Transcript Document

HOME MECHANICAL
VENTILATION: HMV GUIDELINES
Committee Members
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Doug McKim and Jeremy Road : Co-chairs
Nigel Duguid
Debra Morrison
Colleen O’Connell
Francois Maltais
Fabien Cote
Basil Petrof
Monica Avendano, Steve Abdool and Janet Fraser
Robert Skomro
Karen Rimmer
Methodologist Tom Oliver
Observer Ian MacLusky (Paeds)
Admin. Assistance CTS
Not one have a COI with material to be presented .
• Committee formed in 2008
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Evolution of technology
Emerging clinical experience
Care gaps across the country, around the world
Absence of CPG
Significant expense
Ethical issues
EUROVENT STUDY (ERJ , 2005)
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16 countries
483 centers
Wide variation in patients on HMV
Invasive v non invasive
“Lung users” < 1 year ; neuromuscular > 6 years
CTS HMV Guidelines: Disease Specific
Eleven of the areas of most significance for those requiring
Prolonged Assisted Ventilation (PAV) and addressed by the
guidelines were as follows:
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* Airway Clearance in Ventilator Assisted Individuals
* Transition to Home
Amyotrophic Lateral Sclerosis
Central Hypoventilation Syndrome
Stable COPD
Kyphoscoliosis
Obesity Hypoventilation Syndrome
Spinal Cord Injury
Duchenne Muscular Dystrophy
Muscular Dystrophies other than Duchenne and Myopathies
* Ethical Considerations
First CPG on HMV to adapt this process
Methodology
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Guideline development
– AGREE II , GRADE/Grading recommendations table from the ACCP
report, Judging the Strength of Recommendations Consensusbuilding process, external review
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Literature Search Strategy
– MEDLINE (OVID: 1980 through June 2010), EMBASE OVID: (1980
through June 2010), HealthStar (1980 through June 2010), the
Cochrane Library (OVID; Issue 1, 2009), the Canadian Medical
Association InfoBase, and the National Guideline Clearinghouse
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Study selection criteria
– outcomes of interest: survival, pulmonary function, sleep parameters,
airway clearance techniques, VAI and caregiver quality of life,
transition to home, ethical considerations
Guideline Development Process
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Grading system ( GRADE , Guyatt et al Chest 2006)
Quality of evidence : A,B or C
Strength of recommendations 1 or 2
Consensus building meetings
Established key research questions for the future.
External review process: international experts,
Canadian partners including Spinal Cord Network,
ALS and Muscular Dystrophy Societies and CRTS.
External Reviewers Comments
• Strong support for the document
• AGREE II scores were high
• 2 concerns: procedure for updating guideline and
opinions of patients were not sought
• Some wanted more information on “how to”
• Some suggestions re : recommendations and levels of
evidence .
Guideline Development Process
• Compendium (source document).
• Executive summary includes
– Synopsis of section
– Conclusions
– Recommendations with levels of evidence
Collaboration with LTMV Canadian
Community of Excellence Steering Group
• Initial meeting of working group in 2010
• CIHR funded program, April 2011. “Understanding
LTMV in Canada: A Programmatic Approach” . PI :
Louise Rose.
Dissemination and Implementation
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Publication in 2011
Target groups with shared interest
Collaboration with LTMV group
Research opportunities
Central Hypoventilation Syndrome
(CHS)
• Rare condition
• Decreased respiratory drive
• Varied etiology
Etiology of CHS
Acquired: Brainstem Disease
Cerebrovascular accident
Tumor or space occupying lesion
Syringomyelia
Post poliomyelitis
Trauma
Arnold Chiari malformation
CNS infection
Neurodegenerative processes , MSA.
Congenital CHS:
PHOX2B mutations
Perry Syndrome
Nemaline myopathy
Idiopathic
• In pediatric population 4 retrospective chart reviews
(n=6-196)
• In adults mostly case reports on acquired causes of
CHS
• In pediatric population with Congenital Central
Hypoventilation Syndrome ( CCHS) 90% have the
PHOX2B mutation
• Late onset , LO-CCHS in adults can be associated
with the PHOX2B mutation as well, less severe than
pediatric presentation
• First degree relatives should be screened as carriers
can be affected
Recommendations
• 1. The diagnosis of CHS is best established by polysomnography
supplemented with transcutaneous CO2 or early morning ABGs.
(GRADE 1C)
• 2. Once the diagnosis of CHS is established, it is strongly recommended
that acquired causes should be excluded by MRI of the brainstem.
