Paediatric Long Term Ventilation
Canada 2010
A Review
Conflicts:
Financial
Nil
Bias
Definitely
Ian MacLusky MBBS, FRCP(C)
Children’s Hospital of Eastern Ontario
Ottawa
Paediatric Long Term Ventilation
Outline
1.
2.
3.
4.
Current State
Rationale
Structure
Common Problems
Ventilation i.e. Rx of hypercapnoea by MV
Not respiratory support (CPAP /BiPAP for OSA / CHF)
Emersen “Iron Lung”
Rancho Los Amigos Hospital, California. 1953
Long Term Home Ventilation
1. Improved equipment
a. Invasive
b. Non-invasive
2. Changing attitudes / expectations
a. Home better than hospital
b. Care of “invariably fatal” conditions
3. Parental education / information access
4. Home cheaper than hospital (?)
Simonds AK. Eur Resp J. 2003;22(S47):38s-46s
ANTADIR
10,000
5,000
0
http://www.antadir.com/IMG/pdf/OBSERVATOIRE_2006.pdf
Canada?
2006 Canada wide survey*
Non-invasive: c.300 patients
Invasive: c.100 patients
*Jiemin Zhu, RN, MSc (Davis M. MB.ChB.)
Hospital for Sick Children: 1990-2006
40 Invasive. 150 Non-invasive
50
New
Enrolments
Patients Referrred
40
Non-Invasive
Invasive
30
20
10
0
1990
1992
1994
1996
1998
Year
2000
2002
2004
2006
Patient Population
40 patients
Invasive
NM hypoventilation
Thoracic hypoventilation
Central hypoventilation
Obesity hypoventilation
CCAHS
Cardiopulmonary
Non-Invasive
150 patients
Outcomes (SickKids)
• Total 190 enrolled
– Mean age enrolled 8.4 yrs
– Mean duration follow up 5.8 years
• 90 still followed, 100 no longer followed
– 8 Failed Rx
– 8 Referred to local center
– 27 Improved (no longer needing support)
– 28 Transferred, aged >18 years
– 29 Died
Technologies
1. Non-invasive
a. Negative pressure (cuirasse)
b. Mask: BiPAP
2. Invasive
a. Tracheostomy
b. Phrenic Nerve Pacing
Simonds AK. Eur Resp J. 2003;22(sup 47):38s-46s
Toussaint M. Chron Respir Dis. 2007;4(3):167-177
Lewarski JS. Chest. 2007;132(2):671-6
NIPPV
Advantages
• Ease of initiation and removal: undoable!
• Preservation of airway defenses
• Patient can eat, drink and communicate
• Avoidance of complications of intubation
• Less “technology dependency”: caregiver
expertise
NIPPV
Disadvantages
• Mask uncomfortable / claustrophobic
– (poor compliance)
•
•
•
•
EPAP
Airway not protected
Air leaks
Maximum pressure (30 cm H2O)
NiPPV: Problems
*
*
Skin breakdown
Midfacial
hypoplasia
*
+
+
*
+
+
Kasey K. Chest. 2000;117:916-8
Cannot use > 12 hrs /day
NOT GUARANTEED VENTILATION
Invasive Ventilation: IMV
Pressure vs Volume cycled?
Invasive
Advantages
• Guaranteed ventilation
– Return circuit: exhaled volume
• Access to airway (but ?  need)
• Nocturnal?
– Can “cork” during day?
• Speak
• Cough
– If not able to exhale
• Passy-Muir “speaking valve”
Invasive
Disadvantages
• Surgical intervention
• Trach: Interferes with cough + auto PEEP
• Increased caregiver expertise + time
• Bypasses nose (filter/ humidify / sterilize)
• Not readily “portable”
• Not readily “undoable”
– (1/3 do come off /  NIPPV)
Alternatives?
Phrenic nerve pacing
• Portable (24 / 7) @
.
