Optimizing Respiratory Care in ALS

Jennifer Armstrong, RN, MSN, MHALisa Wolfe, MD

Northwestern MedicineDivision of Neuromuscular Medicine

Les Turner/Lois Insolia ALS Center

November 6, 2014The ALS Association 2014 Clinical Conference

Overview

• Hypoventilation

– Testing

– NIV

• Airway Clearance

• Sialorrhea

• Comfort Tips

• Emergency Preparedness

Hypoventilation

Testing

• Restrictive Thoracic Disorders – FVC<50% or

– MIP<-60 or

– O2 sats <88% for >5 mins. or

– PaCO2 >45

• Early initiation of therapy using multiple modality testing– improves survival in ALS (2.7

vs. 1.8 yrs)

– MIP criterion

– FVC attained in the supine position

– overnight oximetry testing

Lechtzin, N., et al., Amyotroph Lateral Scler, 2007. 8(3): p. 185-8.

NO

PSG

Hill N, Leger P, Criner G. Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation—a consensus conference report. Chest. 1999;116:521-534.

A consensus conference was convened by the National

Association of Medical Directors of Respiratory Care in Washington, DC, on February 4 and 5, 1998.

Spirometry

Over night shows

desaturation in clumps.

Most likely cause is stage REM related

central hypoventilation-

apnea

5 mins. <88% to qualify for NIV

Overnight Oximetry

Likely REM related hypoventilation

Total time less the 88% is >5 min

Non-Invasive Ventilation

• Spontaneous (S)– All patients with NMD should have a back up rate

• Spontaneous/Timed (S/T)– Use both patient and device breathing, but Ti time is

not assured in each breath

• Pressure Control (PC)– Inspiratory time is guaranteed with both device and

patient triggered breaths

• Volume Assured Pressure Support (VAPS)– increases pressures to meet patient needs in an

automated fashion, still set mode of S/T or PC

Modes

Setting Options: Ti for S/T & PC Mode

Respironics• S/T –

– A total inspiratory time (Ti) is set on the device; however, the patient only receives this guaranteed time during the apnic breaths.

– During spontaneous breaths the Ti is not employed.

• PC –• A total inspiratory time (Ti) is set

on the device and is guaranteed and fixed as the Ti time during both apnic and spontaneous breaths.

• Autotrack vs Autotrack Sensitive

Resmed• S/T –

– The Ti time applies to every breath spontaneous or device delivered due to apnea.

– The Ti is set with a window of Ti minimum and Ti maximum. • The breath cannot end before

the Ti min

• The breath cannot continue after the Ti max

– If the Ti min is short essentially all breaths are spontaneous

– If the Ti min is long then this is the same as PC mode – every breath is given the window and will be supported.

Setting Options: Auto Modes for NMD

AUTO MODES TO USE

• In the US, options include:– Volume Assured Pressure

Support Devices

• Respironics :

– AVAPS – Average Volume Assured Pressure Support

• ResMed:

– iVAPS - Intelligent Volume Assured Pressure Support

AUTO MODES TO AVOID

• Anything with the name “Auto”, it may seem that they have the ability to provide ventilation……….

but they don’t– VPAP auto

– Bipap auto

– Aflex auto

– Servo Ventilation

4

5

6

7 8

9

1

0

11

12

1

3

14

1

5

Consider VAPS Functionality

• REM to NREM changes

• Worsening disease

• PAP/ pressure intolerance

• Monitored PAP initiation is not available

• Severe aerophagia

Time

Vo

lum

e =

cc/

kg

IDB

wt

IPA

P =

cw

p

8

How to Set Up VAPS

AVAPS1. Choose a mode: S/T or2. Choose an EPAP

1. In NMD minimize EPAP (4 or 5)

3. Choose a goal Target Tidal Volume1. For the average patient set at 8 cc/

kg ideal body weight based on height.

2. For those with: bulbar disease, stiff chest wall, pressure intolerance set at 6 cc/ kg ideal body weight based on height.

4. Set IPAP minimum and maximum1. For de-conditioning set the IPAP

min low2. For most set the IPAP min close to

the target

5. Set Back Up Rate/ Ti min / Rise6. Set Flow Trigger

IVAPS1. Choose an EPAP

1. In NMD minimize EPAP (4 or 5)

2. Set a target alveolar volume based on height (see IVAPS calculator)

3. Set Pressure Support min and max1. For de-conditioning set the PS min

low2. For most set the PS min close to the

target

4. Set back up rate 5. Set trigger/cycle/ rise/

Timin/Timax window6. Learned targets is an option but

may be inappropriate in NMD***

PC

http://www.ardsnet.org/node/77460

IVAPS Calculator

• Input the height

• Set the back up rate a smidge higher then you think and lower than spontaneous

• Then, pick either a vt/kg ideal body weight OR specific vt goal

• Then hit calculate to get the Alveolar volume (Va) to input on the device settings

What Does “AVAPS” Mean?

