Transcript Document

Long Term Ventilator Considerations in
NMS, ALS, and Pediatric Conditions:
Diagnosis and therapy
RET 2264C
Advanced
Mechanical
Ventilation
Special Thanks to:Ove Fondenes
Centre for Home Mechanical Ventilation
University Hospital of Bergen
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A Dramatic Example: Respiratory insufficiency
is a major cause of complaints and the major
cause of early death in ALS
 Mean survival time from diagnosis of respiratory
insufficiency in ALS is 15 – 20 months without ventilatory
support
 With NIV: Additional 1 y? With Trach?: … 5 - 10 y?
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Neuromuscular Diz
 Amyotrophic lateral sclerosis (ALS)
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Congenital childhood hypotonias
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Guillain-Barré syndrome
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Infant botulism
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Muscular dystrophies
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Myasthenia gravis
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Phrenic nerve paralysis
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Polio and postpolio sequelae
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Spinal muscular atrophy
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Myotonic dystrophy
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CNS Injury
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CNS Condions that may Require LTV
 Arnold-Chiari malformation
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CNS trauma
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Cerebrovascular disorders
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Congenital and acquired central control of
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breathing disorders
Myelomeningocele
Spinal cord traumatic injuries
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Other Conditions that may require LTV
SKELETAL DISORDERS
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Kyphoscoliosis
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Thoracic wall deformities
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Thoracoplasty (Surgical Tmt for TB)
CARDIOVASCULAR DISORDERS
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Acquired heart diseases
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Congenital heart diseases
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Pediatric Conditions…generally these
Patients make of the Majority of LTV Pts.
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Pediatric and Adult Conditions for LTV
Upper Airway
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Pierre-Robin syndrome
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Tracheomalacia
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Vocal cord paralysis
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Lower Respiratory Tract
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Bronchopulmonary dysplasia (BPD)
Chronic obstructive pulmonary disease (COPD)
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Complications of acute lung injury
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Cystic fibrosis
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Complications of infectious pneumonias
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Pulmonary fibrotic diseases
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Long Term Commitment To Ventilatory
Support in Many Conditions May:
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Alleviate dyspnea
Ameliorate the consequences of hypoventilation
Improve QoL
Increase lifespan
Contribute to VAPs
..few patients regret the choice of ventilatory
support
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Long Term Care Settings
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Intensive care or acute care units
Long-term acute care facilities
Extended care facilities
The patient’s home
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Ventilator Choices are Many
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First Generation Portable Volume
Ventilators
 Bear 33 (VIASYS, Critical Care Systems, no
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longer manufactured)
PB Companion 2800 and 2801(Puritan Bennett;
no longer manufactured or serviced)
LP-4 and LP-6 (Aequitron, no longer
manufactured)
LP-6 Plus, LP-10, LP-20 (Aequitron, Tyco Health
Care, Plymouth, Minn)
Lifecare PLV 100, and PLV 102
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2nd Generation Portable Ventilators
 Achieva, Achieva PS and PSO2 (Tyco Health
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Care, Plymouth, Minn)
iVent (VersaMed, Hackensack, NJ)
Newport HT-50 (Newport Medical, Newport,
Calif)
Pulmonetics LTV series (Colton, Calif)
T-Bird Legacy (VIASYS, Critical Care Systems,
Palm Springs, Calif)
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i vent, T bird and Newport HT 50,
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Ventilator Selection Considerations
 Reliability: The ventilator must be mechanically dependable
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and trouble-free for extended periods of time without breaking
down or requiring costly maintenance.
Safety: The ventilator must be safe to operate in oxygenenriched environments and have an adequate alarm system
to warn of low ventilating pressure, high ventilating pressure,
patient disconnection, and mechanical failure
Versatility: The ventilator should be portable or able to be
adjusted for travel outside the home, necessitating the use of
a reliable internal and external battery sources and alarms.
User-friendly: Ventilator controls should be easy to
understand and manipulate The circuit should be simple and
easy to change.
Easy patient cycling: The ventilator should be easy to cycle
in the VC-CMV mode; for patients with some spontaneous
effort.