• Minimal caregiver expertise
• (Not interfere cough / speech)
BUT
•
•
•
•
Expensive ($40-$50K, + surgery)
May still need tracheostomy (?50-90%)
Not guaranteed ventilation (fixed RR/TV)
Still surgery: Complex insertion / setup
– Phrenic nerve damage
• Need intact phrenic nerve (?diaphragmatic*)
@Guilleminault C. et al Sleep. 1997;14:369-77
Patient selection?
*DiMarco AF at al. Chest. 2005;127:671-8
Long Term Ventilation:
Justification?
1.
Life expectancy (?)
–
NIPPV in NM disease
Robert D, Argaud L. Crit Care. 2007;11(2):210-219
Increased Life Expectancy?
99 patients 1980-95 (80 died)
1980-87 nil, 87-92 Cuirass, 92 on nIPPV
Yasuma F at al. Chest 1996;109:590
Eagle M Neuromusc Dis 2002;12(10):926-29
Long Term Ventilation Program:
Justification?
1.
2.
Life expectancy
Improve quality vs. quantity of life
•
•
Sleep fragmentation (NIPPV)
Blood gasses (carry over)
Robert D, Argaud L. Crit Care. 2007;11(2):210-219
Long Term Ventilation Program:
Justification?
1.
2.
Life expectancy
Improve quality vs. quantity of life
•
•
•
Sleep fragmentation (NIPPV)
Blood gasses (carry over)
QOL (?)
Markstrom A et al. Chest 2002;122(5):1695-1700
*
Long Term Ventilation Program:
Justification?
1.
2.
Life expectancy
Improve quality vs. quantity of life
•
QOL (?)
Whose evaluating?
-
Patients > family > caregivers
“It’s Okay, it helps me breathe”*
-
Disease vs. ventilation?@
*Earle RJ et al. J Child Health Care 2006;10:270-82
Noyes J. J Advan Nurse 2006;56(4):392-403
@
Mah JK Pediatr Neurol 2008;39(2):102-107
Long Term Ventilation Program:
Justification?
1. Life expectancy
2. Improve quality as well a quantity of life
3.  Costs (?)

Hospital
Impact on Hospitalization?
• 15 NM children
– Age 11.7 (3.4-17.8)
• NPPV at least 1 year
Year before vs. year after
– Days in hospital 85% (48  7.0)
– Days in ICU 68% (12  3.9)
Katz S at al. Arch Dis Child 2004;89:121-124
(Leger P at al. Chest 1994;105:100-105)
Outcomes (SickKids)
• 29 Deaths (9 BiPAP, 20 Invasive)
– Mean age enrolled 5.6 years
– Mean duration follow up 1.3 years
Invasive
Non-Invasive
Home
6
Home
6
Local Hospital
1
Local Hospital
7
SickKids ER / ward
0
SickKids ER / ward
2
SickKids ICU
3
SickKids ICU
4
9/29 deaths SickKids, 7/9 ICU
Long Term Ventilation Program:
Justification?
1. Life expectancy
2. Improve quality as well a quantity of life
3.  Costs (?)
 Hospital
Family (?)
Long Term Ventilation Program:
Family Impact?
1. Parents (mother)
a. Time + financial demands
b. Physical overburden
c. Emotional turmoil
Stress / fear
Child “different” from societal norm
Loss family privacy / independence
d. Social Isolation
Carnevale F. Pediatrics 2006;117(1):e48-e60
Heaton J et al. Health Soc Care Comm 2005;13(5):441-450
Wang K-W K. J Advan Nurs 2004;4(1):36-46
Long Term Ventilation Program:
Family Impact?
1. Parents
2. Siblings
Jealousy / resentment / rivalry
Carnevale F. Pediatrics 2006;117(1):e48-e60
Wang K-W K. J Advan Nurs 2004;4(1):36-46
Long Term Ventilation Program:
Justification?
