Paediatric Long Term VentilationCanada 2010

A Review

Ian MacLusky MBBS, FRCP(C)Children’s Hospital of Eastern OntarioOttawa

Conflicts:

FinancialNil

BiasDefinitely

Paediatric Long Term VentilationOutline

1. Current State

2. Rationale

3. Structure

4. 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 equipmenta. Invasive

b. Non-invasive

2. Changing attitudes / expectationsa. 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

http://www.antadir.com/IMG/pdf/OBSERVATOIRE_2006.pdf

10,000

5,000

0

Canada?

2006 Canada wide survey*

Non-invasive: c.300 patientsInvasive: c.100 patients

*Jiemin Zhu, RN, MSc (Davis M. MB.ChB.)

Year

1990 1992 1994 1996 1998 2000 2002 2004 2006

Pat

ient

s R

efer

rred

0

10

20

30

40

50

Non-InvasiveInvasive

Hospital for Sick Children: 1990-200640 Invasive. 150 Non-invasive

NewEnrolments

Patient Population

Invasive

NM hypoventilationThoracic hypoventilationCentral hypoventilationObesity hypoventilationCCAHSCardiopulmonary

Non-Invasive40 patients 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-invasivea. Negative pressure (cuirasse)b. Mask: BiPAP

2. Invasivea. Tracheostomyb. Phrenic Nerve Pacing

Simonds AK. Eur Resp J. 2003;22(sup 47):38s-46sToussaint M. Chron Respir Dis. 2007;4(3):167-177Lewarski 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

Cannot use > 12 hrs /day

NOT GUARANTEED VENTILATION

Kasey K. Chest. 2000;117:916-8

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*)

Patient selection?

@Guilleminault C. et al Sleep. 1997;14:369-77*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?

Yasuma F at al. Chest 1996;109:590Eagle M Neuromusc Dis 2002;12(10):926-29

99 patients 1980-95 (80 died)1980-87 nil, 87-92 Cuirass, 92 on nIPPV

Long Term Ventilation Program:Justification?

1. Life expectancy

2. 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. Life expectancy

2. 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. Life expectancy

2. 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 yearYear 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 turmoilStress / 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):441-450Wang K-W K. J Advan Nurs 2004;4(1):36-46

Long Term Ventilation Program:Family Impact?

1. Parents

2. SiblingsJealousy / 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 CenterCommunityLines of communicationContinuity of care

Time!“Informed Consent” when child already trach’ed?Parents (and child) time to digestIdentify caregivers: buy in from communityTeam to review family needs / resources

But: How long is actually required?

Ballangrud R. J Advan Nurs 2008;65(21):425-434

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?

• CCAHS• Slowly progressive NM disease

– Rapidly progressive?• SMA1*, tumour

– Don’t know?

*Roper H et al. Arch Dis Child 2010;95:845-849

Yes

No

Yes (?)

Problems1

Availability of Necessary Resources?1. Equipment

a. Ventilator: provision and maintenanceHow long to get?

b. “Expendables”: tubing, etc.?c. Other?

i. Suctionii. Oximeter

2. Trained community caregiversHow 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 maintenanceb. “Expendables”: tubing, etc.?c. Other?

2. Trained community caregivers3. Respite?4. Transition to Adult Care?

Need National Standards?Optimum vs. bare minimum?

Problems3

Organization: Theoretical

Patient

Family

CommunityMD

CommunityCare

Access

School,etc

CommunityCaregivers

TertiaryCare

Center

Organization: Actual

Patient

Family

NurseCoordinator

Respirology

Paediatrics

Gastro/Nutrition

Physio,RT, etc

NeurologyCommunity

MD

CCAC

School,etc

Who do the parents call at 2:00 am Sat?

?

Tertiary care centerCommunity caregivers

ER

Ethics?Ethics?1. Non-invasive: easy

-“Undoable” therefore patient can determine2. 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?

Brazier M. Med Law Rev 2005;13:671-8

The courts

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 Ontariowww.superstarrenee.com/index.php