2 Contents Volume 64 Issue Suppl II THORAXAugust 2009 BTS guidelines for home oxygen in hildren Journal of the British Thorai Soiety Impat Fator: Editor-in-Chief J A Wedziha (UK) Editor S L Johnston (UK) Assoiate Editors J S Brown (UK) J R Hurst (UK) P M A Calverley (UK) D A Lomas (UK) M Dusmet (UK) D M Mannino (USA) J S Elborn (N Ireland) F D Martinez (USA) A J Fisher (UK) C Robertson (Australia) J M FitzGerald (Canada) B Shonhofer (Germany) J A Fleetham (Canada) N M Foley (UK) I Hall (UK) R Hubbard (UK) G A Silvestri (USA) G I Town (New Zealand) M K B Whyte (UK) Statistial Editors R Newson (UK) T M MKeever (UK) L Tata (UK) Images Editors J M FitzGerald (Canada) J R Mayo (Canada) J C Hogg (Canada) Letters Editor J R Hurst (UK) Lung Alert Editors A Bhowmik (UK) J Quint (UK) President, British Thorai Soiety P Ormerod Editorial Offie BMJ Publishing Group Ltd, BMA House, Tavistok Square, London WC1H 9JR, UK T: +44 (0) F: +44 (0) E: thorax@bmjgroup.om ISSN: (print) ISSN: (online) Dislaimer: Thorax is owned and published by the British Thorai Soiety and BMJ Publishing Group Ltd, a wholly owned subsidiary ofthe British Medial Assoiation. The owners grant editorial freedom to the Editor of Thorax. Thorax follows guidelines on editorial independene produed by theworld Assoiation ofmedialeditors and the ode on good publiation pratie of the Committee on Publiation Ethis. Thorax is intended for medial professionals and is provided without warranty, express or implied. Statements in thejournalare theresponsibility of their authors and advertisers and not authors institutions, the BMJ Publishing Group Ltd, the British Thorai Soiety or the BMA unless otherwise speified or determined by law. Aeptane of advertising does not imply endorsement. To the fullest extent permitted by law, the BMJ Publishing Group Ltd shall not be liable for any loss, injury or damage resulting from the use of Thorax or any information in it whether basedon ontrat, tort or otherwise. Readers are advised to verify any information they hoose to rely on. Copyright: E 2009 BMJPublishingGroupLtdandthe British Thorai Soiety. All rights reserved; no part of this publiation may be reprodued, stored in a retrieval system or transmitted in any form or by any means, eletroni, mehanial, photoopying, reording or otherwise without the prior permission of Thorax. Thorax is published by BMJ Publishing Group Ltd, typeset by The Charlesworth Group and printed in the UK on aid-free paper by Latimer Trend & Co Ltd, Plymouth. Thorax (USPS No: ) is published monthly by BMJ Publishing Group and distributed in the USA by Pitney Bowes International Mailing Servies In as mailingagent. Periodials postage paid at Kearny, NJ, USA and additional mailing offies. POSTMASTER: send address hanges to Thorax, PB International Mailing Servies In, 500 US Hwy 46, Clifton, NJ 07011, USA. i Endorsement ii1 1. Introdution 1.1 Aims and target audiene 1.2 Methodology for generation of the guidelines 1.3 Conflit of interest 1.4 Aknowledgements SUMMARY OF BACKGROUND FACTS SUMMARY OF RECOMMENDATIONS ii4 ii9 2. Bakground 2.1 Definitions 2.2 What differenes are there between adult and paediatri pratie? 2.3 What is urrent UK pratie in presribing home oxygen? 2.4 What is the normal oxygen saturation in a healthy infant aged,1 year and a healthy hild aged >1 year? Methodologial issues Healthy infants aged,1 year Healthy hildren aged >1 year 2.5 What are the onsequenes of hroni low oxygen saturation in hildren? Pulmonary arterial hypertension Neurodevelopment Apnoeas/apparent life-threatening events/sudden unexplained death in infany Growth Sleep 2.6 What are the onsequenes of exess oxygen therapy in hildren? 3. Indiations for LTOT 3.1 Chroni neonatal lung disease Survival Symptoms Growth Neurodevelopment Hospitalisation rates Quality of life and psyhologial impat Evidene that home oxygen is preferable to hospital-based oxygen 3.2 Other oxygen-dependent neonatal lung onditions 3.3 Congenital heart disease Cyanoti ongenital heart disease Ayanoti ongenital heart disease 3.4 Pulmonary hypertension Idiopathi pulmonary hypertension Seondary to ongenital heart disease Seondary to pulmonary disease 3.5 Non-ardia intrapulmonary right to left shunt 3.6 Children with reurrent yanoti-apnoei episodes 3.7 Interstitial lung disease 3.8 Obliterative bronhiolitis 3.9 Cysti fibrosis and non-cf bronhietasis 3.10 Obstrutive sleep apnoea syndrome 3.11 Chroni hypoventilation 3.12 Sikle ell disease 3.13 Palliative are/end of life are ii13 4. Speial situations 4.1 Intermittent LTOT Neurodisability Other situations 4.2 Intermittent emergeny oxygen therapy Reurrent life-threatening asthma Epilepsy and status epileptius 4.3 Misellaneous Aute viral bronhiolitis ii15 5. Assessment of need for LTOT 5.1 Assessing gas exhange 5.2 What is the target SpO 2 level to avoid the adverse onsequenes of hypoxaemia? 5.3 Should the right heart be assessed? ii16 6. Ordering and provision of oxygen (equipment) 6.1 Oxygen supply 6.2 Whih delivery system is the best for hildren?