(GRADE 1C)
• 3. Patients with CHS and no known cause should undergo genetic
screening for the PHOX2B gene mutation. (GRADE 1C)
• 4. For patients confirmed to have PHOX2B mutation, first degree
relatives should be screened for hypoventilation. (GRADE 1C)
• 5. CHS patients who require only nocturnal ventilatory support may be
managed by NIV with a back up rate or diaphragmatic pacing. (GRADE
1C)
• 6. Severe CHS mainly seen in C-CHS requires continuous ventilatory
support but daytime diaphragmatic pacing can markedly improve
mobility. (GRADE 1C)
COPD
• RCT ’s of > 3 months duration selected , 7 found.
• Clinical practice guidelines from CTS,ATS/ERS and
GOLD consulted
• 2 systematic reviews
• Outcomes : HRQL, Dyspnea, Exercise Capacity,
Sleep, Hospitalization , Mortality.
Author (Year)
Diagnosis
Blood Gas
NIV
Strumpf, 1991
(20)
FEV1 < 1L
No pre-established
criteria.
Mean PaCO2 = 49
(range 35-67)
IPAP = 15
EPAP = 2
Meecham Jones, 1995
(21)
FEV1 < 50%
predicted
PaO2 < 60 mmHG
PaCO2 > 45 mmHG
IPAP = 18
EPAP = 2
Gay, 1996
(14)
FEV1 < 40%
predicted
PaCO2 > 45 mmHG
IPAP = 10
EPAP = 2
Casanova, 2000
(15)
FEV1 < 45%
predicted
No pre-established
criteria Mean
PaCO2 = 51 mmHG
IPAP = 12
EPAP = 4
Clini, 2002
1(6)
FEV1 < 1.5L
PaCO2 > 50 mmHG
IPAP = 14
EPAP = 2
Duiverman. 2008
(17)
FEV1 < 50%
predicted
PaCO2 > 45 mmHG
IPAP = 20
EPAP = 6
McEvoy, 2009
(18)
FEV1 < 1.5L or <
50% predicted
PaCO2 > 46 mmHG
IPAP = 13
EPAP = 5
Outcomes
Author Year
(Ref)
Study Type
# of
Pts.
Dyspnea
Quality of
life
Exercise
capacity
Sleep
Mortality
Hospitalizatio
n
Strumpf, 1991
(20)
Randomized,
cross-over
NIV vs. usual
care
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No change
Not
assessed
No change
No change
Not
assessed
Not assessed
Meecham Jones,
1995
(21)
Randomized,
cross-over
NIV + LTOT vs.
LTOT alone
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Not
assessed
Improved
SGRQ
symptom
scores
No change
Improved
sleep time
and
efficiency
Not
assessed
Not assessed
Gay, 1996
(14)
Randomized,
parallel group
NIV vs. sham
NIV
13
Not
assessed
Not
assessed
No change
No
improveme
nt
Not
assessed
Not assessed
Casanova, 2000
(15)
Randomized,
parallel group
NIV + LTOT vs.
LTOT alone
52
Reduced
dyspnea
Not
assessed
Not
assessed
Not
assessed
No change
No change at 1
year
Clini, 2002
(16)
Randomized,
parallel group
NIV + LTOT vs.
LTOT alone
90
Reduced
MRC
scores
No change
in SGRQ,
improved
MRF-28
No change
No change
No change
No change
Duiverman, 2008
(17)
Randomized, parallel
group
NIV + rehabilitation
vs. rehabilitation
alone
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No further
improvement
in CRQ
dyspnea
subscale
with NIV
No further
improvemen
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CRQ score
with NIV
Larger
improvemen
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MRF-28
cognition
domain and
total
score with
NIV
No further
improvemen
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exercise
capacity
with NIV
Not
assessed
Not
assessed
Not assessed
McEvoy, 2009
(18)
Randomized, parallel
group
NIV + LTOT vs. LTOT
alone
144
Not
assessed
No change
in SGRQ
Deteriorat
ion in
several
subscales
of the SF36.
Not
assessed
Increased %
REM sleep
Improved
survival
No change
• Dyspnea- weak evidence
• HRQL-conflicting results
• Exercise capacity-2 trials showed increase in 6MWD
53 and 13m,one no change
• Sleep-conflicting results
• Mortality-2 trials no benefit and 1 a benefit (HR 0.63 ,
0.4-0.99)
• Difficult studies to perform ,ill patients with high drop
out rates and non adherence to therapy rates of 1540%.