1. Prolonged Life
2. Improve quality as well a quantity of life
3. Costs (?)
 Hospital
Family / community
Net?
Wang K-W K. J Advan Nurs 2004;4(1):36-46
What is Required to Succeed?
A Plan (prepared and agreed on in advance)
A Team
Tertiary Center
Community
Lines of communication
Continuity of care Ballangrud R. J Advan Nurs 2008;65(21):425-434
Time!
“Informed Consent” when child already trach’ed?
Parents (and child) time to digest
Identify caregivers: buy in from community
Team to review family needs / resources
But: How long is actually required?
Issues1
• When to start ventilation, and on whom?
– 16 European countries
– >10 fold variation frequency + who ventilated
Lloyd-Owen SJ at al. Eur Resp J 2005;25(6):1025-1031
Issues2
• When to start ventilation, and on whom*?
• NIPPV:
– Recurrent pneumonias (NM patients)
– Nocturnal hypoventilation (sleep disturbance)@
– Daytime hypercapnoea
– Prophylactic?
• Ineffective
• Unlikely to be tolerated
*Fauroux
B Resp Med 2009;103:574-581
@Ward S at al. Thorax 2005;60(12):1019-1024
Issues3
• When to start ventilation, and on whom?
• Invasive: ?
– No alternative (sure?)
– Potential for stability or improvement? Yes
• CCAHS
• Slowly progressive NM disease
– Rapidly progressive?
• SMA1*, tumour
– Don’t know?
No
Yes (?)
*Roper H et al. Arch Dis Child 2010;95:845-849
Problems1
Availability of Necessary Resources?
1. Equipment
a. Ventilator: provision and maintenance
How long to get?
b. “Expendables”: tubing, etc.?
c. Other?
i. Suction
ii. Oximeter
2. Trained community caregivers
How much funded?
(Great in theory:- In practice availability)?
Alternatives? i.e. Health Care Support Workers (HCSW)
3. Availability of Respite?
Problems2
Availability of Necessary Resources?
1. Equipment
a. Ventilator: provision and maintenance
b. “Expendables”: tubing, etc.?
c. Other?
2. Trained community caregivers
3. Respite?
4. Transition to Adult Care?
Need National Standards?
Optimum vs. bare minimum?
Problems3
Organization: Theoretical
Community
Caregivers
Community
MD
Community
Care
Access
Patient
Family
School,
etc
Tertiary
Care
Center
Organization: Actual
Community caregivers
Tertiary care center
Neurology
Community
MD
ER
Respirology
Paediatrics
CCAC
?
Patient
Family
Gastro/
Nutrition
Nurse
Coordinator
School,
etc
Physio,
RT, etc
Who do the parents call at 2:00 am Sat?
Ethics?
1. Non-invasive: easy
-“Undoable” therefore patient can determine
2. Invasive
- Promising what we can’t deliver?
- Prolonging life or prolonging death?
Progressive / Terminal case (SMA1, HIE)
Parents “want everything done”?
Whose needs are we meeting?
Who decides?
The courts
Brazier M. Med Law Rev 2005;13:671-8
Ethics (of Finite Resources)?
Conclusions1
• Increasing numbers children home on
chronic ventilation- NIPPV and IMV.
(success?)
• Heterogeneous population, require
complex, coordinated care.
• Significant burden
– Family
– Community (financial, personnel, knowledge)
Conclusion2
• Improvements required
– Improved coordination of healthcare
– Increased resources:
• Increased availability of community care & respite
• Increased training and availability of local health
care resources*
• Alternative to “RN” caregivers?
(Ventilation Support Worker: NHS 2007)
National minimum standards?
National Registry: outcomes?
*Hewitt-Taylor. Inten Crit Care Nurs 2004;20:93-102
http://www.longtermventilation.nhs.uk/
“Suck It Up Princess”
Renee Rodrigues
CMD
TV Ontario
www.superstarrenee.com/index.php