Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset – NIV
Baseline respiratory assessment by neurologist or palliative care consultant at diagnosis
(spirometry – FVC and symptoms1).
Offer just in case kit and physiotherapy referral if appropriate.
Assess psychological state and commence discussion of advance care planning 2 regarding
NIV, feeding, escalation of care, social issues if appropriate.
Discuss with patient whether they would
like to pursue non-invasive ventilation
(when appropriate) for symptom relief
and/or prolongation of life.
Continue symptom
management.
Consider completing
Advance Decision to
Refuse Treatment (pack
available from MND
Association).
Inform GP and others.
No
Yes
Significant
swallowing
difficulties
Monitoring of respiratory function at
3 monthly clinics/by relevant
professionals as needed
(FVC and SpO2)
Discuss insertion of feeding tube:
(See Nutrition Pathway)
Respiratory function
deteriorating (or FVC<50%) or
respiratory symptoms
If respiratory function
deteriorating rapidly, refer
as ‘urgent’
If cough
compromised
Cough
augmentation
pathway
Referral to local respiratory consultant (Dr
Shaw East Dorset, Dr McConnell West Dorset)
Further assessment and discussion including
advance decisions/advance care planning2
Consider providing Just In Case Kit (formerly
Breathing Space Kit) via MND Association.
Out patient
assessment
required
Referral to Respiratory
Centre, University
Hospital Southampton
(UHS)
Regular review – if FVC<50% pred,
check ABGs and overnight oximetry
approx 3 monthly.
Symptoms of dyspnoea,
orthopnoea, early morning
headaches, daytime
fatigue or sleepiness
In patient
assessment
required
Referral to UHS
or Papworth
Hospital (if no
bed at UHS)
± PaCO2 > 6 kPa.
Non-invasive ventilation
provided if appropriate.
Follow up by local respiratory
centre if appropriate.
If patient requests invasive
ventilation, see separate
pathway.
Follow up by respiratory
specialist centre
End of life care pathway3
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset
1. Symptoms which should prompt reassessment of respiratory function:
Table 1 Symptoms and signs of potential
respiratory impairment
Symptoms
Breathlessness
Orthopnoea
Recurrent chest infections
Disturbed sleep
Non-refreshing sleep
Nightmares
Daytime sleepiness
Poor concentration and/or memory
Confusion
Hallucinations
Morning headaches
Fatigue
Poor appetite
Signs
Increased respiratory rate
Shallow breathing
Weak cough
Weak sniff
Abdominal paradox (inward movement of the
abdomen during inspiration)
Use of accessory muscles of respiration
Reduced chest expansion on maximal inspiration
(The use of non-invasive ventilation in the management of motor neurone disease, NICE, 2010)
2. Advance decisions to refuse treatment/advance care planning:
It is advisable to have advance decisions concerning which circumstances will trigger
withdrawal of the ventilator (Gonzalez-Lorenzo & Diaz-Lobato, 2000; Miller et al, 1999;
Polkey et al, 1999; Moss et al, 1996).
3. Withdrawal of NIV
See ‘Guidelines for withdrawal of NIV’
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset – Invasive
Ventilation
Inpatient
Outpatient
Patient invasively
ventilated as emergency
or before diagnosis of
MND made.
Ventilator dependent via
tracheostomy
Referral to Respiratory
Centre, UHS for NIV
respiratory support
Consider referral to
Papworth for weaning to
NIV
NIV commenced
Fails
Consider advance decisions/care
planning2 and patients mental
capacity
Patient requests long term
invasive ventilation is
considered
Clinical decision by
medical team regarding
the appropriateness of
invasive ventilation
Follow up by respiratory
specialist centre
Inappropriate
Explain to patient
and family
Appropriate to take further
Discussion with patient and family about
whether ventilation would or would not
achieve goals of patient and/or family
Relevant commissioning body contacted by local
service or by respiratory specialist centre for CHC
panel approval
Discuss advance decisions2 and care planning
with patient to establish circumstances where
ventilation will be withdrawn




Liaise with ENT to place tracheostomy
Consider referral to respiratory centre for
advice, ongoing care and follow up
Purchase ventilators
Training of carers
Consider referral to
respiratory centre for
advice, ongoing care and
follow up
Follow up by respiratory
specialist centre/local
centre as appropriate
End of life care pathway
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset
Local contact details:
Bournemouth/Christchurch
Respiratory consultant
Respiratory physiotherapist:
West Dorset
Respiratory consultant
Dr T Shaw
Ian Hills
Charlotte Church
01202 303656
01202 726095
01202 704403
Dr W McConnell
01305 255270
Southampton respiratory team:
Respiratory centre (office hours)
02380 794325
Out of hours advice
If this is thought to be necessary patient will have
out of hours pager number. If this is not available,
advice can be obtained via Southampton Hospital
respiratory registrar on call.
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset
Further information available:
For people with MND:
MND Association information sheet 14A: How motor neurone disease (MND) might
affect your breathing
MND Association information sheet 14B: Ventilation in motor neurone disease (MND)
MND Association information sheet 14C: NICE clinical guidelines for non-invasive
ventilation (NIV)
MND Association Information sheet 14D on trouble shooting problems when using
Non Invasive Ventilation
MND Association Information sheet 14E on air travel for people with MND and those
using Non Invasive Ventilation
MND Association information sheet 19: Advance Decisions to Refuse Treatment
(ADRT) explained
MND Association Advance Decision to Refuse Treatment full pack
MND Association information sheet 22D: NHS continuing healthcare
For professionals:
Guidelines for the withdrawal of NIV
MND Association information sheet P6: Management of respiratory symptoms
MND Association information sheet P7: Management of respiratory insufficiency DVD
MND Association reading list on respiratory problems
MND Association information sheet P4A MND Just in Case kit (JIC kit)
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Respiratory Care Pathway for People Living With
Motor Neurone Disease in Dorset
This pathway has been drawn up using the following evidence/guidelines:
Gonzalez-Lorenzo & Diaz-Lobato (2000) Mechanical ventilation in patients with
amyotrophic lateral sclerosis. Revista de Neurologia Jan 1-15;30(1):61-4.
Miller RG, Rosenberg JA, Gelinas DF, et al (1999) Practice parameter: The care of
the patient with amyotrophic lateral sclerosis (An evidence-based review). Muscle
Nerve., 22:1104-1108.
MND Association (2005) Summary of Guidelines for respiratory management in
MND/ALS
http://www.mndassociation.org/document.rm?id=291
Moss AH, Oppenheimer EA, Casey P, Cazzolli PA, Roos RP, Stocking CB, Siegler
M. (1996) Patients with amyotrophic lateral sclerosis receiving long-term mechanical
ventilation. Advance care planning and outcomes. Chest, 110(1):249-255.
National Institute for Clinical Health and Excellence (2010) Motor neurone disease:
The use of non-invasive ventilation in the management of motor neurone disease
http://www.nice.org.uk/nicemedia/live/13057/49885/49885.pdf
Polkey MI, Lyall RA, Davidson AC, Leigh PN,Moxham J. (1999) Ethical and clinical
issues in the use of home noninvasive mechanical ventilation for the palliation of
breathlessness in motor neurone disease. Thorax 54: 367-71.
This pathway has been circulated to the following for comments:
Dr Tim Shaw
Dr Mark Allenby
Dr Will McConnell
Dr Jane Wilkinson
Dr A Lekkas
Sarah Ewles
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013
Iain Hills/Charlotte Church
These pathways have been drawn up with input from all
commissioners and service providers involved.
AF/TS/SD/CH/RP/RS /KD
Final version Dec 2011
Review Dec 2013