Palliative care of children with malignant pontine tumours

Abstract

Objectives: Children with malignant pontine tumours (MPT) including diffuse intrinsic pontine gliomas face a dismal prognosis. Palliation is challenging with complex issues at the end-of-life. This study describes (1) the clinical course of children dying of MPT; (2)symptoms experienced; (3) interventions employed; (4) health professionals involved; (5) decision- making regarding end-of-life issues. Methods: This retrospective cohort includes 41 of 48 consecutive children diagnosed under 18, who died between January 2001 and June 2010 with MPT, at a single institution. Disease-specific treatment and decision- making from diagnosis and symptoms/interventions/ resources used in the final 3 months of palliation were studied. Results: 95% of children received radiation and/or surgery/chemotherapy upon diagnosis. All children progressed with 54% receiving treatment for progression. Death occurred due to progression +/-complications. All children experienced symptoms in their final 3 months, most commonly ambulatory difficulties/ dysphasia/dysphagia/visual impairment/fatigue affecting 70-90%. Intensive interventions were provided to a small subset. This included ventriculoperitoneal shunts which were placed in 5 patients (n=2 in the final 3 months of life); and invasive/non-invasive (n=3/n=2) mechanical ventilation. Palliative care was provided by the neuro-oncology team. 51% were also supported by a specialist palliative care team. For 39% of patients day-to-day care was provided by a paediatric oncologist. For the remaining patients, family doctors/ paediatricians/ palliative care physicians and intensivists managed daily issues. The majority of patients received in-home palliation, and 56% died at home. The remainder died in hospital, generally admitted at parental request. 68% of families agreed to a do-notresuscitate order, instituted a median of 28 days before death. 29% of children received chemotherapy and 5% of families admitted to using complementary therapies in the final month. 30% had an autopsy. Conclusions: This study details the challenging palliative course of children dying of MPT. It will be of value to physicians managing complex symptoms and guiding end-of-life decision-making.