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ALS Survival
%
(Kleopa et al., J Neur Sci 164, 1999: 82 –88)
100
90
80
70
60
Group 1 BiPAP > 4h/dy
50
Group 2 BiPAP < 4h/dy
40
Group 3 no BiPAP
30
20
10
months
0
0
5
10
15
20
25
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Fall in FVC (Forced Vital Capacity)
(Kleopa et al., J Neur Sci 164, 1999: 82 –88)
FVC %
100
Group 1 BiPAP > 4h/dy
Group 2 BiPAP < 4h/dy
50
- 20
- 15
- 10
-5
0
Group 3 no BiPAP
5
10
15
months from BiPAP
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QoL
Bourke, S.C., et al., Noninvasive ventilation in ALS: indications and effect on quality of
life. Neurology, 2003. 61(2): p. 171-7
50
45
SF- 36 MCS
40
35
30
Pre NIV
1
3
5
7
6
5
SAQLI
4
3
Short form 36
Sleep Apnea Quality of life index
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2
Pre NIV
1
3
5
7
QoL
Bourke, S.C., et al., Noninvasive ventilation in ALS: indications and effect on
quality of life. Neurology, 2003. 61(2): p. 171-7
25
20
ALSFRS
15
10
Pre NIV
ALS Functional Rating Scale
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3
5
7
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Non invasive ventilation
Bourke, S.C., et al., Noninvasive
ventilation in ALS: indications and effect on quality of life. Neurology, 2003. 61(2): p. 1717
 Moderate to substantial improvement of QoL
and survival
 .. in spite of disease progression
 ..indicating that treatment did NOT ”prolong
suffering”
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HMV in ALS in Norway
 Total number of ALS patients on ventilator :
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52
Prevalence: 1,8 pr 100 000 > 30 y
Some counties > 5 pr. 100 000
Typical patient ~60 y and male! (2:1 ratio to
W)
One patient treated 9 years, average 2 y
3 out of 10 patients tracheotomized
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HMV in Norway (n=763, october 2005)
Muskeldystrofier
15 %
Obstruktiv
lungesykdom
19 %
Postpolio
10 %
Pickwick
21 %
Annen
Lungesykdom
3%
ALS
7%
Skjelett
4%
Annet
14 %
SMA
4%
Tverrsnittsskade
3%
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Gender distribution
FEMALE
MALE
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Age distribution
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Type of ventilator
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Some challenges
 Bulbar patients are diffucult to treat noninvasively
 Tracheal ventilation (TV) expands life-time, but
is resource intensive and can reduce the QoL of
family
 Healthcare professionals invariably makes
wrong assumptions about the patients perceived
QoL.
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Further challenges
 Patients on TV gradually become more
dependent on ventilatory support to the point
they might be unable to communicate i.e. in a
”locked-in” state.
 .. hence, the use of advance directives is crucial.
 Cognital dysfunction may occur in 20 – 50% of
patients
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Hypoventilation
 Respiratory failure is due to weak inspiratory
effort
”Bellows”function
Diaphragm
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Hypoventilation
 Lack of the ability to maintain adequate
blood-gases due to disease outside the lung
 Carbon Dioxide (PaCO2) (hypercapnia)
initially, and gradually lowering of oxygen
  (PaO2), hypoxemia.
 Initially manifest itself during sleep
Pathological levels of pCO2 > 6 kPa (45.1 mm Hg)
Pathological levels of pO2 < ~10 kPa (75 mm Hg)
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NMD Respiratory problems
• Muscular function during INhalation
• Muscular function during EXhalation
• Upper airways, bulbar function
Ability to cough
Hypoventilation
Infection
Atelectasis
Secretion
stagnation
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Symptoms and signs of respiratory
insufficiency in NMD and ALS
Symptoms
Dyspnoea on exertion or talking
Ortopnoea
Frequent nocturnal awakenings
Excessive daytime sleepiness
Daytime fatigue
Difficulty clearing secretions
Morning headache
Nocturia
Depression
Poor appetite
Poor concentration and/or memory
Signs
Tachypnea
Use of auxillary respiratory
muscles
Paradoxical movement of
abdomen
Decreased chest movement
Weak cough
Sweating
Tachycardia
Weight loss
Confusion, hallucinations,
dizziness
Papilloedema (rare)
Syncope
Mouth dryness
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Questions to Ask The LTV Pt when they
Arrive Home
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Are you feeling anxious?
Are you getting a deep enough breath?
Does is last long enough?
Is it too deep?
To you have enough time to get all your air out?
Do you need more breaths? Or fewer breaths?
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LTV Patient Home Report Elements
 Identification of company, servicing location, date and
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time
Patient name, address, phone, email
Patient diagnosis
Physician, phone number
Prescribed equipment and procedures,
Assessment (vital signs, breath sounds, sputum
evaluation, SpO2, ventilator parameter and alarm
settings, functioning of ancillary equipment,
Caregiver and patient (if possible) comprehension of
equipment and procedures,
Compliance with plan of care
Recommendations of DME company representative
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European Measures of Blood Gases
 SI Unit of Pressure is the Pascal (N/m2 )
 1000 pascals = 1 kPa
 1 kPa = 7.5 mm Hg
 Normal PaCO2 = 4.67-6 kPa (35-45 mm Hg)
 Normal PaO2 = 10.67-13.33 kPa (80-100 mm Hg)
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Night-time measurements
Oxygen saturation= 90%
kPa
12
12
10
10
8
8
6
6 Capillary pCO2
4
24
4
Oxygen
01
02
03
04
kl
05
06
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Other Assesment tests
 Spirometry
 Vital capacity < 50 %
= increased risk of
hypoventilation
 Vital capacity < 30 % ( < 1 - 1,2 l ) = substantially
increased risk
 Sitting and lying flat
Difficult to do
measuremnts in
bulbar patients!