3 Contents Volume 64 Issue Suppl II THORAX August Conentrator vs ylinder Standard vs light weight ylinders for portable use Liquid oxygen Low flow vs very low flow meters Should a onserver devie be used? Is humidifiation neessary? Fae mask vs nasal annulae 6.3 Should parents have a home saturation monitor? ii19 7. Disharge planning 7.1 Disharge riteria Infants with CNLD Older hildren 7.2 Eduating the families ii20 8. Follow-up after disharge 8.1 What follow-up is required? 8.2 Withdrawal of supplemental oxygen ii22 9. Oxygen outside the home 9.1 Shool/nursery 9.2 Travel Cars Holidays Air flight ii Potential disadvantages 10.1 Safety issues 10.2 Psyhosoial issues ii Audit points for loal pratie ii Appendies (online only) Appendix 1: Searh strategy Appendix 2: Cheklist of topis for eduating those involved with the are of the hild ii Authors affiliations ii Abbreviations ii Referenes

4 for home oxygen in hildren BTS guidelines I M Balfour-Lynn, D J Field, P Gringras, B Hiks, E Jardine, R C Jones, A G Magee, R A Primhak, M P Samuels, N J Shaw, S Stevens, C Sullivan, J A Taylor, C Wallis, on behalf of the Paediatri Setion of the Home Oxygen Guideline Development Group of the BTS Standards of Care Committee Appendies 1 and 2 are published online only at thorax.bmj.om/ontent/vol64/ issuesupplii Correspondene to: Dr I M Balfour-Lynn, Department of Paediatri Respiratory Mediine, Royal Brompton & Harefield NHS Trust, Sydney Street, London SW3 6NP, UK; i.balfourlynn@i.a.uk This guideline has been endorsed by the Royal College of Paediatris & Child Health. Reeived 3 Marh 2009 Aepted 8 April 2009 as we know, there are known knowns; there are things we know we know. We also know there are known unknowns; that is to say we know there are some things we do not know. But there are also unknown unknowns the ones we don t know we don t know. D Rumsfeld, INTRODUCTION 1.1 Aims and target audiene The aims of these guidelines are to present the evidene base for the pratie of administering supplemental oxygen to hildren outside hospital and to make reommendations for best pratie. For many aspets high-quality evidene is laking, and suggestions are made based on linial experiene. It is hoped the guideline will highlight areas where researh is needed to further inform liniians. The target audiene is liniians who presribe home oxygen for hildren, prinipally those in hospital pratie. It is also intended for other professionals involved with the whole proess, whih may inlude ommunity paediatriians, paediatri neurodisability speialists, nurse speialists, shool nurses, oupational therapists and physiotherapists; this is refleted by the multidisiplinary nature of the guideline ommittee (setion 13). 1.2 Methodology for generation of the guidelines The initial literature searh was arried out by the Centre for Reviews and Dissemination at the University of York. Further searhes were then arried out by members of the working group who onentrated on their own topis. Details of the searh strategy are given in Appendix 1 available online. Eah setion of the guideline was researhed and drafted by a subgroup of the Paediatri Setion of the British Thorai Soiety (BTS) Home Oxygen Guideline Development Group (itself a subommittee of the BTS Standards of Care Committee). Publiations were rated aording to the SIGN 50 riteria for the alibre of the methodology of the researh to give levels of evidene (see box 1). One all parts were merged into one doument, the whole group then met to disuss the first draft before redrafting took plae. This draft was based, where possible, on the published evidene, but this was then ombined with linial expertise as required. The resulting draft is therefore a blend of published evidene and linial experiene. This was sent to a group of speialist reviewers listed in the Aknowledgements. The manusript was then amended in the light of their omments and the doument was reviewed by the BTS Standards of Care Committee and the Quality of Pratie Committee of the Royal College of Paediatris and Child Health. After a further redrafting and final approval from the BTS Standards of Care Committee, the guidelines were submitted for publiation. Bakground fats are shown in the text in italis. Reommendations are shown in bold and plaed above the text aompanied by the grade for that reommendation. 1.3 Conflit of interest All the members of the Guideline Committee submitted a written reord of possible onflits of interest to the Standards of Care Committee of the BTS. IMB-L, BH, RAP and NJS are involved with the Children s Home Oxygen Reord Database (CHORD) whih has reeived funding from British Lung Foundation and Carburos Metallios, the researh arm of Air Produts (based in Spain). These are available for inspetion on request from the Chairman of this Committee. 1.4 Aknowledgements Funding for the literature searh and travel to the guideline meeting was kindly provided by the British Thorai Soiety. The authors thank Lisa Stirk at the Centre for Reviews and Dissemination at the University of York for the literature searh; Professor Fenella Kirkham, Paediatri Neurologist at the Institute of Child Health, London for advie on sikle ell disease; Dr Gerry Coghlan, Consultant Cardiologist at the Royal Free Hospital, London for his omments on pulmonary hypertension; and Dr Renee MCulloh, Consultant in Palliative Care at Great Ormond Street Hospital, London for omments on palliative are. The following ated as speialist reviewers: Clinial Assoiate Professor Domini Fitzgerald, Paediatri Respiratory Mediine, Children s Hospital, Westmead, Sydney; Professor Sheila G Haworth, Professor of Developmental Cardiology, Institute of Child Health, London; Dr Jeremy Hull, Consultant in Paediatri Respiratory Mediine, Oxford Children s Hospital; Professor Neena Modi, Professor of Neonatal Mediine, Imperial College London and Honorary Consultant, Chelsea and Westminster NHS Foundation Trust; Dr Win Tin, Consultant Paediatriian and Neonatologist, James Cook University Hospital, Middlesbrough; Dr Jane Williams, Nottingham University College Hospital NHS Trust on behalf of the British ii1

