Transcript Powerpoint - University of Miami ALS Clinical and

University of Miami ALS Annual Symposium
January 21, 2017
Marriot Biscayne Bay
Miami, Fl.
Respiratory Care in ALS
Lisa F. Wolfe MD
Northwester University Feinberg School of Medicine
Chicago, Illinois
Overview
Day
• Nose
• Mouth
• Saliva
• Oral Care
• Chest wall
• Lung
• Airway
Recruitment
• Positioning
Night
Day to Night
• Noninvasive
ventilation
• Devices
• Masks
• Monitoring
• Politics
Nose
• Medications
• Sprays
• Astelin – for thick nasal secretions
• Atrovent – for watery nasal secretions
• Limitation – limited ability to sniff
• Pills
• Avoid Decongestants
• Non- sedating antihistamine
• OTC – Zyrtec, Allegra, Claritin
• Sedating antihistamine
• OTC – Benedryl, Chlor-Trimeton etc
• Alternative anti- allergy
• RX – Singulair
• Devices
• Little sucker
• Nebulizer
• Humidifier
Mouth
• Aspiration is the common
mechanism for pneumonia
development for those with
chronic illness, aging or in the
hospital
• Clearing flora from the airway will
decrease bacterial burden within
the lung
IMAJ 2003;5:329±332
Mouth
• Oral Hygeine
• Oral Bacteria and Pneumonia
• Dental Cleanings
• Chlorhexadin
• Hydration
• Suction
• No Bite V
Mouth - Saliva
Thick vs Thin
• Thick
•
•
•
•
Humidifier
Steam shower
Table top facial
Saline Nebulizer
• Thin
•
•
•
•
Medications
Injections
Surgery
Radiation
Why am I to wet and to dry at the
same time?
Mouth - Saliva
• Thick Saliva/ Dry Mouth
•
•
•
•
•
•
•
Artificial Saliva
Improved Hydration
Concord Grape Juice
Papaya
Steam/ Humidity
Lemon Drops
Avoid Dairy
Mouth - Saliva
Surgery
Radiation
Scopolamine patch
Ipratropium
- Spray / Neb
Tricyclic
antidepressants
• amitriptyline
• imipramine
• clomipramine
Injecting
botulinum
toxin (Botox or
Myobloc) into
the parotid
glands is one
alternative for
Acetylcholine
blockade
Neural pathways for salivary secretion.
Journal of Clinical Gastroenterology. 39(2):89-97, 2005 Feb
Vocal 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 Intensol
• Baclofen
Chest Wall - Range of Motion
Physical Therapy
• Chest wall stiffness
• Mechanism
• Intra - articular Adhesions
• Improved work of breathing
• Pain prevention
• When 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
Chest Wall
• Chest Wall changes due to
disuse:
• Osteoperosis,
• extraarticular contractures,
• intraarticular adhesions
American Review of Respiratory Disease.
128(6):1002-7, 1983 Dec
Chest Wall - ROM
Chest Wall - ROM
Lung Volume Recruitment
Positioning
Breathing and cough is better when
the body is straight
Mobility solutions make a difference
Day to Night
Night
• Insomnia
• Noninvasive
ventilation
• Devices
• Masks
• Monitoring
• Politics
Why do we care about sleep?
Muscle factors
• In “Dream Sleep”
• Natural muscle weakness
• Laying down
• Reduces the ability of “Accessory
Muscles” to assist with breathing
• Reduces 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
Insomnia
Non – Medical options
• Avoiding naps
• Encourage
• Bright light
• Activity
• Improved respiratory support
• Elevating the head of bed
• What is “anxiety”?
• Stretching – the need to move
• Firm mattress
• Mind 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
Ventilation Options
Non-Invasive Ventilation
Invasive Ventilation
• Uses a “mask” interface
• Should be started early based on
lung function tests
• Requires an internal airway
• Increases cost and availability of
caregivers
• May increase infections
• Will not stop the progression of
ALS
• Slow progression of muscle
weakness and reduction of lung
function
• Therapy for ALS not just a way to
cover symptoms
• Sleep testing is not a “need to”
Ventilation Options
Non-Invasive Ventilation
Invasive Ventilation
Ventilation Options
Non-Invasive Ventilation –
SIP ventilation
• Reduces CO2
• Allows for improves speech and
cough
• An alternative to a trach for 24
hour ventilation
Marie-Eve Bédard and Douglas A McKim
Respiratory Care October 2016, 61 (10) 1341-1348
Ventilation Options - Monitoring
Oxygen monitoring
CO2 monitoring
Monitoring
Device Downloads
• What we look for
•
•
•
•
•
Hours of use
Mask leak
Size of breaths
Frequency of breaths
Breathing pauses
• How do we look
• Modems
• Cards
• The device it self
Ventilation Options
What does a mechanical ventilator
have to offer?
What does a RAD offer?
Ventilation Options
What does a mechanical ventilator
have to offer?
• Battery
• Safety
• Portability
• Sip Ventilation
• CO2
• Mortality
• Breath stacking
• LVR
• Cough
• Swallow
• Communication
• 24 hour NIV
• Cons
• Cost
• Humidity
• Remote control
• Sleep 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
2016 – 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
2014 – CMS OIG report
Reimbursement
Floor &Ceiling / mo.
2014 - CMS starts “imminent death criterion”
Some limitations are placed on the use
of Vent with Mask
• Ventilators are covered for the
treatment of
• neuromuscular diseases
• thoracic restrictive diseases
• chronic respiratory failure consequent to
chronic obstructive pulmonary disease
• Ventilators 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.
Interruption of respiratory
support would be life-threatening
or lead to serious harm =
• Imminent death after 4 hours
without the vent
2016 – OIG report
2016 – 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 inpatient care. If these have been successfully used on an in pt basis they should be
available on an out patient basis
Peter Gay
2016 – Where do we go from here?
When should we continue to use home based full MV?
1. 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
tracheostomy
2. Hypcapnea:
• PaCO2 > 45 (even with nocturnal NIV) suggests need for daytime support and the use of a portable MV
• In NMD use of daytime support in this group has been shown to stabilize vital capacity, and improve
survival.
3. 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 oxygenation
4. Daytime 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.
5. 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
2016 – Where do we go from here?
When should we continue to use home based full MV?
6. Swallow:
• In NMD those with fatigue and microaspiration augmented tidal volume can improve safety
with eating.
7. 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.
8. 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
Support / Thanks