Challenging clinical cases CC8 Noninvasive respiratory aids for continuous support in neuromuscular disorders

Transcription

1 ERS Annual Congress Milan September 2017 Challenging clinical cases CC8 Noninvasive respiratory aids for continuous support in neuromuscular disorders Tuesday, 12 September :00-08:15 Blue 1 (North) MICO

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3 Challenging clinical cases : CC8 Noninvasive respiratory aids for continuous support in neuromuscular disorders Aims : To describe how to set up non-invasive ventilation that is complemented by assisted cough techniques, thereby safely extending the full-time non-invasive support of neuromuscular disease patients and avoiding tracheostomy/invasive ventilation. Tracks: Acute and chronic respiratory failure/sleep Tags: Clinical Target audience: Intensivist/critical care physician - Nurse - Physiotherapist - Pulmonologist - Respiratory therapist Chairs : João Carlos Winck (Vila Nova de Gaia, Portugal) Chronic respiratory failure in a patient with ALS Capucine Morelot-Panzini (La Varenne St-Hilaire, France) Chronic respiratory failure in a patient with chest wall deformity Collette Menadue (Leichhardt, NSW, Australia)

4 NONINVASIVE VENTILATION... how to do it, why to do it, when to do it and when to stop! ERS Practical Handbook of Noninvasive Ventilation Edited by Anita K. Simonds ISBN (print) ISBN (ebook) (ERS members) 40 (non-members) The ERS Practical Handbook of Noninvasive Ventilation provides a concise why and how to guide to NIV from the basics of equipment and patient selection to discharge planning and community care. Editor Anita K. Simonds has brought together leading clinicians and researchers in the field to provide an easy-to-read guide to all aspects of NIV. Topics covered include: equipment, patient selection, paediatric indications, airway clearance and physiotherapy, acute NIV monitoring, NIV in the ICU, long-term NIV, indications for tracheostomy ventilation, symptom palliation, discharge planning and community care, and setting up an NIV service. This Practical Handbook is a valuable reference and training resource for all NIV practitioners. User-friendly format with key point summaries Focused on practical aspects and problem solving Multiple choice questions to enable self-assessment To buy printed copies, visit the ERS Bookshop in the World Village at the ERS International Congress Electronic: Print:

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6 CC8 Noninvasive respiratory aids for continuous support in neuromuscular disorders Chronic respiratory failure in a patient with chest wall deformity Collette Menadue, PhD Senior Physiotherapist Respiratory Failure Service Royal Prince Alfred Hospital Sydney, Australia

7 Conflict of interest disclosure I have no real or perceived conflicts of interest that relate to this presentation. I have the following real or perceived conflicts of interest that relate to this presentation: Affiliation / Financial interest Grants/research support: Commercial Company Honoraria or consultation fees: Participation in a company sponsored bureau: Stock shareholder: Spouse / partner: Other support / potential conflict of interest: This event is accredited for CME credits by EBAP and EACCME and speakers are required to disclose their potential conflict of interest. The intent of this disclosure is not to prevent a speaker with a conflict of interest (any significant financial relationship a speaker has with manufacturers or providers of any commercial products or services relevant to the talk) from making a presentation, but rather to provide listeners with information on which they can make their own judgments. It remains for audience members to determine whether the speaker s interests, or relationships may influence the presentation. The ERS does not view the existence of these interests or commitments as necessarily implying bias or decreasing the value ofthe speaker s presentation. Drug or device advertisement is forbidden.

8 Introduction AIMS: 1. Discuss timing and strategies to augment cough and airway clearance in people with severe chest wall deformity and respiratory muscle weakness 2. Discuss non-invasive ventilatory aids to avoid intubation and improve outcomes during an acute respiratory illness 3. Discuss the role of non-invasive ventilation for long-term continuous ventilatory support in patients with severe chest wall deformity and respiratory muscle weakness

9 Chest wall deformity: Pathophysiology C rs + respiratory mm weakness TLC, VC, FRC work of breathing Rapid shallow breathing pattern ( V D /V T ) REM hypoventilation Resetting of central chemoreceptors Daytime respiratory failure (Bergofsky 1959 Medicine, Casas 2003 Arch Bronconeumol)

10 Post polio syndrome (PPS) New or muscle weakness, occurs average 35yrs after acute polio Atrophy, pain, fatigue (Jubelt 2000 JAMA, Farbu 2006 Eur J Neurol) Prevalence 15-80% (Farbu 2006 Eur J Neurol) Sleep disordered breathing common (OSA/hypoventilation) (Hsu1998 Mayo Clin Proc) Chest wall deformity s risk of nocturnal hypoventilation (Howard 2003 Pract Neurol) Usually a slowly progressive neuromuscular disorder (Trojan 2005 Muscle Nerve)

11 Case yo man with severe kyphoscoliosis and post polio syndrome referred for sleep study Presents with: Dyspnoea, daytime sleepiness, sleep fragmentation, fatigue Past Medical Hx: Polio in Lebanon (age 2 ½), required short-term tracheostomy Wheelchair for mobility Severe muscle weakness lower limbs and trunk, weak upper limbs Obese (BMI 31.6) Snorer

12 Pulmonary function tests Spirometry Case FEV L (19% pred), 0.87L FVC (17% pred), ratio 92% Static lung volumes TLC 1.56L (22% pred), VC 0.98L (19% pred), FRC 0.81L (25% pred) Respiratory muscle strength ABG MIP -55cmH 2 O (50% pred), MEP 60cmH 2 O (39% pred) * Arm span used to calculate % pred values (Linderholm 1978 Acta Orthop Scand) pm ph 7.36, PaCO 2 55, PaO 2 58, HCO 3-33, BE 5, SaO 2 88% (room air) Diagnostic sleep study AHI 32/hr, min SpO 2 65% in REM with 12mmHg TcCO 2, PaCO 2 8mmHg overnight

13 Questions 1. The patient requires treatment with nocturnal NIV. Is volume preset ventilation associated with better outcomes than pressure preset ventilation (e.g. bilevel NIV) in people with severe chest wall deformity (CWD)? Equivalent outcomes in ABGs, sleep, health status, provided PS adequate (Tuggey 2005 Thorax, Struik 2011 Respir Care) PaCO 2 >50mmHg at 1/12 follow-up independent predictor of mortality in CWD (HR 3.42, 95% CI ) (Marti 2010 Respir Med)

14 Case Bilevel (pressure preset) titration study performed: Spontaneous mode, IPAP 18 cmh 2 O, EPAP 6 cmh 2 O via nasal mask and chin strap prevented OSA and hypoventilation

15 NIV for chronic hypercapnia in chest wall deformity NIV is a standard of care No RCTs unethical Retrospective studies and prospective observational studies with long term follow-up consistently showing: Good survival (Simonds 1995 Thorax, Duiverman 2006 Respir Med, Gustafson 2006 Chest, Laub 2007 Respir Med) Good HRQL Improvements in ABGs, hypoventilation symptoms, exercise tolerance Inconsistent effects on pulmonary function (Leger 1994 Chest, Chailleux 1996 Chest, Schonhofer 2001 Chest, Nauffal 2002 Respir Med, Gonzalez 2003 Chest) Restrictive pathologies <10% HMV Australia & New Zealand (Garner 2013 Eur Respir J)

16 NIV for chronic hypercapnia in chest wall deformity: Mechanisms ventilatory response to CO 2 Minimal change respiratory mm strength No significant changes in C RS

17 Case 1993 to 2007 (14yrs) Responded well to NIV - ABGs normal - Symptoms resolved - Using NIV 7-8hr/night Back to work - Disability Advocate - Teacher Disability Services - After hours emergency care coordinator Wheelchair sports Married with 2 children

18 Case 2010 (47yo) Failed to attend follow-up NIV usage average 8.5hr/day 13hr/day Waking with choking sensation Harder to cough

19 NIV review sleep study: Case Unable to trigger in REM diaphragmatic strength Residual upper airway obstruction in NREM weight gain 2 min New settings ST mode Rate 16 bpm EPAP 12 cmh 2 O IPAP 22 cmh 2 O Ti 1.4s Rise time 2

20 Case Discussed Advanced Care Plan For trial of full active Rx, including I+V Referral to dietician Cough assessment peak cough flow (PCF) 180L/min

21 Questions 2. PCF 160L/min = minimum threshold for an effective cough (Bach 1996 Chest). Should strategies to augment cough be introduced now (PCF 180L/min)? Yes, respiratory mm strength s during URTI PCF (Poponick 1997 Am J Respir Crit Care Med) PCF <270L/min threshold for starting techniques (Tzeng 2000 Chest, McKim 2011 Can Respir J) 3. What strategies can be used to PCF? Manually Assisted Cough (MAC) Lung Volume Recruitment (LVR) +/- MAC Mechanical in-exsufflation (MI-E) +/- MAC

22 Strategies to augment cough

23 Questions 4. Are LVR and MI-E safe to perform in people with severe CWD? Extremely low complication rates (Bach 2008 Am J Phys Med Rehab, Bach 2014 J Neurorestoratology) Case reports of pneumothorax LVR: 72yo woman with PPS, scoliosis, asthma after 3yrs of LVR (Westermann 2013 J Bras Pneumol) MI-E: 26yo man with DMD, scoliosis on CNVS; 58yo man SCI + COPD on noct NIV (Suri 2008 Am J Phys Rehab) Avoid if previous/current pneumothorax, bullous emphysema, hypotension Transient MAP 10mmHg ( 50cmH 2 O) (Molgat-Seon 2017 ERJ Open Res) Urgent medical r/v if sudden chest pain/dyspnoea following LVR or MI-E

24 Case LVR Dose: Recommended insufflation cycles, x2-3/day (Kang 2000 Chest) Short trial of MI-E 45cmH 2 O Missed follow-up in 2011

25 Case Admitted to hospital with URTI High-flow O 2 in ambulance drowsy (GCS 14) For full active treatment ABG: ph 7.27, PaCO 2 65, PaO 2 110, HCO 3-31, SaO 2 99% (8L/min O 2 mask) Placed on NIV via BiPAP Vision in the Emergency Dept: - IPAP 12, EPAP 6, FiO 2.35 via an oronasal mask - Audible mask leak, intermittent trigger failure - Audible upper airway noises, weak non-productive cough Repeat ABG (1hr): ph 7.27, PaCO 2 67, PaO 2 63, HCO 3-31, SaO 2 89%

26 Questions 5. Would you continue with NIV or intubate the patient? NIV is not currently optimised and signs of secretion retention, continue NIV but: a) Manage pt in location where intubation is readily available (Ambrosino 2009 Eur Respir J) b) Optimise NIV and trial for finite period Back-up rate PS (and EPAP for sleep) Minimise leak and reduce PVAs (Vignaux 2009 ICM, Davidson 2016 Thorax) c) Airway clearance using mechanical in-exsufflation (MI-E) (Bach 1993 Arch Phys Med Rehab ) d) Target SpO % (O Driscoll 2011 Clin Med) e) Humidification

27 Case 2012 Transferred to HDU NIV dependent (STx16bpm, IPAP 25, EPAP 12) Regular chest PT + MI-E (E70) at 50 cmh 2 O PCF 300L/min Able to clear sputum Supplemental O 2 weaned to 1L/min Repeat ABG (1hr): - ph 7.32, PaCO 2 58, PaO 2 65, HCO 3-30, SaO 2 91% Plan: continue NIV + regular chest PT with MI-E

28 Case Progress next day: ph 7.36, PaCO 2 54, PaO 2 67, HCO 3-30 Still NIV dependent + ongoing MI-E Reddened nasal bridge Alternative interfaces, pressures daytime Download of home BiPAP machine

29 Case NIV usage past 6/12 >19 hr/day ventilator dependent (Tzeng 2000 Chest) Using NIV for breathlessness Stopped regular LVR, thought daytime NIV was adequate Stopped work, housebound Implications for weaning and d/c planning (equipment and safety)

30 Questions 6. As the patient will remain ventilator dependent (>16-18hr/day) long term, would you change to tracheal ventilation or continue with NIV to prepare for d/c? No RCTs Continuous NIV (including mouthpiece ventilation) in NMD and CWD: Safe, good survival (Bach 1987 Chest, Bach 1993 Chest, Bach1993 Arch Phys Med Rehab, Toussaint 2006 Eur Respir J, McKim 2013 Can Respir J, Nicolini 2016 Rev Port Pneumol) complications, chest infections (Bach 1993 Chest, Bach 1998 Am J Phys Med Rehabil, Souden 2008 Chron Resp Dis) complex care, live at home, cost (Souden 2008 Chron Resp Dis, Bach 2015 Am J Phys Med Rehab) Preferred by patients (Bach 1993 Chest (vol 103), Bach 1993 Chest (vol 104), McKim 2013 Can Respir J) Case: Life support ventilator (Trilogy 100), weaned to usual nocturnal settings Trial of daytime mouthpiece ventilation (MPV)

31 Open-circuit mouthpiece ventilation (MPV) Speech Eating/drinking easier Avoid pressure areas Advantages Breath stacking (ACV mode) Little dead space Aesthetics Limitations to MPV/continuous NIV: Severe bulbar dysfunction Inability to protect the airway PCF <160L/min despite LVR and MI-E Uncooperative Disadvantages Limited to daytime (awake) use Aerophagia (ACV mode) Orthodontic deformity Hypersalivation Nuisance alarms* Role for tracheal ventilation in selected individuals (Bach 1987 Chest, Bach 1993 Chest, McKim 2013 Can Respir J, Garuti 2014 Rev Port Pneumol, Khirani 2014 Respir Care)

32 Open-circuit Mouthpiece Ventilation (MPV) Equipment: 15mm or 22mm angled mouthpiece, or straw Circuit Single limb, non-vented circuit with active expiratory valve Trilogy 100 MPV mode passive circuit no expiratory valve required Mouthpiece circuit support arm Ventilator (with volume pre-set mode) Bracket/bag to mount ventilator on w/chair

33 Mouthpiece ventilation (MPV) Settings Mode: predominantly volume controlled ventilation (ACV mode) (Khirani 2014 Respir Care) Can also use pressure controlled ventilation/pacv (cannot breath stack) V T : adults mL (Hess 2012 Respir Care, Bach 2014 J Neurorestoratology) PEEP/EPAP: zero (Nicolini 2014 Phys Med Rehabil Int) Rate: zero/lowest if can spontaneously trigger, otherwise set as needed* *Trilogy 100 MPV mode kiss trigger (reverse flow trigger) no rate required (doesn t blow on face) Ti, Flow shape, Trigger sensitivity Alarms Low pressure alarm: off or minimum Angled mouthpiece/straw provides resistance to maintain pressure in circuit (15mm > 22mm) Apnea alarm and Disconnect alarm: off or maximum duration

34 Yes Yes

35 Case - Trilogy 100 script

36 LVR (breath stacking) with mouthpiece ventilation

37 Safety considerations for NIV dependent patients

38 Case 2012 to 2017 Regular follow-up SpO 2 98% on room air, chest infections 24/24 NIV dependent, daily LVR Returned to work Teacher Disability Services 4-5 talks/week! Weight stable Plans to travel to Nth America 2018

39 Conclusion Regular follow-up and monitoring is important to allow timely introduction of non-invasive respiratory aids Strategies to augment cough and facilitate airway clearance are integral to the success of NIV in patients with severe CWD and respiratory muscle weakness Non-invasive ventilation and assisted cough strategies may be used to avoid intubation in selected individuals with acute on chronic hypercapnic respiratory failure Individuals with severe CWD and respiratory muscle weakness can be successfully managed with continuous NIV with good long term survival and quality of life

40 Questions?