5 Box 1 Revised SIGN grading systems for grades of reommendation and levels of evidene (Annex B of SIGN 50 available at Levels of evidene 1++ High quality meta-analyses, systemati reviews of randomised ontrolled trials (RCTs) or RCTs with a very low risk of bias 1+ Well-onduted meta-analyses, systemati reviews or RCTs with a low risk of bias 12 Meta-analyses, systemati reviews or RCTs with a high risk of bias 2++ High quality systemati reviews of ase-ontrol or ohort studies, or high quality ase-ontrol or ohort studies with a very low risk of onfounding or bias and a high probability that the relationship is ausal 2+ Well-onduted ase-ontrol or ohort studies with a low risk of onfounding or bias and a moderate probability that the relationship is ausal 22 Case-ontrol or ohort studies with a high risk of onfounding or bias and a signifiant risk that the relationship is not ausal 3 Non-analytial studies (eg, ase reports, ase series) 4 Expert opinion Grades of reommendations A At least one meta-analysis, systemati review or RCT rated as 1++ and diretly appliable to the target population; or a body of evidene onsisting prinipally of studies rated as 1+, diretly appliable to the target population and demonstrating overall onsisteny of results B A body of evidene inluding studies rated as 2++, diretly appliable to the target population and demonstrating overall onsisteny of results; or extrapolated evidene from studies rated as 1++ or 1+ C A body of evidene inluding studies rated as 2+, diretly appliable to the target population and demonstrating overall onsisteny of results; or extrapolated evidene from studies rated as 2++ D Evidene level 3 or 4; or extrapolated evidene from studies rated as 2+ Good pratie points 3 Reommended best pratie based on the linial experiene of the Guideline Development Group. Aademy of Childhood Disability. The authors also thank the Quality of Pratie Committee of the Royal College of Paediatris and Child Health for reviewing the guidelines. SUMMARY OF BACKGROUND FACTS Normal oxygen saturations (Setion 2.4) 1. Oximeters from different manufaturers may give different oxygen saturation readings depending on whether frational or funtional oxygen saturation is being measured. 2. The median baseline saturation in healthy term infants during the first year of life is 97 98%. 3. In only 5% of healthy infants is the arterial oxygen saturation measured by pulse oximetry (SpO 2 ),90% for.4% of the time. 4. The median baseline SpO 2 in healthy hildren >1 year old is 98% with a 5th entile of 96 97%. ii2 5. A healthy hild aged 5 11 years spends no more than 5% of the time below a SpO 2 of 94% while asleep. Consequenes of hroni low oxygen saturation (Setion 2.5) 1. Hypoxaemia auses pulmonary hypertension but the preise severity and duration of hypoxaemia needed to do this are not known. The fators affeting individual suseptibility are also unknown. 2. SpO 2 levels.94 95% appear to redue pulmonary hypertension, while levels,88 90% may ause pulmonary hypertension. This does not apply to hildren with ongenital ardia defets and idiopathi pulmonary arterial hypertension. 3. Hypoxia may have adverse effets on ognition and behaviour at SpO 2 levels of (85%, but the effets of milder hypoxia are less lear. 4. In infants with hroni neonatal lung disease (CNLD), SpO 2,90% is assoiated with an inreased risk of apparent life-threatening events while SpO 2 >93% is not. 5. In infants with CNLD, SpO 2,92% may be assoiated with suboptimal growth. 6. In infants with CNLD, SpO 2 (90% impairs sleep quality but SpO 2.93% does not. SUMMARY OF RECOMMENDATIONS Consequenes of exess oxygen therapy (Setion 2.6) 1. Exess arterial and intra-alveolar oxygen onentrations are toxi in preterm infants and must be avoided by appropriate monitoring and adhering to the target SpO 2 level; there are no data in older hildren. [D] Indiations for long-term oxygen therapy (LTOT) (Setion 3) Chroni neonatal lung disease (Setion 3.1) (fig 1) 1. Supplementary oxygen should be given to infants with hroni neonatal lung disease: a. to redue or prevent pulmonary hypertension, redue intermittent desaturations, redue airway resistane and promote growth; [C] b. as it is likely to be benefiial for neurodevelopment in infants with CNLD; [D]. as it may redue the assoiated risk of sudden unexplained death in infany; [D] d. as oxygen at home is preferable to a prolonged hospital stay for both quality of life and psyhologial impat for the infant, parents and family; [D] e. as it saves days in hospital due to earlier disharge despite a signifiant readmission rate. [C] Other neonatal lung onditions (Setion 3.2) 1. Home LTOT should be offered to infants with other oxygen-dependent neonatal lung onditions who are otherwise ready for hospital disharge. [3] Congenital heart disease (Setion 3.3) 1. Home oxygen should not be used for yanoti ongenital heart disease unless aompanied by other respiratory problems. [3] 2. In ayanoti heart disease there is no role for LTOT. [3] Pulmonary hypertension (Setion 3.4) 1. In idiopathi pulmonary hypertension, supplementary oxygen is reommended for sleep-assoiated desaturations and for emergeny use. [D]

6 2. In pulmonary hypertension assoiated with ongenital ardia defets, some hildren may gain symptomati benefit and a small open study has suggested it may improve survival. However there is a lak of good evidene that LTOT is of benefit and it is not reommended. [D] 3. LTOT is reommended for pulmonary hypertension seondary to pulmonary disease. [D] Intrapulmonary shunting (Setion 3.5) 1. The benefits of LTOT in non-ardia intrapulmonary shunting are unknown with no relevant publiations; however, it should be onsidered if it leads to symptomati improvement. [3] Reurrent yanoti-apnoei episodes (Setion 3.6) 1. LTOT should be onsidered for infants and hildren who have reurrent yanoti-apnoei episodes severe enough to require ardiopulmonary resusitation, assuming any anaemia has been orreted. [D] Interstitial lung disease (Setion 3.7) 1. LTOT should be offered to hypoxi hildren with interstitial lung disease who are otherwise ready for hospital disharge. [3] Obliterative bronhiolitis (Setion 3.8) 1. LTOT should be offered to hypoxi hildren with obliterative bronhiolitis who are otherwise ready for hospital disharge. [3] Palliative are (Setion 3.13) 1. LTOT should be onsidered for hypoxaemi hildren undergoing palliative are who obtain symptomati relief from supplemental oxygen. [3] Speial situations (Setion 4) Intermittent LTOT (Setion 4.1) 1. In hildren with neurodisability, oxygen may be given in the presene of hypoxia seondary to an aute lower respiratory trat infetion. Children will usually be hospitalised but, where families opt for home treatment, failities for home oxygen may be required if the infetions are reurrent. [3] 2. The use of home oxygen in hildren with severe neurodisability and low SpO 2 should