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King’s MND Care & Research Centre

Guidelines : Non Invasive Positive Pressure Ventilation
Increasing respiratory muscle weakness may present insidiously, and signs and
symptoms may be subtle and easily overlooked. Clinical vigilance and serial
measurements of vital capacity (VC) can provide a planned approach to managing
respiratory problems.

Diagnosis of respiratory insufficiency
Symptoms (see Appendix 1) may include:
 dyspnoea on exertion
 excessive fatigue
 disturbed sleep
 orthopnoea
 daytime hypersomnolence
 morning headaches
 impaired cognition
 decreased appetite
 “nocturia” secondary to frequent sleep arousals

1.2 Physical signs may include:
 breathlessness on exertion
 breathlessness at rest
 weak cough/sniff
 use of accessory muscles
 paradoxical movement of the abdomen on inspiration

Initial clinical measurements which may be helpful in diagnosis:
 Slow VC (lying and standing)
 Sniff nasal pressures
 serum bicarbonate
 blood gases (if possible)
NB – if there are symptoms and the clinician strongly suspects respiratory
compromise, despite reasonable vital capacity and serum bicarbonate levels,
then the patient should be referred to respiratory medicine for a diagnostic


Clinic procedure for monitoring respiratory function


Assessment of symptoms and physical signs
Respiratory monitoring form (Appendix 2) to be completed at each clinic visit
for any patient with respiratory symptoms or a VC of less than 70% of
predicted. Examination of the patient for physical signs if any concern.
(NB. Sensitivity is needed in asking and examining for respiratory signs and
symptoms – in early stages a patient may not be aware of any change and be
very fearful of this.)

but for accurate measurement of diaphragm strength and blood gases. 3. physiotherapy with techniques such as assisted cough. Prior to referral to the respiratory team.3 The need for referral to the community and palliative care teams for home management of NIPPV and the important role played by the primary carer should also be briefly discussed. 3. after all these points have been raised. sex and height.1 Slow VC to be done at initial and subsequent clinic visits and recorded on respiratory monitoring form. If. If possible. this may not be for a full assessment for non-invasive positive pressure ventilation (NIPPV). 2.3 Sniff nasal pressures if machine available. it will be necessary to refer for further respiratory assessment to the department of respiratory medicine (see below). (Written information . but the latter may not be easily done in the clinic.2. In either case this should be documented in the notes).2 Initial measurements in clinic setting 2. treatment of chest infections. 2. Measurements should be given as a % of that predicted for age. then a formal referral for a full assessment . At this stage. the following points may need to be covered: 3.1 Treatment options include medication to alleviate breathlessness.Appendix 3 to be given about this to be given if appropriate). even at this early stage.3 Further respiratory assessment Where there are signs and/or symptoms and it is not possible to adequately measure VC or blood gases in the clinic.King’s MND Care & Research Centre Page 2 Ventilation Guidelines 2. or may be omitted due to patient distress. VC should be measured both standing/sitting and lying (NB – measurement of VC may be impossible due to poor bulbar function. (Appendix 2).2 The initial discussion of NIPPV must include the need for a full assessment by the respiratory team for NIPPV suitability This should include a description of the possible assessment procedures such as balloon trans-diaphragmatic pressure measurement and overnight polysomnography. the patient is keen to be further investigated for NIPPV suitability.2 Serum bicarbonate and chloride levels and/or blood gases can be helpful.2. and initiation of NIPPV if appropriate. 2. Discussion of the management of respiratory problems in the clinic 3.4 The long-term implications of NIPPV.2. tracheostomies and end of life decisions should be broached. 3.

King’s MND Care & Research Centre Ventilation Guidelines by the respiratory team should be made. Page 3 .

4.King’s MND Care & Research Centre Page 4 Ventilation Guidelines 4. this discussion should include the neurologist. plus the patient’s telephone number and GP details. particularly with regard to decisions about tracheostomy. .results and liaison with community services 5.2 If NIPPV indicated. These should be sent directly to Professor Leigh/Dr Shaw. This is particularly important if the patient lives alone and has no full-time carer at home. (Ideally.e. The following should be included in this discussion:        Need for carer(s) to be involved Practical problems (nose ulceration.1 Referral letter to Professor John Moxham. Need for early palliative care involvement for all patients receiving NIPPV The pros and cons of an Advance Directive (see Appendix 4). a member of the respiratory team. 5. and contain brief clinical details and copies of any relevant letters. It should also state the degree of urgency of the referral. Referral for an assessment for NIPPV 4. end of life decisions. having discussed all of the above.1 Obtain results of investigations done in respiratory medicine. DN and palliative care team prior to seeing patient. KCH. palliative care. leaking mask.2 This referral letter should be sent within one week. 5. Department of Respiratory Medicine. If the patient is keen to proceed. Possibility of 24 hrs dependence on NIPPV Management of symptoms once NIPPV not effective – i. Post assessment . power failure etc) NIPPV will not prevent disease progression. Some district nursing services offer a “twilight” evening service and may agree to visit every evening to put the NIPPV mask on the patient if there is no carer. admission for a trial of NIPPV will need to be organised. symptom relief. requesting respiratory assessment (with copy to GP and to palliative care team if referral to the latter has already been made). and at some point breathing difficulties will not be helped by NIPPV. care co-ordinator/MND nurse specialist and patient with primary carer). 5. but this may not be possible in all areas. to check that they are happy to support the patient at home with a NIPPV.3 Arrange a clinic appointment as soon as possible (and within 4 weeks of testing) to discuss NIPPV. contact GP.

