Disease specific outcome in paediatric intensive care

Medical care in general and paediatric intensive care specifically have greatly advanced over the years. This has resulted in a decrease in mortality over time for a large number of diseases. However, for several diseases there still is considerable uncertainty about the outcome of intensive care treatment. This holds true especially for rarely occurring diseases, or for diseases which traditionally were considered to have an unfavourable outcome. The studies described in this thesis assessed epidemiological characteristics, clinical courses and ICU mortality rates for several of such conditions. In the first study we retrospectively examined outcomes of mechanical ventilation in patients with cystic fibrosis over a period of 15 years. Results revealed that children nowadays have a good prognosis. Their long-term outcome seems identical to unventilated controls. Adult cystic fibrosis patients still have high ICU mortality rates, especially when they have acute on chronic respiratory failure. For children requiring mechanical ventilation after haematopoietic stem cell transplantation, there is a widely held impression that ICU mortality has clearly decreased over time. However, we could not confirm this with a meta-analysis of available literature (study 2). Since recent data in this group of patients were scarce, we subsequently assessed outcome in the 35 most recently ventilated children after haematopoietic stem cell transplantation from our own department (study 3). We found an improvement of ICU survival and in survival six months after discharge from ICU. Children with severe neurological impairment often have respiratory problems. The decision to initiate or withhold mechanical ventilation in this population requires careful consideration. Unfortunately, literature does not provide any evidence to make this decision. In the fourth study we showed that in our department, uneventful survival in these children was low. Repeated, prolonged and complicated admission to ICU was often required. However, outcome was not uniformly dismal, which underscores the need for further research. In the fifth study we reviewed the outcomes for 239 consecutive patients with meningococcal disease treated in our ICU. Over time, children were more severely ill on admission. Mortality corrected for severity of disease decreased in the same time period, suggesting improvements in ICU care. More fluids were given over time and vaso-active drugs were started earlier in the course of the disease. Yet, the increase in survival could not be explained by this more aggressive resuscitation of shock. In the sixth study we reviewed 34 episodes of refractory status epilepticus (RSE) treated in our ICU. Thiopental was effective in most patients, but there were serious side-effects. Propofol was used according to a strict protocol. It was effective in most patients, so that thiopental was not needed. Side-effects were infrequent, of minor severity and fully reversible. We suggest the use of propofol before thiopental. Overall it can be concluded that advancements in intensive care have resulted in increased survival over time, but many questions remain. Future research in these complex groups of patients would be helped with prospectively collected databases by a group of dedicated ICUs.