• In Eurovent study COPD is common indication and
use increasing in some countries.
Recommendations
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The use of long-term NIV cannot be widely recommended in
patients with stable COPD. (GRADE 1B)
Long-term NIV in COPD should only be considered on an
individual basis. One subgroup of patients with COPD in which
long-term NIV could be considered are patients with severe
hypercapnia (e.g. PaCO2 greater than 55 mmHG) experiencing
repeated episodes of acute hypercapnic respiratory failure that
require in-hospital ventilatory support. However, definitive proof
of efficacy of long-term NIV in these patients will need to await
further studies. (GRADE 2C)
The overlap syndrome (concomitant COPD and obstructive
sleep apnea syndrome) should be differentiated from chronic
respiratory failure that is solely due to advanced COPD.
(GRADE 1B)
Duchenne Muscular Dystrophy (DMD)
• Studies selected included outcomes of : survival,
HRQL, hospitalizations, PFTs and ABGs.
• 11 observational studies (n=10-42)
• 8 retrospective chart reviews (n=15-243)
• 2 small RCTs (n= 60)
• Absence of dystrophin
• Decline in VC 8-12% per year
• VC < 40% or MIP < 30 cm H20,at risk for
hypoventilation
• Diurnal hypercapnia = high one year mortality and
reduced HRQL
• FVC<1L. 5 year survival is 8%.
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Largest RCT Rafael et al (Lancet 1994,343:1600)
Does early NIV improve survival (n= 35 in each arm)
Mortality > in NIV ( 8v2 ) study stopped
Problems : infections managed at home, groups not
controlled for cardiac disease, infrequent follow up
and airway clearance techniques were sub optimal.
• Vianello et al (Chest 1994,105:445)
• 10 DMD patients with daytime hypercapnia ,followed
for 2 years matched by VC and comorbidity.
• 5 refused NIV 4 died, mean survival 9.7 months
• NIV group 100 % survived at 2 years
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Danish experience
243 cases (retrospective chart review)
1988-2002 vent use increased from 0.9-43%
Mortality rate 3.8 v 9.8% in vent users
2,4,6 year survival 93,91 and 81% in vent users
• Japanese experience
• 157 DMD
• Mean age at death 31 yrs in vented group v 20 yrs .
• ABGs improve : from pCO2 60 to 45 mmHg on NIV
• HRQL improves
Recommendations for Monitoring
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Carefully question and educate patients to report symptoms
consistent with hypoventilation, including disturbed sleep,
excessive daytime sleepiness, headache and weight loss.
(GRADE 1B)
Measure VC, MIP, MEP, peak cough flow, and awake
oxyhemoglobin saturation by pulse oximetry at least yearly; if
VC < 40% predicted, also monitor awake CO2 tension by noninvasive methods or blood gas analysis. (GRADE 1B)
Perform an evaluation of respiration during sleep if there are
symptoms consistent with nocturnal hypoventilation or other
forms of sleep-disordered breathing (1B). In the absence of
such symptoms, periodic screening for sleep-disordered
breathing should also be considered once FEV1 or FVC < 40%
predicted. (GRADE 2B)
Recommendations for Treatment
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Offer nocturnal NIV to patients with diurnal hypercapnia
(daytime arterial pCO2 > 45 mmHg), or when there is
documented nocturnal hypercapnia and the presence of
symptoms consistent with hypoventilation. (GRADE 1B)
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Institution of NIV during sleep should also be strongly
considered in patients demonstrating a major degree of
nocturnal gas exchange disturbance, even if asymptomatic.
(GRADE 1C)
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When bilevel ventilation is used, back up respiratory rates are
recommended during sleep while on NIV to reduce the work of
breathing associated with spontaneous breath initiation (Grade
1C)
• Individualize the decision about the transition from nocturnal NIV
to daytime ventilation by carefully evaluating patient factors
(symptoms, bulbar involvement, patient preference, etc.) and
available resources. In patients requiring daytime ventilation,
strongly consider NIV as an alternative to invasive tracheostomy.
(GRADE 1B)
• Lung volume recruitment maneuvers should be introduced with
declining vital capacity. (see Airway Clearance section) (GRADE
1C)
• Methods to assist secretion clearance should be initiated when
peak cough flow is less than 270 l/min. (see Airway Clearance
section) (GRADE 1C)
• Thank you