A Box A Mode An add on

This NIV is named the “AVAPS” it can provide many

modes S/ST/PC/T

This NIV is named the Trilogy it can provide an unique

MODE called “AVAPS- AE”

Also has MPV, 2 channels, Battery Back Up

Both of these devices can provide “AVAPS”function as an ADD-

ON to any mode such as ST/PC or S

Consider Other Ventilation Modes

AVAPS - AE

• This adds the ability to set auto modes for BOTH:– EPAP based on an algorithm to

resolve upper airway obstruction

– PS based on an algorithm to assure, on average adequate tidal volume

– Back up rate is monitored with a goal based on alert rate****

– HOWEVER: in NMD – you don’t have to worry about the auto EPAP because their upper airway will not collapse (they are not strong enough to collapse it) and the AVAPS-AE is in ST mode

Kiss Ventilation Mouth Piece Ventilation (MPV)

• Daytime ventilation support

• Used for – Relief of acute dyspnea

– Improve speech

– Improve swallow

– Assist with cough

– Assist with clearing sinuses

Consider Other Ventilation Modes

MPV• Recommended settings thanks to Doug McKim and

Carole LeBlanc (Ontario)

• MPV Support System (PN 1102862)– MPV : ON

Tidal Volume (Vt) : larger than patient’s spontaneous Vt, enabling LVR to maximum insufflation capacity (MIC) within 2-3 stacked breathsBreath Rate (BPM) : 0 if the patient has sufficient ventilator free breathing time (VFBT) and up to 12 if more dependentInspiratory Time : 1.2 to 1.5 seconds (adjust as per comfort and desired patient peak inspiratory flow (PIF); PIF will be dependent on VtFlow Pattern : Ramp or Square (adjust as per comfort)PEEP : 0 cmH20Low Inspiratory Pressure : 1-2 cmH20High Inspiratory Pressure : up to 70 cmH20 (for optimal LVR). To allow for LVR up to MICApnea and Circuit Disconnect alarms MUST be enabled if patient has limited ventilator free breathing time or if close monitoring is required.Informed consent is recommended for alarm settings

Trilogy Order WordingTrilogy - Software Version 13.2

PrimarySet AVAPS-PC Mode, Vt=*** ml, IPAP min 8, IPAP max 15, EPAP 4, Back-up Rate 12, Rise 6, Ti min 1.0s

Flow Trigger - AutoTrak SensitiveRate of Change - ***Alarms Off

Secondary:MPV Support System (PN 1102862)Passive circuit (absent active exhalation valve)Mode of ventilation : Assist / Control (A/C)MPV : ONTidal Volume (Vt) : ***ml = larger than patient’s spontaneous Vt, enabling LVR to maximum insufflation capacity (MIC) within 2-3 stacked breathsBreath Rate (BPM) : 0 if the patient has sufficient ventilator free breathing time (VFBT) and up to 12 if more dependentInspiratory Time : 1.2 to 1.5 seconds (adjust as per comfort and desired patient peak inspiratory flow (PIF); PIF will be dependent on VtFlow Pattern : Ramp or Square (adjust as per comfort)PEEP : 0 cmH20Low Inspiratory Pressure : 1-2 cmH20High Inspiratory Pressure : up to 70 cmH20 (for optimal LVR)To allow for LVR up to MICApnea and Circuit Disconnect alarms MUST be enabled if patient has limited ventilator free breathing time or if close monitoring is requiredInformed consent is recommended for alarm settings

Swift LT w/ chinstrap, Heated Humidifier

Download monthly - email .pdf to jarmstrong@nmff.org and shesser@nmff.org or fax to 312-695-3166

Update Mask, Hoses, Filters, Humidifier Chambers Routinely.

Consider Mechanical Ventilation Modes

Benefits of Sip Ventilation

• Prolongs survival

• Stabilizes vital capacity

• Improves hypercapnea

• Augments cough

• Improves VC

Improvement in CO2

M. Toussaint; Eur Respir J 2006; 28: 549–555

Consider Mechanical Ventilation Modes

Why use a vent?

• Battery– Use for more

then 12 hours a day

• Concern for disease progression– ALS

• Need for very high pressures

Why not use a vent?

