January 21 2017 Marriot Biscayne Bay Miami Fl Lisa F Wolfe MD Northwester University Feinberg School of Medicine Chicago Illinois Overview Day Nose Mouth Saliva Oral Care Chest wall ID: 759089
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Slide1
Respiratory Care in ALS
University of Miami ALS Annual SymposiumJanuary 21, 2017Marriot Biscayne BayMiami, Fl.
Lisa F. Wolfe MD
Northwester University Feinberg School of Medicine
Chicago, Illinois
Slide2Overview
DayNoseMouthSalivaOral CareChest wallLung Airway RecruitmentPositioning
Day to Night
Night
Noninvasive ventilation
Devices
Masks
Monitoring
Politics
Slide3Nose
MedicationsSprays Astelin – for thick nasal secretionsAtrovent – for watery nasal secretionsLimitation – limited ability to sniffPillsAvoid DecongestantsNon- sedating antihistamineOTC – Zyrtec, Allegra, ClaritinSedating antihistamineOTC – Benedryl, Chlor-Trimeton etcAlternative anti- allergyRX – SingulairDevicesLittle suckerNebulizerHumidifier
Slide4Aspiration is the common mechanism for pneumonia development for those with chronic illness, aging or in the hospitalClearing flora from the airway will decrease bacterial burden within the lung
Mouth
IMAJ 2003;5:329±332
Slide5Mouth
Oral HygeineOral Bacteria and PneumoniaDental CleaningsChlorhexadinHydrationSuctionNo Bite V
Slide6Mouth - Saliva
Thick vs Thin
ThickHumidifierSteam showerTable top facialSaline NebulizerThinMedicationsInjectionsSurgery Radiation
Why am I to wet and to dry at the same time?
Slide7Mouth - Saliva
Thick Saliva/ Dry MouthArtificial SalivaImproved HydrationConcord Grape JuicePapayaSteam/ HumidityLemon DropsAvoid Dairy
Slide8Mouth - Saliva
SurgeryRadiationScopolamine patchIpratropium- Spray / NebTricyclic antidepressants amitriptyline imipramine clomipramine
Neural pathways for salivary secretion. Journal of Clinical Gastroenterology. 39(2):89-97, 2005 Feb
Injecting botulinum toxin (Botox or Myobloc) into the parotid glands is one alternative for Acetylcholine blockade
Slide9Vocal Cords
Vocal Cord Spasticity may present as “cough” or a “wheeze” in the neuromuscular patient.These episodes are scary with a feeling of in ability to inhale.
Speech therapy will not be enough in the setting of neuromuscular disease
Therapy:Lorazepam IntensolBaclofen
Slide10Chest Wall - Range of Motion
Physical Therapy
Chest wall stiffnessMechanismIntra - articular AdhesionsImproved work of breathingPain preventionWhen to start ?Is it ever to early?
Lung Volume Recruitment
Mechanisms
Prevent basilar atelectasis
Reduced work of breathing
Techniques
Ambu
with breath stacking
Cough assist
Meta- Neb
Therapy Vest
Slide11Chest Wall
Chest Wall changes due to disuse: Osteoperosis, extraarticular contractures, intraarticular adhesionsAmerican Review of Respiratory Disease. 128(6):1002-7, 1983 Dec
Slide12Chest Wall - ROM
Physical Therapy
Slide13Chest Wall - ROM
Lung Volume Recruitment
Slide14Positioning
Mobility solutions make a difference
Breathing and cough is better when the body is straight
Slide15Day to Night
NightInsomniaNoninvasive ventilationDevices MasksMonitoringPolitics
Slide16Why do we care about sleep?
Muscle factors
In “Dream Sleep”Natural muscle weaknessLaying downReduces the ability of “Accessory Muscles” to assist with breathingReduces the ability to move
Brain factors
Smaller breaths
Occasional failure to send the signal to initiate a breath
CO2 elevation occurs because of changes in control of breathing
Slide17Insomnia
Non – Medical options
Avoiding napsEncourage Bright lightActivityImproved respiratory supportElevating the head of bedWhat is “anxiety”?Stretching – the need to moveFirm mattressMind clearing exercise Cognitive behavioral therapy
Medical Options
Treat pain and spasticity
Consider medications that may help with saliva AND sleep (amitriptyline)
Consider medications that will not impact breathing
Doxepin, The “Z’s”,
Ramelteon
Slide18Ventilation Options
Non-Invasive Ventilation
Uses a “mask” interfaceShould be started early based on lung function testsSlow progression of muscle weakness and reduction of lung functionTherapy for ALS not just a way to cover symptomsSleep testing is not a “need to”
Invasive Ventilation
Requires an internal airway
Increases cost and availability of caregivers
May increase infections
Will not stop the progression of ALS
Slide19Ventilation Options
Non-Invasive Ventilation
Invasive Ventilation
Slide20Ventilation Options
Non-Invasive Ventilation – SIP ventilation
Reduces CO2Allows for improves speech and coughAn alternative to a trach for 24 hour ventilation
Marie-Eve
Bédard
and Douglas A
McKim
Respiratory Care October 2016, 61 (10)
1341-1348
Slide21Ventilation Options - Monitoring
Oxygen monitoring
CO2 monitoring
Slide22Monitoring
Device Downloads
What we look for Hours of useMask leakSize of breathsFrequency of breaths Breathing pausesHow do we lookModemsCardsThe device it self
Slide23Ventilation Options
What does a mechanical ventilator have to offer?
