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Beyond CPAP

Kristie R Ross, M.D.November 12, 2015

Sponsored byThe Warren Alpert Medical School

of Brown University

No relevant financial conflicts of interest

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

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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)

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◦ 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

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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

◦ Email 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

Wallis CJ Ach Dis Child 2011; 96:998-1002

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; 81:411-415

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

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Children’s hospital inpatient setting

Sleep laboratory◦ Adequate staff – number and experience

Level and type of monitoring will depend on the child◦ Pulse oximetry

◦ CO2 measurement- generally will need tcCO2

◦ 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 sec120 sec

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120 sec

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

Diagnostic AHI 64, SpO2 < 90% for 10% sleepTcCO2 > 50 mmHg for 70% of sleep

Persistent hypoventilation on BiPAP◦ Elevated tcCO2

◦ Borderline SpO2

◦ Sleep fragmentation

What other options do we have?

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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 H2O

◦ IPAP max (initial) 20 cm H2O

IPAP max 25 cm H2O

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

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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

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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

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A: documentation in the record of◦ Neuromuscular disease

◦ Severe thoracic cage abnormality

B: gas exchange/pulm function:◦ Awake PaCO2 > 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 PaCO2 > 45 mmHg AND

Spirometry FEV1/FVC > 70% and FEV1 > 50% predicted AND

PaCO2 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

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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 YouKristie.Ross@case.edu