be driven by quality of life issues rather than oxygen saturation targets. [3] Intermittent emergeny oxygen therapy (Setion 4.2) 1. Although most hildren with asthma should reeive bronhodilators via a spaer devie, for those using a home nebuliser, unless there is a signifiant o-morbidity or the hild has life-threatening aute exaerbations, it should be run off room air. [3] 2. Intermittent aute oxygen therapy at home should be onsidered for the few hildren with reurrent episodes of severe life-threatening asthma, as a temporary therapy prior to ambulane transfer to hospital. [3] 3. Intermittent aute oxygen therapy at home is not routinely reommended for seizures as there is no evidene that it redues their duration, redues harm from prolonged seizures or improves quality of life for the hild or family. [3] Cysti fibrosis (Setion 3.9) 1. LTOT should be onsidered for hypoxi hildren with ysti fibrosis as a means to improve shool attendane [B], and for those who obtain symptomati relief. [D] 2. In ysti fibrosis, monitoring of CO 2 levels should be arried out when oxygen therapy is initiated. [C] Obstrutive sleep apnoea (Setion 3.10) 1. In obstrutive sleep apnoea, ontinuous positive airway pressure (CPAP) or oasionally non-invasive ventilation (NIV) is the therapy of hoie if the upper airway obstrution annot be relieved surgially. If this is not possible, LTOT should be used to improve the SpO 2,butCO 2 levels need to be monitored at initiation of treatment. [C] Chroni hypoventilation (Setion 3.11) 1. LTOT should be given in addition to ventilatory support if there is a hypoxaemi omponent of hypoventilation (assuming the hild is optimally ventilated). On oasions when ventilatory support is not possible, supplemental oxygen may be the only alternative. [3] Sikle ell disease (Setion 3.12) 1. LTOT should be onsidered for hildren with sikle ell disease and persistent noturnal hypoxia to redue the risk of stroke and painful rises. [C] Misellaneous situations (Setion 4.3) 1. Infants with bronhiolitis requiring oxygen (SpO 2 (92%) should be admitted to hospital and an be onsidered for disharge when their SpO 2 is.94% and they no longer require oxygen (for at least 8 12 h). [3] Assessment of need for LTOT and target oxygen saturations (Setion 5) 1. Suitability for home oxygen therapy should be assessed by a speialist with appropriate experiene. [3] 2. Pulse oximetry should be used for assessing hildren rather than arterial blood sampling. [C] 3. Children should be assessed for at least 6 12 h and during all levels of ativity, inluding sleep and feeding. [D] 4. Lower limit target SpO 2 should be met for at least 95% of the stable reording period. [3] 5. There is no need to regularly assess CO 2 levels in infants with CNLD who are at home [3], but it may be useful in some neonates with other onditions [3] and older hildren [C], espeially when initiating home oxygen therapy. 6. In CNLD, oxygen therapy should be given to maintain an SpO 2 of >93%. [C] 7. There are no data to guide target levels for SpO 2 in hildren with other respiratory onditions, but the reommendation is to maintain SpO 2 at >93%, although >94% may be appropriate for sikle ell disease and >90% for ysti fibrosis. [3] ii3

7 8. In infants with CNLD, prior to disharge an ECG or ehoardiogram is useful to assess the right heart in order to exlude signifiant pulmonary hypertension. [3] Ordering and provision of oxygen (equipment) (Setion 6) 1. The deision that a hild requires home oxygen and its ordering should be undertaken by paediatri speialists rather than primary are. [3] 2. Oxygen onentrators should be provided for LTOT, unless it is likely that the hild will only require low flow oxygen for a short while. [3] 3. While low weight ylinders are easier to handle, they empty more quikly. Parent hoie should be onsidered. [3] 4. Portable equipment should be available for all hildren as part of the provision of home oxygen unless oxygen is only required at night. [3] 5. Continuously delivered liquid oxygen annot be used at flows of,0.25 l/min, although breath-ativated systems an allow lower flows. It has limited appliations for hildren, so is generally not reommended. [3] 6. Low flow meters are preferable, so very low flow meters are not reommended. [3] 7. Oxygen onservers are not indiated for young hildren but an be onsidered for older hildren apable of triggering the devie. [3] 8. Humidifiation should be onsidered for high oxygen flows when given by fae mask, espeially for ysti fibrosis; a old water bubble-through humidifier may be adequate for this purpose. [3] 9. When oxygen is given via a traheostomy, heated humidifiation is generally reommended; a heat-moisture exhanger with an oxygen attahment may be an adequate alternative. [3] 10. Nasal annulae are preferable for infants and young hildren for flows of (2 l/min. Patient hoie should be onsidered for older hildren. [3] 11. There is no evidene on whether the routine use of a saturation monitor at home is of benefit or harm, and it annot be reommended. Nevertheless, some liniians and parents may find it helpful in ertain irumstanes. [3] Disharge planning (Setion 7) 1. A omprehensive written parent-held disharge plan with multidisiplinary follow-up is reommended to ensure a safe and smooth transition into the ommunity and to avoid repeated or unneessary hospitalisations. [3] 2. Children an be disharged from the neonatal unit when their oxygen requirement is stable with a mean SpO 2 of >93% and without frequent episodes of desaturation. This usually orresponds with an oxygen flow (0.5 l/min. [D] 3. The SpO 2 should not fall below 90% for more than 5% of the artefat-free reording period. [3] 4. There should be no other linial onditions preluding disharge and the hild must be medially stable. [3] 5. Careful preparation with a strutured eduational programme should be implemented. [D] Follow-up after disharge (Setion 8.1) 1. The ommunity hildren s nurse or nurse speialist should visit the hild within 24 h of disharge. [D] 2. Infants with CNLD should have their SpO 2 monitored within a week of disharge, with subsequent reordings as linially indiated (but not usually less often than 3 4 weekly); monitoring should inlude various ativity states. [D] 3. Older hildren with other onditions who are linially stable are likely to need home SpO 2 reordings performed less often than infants with CNLD. [D] Withdrawal of supplemental oxygen (Setion 8.2) 1. One the oxygen requirement is down to 0.1 l/min, onsideration should