• Very high cost• CMS may

reduce access

• Reduced number of vendors in a post competitive bid world• May see a

180 on this issue

Negative Pressure Ventilation

• Negative ventilation– Modern negative pressure devices

• Diaphragm Pacers– Not a mode of ventilation

– These devices are there to help reduce muscle loss

• Biphasic Cuirass Ventilation– Will still have potential upper airway

obstruction

– Comfort has been an issue due to a square wave form

– Some clinics are using these devices for bulbar patients that have failed NIV

• Porta Lung– Older devices are no longer manufactured

however there are still patients in the community using these original devices.

NIV Monitoring - Downloads

• Compliance

• Mask Fit

• Efficacy

• Many different types of software

• Mask fitting goals change with type of device– Resmed – 24 L/min

– Respironics – Time out of range

• Efficacy Goals– Tidal Volume

– % spontaneous Trigger

C

M

E

Step 1: Tidal Volume Assessments

Work of Breathing

WOB is high if the respiratory rate is much higher than the set rate and if the shallow breathing index is higher than 60.

To calculate work of breathing, use the shallow breathing index: f/Vtf=average respiratory rateVt=average tidal volume

To decrease the WOB, may need to increase the IPAP, ti min, or increase the back up rate. Other airway clearance modalities may be considered to decrease the total work of breathing.

http://www.ardsnet.org/node/77460

Kleopa, K.A., Sherman, M.,

Neal. B., et al. (1999). Bipap

improves survival and rate of

pulmonary function decline in

patients with ALS. Journal of

Neurological Science. 64:82-88

Step 2: Usage Assessments

NIPPV INTERFACE DESENSITIZATION STEPS

1) Wear the mask at home while awake for 5-10 minutes at a

time, goal of one hour each day.

2) Attach the mask to the NIPPV device, and switch the unit

“on". Practice breathing through the mask for short segments

while watching television, reading or performing some other

sedentary activity. Goal is four hours a day.

3) Use the NIPPV during scheduled naps at home. Goal

remains four hours a day.

4) Use NIPPV during initial 4 hours of nocturnal sleep.

5) Use NIPPV through an entire night of sleep.

Comfort Features

– Heated wire circuits

– Hose lift system

– Under chin design

• Magnetic Clips for Hand Issues

Skin care

• REM- ZZZ

• Desitin

• Gecko

• Acclovate

• Replace cushion regularly

Step 3: Mask Leak Assessments

Qureshi, M.M., et al. (2007).

Increased incidence of deep

venous thrombosis in ALS.

Neurology. Vol. 68: 76-77.

Step 4: Minute Ventilation

Assessment

Assess minute ventilation.

The measurement may be 5-8

L/min.

High minute ventilation may

indicate pain, fever, infection,

pulmonary embolism, or high

caloric needs.

Review previous reports to

compare trends in the minute

ventilation and discuss

symptoms with patient.

Step 5: Pulse Oximetry

Assessments

Some NIPPV machines

incorporate pulse oximetry. With

others, overnight pulse oximetry

may need to be ordered separately

for monitoring efficacy.

Pulse oximetry will assess if the

NIPPV is providing the correct

support to maintain oxygenation

at greater than 90% throughout

the night.

Address complaints of dyspnea by

checking pulse oximetry. May be

indicated to monitor 24 hour pulse

oximetry depending on usage of

NIPPV.

Daytime use may be indicated.

Step 6: Apnea/Hypopnea

Assessments

The Apnea/Hypopnea Index (AHI)

should remain at zero if the NIPPV

device is correcting the sleep disordered

breathing associated with ALS.

An abnormality in the AHI usually

indicates a need to increase the EPAP.

With ALS, the EPAP should be low as

to keep the WOB low.

A high apnea index in ALS may indicate

the need to increase the back-up rate or

adjust the trigger setting.

A high hypopnea index in ALS may

indicate the need to increase the IPAP

setting.

The target tidal volume in

ALS is 6-8 cc/kg of ideal body weight.

ALS- NIV: Issues Impacting Non-compliance

• ALS patients diagnosed and followed over a 4-year time period.

• Tolerance was six times more likely in limb-onset than bulbar-onset ALS patients, with a trend toward reduced tolerance in those with lower forced vital capacity

• Age, gender, and duration of disease were not predictors of NIV tolerance.

Gruis KL. (2005). Muscle & Nerve. 32(6):808-11.

FTD related Non Compliance with NIV contributes to an impressive decrease in survival.

Forshew, D. (2005). The effects of executive and behavioral dysfunction on the course of ALS. Neurology. 65:1774–1777.