6. admissions office. For audit purposes. This will involve contacting the GP. telephone contacts and any problems identified should be recorded in the medical notes. Appendix 5) 6. ward. and pressure ulcers developing over the nose where the mask sits. (Identify who will do this and see discharge planning form for NIPPV.3 Patient and carer must be given written information about NIPPV and appropriate contact numbers for trouble-shooting (MND clinic numbers. if they are not already aware of patient’s admission.1 Bed to be booked on Golla (ideally for a Monday–Friday admission).2 A clinic appointment should be offered following NIPPV initiation. Common problems include ill-fitting masks. The timing of this will depend on the patient’s condition. Any problems should be discussed with the department of respiratory medicine. or as frequently as needed depending on the level of support in the home and other professionals involved.King’s MND Care & Research Centre Page 5 Ventilation Guidelines 6. district nurse and local palliative care team. Follow-up contact following NIPPV initiation 7. All patients should be followed up with telephone contact by MND nurse specialist/care co-ordinator within 1 week of discharge from the ward. patient and carer. Telephone contact should then be offered. and the King’s switchboard number to request the on-call Firm A registrar for out-of –hours emergency contact) 7. . 7. respiratory medicine numbers.1 Telephone contact. and contact made with the district nurse/palliative care team. Liaison with respiratory medicine (? who will ventilate).2 Plan discharge as soon as patient is admitted. and continued on a monthly basis. but should be within 2 months and may need to be much sooner than this. Hospital admission for NIPPV trial 6.

it can be assumed that the NIPPV is effective.2 Ear-lobe blood gases should be arranged at the follow-up clinic appointment. We should remember that people may change their minds regarding these decisions. 8.1 Discussion of discontinuation of ventilation needs to be addressed in the context of palliative care (and should have been initially discussed when initiating NIPPV).2 Some patients may wish to make an advance directive to clarify their position regarding end of life decisions. A key aspect of NIPPV is its’ impact on quality of life both for patients and carers. using the SF36. 8.1 Information about the efficacy of ventilation can be gained by asking basic questions about sleep. Where an advance directive is wanted. and overnight oximetry may be necessary to confirm efficacy. and perceived breathlessness. (This can be done by either the neurologist. Monitoring efficacy of ventilation 8. 9. The MNDA leaflet for GP’s “Motor Neurone Disease – A problem solving approach” contains a useful section on dealing with dyspnoea and choking (Appendix 6) . including patients receiving NIPPV. 9. and at three monthly intervals during routine neurological follow-up. absence of morning headaches. Coping with the progression of the disease in the context of ventilation 9. mood.3 Quality of life. concentration. energy levels and appetite. NIPPV alone will not alleviate all breathlessness and when a decision to discontinue NIPPV is reached medication should be offered to ease dyspnoea and anxiety. (MND/respiratory nurse specialist could be taught to do these). 9. This should be monitored before initiating NIPPV. As the disease progresses. or MND nurse specialist). specialist respiratory nurse. we would recommend using the Patients Association leaflet “Advance Statements About Future Medical Treatment” (Appendix 4). and that any advance directive will need to be regularly reviewed. Patients and their carers should be included in all discussions and management decisions.King’s MND Care & Research Centre Page 6 Ventilation Guidelines 8. particularly regarding tracheostomy and discontinuation of NIPPV. If many of the presenting symptoms have been alleviated.3 Medication to alleviate breathlessness and anxiety should be considered for all patients who experience these symptoms. Protocols may need to be developed in conjunction with local palliative care teams and hospices.

King’s MND Care & Research Centre Ventilation Guidelines APPENDIX 1 SIGNS AND SYMPTOMS OF RESPIRATORY INSUFFICIENCY Symptoms include:              Dyspnoea Orthopnoea Excessive fatigue Disturbed sleep – frequent awakenings. tachycardia “Nocturia” secondary to frequent sleep arousals Physical signs and clinical examination includes: In Neurology Out-Patient Clinic:       Dyspnoea on exertion Dyspnoea at rest Use of accessory muscles of respiration Weak cough Paradoxical movement of the abdomen on inspiration Slow Vital Capacity measurements – standing/sitting and lying Record both actual measurement and predicted for sex & height (< 50% of predicted likely to cause symptoms) Record both standing/sitting and lying (>25% difference in VC means diaphragm weakness likely) In Respiratory Medicine:        Nocturnal oximetry Full polysomnography Measurement of trans-diaphragmatic pressures Maximum inspiratory pressure Vital Capacity –standing and lying Blood gases Serum bicarbonate levels . aggression Recurrent or chronic URTI’s Sweating. nightmares Morning headache Daytime hypersomnolence Reduced appetite Depression/anxiety Cognitive changes – poor concentration Personality changes – irritability.

