BACKGROUND: More than 90% of patients with diffuse intrinsic pontine glioma (DIPG) will die within 2 years of diagnosis. Patients deteriorate rapidly during the disease course, which severely impairs their quality of life. To date, no specific research on this clinically important subject has been conducted. This study aimed to compile an inventory of symptoms experienced, interventions applied, and current service provision in end-of-life care for DIPG. METHODS: We performed a retrospective cohort study of children with DIPG, aged 0-18 years, who received treatment under the care of 2 London hospitals. Symptoms, interventions, and services applied during the 12 weeks before death were analyzed. In addition, we conducted a global questionnaire-study among health care professionals. RESULTS: In more than 78% of DIPG patients, problems concerning mobility, swallowing, communication, consciousness, and breathing arose during end-stage disease. Supportive drugs were widely prescribed. The use of medical aids was only documented in <15% of patients. Palliative and end-of-life care was mostly based on the health care professional's experience; only 21% of the questionnaire respondents reported to have a disease-specific palliative care guideline available. CONCLUSIONS: This research assessed the current state of palliative and end-of-life care for children with DIPG. Our results show the variability and complexity of symptoms at end-stage disease and the current lack of disease-specific guidelines for this vulnerable group of patients. This first descriptive paper is intended to act as a solid basis for developing an international clinical trial and subsequent guideline to support high-quality palliative and end-of-life care.
BACKGROUND: More than 90% of patients with diffuse intrinsic pontine glioma (DIPG) will die within 2 years of diagnosis. Patients deteriorate rapidly during the disease course, which severely impairs their quality of life. To date, no specific research on this clinically important subject has been conducted. This study aimed to compile an inventory of symptoms experienced, interventions applied, and current service provision in end-of-life care for DIPG. METHODS: We performed a retrospective cohort study of children with DIPG, aged 0-18 years, who received treatment under the care of 2 London hospitals. Symptoms, interventions, and services applied during the 12 weeks before death were analyzed. In addition, we conducted a global questionnaire-study among health care professionals. RESULTS: In more than 78% of DIPGpatients, problems concerning mobility, swallowing, communication, consciousness, and breathing arose during end-stage disease. Supportive drugs were widely prescribed. The use of medical aids was only documented in <15% of patients. Palliative and end-of-life care was mostly based on the health care professional's experience; only 21% of the questionnaire respondents reported to have a disease-specific palliative care guideline available. CONCLUSIONS: This research assessed the current state of palliative and end-of-life care for children with DIPG. Our results show the variability and complexity of symptoms at end-stage disease and the current lack of disease-specific guidelines for this vulnerable group of patients. This first descriptive paper is intended to act as a solid basis for developing an international clinical trial and subsequent guideline to support high-quality palliative and end-of-life care.
Authors: Tammy Kang; K Sarah Hoehn; Daniel J Licht; Oscar Henry Mayer; Gina Santucci; Jean Marie Carroll; Carolyn M Long; Malinda Ann Hill; Jennifer Lemisch; Mary T Rourke; Chris Feudtner Journal: Pediatr Clin North Am Date: 2005-08 Impact factor: 3.278
Authors: Sophie E M Veldhuijzen van Zanten; Marc H A Jansen; Esther Sanchez Aliaga; Dannis G van Vuurden; W Peter Vandertop; Gertjan J L Kaspers Journal: Expert Rev Anticancer Ther Date: 2014-11-29 Impact factor: 4.512
Authors: Eve Namisango; Katherine Bristowe; Matthew J Allsop; Fliss E M Murtagh; Melanie Abas; Irene J Higginson; Julia Downing; Richard Harding Journal: Patient Date: 2019-02 Impact factor: 3.883
Authors: Sophie E M Veldhuijzen van Zanten; Joshua Baugh; Brooklyn Chaney; Dennis De Jongh; Esther Sanchez Aliaga; Frederik Barkhof; Johan Noltes; Ruben De Wolf; Jet Van Dijk; Antonio Cannarozzo; Carin M Damen-Korbijn; Jan A Lieverst; Niclas Colditz; Marion Hoffmann; Monika Warmuth-Metz; Brigitte Bison; David T W Jones; Dominik Sturm; Gerrit H Gielen; Chris Jones; Esther Hulleman; Raphael Calmon; David Castel; Pascale Varlet; Géraldine Giraud; Irene Slavc; Stefaan Van Gool; Sandra Jacobs; Filip Jadrijevic-Cvrlje; David Sumerauer; Karsten Nysom; Virve Pentikainen; Sanna-Maria Kivivuori; Pierre Leblond; Natasha Entz-Werle; Andre O von Bueren; Antonis Kattamis; Darren R Hargrave; Péter Hauser; Miklos Garami; Halldora K Thorarinsdottir; Jane Pears; Lorenza Gandola; Giedre Rutkauskiene; Geert O Janssens; Ingrid K Torsvik; Marta Perek-Polnik; Maria J Gil-da-Costa; Olga Zheludkova; Liudmila Shats; Ladislav Deak; Lidija Kitanovski; Ofelia Cruz; Andres Morales La Madrid; Stefan Holm; Nicolas Gerber; Rejin Kebudi; Richard Grundy; Enrique Lopez-Aguilar; Marta Zapata-Tarres; John Emmerik; Tim Hayden; Simon Bailey; Veronica Biassoni; Maura Massimino; Jacques Grill; William P Vandertop; Gertjan J L Kaspers; Maryam Fouladi; Christof M Kramm; Dannis G van Vuurden Journal: J Neurooncol Date: 2017-01-21 Impact factor: 4.130
Authors: Fatma E El-Khouly; Sophie E M Veldhuijzen van Zanten; Vicente Santa-Maria Lopez; N Harry Hendrikse; Gertjan J L Kaspers; G Loizos; David Sumerauer; Karsten Nysom; Kaie Pruunsild; Virve Pentikainen; Halldora K Thorarinsdottir; Giedre Rutkauskiene; Victor Calvagna; Monika Drogosiewicz; Monica Dragomir; Ladislav Deak; Lidija Kitanovski; Andre O von Bueren; Rejin Kebudi; Irene Slavc; Sandra Jacobs; Filip Jadrijevic-Cvrlje; Natacha Entz-Werle; Jacques Grill; Antonis Kattamis; Peter Hauser; Jane Pears; Veronica Biassoni; Maura Massimino; Enrique Lopez Aguilar; Ingrid K Torsvik; Maria Joao Gil-da-Costa; Ella Kumirova; Ofelia Cruz-Martinez; Stefan Holm; Simon Bailey; Tim Hayden; Ulrich W Thomale; Geert O R Janssens; Christof M Kramm; Dannis G van Vuurden Journal: J Neurooncol Date: 2019-09-14 Impact factor: 4.130