ALS- NIV: Issues Impacting Non-compliance

Barriers

Airway Clearance Protocol

• Rhinitis/Sinus/Oral Hygeine• Positioning• Salivary Control• Upper Airway Health• Non-Invasive Ventilation• Nebulizer (face mask)• Abdominal Muscle Support• Lung Volume Recruitment or Breathstacking (w/Ambu®)• High Frequency Chest Wall Oscillation• Mechanical In/Ex-sufflation

– Manual (w/Ambu®)– Device

• Nutritional Support

Oral Cavity

• Rear roof of mouth becomes dry as tongue motility decreases

• Keep bacteria down– Diluted mouthwash (baking soda

varieties)

– Mouth Swabs

– Oral Rinse Systems (Waterpik®)

– Suctioning during or after

brushing• Attachments or Separate Units

Positioning

• Use medication to decrease acid reflux

• Preventing aspiration

– Meal Positioning• Upright at 90 degrees

• Chin tuck with swallowing

– Sleep Positioning• Not laying down until 30-60 minutes

after meals

• Head of Bed up 30-45 degrees during sleep

Salivary Control

• Medication Management– Tricyclic Antidepressants– Scopolamine Patch or Gel– Drops or Compound

Pharmacy Preps

• Injecting botulinum toxin (Botox® or Myobloc®) into the parotid glands and/or submandibular glands is one alternative for Acetylcholine blockade

Salivary Control

• Thick Saliva/Dry Mouth

– Improved Hydration

– Hypertonic Saline Nebs

– Artificial Saliva Sprays

– Concord Grape Juice

– Papaya Enzyme/Juice

– Meat Tenderizer

– Steam/Humidity

– Avoid Dairy Products

Salivary Control

• Portable Suction Device with a Yankaur suction wand

– Use mouthwash to color and freshen secretions in canister

– Consider portable power sources for extended trips from home

Neotech Little Sucker Nasal Tip Aspirator

Hydration and Humidity

• Mucus and other secretions respond to hydration and humidity– Humidify living spaces (cool

temp w/ Hepafilters)

– Humidify NIV and Oxygen Sources (prevent rain-out)

– Encourage 6-8 glasses of liquids in addition to meals per day

Laryngospasm

• Triggers– Reflux– Nasal Drip– Saliva– Particles in Airway

• Spasm of the vocal cords can occur with fatigue, dehydration or chemical airway reactivity– Maintain hydration and humidity– Utilize energy conservation in speech – Can use fast-acting benzodiazepines for muscle

relaxation of spasms

Nebulizer Therapy

• Inability to generate adequate flow for metered dose inhalers

• Use a facemask due to

lack of facial muscle strength

• Medications can help to thin out the mucus and saliva making it easier to mobilize

• Covers nose to help dry nasal secretions

Abdominal Binders

• A meta analysis of vital capacity’s supports that the use of abdominal binders improves upright breathing.

Wadsworth, BM, et al. (2009) Abdominal binder use in people with spinal cord injuries: a systematic review and meta-analysis. Spinal Cord 47, 274–285.

Lung Volume Recruitment

• Breath Stacking

• Chest wall range of motion

• Complete opening of basilar lung segments

LVR: Improves Outcomes

McKim, DA. (2011). American Thoracic Society, Presentation.

Breath-stacking Exercises

• Encourages Chest Wall Mobility & Opens Air Sacs

– Improves tolerance with other therapies later in disease

– Quality of Life Exercise

• Unassisted

– Sitting tall, 10-15 slow deep breaths 2-3 times per day

• Assisted

– Using resuscitation bag to give stacking breaths

• Perform 10 breaths, 2-4 times per day

• Can be done by self or by caregiver

– Can use mouthpiece or facemask

Lechtzin, N., et al. (2006). Supramaximal Inflation Improves Lung Compliance in Subjects With Amyotrophic Lateral Sclerosis. Chest, 129(5), 1322-9.

High Frequency Chest Wall Oscillation

Hill-Rom®

ElectroMedRespirTech®

HFCWO

• Although there is early data in ALS and other motor neuron diseases– In the setting of children/young adults with neuromuscular

disease the HFCWO has been effective in reducing mucus plugging and reducing respiratory complaints

– Adults with spinal cord injury and post polio syndrome have also been reported to have good success with the device

– Studies with ALS have shown an improvement in quality of life and have reduced progression of disease when initiated with FVC 70% - 40%.

Lange, et al. (2007). Early use of non-invasive ventilation prolongs survival in subjects with ALS. Amyotrophic Lateral Sclerosis. 8: 185–188.