be given to withdrawing supplemental oxygen. [3] 2. The same target saturations used to deide initiation of supplementation should be used for withdrawal purposes (ie, >93%). [3] 3. Children an be weaned from ontinuous low flow oxygen to night-time and naps only, or remain in ontinuous oxygen throughout the 24 h until the hild has no requirement at all. It is not possible to reommend whih strategy is superior. [3] 4. Oxygen equipment should be left in the home for at least 3 months after the hild has stopped using it. If this is in a winter period, it is usually left until the end of winter. [3] 5. In CNLD, failure to redue oxygen supplementation after 1 year should lead to a speialist review to rule out onomitant onditions. [3] Oxygen outside the home (Setion 9) 1. An appropriately trained individual should be present while the hild is using the oxygen, but this does not neessarily have to be a shool nurse or health professional. [3] 2. Children will need higher oxygen flows during air flights or at high altitude, whih should be determined by a fitnessto-fly test. [B] 3. If a hild has stopped supplemental oxygen within the last 6 months, they will need a fitness-to-fly test. [3] Potential disadvantages (Setion 10) 1. Parental/arer smoking must be strongly disouraged. [3] 2. Parents/arers (and older hildren) must be made aware of the potential hazards of home oxygen. [3] 3. It is ritial that parents and arers reeive suffiient emotional support from their family, friends and the healthare servies. [3] 2. BACKGROUND 2.1 Definitions Although domiiliary refers to the home, in the ontext of oxygen therapy it refers to delivery of supplemental oxygen outside the hospital as it may also be used outside the home, espeially by hildren. Modes of delivery fall into several ategories (fig 2). Long-term oxygen therapy (LTOT) is defined as the provision of oxygen for ontinuous use at home for patients with hroni hypoxaemia (due to any ause) in order to maintain target oxygen saturations. It may be required 24 h per day (ontinuous LTOT) or during periods of sleep only (sleep-related LTOT). The latter may be given at night alone (noturnal LTOT) or, in young hildren, for daytime naps as well. Portable oxygen therapy is LTOT outside the home (or in the garden). It refers to the provision of oxygen that an be wheeled on a trolley or pram, worn in a bakpak or arried. When ii4

8 Figure 1 Long-term oxygen therapy (LTOT) pathway for an infant with hroni neonatal lung disease. SpO 2, oxygen saturation measured by pulse oximetry. ii5

9 Figure 2 Modes of home oxygen. Long-term oxygen therapy (LTOT) is the provision of oxygen for ontinuous use at home for patients with hroni hypoxaemia. It may be required 24 hours per day (ontinuous LTOT) or during periods of sleep only (sleep-related LTOT). The latter may be given at night alone (noturnal LTOT) or, in young hildren, for daytime naps as well. Portable oxygen therapy is the provision of oxygen to deliver LTOT outside the home; when arried by the patient it is known as ambulatory oxygen therapy. Intermittent oxygen therapy is a less ommon situation whereby the hild reeives oxygen in an episodi manner but, beause of the reurrent nature of the underlying ondition, oxygen needs to be permanently available in the hild s home. Intermittent oxygen use may last days or weeks (intermittent LTOT), or it ould be used during an aute emergeny situation only (intermittent emergeny oxygen therapy). arried by the patient it is termed ambulatory oxygen. All hildren on LTOT require failities for portable oxygen unless they only use it at night. There are no situations where a hild reeives portable oxygen that is not part of an LTOT regimen. Children are rarely housebound, and it is important to enable them (and their parents) to go outside the home in order to lead as normal a family life as possible. Intermittent oxygen therapy desribes a less ommon situation whereby the hild reeives oxygen in an episodi manner but, beause of the reurrent nature of the underlying ondition, oxygen needs to be permanently available in the hild s home. An example would be a hild with neurodisability who requires oxygen for aspiration pneumonia being treated at home (setion 4.1), and who usually reeives it for 1 2 weeks every few months. This is known as intermittent LTOT. In another situation an autely hypoxaemi hild may reeive oxygen for a short while for an emergeny situation at home, for example,. life-threatening asthma waiting for an ambulane (setion 4.2). This is known as intermittent emergeny oxygen therapy. Hypoxaemia refers to low oxygen tension or partial pressure in the blood. Hypoxia is less speifi and refers to lak of oxygen in a partiular ompartment (eg, alveolar or tissue hypoxia). It is usually as a result of hypoxaemia (hypoxaemi hypoxia), dereased tissue blood flow (stagnant hypoxia), anaemia (anaemi hypoxia) or an inability of the tissues to utilise oxygen (histotoxi hypoxia). 2.2 What differenes are there between adult and paediatri pratie? Diagnosis. The range of onditions seen in hildren is quite distint from adults. There is a tendeny for hildren s diseases to improve with time, whereas with adults they tend to deteriorate. Exeptions in hildren inlude ysti fibrosis and neuromusular disease. Ordering oxygen. In hildren almost all oxygen therapy is presribed by hospital speialists (onsultant paediatriians) rather than in primary are. Assessment. In hildren, almost all oxygen assessments are done by pulse oximetry and not arterial blood sampling. ii6 Growth and neurodevelopment. These are important onsiderations in hildren. Equipment. Speifi equipment is required to allow for low oxygen flows. Almost all hildren reeiving LTOT also require portable oxygen therapy (they are rarely housebound). Many older hildren have LTOT for,15 h per day. Care and safety onsiderations. All hildren require supervision from a parent/arer. Preshool/shool. Provision of oxygen may be neessary at nursery or shool. 2.3 What is urrent UK pratie in presribing home oxygen? Data are available from the BTS Home Oxygen Database whih reeives anonymised data for England and Wales from the four oxygen suppliers; and also from the Children s Home Oxygen Reord Database (CHORD) whih reeives opies of the Home Oxygen Order Form one the parents have signed onsent. In June 2007 there were 3136 hildren under 17 years of age in England and Wales reeiving home oxygen, whih represents 4% of all patients (adult and hildren) reeiving it. 1 From CHORD inidene data (available Deember 2008) on 828 hildren, the ommonest underlying diagnoses are hroni neonatal lung disease (60%), neurodisability (7%), paediatri ardia disease (5%), neuromusular disease (3%) and interstitial lung disease (2%). 