title: Palliative and end-of-life care for children with diffuse intrinsic pontine glioma: results from a London cohort study and international survey.
abstract: 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.
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Sophie E M
authors.initials: SE
authors.lastname: Veldhuijzen van Zanten
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Charlotte L L I
authors.initials: CL
authors.lastname: van Meerwijk
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Marc H A
authors.initials: MH
authors.lastname: Jansen
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Jos W R
authors.initials: JW
authors.lastname: Twisk
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Anna-Karenia
authors.initials: AK
authors.lastname: Anderson
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Lucy
authors.initials: L
authors.lastname: Coombes
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Maggie
authors.initials: M
authors.lastname: Breen
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Olivia J
authors.initials: OJ
authors.lastname: Hargrave
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: June
authors.initials: J
authors.lastname: Hemsley
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Finella
authors.initials: F
authors.lastname: Craig
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Ofelia
authors.initials: O
authors.lastname: Cruz
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Gertjan J L
authors.initials: GJ
authors.lastname: Kaspers
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Dannis G
authors.initials: DG
authors.lastname: van Vuurden
authors.affiliation: Department of Pediatrics, Division of Oncology/Hematology, VU University Medical Center Amsterdam, Netherlands (S.E.M.V.v.Z., C.L.L.I.v.M., M.H.A.J., G.J.L.K., D.G.v.V.); Department of Epidemiology and Biostatistics, VU University Medical Center Amsterdam, Netherlands (J.W.R.T.); Paediatrics Unit, Royal Marsden NHS Foundation Trust, Sutton, UK (A.-K.A., L.C., M.B.); Department of Oncology, Great Ormond Street Hospital, London, UK (O.J.H., J.H., F.C., D.R.H.); Department of Pediatric Oncology, Hospital Sant Joan de Déu, Barcelona, Spain (O.C.).
authors.forename: Darren R
authors.initials: DR
authors.lastname: Hargrave
mesh_terms: D000293:Adolescent; D020295:Brain Stem Neoplasms / Q000628:therapy*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005500:Follow-Up Studies; D005910:Glioma / Q000628:therapy*; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007390:International Agencies; D008131:London; D008297:Male; D009367:Neoplasm Staging; D010166:Palliative Care; D011379:Prognosis; D011788:Quality of Life; D012189:Retrospective Studies; D011795:Surveys and Questionnaires; D013727:Terminal Care
keywords: diffuse intrinsic pontine glioma (DIPG); end-of-life care; palliative care; pediatric oncology; quality of life