What is really needed in monitoring children on Long Term Ventilation

1. What is really needed in monitoring children on Long Term Ventilation? Peter Fleming May 2004

2. Monitoring children on LTV: Purpose of monitoring Prevent complications of treatment or the underlying condition Optimise ventilatory support as the child grows and develops Maintain as normal a life as possible Support and facilitate the family and carers Minimise hospital admissions

3. Monitoring Children on LTV Children on LTV are first and foremost CHILDREN. - i.e. The support and monitoring should aim to give them and their families as positive and normal an experience of life as possible.

4. Monitoring children on LTV Depends on the nature of the underlying condition: Airway problems - e.g. tracheobronchomalacia Neuromuscular- e.g DMD, SMA, Myopathy Respiratory control disorder- e.g. CCHS, Rett, PWS, Joubert Pulmonary - e.g. BPD, CF, Other -e.g. complex cardiac, metabolic.

5. Monitoring children on LTV Multiprofessional Home-based General Specific to LTV Specific to underlying condition Hospital based

6. Monitoring children on LTV: Multiprofessional reviews Led by community paediatrician Involves family and carers Attended by hospital based staff also Every 6-12 months Structured care plan reviewed and restated.

7. Monitoring children on LTV:Home based monitoring Adequacy of ventilatory support – record SpO2 and ETCO2 – every 3-6 months when well – more frequently when unwell – e.g. capnocheck oxygen monitoring alone is NOT adequate, as significant under- or over- ventilation will be missed. Monitoring without recording will also potentially miss significant problems

8. Monitoring children on LTV:General Growth Development Underlying condition Effectiveness/safety of support – people and equipment.

9. Monitoring children on LTV:Specific to LTV Adequacy of ventilatory support ECG Echocardiogram Blood pressure (Chest Xray) Lung function

10. Monitoring children on LTV: Specific to the underlying condition CCHS – other autonomic problems – e.g. Hirschsprungs, temperature, fluid balance, hormonal,arrhythmias, tumours, Neuromuscular – muscle tone & power, mobility, deformity, swallowing, secretions……

11. Monitoring children on LTV: Hospital based monitoring I Review progress of underlying condition Assess continuing need for support Assess adequacy of current support, and modify ventilator settings Non-invasive assessment of cardiorespiratory status – e.g. ECG, Echocardiogram, Lung function

12. Monitoring children on LTV: Hospital based monitoring II Assess spontaneous ventilation – Vt, f, Ve, awake, in REM and in Non-REM sleep – direct measurement plus measurement of SPO2 and ETCO2. NB: ventilation is generally worse in REM in neuromuscular disorders or in airway obstruction, whilst in CCHS it is worse in Non-REM sleep.

13. Monitoring children on LTV: Hospital based monitoring III Assess residual hypoxic respiratory drive – safest by giving inhaled oxygen and monitoring effect on VT, frequency and Ve – or by monitoring ETCO2. Children with depressed CO2 responses – e.g. CCHS, or chronic neuromuscular disorders – may rely on hypoxic drive, and develop severe respiratory failure if given inhaled oxygen.

14. Monitoring children on LTV: Hospital based monitoring IV ? Assess response to inhaled CO2.- less important – technical difficulties - ? Steady state (i.e. central chemoreceptor) or immediate (i.e airway +/- peripheral chemoreceptor). - Predominantly a research tool at present.

15. Monitoring children on LTV: Hospital based monitoring VI Assess adequacy of current support, and modify ventilator settings – may involve use of invasive blood gas measurements – though seldom needed. Non-invasive assessment of cardiorespiratory status – e.g. ECG, Echocardiogram, Lung function

16. Monitoring children on LTV: Problems I. Non-invasive ventilation: very difficult to record ETCO2 – capnocheck is promising, but not fully validated – need to view and check the wave form to assess accuracy All ETCO2 monitors tend to under-read in Non-invasive ventilation

17. Monitoring children on LTV: Problems II. No commercially available suitable equipment to measure VT or Ve in children. Extreme difficulty of measuring VT or Ve in Non-invasively ventilated children

18. Monitoring children on LTV: Problems III. Children seldom sleep normally when in hospital – need a combination of home and hospital studies to assess condition and care.

19. Monitoring children on LTV: Problems IV. Deciding when to switch from invasive to non-invasive respiratory support Risks/benefits of two modes Child’s cooperation – invasive ventilation may be more comfortable – child must want to change! Risk of mid-facial injury from face mask -? Up to what age

20. Monitoring children on LTV: Purpose of monitoring Prevent complications of treatment or the underlying condition Optimise ventilatory support as the child grows and develops Maintain as normal a life as possible Support and facilitate the family and carers Minimise hospital admissions

21. Monitoring children on LTV Thanks to all the children and their families.