2.4 What is the normal oxygen saturation in a healthy infant aged,1 year and a healthy hild aged >1 year? Methodologial issues Oximeters from different manufaturers may give different oxygen saturation readings depending on whether frational or funtional oxygen saturation is being measured. Normal oxygen saturation (SaO 2 ) is an oversimplifiation of a omplex measurement. Assessment of SpO 2 (oxygen saturation measured by pulse oximetry) an be made in different behavioural states and using different mahines. An oximeter whih measures frational oxygen saturation (eg, Ohmeda 3470) may read 1.6% lower than one measuring funtional saturation (eg, Nellor N3000). 2 Frational saturation refers to the ratio of oxyhaemoglobin to all haemoglobin measured inluding dyshaemoglobins (eg, arboxyhaemoglobin or

10 methaemoglobin), whereas funtional saturation refers to the ratio of oxyhaemoglobin to all haemoglobin that is apable of arrying oxygen. The normal values from published data an be onfusing as they may be derived from data whih averages the summary data generated from a number of subjets where eah reording generates its own mean, median and entiles. Thus, referene values might refer to the median of mean oxygen levels or even the 5th entile for 5th entile oxygen levels. Generation of the lower limit of normal is ompliated by the fat that data are often given as mean and standard deviation (SD), but SpO 2 is not normally distributed whih means that the SD annot be used reliably to generate lower 95% onfidene intervals for a population. Many studies generating referene values are partiularly foused on desaturation episodes, and baseline levels may be seleted from periods of normal breathing. Conerning hildren on home oxygen, the main purpose is to maintain a stable baseline level of oxygenation, and it has been reommended that assessments should be made of summary data on a reording. Thus, the most relevant studies are those whih inlude all data from different behavioural states and give summary data to allow the generation of lower limits of normal values. Artefat rejetion is addressed by some studies, usually by visual inspetion of the waveform or by disparity between oximeter heart rate and ECG. In more reent studies, automated artefat rejetion by ommerial monitors is more usual. Studies have been inluded for this setion if they were of healthy hildren and where summary data were available for mean, median or distributions of values for SpO 2. Studies whih were restrited to desaturation indies or other measures of episodi desaturation were not inluded without the above measures. In all, 20 studies were relevant for normative values, 14 in infants and 6 in older hildren. Seven of the 14 studies in infants are from the same group of investigators Healthy infants aged,1 year The median baseline saturation in healthy term infants during the first year of life is 97 98%. In only 5% of healthy infants is the SpO 2,90% for.4% of the time. There are two longitudinal studies of infants in the first 6 12 months. 3 4 Masters et al 4 found a slight inrease in baseline levels with age but, in ontrast, Hunt et al 3 found no hange in baseline with age but a derease in variability. The five infant studies from the Stoke group 5 9 used a modified oximeter, operating in a beat-to-beat mode (not available for linial use), and restrited analyses of oxygen baselines to periods of regular breathing (table 1). This makes their data diffiult to apply to studies in linial pratie using averaging oximeters, and where all data are inluded irrespetive of breathing pattern. In turn, this makes omparison of linial studies diffiult when they use different oximeters and different averaging, thus inluding pauses, sighs and periodi breathing to different degrees. The median oxygen baseline in these studies ranged from 97.6% to 99.8% with a 5th entile ranging from 91.9% to 95.5%. Three of the studies were on infants born prematurely and without signifiant lung disease when ready for disharge at term, and the results were similar to those in healthy term infants. One paper 11 looked at normal values in healthy preterm infants prior to term, reporting median values of 97%, measuring frational saturation. The remaining four studies were of healthy term infants, using either short-term or overnight monitoring with different monitors. Median SpO 2 levels ranged from 97% to 98.2%. The mean perentage of time spent,90% has been reported in three studies of term infants, and ranges from 0% to 2%; in preterm babies it was approximately 2.5%. 11 However, the 95th entile of the time spent,90% was 0.1 4% in term babies 14 and approximately 11.5% in preterm babies Healthy hildren aged >1 year The median baseline SpO 2 in healthy hildren >1 year old is 98% with a 5th entile of 96 97%. A healthy hild aged 5 11 years spends no more than 5% of the time below a SpO 2 of 94% while asleep. Studies in this age group were all onfined to sleep reordings or reordings done while in bed. One of the six studies used a beatto-beat oximeter and analysed only regular breathing periods; this found median values of 99.5% with a 5th entile of 96.6%. 10 Another study, measuring frational saturation, gave only median figures for the 5th entile of SpO 2, whih was 96% with a 1st entile of 95%. 16 Three more studies gave only mean data, ranging from 97% to 97.8% The most definitive study in this area is that of Urshitz et al, 20 whih is a arefully validated study setting out to generate normal values for primary shool hildren. The median value in this study is 98%, with a 5th entile of 97% and range %. However, this study also gives the 5th and 2.5th entiles for the value below whih the subjets spent 5% and 10% of the reording (SAT5 and SAT10). These are partiularly useful for assessment of normal oxygenation and were 95% and 94% for SAT5 and 96% and 94% for SAT What are the onsequenes of hroni low oxygen saturation in hildren? Pulmonary arterial hypertension Hypoxaemia auses pulmonary hypertension but the preise severity and duration of hypoxaemia needed to do this are not known. The fators affeting individual suseptibility are also unknown. SpO 2 levels.94 95% appear to redue pulmonary hypertension, while levels,88 90% may ause pulmonary hypertension. This Table 1 Normal SpO 2 (%) levels in healthy hildren measured by the same group of investigators using Nellor N200 (or N100 with N200 software) pulse oximetry in beat-to-beat mode Age No Median (%) 5th entile (%) Range (%) Referene Preterm (normal lungs) first week Ex-preterm babies at term Full term first 24 h Full term first week Full term 2 4 weeks Full term 4 8 weeks Full term 2 16 (mean 8) years ii7

