Martin Samuels

University Hospital of

North Staffordshire

why?

when ?

how ?

where?

what with?

Failure of

resp pacemaker

nerve conduction

muscle contraction

airway patency

gas exchange

combination

CCHS

infection, trauma

DMD, SMA

severe TBM

CLD of prem, CF

neurodisability,

obesity

at presentation:

birth

after trauma

after infection

with acute

respiratory illness

eg

CCHS, CLD

operative, RTC

myelitis

myopathy, CF

Conditions Presenting with

Progressive Respiratory

Failure

myopathy

neurodisability

obesity

Hereditarymuscular dystrophiesmyopathiesspinal muscular atrophyhereditary sensory nmyotonic dystrophy

Work with colleagues in muscle disorders

AcquiredpolioGuillain-Barrepolymyositismyasthenia gravis

Dubowitz et al (from JTSMA)

myotonic dystrophy

spinal muscular atrophy

II

neuropathy

nemaline rod myopathy

Duchene MD

- median fall FVC 0.18L/y

- median survival 3.1y

- 5y survival 8%

Phillips et al, 2001

early recognition

symptoms

LFT’s

SaO2 & CO2 monitoring

awareness of treatment options

SenTecSaO2 & tcPCO2

Capnocheck SaO2 & ET-CO2

Apr 2002 - 14y - DMD care plan for terminal care: no CPR,

intubation or ‘active’ intervention referral for respiratory assessment

May 2002 Found unresponsive at home… A&E CO2 found to be 11.6 kPa

May 2002

admitted for sleep study

would not wake: pCO2 27 pH 7.0

bagged: pCO2 19

nPPV no better – agreed not for ETT

family counselled

Intubated for 3/7

Prednisolone

Extubated

Discharged nPPV

Cough Assist

Insidious onsetpatients appear normal when awake

REMsleep

all sleep awake

• Nocturnal arousals

• Behavioural and cognitive problems

• Daytime drowsiness / poor concentration

• Failure to thrive

• Morning headaches

• Recurrent / severe LRTI’s

• Cor pulmonale (late)

Fall in VC hypoxaemic-apnoeic episodes rise in CO2 & fall in SpO2 in REM sleep first lastly, during day

Overnight record of SaO2 & CO2 ? age 10 – 12y ? VC <30%, 50%, 60% …

SaO2

Whole night: 8h

Heart rate

Transcutaneous pCO2

Mail questionnaire: Canadian physicians

Response rate 45/60

25% do not discuss mech vent with all

patients & families

Most frequently cited reason for advising

against / withholding ventilation was

poor quality of life (52.6%)

progressive respiratory failure quality of life reduced:

symptoms repeat / severe LRTI hospitalisation

compliance likely

improves symptoms

keep out of hospital

ease care by parents

reduce complications

use in overall care

plan

“No purpose” situation: degree of physical or mental impairment will be so great that it is unreasonable to expect them to bear it

“Unbearable” situation: child and/or family feel that in the face of progressive and irreversible illness further treatment is more than can be borne

Assessment Consult Discuss with family “Decisions must

never be rushed and must always be made by the team with all evidence available.”

Hospital v Home

training

troubleshooting

adjustments

PICU v HDU v ward

Breas Vivo

Respironics Synchrony

Resmed VPAP

B&D Nippy

Face v nasal mask v prongs

Sizing Humidity Complications Monitoring

Mask intolerance

Skin sores

Dry eyes

Rhinitis

Air swallowing

? Facial deformity

30 second page

physiotherapy

immunisation

antibiotics

nutrition

Rx of GORD

in-exsufflator

Why are we initiating this?

Have we consulted / discussed?

How are we going to do it?

Where are we doing this?

What are we going to monitor?

What about discharge?