HFCWO

• Match device to patient body type– Vests and Wraps are sized from infant to obese– Presence of gastrostomy may indicate use of different

interface

• Begin at low settings– Desensitize patient to oscillations

• Start at a frequency of 9 and pressures at 6 (traditionally are 14, 10)

• Monitoring– Compliance Monitoring– Demand Monitoring

HFCWO Documentation

• Electronic Medical Records

– Smart Phrases

• Orders• Progress Notes

• “Due to a neuromuscular disease, Mr. X has an impaired ability to clear secretions. A high frequency chest wall oscillator is medically necessary to clear secretions and prevent respiratory infections which may lead to unnecessary hospitalizations. Mr. X is unable to tolerate positioning required for other devices and is unable to create enough expiratory force to use other devices due to his reduced vital capacity.”

HFCWO Order Wording

• High Frequency Chest Wall Oscillator

• Use low profile wrap garment

• Lifetime Use = 99 months or 30-day trial

• Set frequency at 8-12 htz

• Use 10-20 minutes, 1-2 times per day

Metanebs• Inpatients – S/P PEG or ICU Stays

Insufflation-Exsufflation

• MI-E (Mechanical Insufflator-Exsufflation) is able to generate clinically effective Peak Cough Flow

• Except those with bulbar dysfunction – severe dynamic collapse of the upper

airways during the exsufflation cycle.

• Although now we have PAP on Pause

Insufflation-Exsufflation

Sancho, J. et al. (2004) Chest 125:1400-1405

Oropharynx CT scan of a bulbar ALS patient with PCFMI-E < 2.7 L/s. Left, A: baseline. Right, B: during the exsufflation cycle.

Insufflation - Exsufflation

• Tracheostomy use of the device• Attach inline, adjust pressure to patient comfort

• Oral use of the device– Mouthpiece or Facemask

• Clear Nasal Secretions Using Facemask

– Manual Cycle vs Automatic Cycle

– Start at pressures of 35cm on + and – pressures• Increase in increments of 5cm to patient comfort

Documentation

• Electronic Medical Records

– Smart Phrases

• Orders

• Progress Notes

• “Due to a neuromuscular disease, Mr. X has an impaired and ineffective cough. A mechanical in/ex-sufflator is medically necessary to clear secretions and prevent respiratory infections which may lead to unnecessary hospitalizations.”

MI-E Order WordingCough Assist T70Length of Need = 99 mos

Cough Therapy:Provide Mouthpiece and FacemaskStart pressures @ +/- 35cm H2O & titrate in increments of +/-

5cm to pt comfort; Inhalation Time 2.0s; Exhalation Time 2.0s; Pause Time 2.0s; all settings may be titrated to patient comfort - perform 4-6 cycles BID routinely, may perform as much as needed for cough.

Advanced Options:• Clearing Nasal Secretions: use with facemask, use

mouth open through inhalation; close mouth through exhalation to blow out through the nose.

• For Daily Lung Volume Recruitment (LVR or Breathstacking): use manual mode for inhale only, 4-6 cycles BID

• Encourage use for Cough Therapy or LVR before meals to improve breathing.

• Replace circuit and interface every 30 days for 12 months.

VitalCough

Length of Need = 99 mos

Cough Therapy:

Provide Mouthpiece and Facemask

Start pressures @ +/- 35cm H2O & titrate in increments of +/-5cm to pt comfort; Inhalation Time 2.0s; Exhalation Time 2.0s; Pause Time 2.0s; PAP on Pause at 6-10cm H2O; all settings may be titrated to patient comfort -perform 4-6 cycles BID routinely, may perform as much as needed for cough

Advanced Options:

• Clearing Nasal Secretions: use with facemask, use mouth open through inhalation; close mouth through exhalation to blow out through the nose.

• For Daily Lung Volume Recruitment (LVR or Breathstacking): use manual mode for inhale only, 4-6 cycles BID

• Encourage use for Cough Therapy or LVR before meals to improve breathing.

• Replace circuit and interface every 30 days for 12 months.

Not for Everyone, but…

• Toolbox

• Portability

Effort of Airway Clearance

• Activity requires energy

• Ensure adequate caloric intake

• Metabolism Monitoring

• Involve the dietician with respiratory efforts

Power Optionshttp://www.cpap.com/productpage/resmed-power-station-battery-kit-s9-cpap-machines.html

Portability

Take Charge Not Chanceshttp://www.ventusers.org/vume/

Non-Invasive Issues, Too!

Thanks to Our Team!

Thank You!!!

jarmstrong@nmff.org