11 does not apply to hildren with ongenital ardia defets and idiopathi pulmonary arterial hypertension. Hypoxia may have adverse effets on ognition and behaviour at SpO 2 levels of (85%, but the effets of milder hypoxia are less lear. Chroni hypoxaemia is a well-established ause of pulmonary hypertension. In animals, the ritial level of alveolar PO 2 at whih the hypoxi pulmonary vasoonstrition reflex is triggered is 100 mm Hg (13.3 kpa). 21 In human adults the SaO 2 at whih pulmonary hypertension ours is believed to be 88 90%, 22 although the duration of hypoxaemia required is not known. The development of pulmonary hypertension in hildren who have intermittent noturnal hypoxaemia due to obstrutive sleep apnoea suggests that hypoxia does not have to be ontinuous. 23 In the past, pulmonary hypertension was often observed in hildren with CNLD, 24 and in one series it was fatal in 5/17 subjets pulmonary hypertension in CNLD and, Supplemental oxygen autely ameliorates in a prospetive ase series, right ventriular hypertrophy resolved in infants on a home oxygen programme with saturations maintained above 94 95% Neurodevelopment Hypoxia may have adverse effets on ognition and behaviour at SpO 2 levels of (85%, but the effets of less severe hypoxia are less lear. A systemati review of the ognitive effets of hroni hypoxia in hildren was onduted in The evidene ame almost exlusively from studies of sleep-related breathing disorders and from yanoti heart disease in hildren. The onlusion was that there was strong evidene for adverse ognitive and behavioural effets of hypoxia. A subsequent re-analysis of a ommunitybased study of noturnal oximetry in 995 primary shool hildren found that mildly abnormal nadirs of SpO 2 (91 93%) were assoiated with worse aademi performane in mathematis, although this effet was not signifiant when habitual snoring was exluded. 31 There are problems with the extrapolation of these onlusions to hildren with lung disease. Sleeprelated breathing disorders may ause neuropsyhologial effets from sleep fragmentation or deprivation as well as from hypoxia, and the mean saturation of the hildren with yanoti heart disease in the studies ited was 85%, whih may be lower than that to whih pulmonary patients are exposed. The Benefits of Oxygen Saturation Targeting (BOOST) study, whih ompared target SpO 2 levels of 91 94% vs 95 98%, did not find any differenes in developmental status at 1 year, although this does not exlude more subtle later effets on ognition. 32 See also setion Apnoeas/apparent life-threatening events/sudden unexplained death in infany In infants with CNLD, SpO 2,90% is assoiated with an inreased risk of an apparent life-threatening event while SpO 2 >93% is not. In both term 33 and preterm infants, 34 redued fration of inspired oxygen (FiO 2 ) may lead to an inrease in periodi breathing, hypoventilation and entral apnoeas, thus hypoxaemia may predispose to apparent life-threatening events (ALTEs). When a group of infants with hroni lung disease was kept at a higher SpO 2 (94 96%), they experiened fewer desaturations to,80% ompared with when their baseline SpO 2 was maintained at 87 91%. 35 Baseline hypoxaemia (,95%) was found in 25% of 91 preterm infants who had suffered ALTE/ ii8 yanoti episodes, and abnormal hypoxaemi episodes were found in 40%. 36 In a small study of 10 infants with bronhopulmonary dysplasia (BPD) who had reently stopped supplemental oxygen (within 7 days), their mean SpO 2 was signifiantly lower and they had signifiantly more entral apnoeas ompared with 10 healthy preterm babies. 37 When the SpO 2 was improved with supplemental oxygen, both entral apnoea and periodi breathing densities delined. 37 A ohort study of 78 infants with CNLD on home oxygen (target SpO %) and 78 mathed preterm ontrols found no differene in the inidene of ALTEs in the two groups (8.9% and 10.5%), and no sudden infant deaths in either group. 38 These figures were felt to ompare favourably with historial ontrols from other authors Growth In infants with CNLD, SpO 2,92% may be assoiated with suboptimal growth. Two observational ase series have found normal growth along entiles in babies with CNLD where saturations were maintained at or above 92% 39 or 93%. 40 In both studies, weight gain faltered if supplemental oxygen was disontinued prematurely. In one of the studies, faltering growth was seen at a SpO 2 of 88 91%. 39 The BOOST study did not find any advantage in growth at a orreted age of 12 months in those whose SpO levels had been maintained at 95 98% ompared with 91 94%. 32 See also setion Sleep In infants with CNLD, SpO 2 (90% impairs sleep quality but SpO 2.93% does not. Infants with CNLD who have SpO 2 levels around 90% had impaired sleep quality whih improved when supplemented with 0.25 l/min via nasal annulae; 41 this effet was not seen in infants with CNLD in whom the SpO 2 was inreased from.93% to.97% What are the onsequenes of exess oxygen therapy in hildren? Exess arterial and intra-alveolar oxygen onentrations are toxi in preterm infants and must be avoided by appropriate monitoring and adhering to the target SpO 2 level; there are no data in older hildren. [D] Oxygen toxiity an broadly be divided into two omponents: the effets of high arterial blood oxygen onentrations (PaO 2 ) and the effets of high intra-alveolar oxygen onentrations (PAO 2 ). There are no trials that address the question of whether a high PaO 2 in term hildren on home oxygen is harmful. Most literature on high arterial blood onentration fouses on infants who are still premature; for example, the effets on the developing retina are well established. 43 High alveolar oxygen levels in premature infants an inhibit lung healing and ontribute to ongoing lung injury, possibly through the formation of reative oxygen intermediates and peroxidation of membrane lipids. 44 Oxidative stress from a high oxygen onentration may be a ontributing fator to the development of BPD, 45 and it is suggested that an FiO 2 of for 24 h is assoiated with the ourrene of BPD. 46 The BOOST study (see setion 5.2 for fuller ritique) showed a non-signifiant exess of deaths from pulmonary auses in the premature babies kept at a higher SpO The STOP-ROP study (see setion 5.2 for fuller ritique) found an inreased rate of adverse pulmonary sequelae

