Outcome of children with neuromuscular disease admitted to paediatric intensive care

Abstract

Aims: To determine the outcome of children with neuromuscular disease (NMD) following admission to a tertiary referral paediatric intensive care (PICU). Methods: All children with chronic NMD whose first PICU admission was between July 1986 and June 2001 were followed up from their first PICU admission to time of study. The outcomes recorded were death in or outside of PICU, duration of PICU admission, artificial ventilation during admission and following discharge from PICU, and readmission to PICU. Results: Over 15 years, 28 children were admitted on 69 occasions. Sixteen (57%) children had more than one admission. The median duration of PICU admission was 4 days (range 0.5-42). Twenty three per cent of unplanned admissions resulted in the commencement of respiratory support that was continued after discharge from the PICU. Severity of functional impairment was not associated with longer duration of stay or higher PRISM scores. Ten children (36%) died, with four (14%) deaths in the PICU. A higher proportion of children with severe limitation of function were among children that died compared to survivors. Conclusion: Most children with NMD admitted to the PICU recover and are discharged without the need for prolonged invasive ventilation. However, in this group of children, the use of non-invasive home based ventilation is common and they are likely to require further PICU admission.A cute respiratory insufficiency is a common complication of chronic neuromuscular disorders (NMD) such as Duchenne muscular dystrophy (DMD) or spinal muscular atrophy (SMA). A common fear is that once intubated for an acute illness, children with NMD will not recover to be weaned from ventilation.1 In a UK study, 27% of all PICU admissions requiring ventilation for more than 28 days after an acute illness had an underlying neuromuscular disorder, and 45% of this group died.

Outcome of goal-directed non-invasive ventilation and mechanical insufflation/exsufflation in spinal muscular atrophy type I

“…By teaching the parents and acclimatising children to the use of NIPPV, they will be ready to use it as and when required. Yates et al 20 confirmed that there might be a role for NIPPV preventing readmission to the paediatric intensive care unit. In our children with SMA type I, we found that at about 12–18 months of age, they began to have increased difficulty with sputum clearance.…”

British Thoracic Society guideline for respiratory management of children with neuromuscular weakness

“…More encouraging results250–252 have shown physicians offering choice regarding ventilatory support; however this is often reported as being during an episode of respiratory failure. Yates and colleagues253 reviewed data for 28 children with neuromuscular disease admitted to the intensive care units over 15 years and found that 66% of admissions (47/69) were unplanned; 11 of the 28 children admitted as an emergency for respiratory failure required ventilatory support after discharge from the intensive care unit and 6 of these continued on home support. One study of 13 infants with SMA type 1 describes the use of NIV and MI-E to support clearly defined, goal-orientated care 96.…”

Specialty services for children with special health care needs: supplement not supplant the medical home

“…Several studies have demonstrated the extensive use of emergency services by CSHCN4 – 6 as well as general inpatient7 8 and critical care services 9 – 12. Dosa et al showed that children with any chronic health condition had a relative risk of 3.3 for unscheduled intensive-care admission versus compared with those without chronic illness.…”