What does a RAD offer?
Slide24Ventilation Options
What does a mechanical ventilator have to offer?
Battery SafetyPortabilitySip VentilationCO2MortalityBreath stackingLVRCoughSwallow Communication24 hour NIVConsCostHumidityRemote controlSleep lab are not prepared
What does a RAD offer?
Less expensive
More convenient for travel
Smaller
Quieter
Better humidity
Remote access and control *
Cons
Covered under the
medicare
BID
Fewer companies can provide
Guidelines to obtain the device are more challenging
Slide252016 – OIG report
Because of increased billing for home based ventilators with mask, expenditures surged from 2009 to 2015, increasing 89-fold (from $3.8 million to $340 million)No change in cost for RADS over the same period
Slide262014 – CMS OIG report
Reimbursement
Floor &Ceiling / mo.
Slide272014 - CMS starts “imminent death criterion”
Some limitations are placed on the use of Vent with Mask
Ventilators are covered for the treatment of neuromuscular diseasesthoracic restrictive diseaseschronic respiratory failure consequent to chronic obstructive pulmonary diseaseVentilators are considered “reasonable and necessary” only when the pt has a severe condition in which the interruption of respiratory support would be life-threatening or lead to serious harm.
I
nterruption of respiratory support would be life-threatening or lead to serious harm =
Imminent death after 4 hours without the vent
Slide282016 – OIG report
Slide292016 – Where do we go from here?
RAD guidelines need to be fixed
We believe that for may patients, NPPV is appropriate and if we can make it easier this may solve the problem
Options to
update guidelines
–
Drop the need for BOTH hypercapnia AND hypoxemia –
allow one or the other
Use
compliance
as a marker for successful / necessary therapy to continue home use
Broaden the use of
ST devices
Acknowledge that NPPV with back up rate (ST or PC devices) are the standard for in-patient care. If these have been successfully used on an in
pt
basis they should be available on an out patient basis
Slide302016 – Where do we go from here?
When should we continue to use home based full MV?
Greater than 8 hours per night of use : Use outside the nocturnal period supports the need for portability and backup battery. In NMD use of portable MV with oral or nasal interfaces can prolong life and delay the need for tracheostomyHypcapnea: PaCO2 > 45 (even with nocturnal NIV) suggests need for daytime support and the use of a portable MVIn NMD use of daytime support in this group has been shown to stabilize vital capacity, and improve survival. Hypoxemia: If PaO2 is < 60 or O2 saturation is <88-92% while awake breathing room air, and a trial of either mask or mouth piece ventilation is shown to normalize oxygenationDaytime dyspnea: In NMD resting modified Borg score of >2.5 as been demonstrated to be a harbinger of the development of daytime hypercapnea and risk of developing a need for round the clock ventilatory support. Speech: In NMD he presence of a reduced VT will cause poor speech volume and early fatigability with speaking.
NMDRC letter to CMS 6-24-2015
: https://www.namdrc.org/sites/default/files/files/NAMDRC%20Coding%20Change%20Ltr.pdf
Peter Gay
Slide312016 – Where do we go from here?
When should we continue to use home based full MV?
Swallow: In NMD those with fatigue and microaspiration augmented tidal volume can improve safety with eating. Very Low Lung Function (FVC < 30%) : In NMD this finding predicts the development of daytime hypercapnea, which should be addressed with the initiation of daytime mouthpiece sip ventilation or mask based daytime ventilatory rest.Nocturnal RAD failure: In those who fail to normalize oxygenation and/ or ventilation during sleep with a NPPV at optimal settings, considered a MV to allow for higher pressures or volume cycled modes.9. Alarms: In young children, or those with very unstable medical conditions, robust alarm systems are needed and this may require the use of a MV.
NMDRC letter to CMS 6-24-2015
: https://www.namdrc.org/sites/default/files/files/NAMDRC%20Coding%20Change%20Ltr.pdf
Slide32Support / Thanks