Child Neurology

  • Monica E. LemmonEmail author
  • Renee D. Boss


Conditions impacting children with neurologic disease and their families range from congenital conditions diagnosed in the fetal period to acute brain injury in otherwise healthy teenagers. This chapter focuses on those conditions that may not have an adult correlate and provides an overview of how clinicians can provide a palliative approach towards their care of children with neurologic disease. Specific elements of palliative care are discussed, including estimating prognosis and communicating prognostic uncertainty, managing both pain and non-pain symptoms, providing targeted support for families and caregivers, and addressing the unique end of life care needs of pediatric neurology patients and their families. This chapter concludes by assessing current gaps in knowledge and training, and proposes an agenda for future research.


Fetal Congenital Pediatric Family Prognostic uncertainty Shared decision making 


  1. 1.
    Ferriero DM, Bonifacio SL. The search continues for the elusive biomarkers of neonatal brain injury. J Pediatr. 2014;164(3):438–40.PubMedGoogle Scholar
  2. 2.
    Ancora G, Testa C, Grandi S, et al. Prognostic value of brain proton MR spectroscopy and diffusion tensor imaging in newborns with hypoxic-ischemic encephalopathy treated by brain cooling. Neuroradiology. 2013;55(8):1017–25.PubMedGoogle Scholar
  3. 3.
    Barkovich AJ, Miller SP, Bartha A, et al. MR imaging, MR spectroscopy, and diffusion tensor imaging of sequential studies in neonates with encephalopathy. AJNR. Am J Neuroradiol. 2006;27(3):533–47.PubMedGoogle Scholar
  4. 4.
    Nash KB, Bonifacio SL, Glass HC, et al. Video-EEG monitoring in newborns with hypoxic-ischemic encephalopathy treated with hypothermia. Neurology. 2011;76(6):556–62.PubMedPubMedCentralGoogle Scholar
  5. 5.
    Tsuchida TN, Wusthoff CJ, Shellhaas RA, et al. American clinical neurophysiology society standardized EEG terminology and categorization for the description of continuous EEG monitoring in neonates: report of the American Clinical Neurophysiology Society critical care monitoring committee. J Clin Neurophysiol: Off Publ Am Electroencephalographic Soc. 2013;30(2):161–73.Google Scholar
  6. 6.
    Shellhaas RA, Thelen BJ, Bapuraj JR, et al. Limited short-term prognostic utility of cerebral NIRS during neonatal therapeutic hypothermia. Neurology. 2013;81(3):249–55.PubMedPubMedCentralGoogle Scholar
  7. 7.
    Hill SJ, Withington DE. Too clever by half? Can bilateral or unilateral NIRS monitoring improve neurological outcome from pediatric cardiopulmonary bypass? Paediatr Anaesth. 2006;16(7):709–11.PubMedGoogle Scholar
  8. 8.
    Srivastava S, Cohen JS, Vernon H, et al. Clinical whole exome sequencing in child neurology practice. Ann Neurol. 2014;76(4):473–83.Google Scholar
  9. 9.
    Lemmon ME, Boss RD, Bonifacio SL, Foster-Barber A, Barkovich AJ, Glass HC. Characterization of death in neonatal encephalopathy in the hypothermia era. J Child Neurol. 2017;32(4):360–5.PubMedGoogle Scholar
  10. 10.
    Shankaran S, Laptook AR, Ehrenkranz RA, et al. Whole-body hypothermia for neonates with hypoxic-ischemic encephalopathy. N Engl J Med. 2005;353(15):1574–84.PubMedGoogle Scholar
  11. 11.
    Shankaran S, Laptook AR, Pappas A, et al. Effect of depth and duration of cooling on deaths in the NICU among neonates with hypoxic ischemic encephalopathy: a randomized clinical trial. JAMA. 2014;312(24):2629–39.PubMedPubMedCentralGoogle Scholar
  12. 12.
    Azzopardi D, Brocklehurst P, Edwards D, et al. The TOBY Study. Whole body hypothermia for the treatment of perinatal asphyxial encephalopathy: a randomised controlled trial. BMC Pediatr. 2008;8:17.PubMedPubMedCentralGoogle Scholar
  13. 13.
    Lemmon ME, Donohue PK, Parkinson C, Northington FJ, Boss RD. Parent experience of neonatal encephalopathy: the need for family-centered outcomes. J Child Neurol. 2016;32(3):286–92.PubMedPubMedCentralGoogle Scholar
  14. 14.
    Lemmon ME, Donohue PK, Parkinson C, Northington FJ, Boss RD. