11/20/2015. Beyond CPAP. No relevant financial conflicts of interest. Kristie R Ross, M.D. November 12, Describe advanced ventilation options

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1 Beyond CPAP Kristie R Ross, M.D. November 12, 2015 No relevant financial conflicts of interest Sponsored by The Warren Alpert Medical School of Brown University Describe advanced ventilation options Compare ventilation modes using case examples and choose the best option Determine appropriate ventilation settings using titration case examples Increasing number of children requiring respiratory support Range of underlying conditions Partner with your pulmonology colleagues to care for these children Be prepared Orders Sensors Staff preparation Actions during the study Ongoing management issues Non-invasive positive pressure Pressure regulated CPAP alone BiPAP ventilation More advanced modes of ventilation Volume regulated not typically used Positive pressure ventilation via tracheostomy Volume regulated Pressure regulated 12 year old morbidly obese boy Chief complaint of excessive daytime sleepiness Loud snoring, witnessed apneas Falling asleep in classes BMI 44, Tonsils 3+, acanthosis nigricans PSG 3 years prior showed severe SDB AHI 50 Parent did not recall receiving results PAP titration ordered Prepare child and family TOSCA Hospital lab 1

2 Summary of CPAP titration CPAP pressures 4 to 15 cm H2O explored AHI >50 SpO2 in the 50-60s TcCO2 climbed up to the 60 s CPAP Continuous single pressure Maintain airway patency at expiration Titrate to clear obstructive apneas Bilevel EPAP : Expiratory Positive Airway Pressure IPAP : Inspiratory Positive Airway Pressure Pressure support: IPAP EPAP Bilevel used when CPAP pressures that control OSA aren t tolerated There is hypoventilation Ability to raise tidal volume depends on Lung mechanical properties Difference between PIP and PEEP Inspiratory pressure Provide support for inspiration Unload respiratory muscles Increase tidal volume Restore alveolar ventilation Expiratory pressure (PEEP) Maintain upper airway patency at end expiration Maintain adequate end expiratory lung volume (FRC) 2

3 EPAP Maintain airway patency on expiration Titrate to clear obstructive apneas IPAP Provide support with inspiration Maintain PS (IPAP-EPAP) of at least 4 To treat hypoventilation Increase the difference (increase IPAP only) Bilevel titration Best practice would be to choose EPAP that cleared OA s Increase IPAP for hypoventilation Improvement in discrete respiratory events Persistent hypoventilation Deliver ventilatory support with a nasal or nasal-oral mask Treat wide range of disorders of hypoventilation without an artificial airway Evidence of improved outcomes in adults compared with invasive ventilation Evidence in children (chronic use) primarily limited to case series Can be delivered using a variety of modes 3

4 Neuromuscular disorders Rib cage and chest wall anomalies Obesity hypoventilation disorders Overlap syndromes- obstructive and restrictive components Chronic upper airway obstruction that does not respond to CPAP Chronic obstructive airway disease that is severe (cystic fibrosis) Central alveolar hypoventilation disorders Survey of use of LTV in the UK Long term ventilation working group forum of health care personnel Yearly meetings discussion group Most children on LTV fall under care Group surveyed in Sept 2008 Any child below age 17 Medically stable Mechanical aid for breathing all or part of day Via mask or tracheostomy Maintain speech Early NIPPV in chronic respiratory failure due to neuromuscular disorders Improves daytime CO2 elimination Improves sleep continuity May prevent tracheostomy May improve survival Gomez-Merino E Am J Physical Med Rehab 2002; 81: Wallis CJ Ach Dis Child 2011; 96: Midface hypoplasia Aspiration Reduced cardiac output Inadequate control of ventilation Skin breakdown Abdominal distention Approach and timing varies with disease process Patience and perseverance required Experienced personnel for mask fit key Wide variety of nasal and nasal-oral masks Nasal cushions may be feasible in older children High flow cannula systems for infants Desensitization procedures may help 4

5 Children s hospital inpatient setting Sleep laboratory Adequate staff number and experience Level and type of monitoring will depend on the child Pulse oximetry CO 2 measurement- generally will need tcco 2 Additional sensors typically used in a sleep lab Initiation at home? Volume targeted More often used for invasive ventilation Can measure inspired and expired tidal volumes Less effective ability to adjust for mask leak Pressure targeted More often used in NIPPV Cheaper and lighter machines Able to estimate unintentional leak and adjust for it More advance machines can measure expired tidal volumes Devices designed for NIPPV in adults may not work for children Get to know the equipment available locally Pressure limited, flow sensitive ventilator Individually set inspiratory and expiratory pressure supports as we ve discussed Modes: Spontaneous (S) Patient sets rate, PS delivered with each triggered breath Timed (T) Set rate with delivery of PS with each delivered breath Spontaneous/timed (S/T) modes PS to every spontaneous breath + back up rate if fails to trigger Diagnoses Severe OSAS G47.33 Obesity related hypoventilation E66.2 Admitted to inpatient unit Hospital bed ordered for home BiPAP ordered: 24/16 Echocardiogram- no evidence for PAH ENT consult AT performed 4 weeks later Repeat titration study: Able to control OSA with BiPAP pressures of 20/10 Some residual hypoventilation 120 sec 120 sec 5

6 18 year old morbidly obese young woman Loud snoring, witnessed apneas, daytime sleepiness (ESS 16/24). Co-morbid HTN, insulin resistance BMI 45 Tonsils 1+, no waking nasal airflow obstruction, waking SpO2 96% Split night PSG ordered 120 sec Diagnostic AHI 64, SpO2 < 90% for 10% sleep TcCO2 > 50 mmhg for 70% of sleep Persistent hypoventilation on BiPAP Elevated tcco2 Borderline SpO2 Sleep fragmentation What other options do we have? 6

7 Average Volume Assured Pressure Support Pressure support is adjusted by the machine to deliver a set tidal volume You set Tidal volume: based on ideal body weight for height EPAP : start with what cleared OA s IPAP min: at least 4 above EPAP IPAP max: max of 25, may want to start lower Rate: resting RR - 2 I-time Rise time Return for AVAPS titration Tidal volume based on IBW EPAP 10 cm H2O OA s cleared IPAP min 12 cm H 2 O IPAP max (initial) 20 cm H 2 O IPAP max 25 cm H 2 O Adaptive Servo Ventilation Central apnea/complex apnea/cheyne Stokes Rapid adjustments to stabilize CO2 Automatically adjusting EPAP and PS Back up breath rate- auto or set Controversy in heart failure patients Delivered via port on the mask Low flow Doesn t interrupt circuit Hard to measure FiO2 accurately Delivered via the circuit Requires a high flow rate to maintain FiO2 In older models may violate manufacturer s instructions 7

8 13 year old with lysosomal storage disease (I- cell) Growth restriction, slowly progressive Lipid deposition in multiple organ systems Referred for discussion of anesthesia risk for MRI Significant muscle weakness, unable to walk Long standing symptoms of obstructive sleep apnea Multiple previous PSG s showing moderate to severe OSA Physical Exam / Direct laryngoscopy Micrognathia Macroglossia Stiff laryngeal structures due to lipid deposition Enlarged epiglottis No surgical options for OSA Anesthesia unable to intubate secondary to abnormal upper airway structures Intermittent follow up returns 2 years later Progression of disease Voice softer More difficulty swallowing Hearing/vision problems Hepatosplenomegaly Admissions for pneumonia Chronic atelectasis Sleepier despite use of CPAP 8

9 Unable to trigger due to weakness Not interested in pursuing invasive ventilation Not interested in pursuing feeding tube Unable to adequately support with standard BiPAP machine Inpatient NIPPV study Ventilator appropriate for small pediatric patient AVAPS mode EPAP 5 cm H2O IPAP min 9 cm H2O, IPAP max 20 cm H2O Tidal volume 150 ml (weight is 16 kg) Long term goals Reduce work of breathing Improve somatic and pulmonary growth Improve daytime and nocturnal gas exchange Reduce unplanned admissions due to pulmonary infections Improve daytime functioning/school performance Prolong life Avoid tracheostomy Monitoring Skin care Midface morphology? Growth Lung function Overall health, quality of life Annual echocardiogram in some cases Diagnostic reports from the equiptment Annual sleep study Number of devices FDA approved for non invasive use Know your options Age and size important for interfaces and equipment Four categories Restrictive thoracic disorders Hypoventilation Central Sleep Apnea COPD 9

10 A: documentation in the record of Neuromuscular disease Severe thoracic cage abnormality B: gas exchange/pulm function: Awake P a CO 2 > 45 mmhg OR Sleep oximetry SpO2 < 88% for 5 minutes OR MIP < 60 cm H2O or FVC < 50% predicted C: COPD does not contribute to the patient s condition Awake P a CO 2 > 45 mmhg AND Spirometry FEV 1 /FVC > 70% and FEV 1 > 50% predicted AND P a CO 2 worsens > 7 mm Hg during/immediately after sleep OR PSG sleep oximetry SpO2 < 88% for 5 minutes not explained by upper airway obstruction AHI > 5 More than 50% of the apneas and hypopneas are central Daytime sleepiness or disrupted sleep Continued coverage beyond the first three months requires re-evaluation Documentation about symptoms and usage Average use of 4 hours/day on 70% or more of nights in reporting period Report format and data will depend on the equipment Consider reporting ability when choosing equipment 10

11 Growing experience with delivering ventilation in children without a tracheostomy Machine and interface options are improving Requires Risk benefit analysis and discussions Patient centered goals In lab titration (likely several) Relationship with local DME companies to know equipment options, coding requirements Monitoring in and out of the sleep lab Thank You Kristie.Ross@case.edu 11