 Diaphragm function
 X-ray (”sniff-test”)
 Maximal pressures (In- and out-) (MIP/ MEP)
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Proposed criteria for NIV
1. Symptoms related to respiratory muscle
weakness. At least one of the following:
a. Dyspnea
b. Orthopnea
c. Disturbed sleep not because of pain
d. Morning headache
e. Poor concentration
f. Loss of appetite
g. Excessive daytime sleepiness (ESS > 9)
2. Signs of respiratory muscle weakness
(FVC < 80% or MIP <40cmH2O)
3. Evidence of either:
a. Significant nocturnal desaturation on overnight
oximetry, or
b. Morning blood-gas pCO2 >65 mm Hg..
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Ventilator choices
NIV
Bilevel CPAP
0%
8
Hazard
Self-sufficiency
100 %
TV
IPPV
Need for ventilatory support
hours
24
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NIV Options: Tank Ventilator vs. Chest
Cuirass
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Ventilator choices
Others
Pressure cycled
Volume cycled
Pressure Bi-level
Positive
Support
Ventilation Airway
Pressure devices
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The advantages and drawbacks of invasive
ventilation tracheostomy
1. Advantages
a) preventing aspiration
b) more secure ventilator – patients interface
c) ability to provide higher ventilator pressures
2. Drawbacks
a. more secretions are generated
b. impairing swallowing risk
c. increasing aspiration
d. increasing risk of infections
e. tracheoesophageal fistula
f. tracheal stenosis or tracheomalacia
g. costs
h. 24 h nursing care
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Ventilatory support – practice points
1. Check symptoms or signs of respiratory
insufficiency at each visit.
2. Measure VC and/ or MIP - If possible both
standing/ sitting and lying.
3. Nocturnal oximetry, available at home, is
recommended in patients with symptoms of
nocturnal hypoventilation.
4. Symptoms or signs of respiratory insufficiency
should initiate discussions with the patient and
the caregivers about all treatment options.
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Ventilatory support – practice points
5. NIV should be considered before Tracheal V.
6. TV undertaken only after full discussion of the
pro’s and con’s with the patient and carers.
7. Unplanned (emergency) TV should be avoided
at all costs.
8. Oxygen therapy alone should be avoided - may
exacerbate CO2 retention and mouth dryness.
9. Medical treatment of intermittent dyspnea:
a) short dyspneic bouts: relieve anxiety and give shortacting benzodiazepines
b) longer phases of dyspnea (>30 min): give morphine.
10.Medical treatment of chronic dyspnea: start
with morphine 2.5 mg orally four to six times
daily. For severe dyspnea give morphine sc or
iv infusion. Start with 0.5 mg/h and titrate.
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Evaluation of Power Requirements in the Home
 1. Place a number by each of the circuit breakers or fuses in the
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main electrical panel.
2. Using a piece of paper for each room in the home, draw a square
representing the room and mark each receptacle or light in the
room.
3. Make sure each receptacle has a small appliance or light plugged
into it. Turn on all the lights and small appliances you have plugged
in.
4. Turn off the number one circuit and note the appliances affected
(they will be off). Mark the circuit number by the receptacles and
lights in that room on the drawing for that room.
5. Turn that circuit back on and turn off the next circuit in the
numbering sequence.
6. Continue until all appliances or lights in all rooms have been
assigned to a circuit.
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Flowchart for the management of
respiratory dysfunction in ALS
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Bronchial secretions
Good practice points
1.
2.
3.
4.
5.
6.
Teach the patient and caregivers the technique of assisting expiratory
movements using a manual assisted cough (can also be performed by a
physical therapist).
Provide a portable home suction device and a room humidifier.
Consider using a mucolytic like N-acetylcysteine, 200–400 mg three times
daily.
If these measures are insufficient, try a nebulizer with saline and a b-receptor
antagonist and/or an anticholinergic bronchodilator and/or a mucolytic and/ or
furosemide in combination.
The use of a mechanical insufflator-exsufflator may be helpful, particularly in
the setting of an acute respiratory infection.
Cricopharyngeal myotomy may be helpful in the rare cases with frequent
episodes with cricopharyngeal spasm and severe bronchial secretions.
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Complications of LTV
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We will Discuss the Real World issues of Long Term
Ventilation with Milennium RTs next week....
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