12 (pneumonia and exaerbations of CNLD) although not deaths in the high saturation group; this group also had more infants still requiring supplemental oxygen at 3 months. 43 In summary, six studies of extremely low birthweight infants have shown that retinopathy of prematurity and hroni lung disease are signifiantly redued if the SpO 2 is kept,93 95% ompared with higher saturations (when under 36 weeks gestation). 47 It is plausible that some of the adverse effets attributed to hypoxaemia may in fat be due to flutuating rather than low oxygen delivery, and avoidane of flutuations in SpO 2 also seems to be important. 47 There is some evidene that high oxygen levels an also be toxi to the term and mature lung. There has been a ase report of a newborn infant with a massive left to right shunt (seondary to a erebral arteriovenous malformation) who required ontinuous oxygen therapy in high onentrations. 48 Despite a high alveolar PAO 2, the infant maintained low to normal arterial PaO 2 onentrations. Light and ultrastrutural studies of the lungs demonstrated typial hanges of aute pulmonary oxygen toxiity. These observations may onfirm earlier experimental animal studies whih demonstrated that the PAO 2 onentration and not the PaO 2 is the major fator ontributing to oxygen toxiity within the lungs. 48 The pathogenesis of oxygen toxiity remains unknown but may involve leuoyte-mediated injury and leukotriene B 4 prodution INDICATIONS FOR LTOT For a variety of onditions, we have assessed the evidene (aepting it is often laking) as to whether supplemental oxygen is benefiial to patients and home oxygen is preferable to hospital-based oxygen. Benefit is onsidered in terms of survival, symptoms (breathlessness, respiratory distress, exerise tolerane), growth and neurodevelopment, shool attendane and hospitalisation rates, quality of life and psyhologial impat. Obviously these parameters are not appliable to all patient groups. 3.1 Chroni neonatal lung disease Supplementary oxygen should be given to infants with hroni neonatal lung disease: to redue or prevent pulmonary hypertension, redue intermittent desaturations, redue airway resistane and promote growth; [C] as it is likely to be benefiial for neurodevelopment in infants with CNLD; [D] as it may redue the assoiated risk of sudden unexplained death in infany; [D] as oxygen at home is preferable to a prolonged hospital stay for both quality of life and psyhologial impat for the infant, parents and family; [D] as it saves days in hospital due to earlier disharge despite a signifiant readmission rate. [C] For the purposes of these guidelines, the diagnosis of CNLD is defined as an infant requiring supplemental oxygen at a orreted age of 36 weeks gestation who is at least 28 days old. The pathophysiologial effets of hroni hypoxia support the use of supplementary oxygen in infants with CNLD. Pulmonary hypertension is a relatively ommon ompliation of CNLD that an ause diminished right ventriular performane and, eventually, or pulmonale. 50 Infants with CNLD who have pulmonary hypertension generally have reative pulmonary vasular beds responsive to supplemental oxygen. Right ventriular hypertrophy and pulmonary hypertension an resolve with home oxygen therapy. 29 Supplementary oxygen in CNLD may also redue the frequeny of intermittent desaturations. Oxygen given to mildly hypoxi infants may ause a derease in total pulmonary resistane. 53 The pathogenesis of left ventriular hypertrophy has been attributed to the metaboli effets of hroni hypoxaemia in addition to hyperarbia and aidosis. If the hypertrophy is severe enough, it may ause an inrease in left atrial pressure, thereby potentially ontributing to pulmonary oedema and the severity of CNLD Survival Home oxygen might in part be responsible for improved survival in CNLD through its role in the treatment or prevention of pulmonary hypertension. In addition, beause patients with CNLD may have an abnormal response to hypoxia that an lead to prolonged apnoea and bradyardia, maintaining the SpO 2 at appropriate levels may derease the higher inidene of sudden unexplained death in infany in this patient group. 55 For infants who have had an ALTE while already on home oxygen, an insuffiient amount of supplementary oxygen may have been given. 56 Previously, preterm infants with CNLD not on home oxygen at disharge but who subsequently suffered an ALTE have been shown to have episodi or baseline hypoxaemia whih improves with home oxygen. 36 In this study, 33 premature babies who had suffered ALTEs were given supplemental oxygen ( l/min via nasal annulae) for up to 17 months (median 3.9 months). There were no further ALTEs in 24 and a redution in severity in 7 (in 2 of whom other auses were found). 36 The provision of home oxygen when appropriate should eliminate episodes of unreognised and untreated hypoxaemia so that preterm infants with CNLD are no longer at inreased risk from sudden infant death ompared with other preterm infants. It is thought that infants with CNLD who die suddenly may have had linially unreognised periods of hypoxaemia Symptoms Supplemental oxygen an redue the demands on an already stressed respiratory system by dereasing the respiratory rate and the work of breathing needed to provide improved oxygenation, thus reduing symptoms Growth It is suggested that supplemental home oxygen improves growth in infants with CNLD when the saturations are kept above 92% Also eliminating sleep-assoiated hypoxia improves growth in infants with CNLD See also setion Neurodevelopment As so many fators at as onfounders for neurodevelopment in this group of hildren, the relative ontribution of CNLD (with or without oxygen supplementation) remains unertain. Gestational age, birth weight, hypoxi-ishaemi events and intraranial haemorrhage are all independently assoiated with worse neurodevelopmental outomes Many studies fail to ensure adequate length of follow-up. This is a ruial methodologial problem given the inreased reognition that preterm hildren are at risk of a range of more subtle neurodevelopmental problems whih have onsiderable impat on daily living and shool performane. These will not be piked up reliably by routine follow-up or even speifi testing until around shool entry age at 5 years. 46 ii9