sitting Observed Value PO2 (kPa) PCO2 (kPa) Bicarbonate Chloride Sniff nasal pressure (cms water) % Predicted Date ? Significant Date .NO: DOB: VC AT FIRST VISIT: DATE: Signs and Symptoms Date Noted Dyspnoea on exertion Dyspnoea at rest Orthopnoea Weak cough Accessory muscle use Paradoxical movement of abdomen Morning headaches Daytime hypersomnolence Disturbed sleep Reduced appetite Depression/anxiety Recurrent/chronic URTI’s Sweating/tachycardia Reduced concentration/cognitive changes Personality change/irritability/aggression CLINICAL MEASUREMENTS: Measure Slow VC – standing Slow VC .King’s MND Care & Research Centre Ventilation Guidelines APPENDIX 2 RESPIRATORY MONITORING FORM NAME: HOSP.

No: Main Carer: Ward: Admission Date: Discharge Date: Consultant: Date of NIPPV Initiation: Dr.King’s MND Care & Research Centre Ventilation Guidelines APPENDIX 5 NIPPV DISCHARGE PLANNING FORM Name: DOB: Hosp. OT/ Physio/ SLT/ dietitian/ social services/ carers) Name of person completing this form: Date: .g. Initiating NIPPV: Phone/bleep: GP: Date contacted: Address: Tel: District Nurse: Date Contacted: Address: Tel: Palliative Care: Date Contacted: Hospice: Address: Tel: Home Care: Date Contacted: Named Nurse: Tel: Others: (e.

King’s MND Care & Research Centre Ventilation Guidelines .

King’s MND Care & Research Centre Ventilation Guidelines APPENDIX 4 ADVANCE STATEMENTS ABOUT FUTURE MEDICAL TREATMENT (ADVANCE DIRECTIVES) A Guide for Patients Published by the Patients Association 1996 .

King’s MND Care & Research Centre Ventilation Guidelines APPENDIX 6 MANAGEMENT OF DYSPNOEA AND CHOKING (from the Motor Neurone Disease Association leaflet Motor Neurone Disease – A problem solving approach 1998 2nd Edition The MND Association) .

King’s MND Care & Research Centre Ventilation Guidelines NIPPV .SUMMARY AND AUDIT SHEET 1) Diagnosis and Assessment Done? Date Why not done (code) Done? Date Why not done (code) Done? Date Why not Done (code) Respiratory monitoring form completed(appendix 2): At first appt Follow up 1 Follow up 2 Follow up 3 Follow up 4 Follow up 5 Follow up 6 2) Measurements in clinic setting Slow Vital Capacity Measurements 1st Appt Sitting/Standing Follow up 1 Follow up 2 Follow up 3 Follow up 4 Follow up 5 Follow up 6 Lying Sitting/Standing Lying Sitting/Standing Lying Sitting/Standing Lying Sitting/Standing Lying Sitting/Standing Lying Sitting/Standing Lying Serum bicarbonate Serum chloride Blood gases Sniff pressures If signs and/or symptoms 3) Discuss management and referral for further assessment Discussion in clinic of respiratory assessment procedures Written information given about assessment procedures Initial NIPPV discussion in clinic(document in notes) Discussion of need for carer involvement & palliative care Discussion of long term implications of NIPPV If patient agrees 4) Referral to respiratory medicine for assessment .

King’s MND Care & Research Centre Ventilation Guidelines Referral letter to Professor Moxham within one week 5) Post assessment – results and liaison with community Done? services Date Contact community services pre NIPPV GP DN Pall.3 guidelines) Why not done (code) If patient agrees: 6) Hospital admission for NIPPV trial Done? Date Why not done (code) Done? Date Why not done (code) Done? Date Why not done (code) Bed booked on Golla Liaison with respiratory medicine re Dr to ventilate Discharge planning sheet completed Written information given to patient/carers 7) Follow-up contact following NIPPV initiation Telephone contact within 1 week of discharge Clinic appointment within 2 months of discharge 8) Monitoring efficacy of ventilation Subjective information from patient/carer Ear lobe blood gases Overnight oximetry Quality of life information – SF36 Before NIPPV 1st F/Up appt 2nd F/Up appt 3rd F/UP appt 4th F/Up appt 5th F/Up appt 6th F/Up appt CODES FOR ACTIVITY NOT DONE 1 2 3 4 5 6 Not applicable/appropriate Patient declined Forgotten Patient left before activity done Not enough time to do No staff available to do .Care Clinic appointment for patient discussion < 4 weeks Document in notes what is discussed (see 5.

King’s MND Care & Research Centre Ventilation Guidelines 7 No equipment available APPENDIX 3 .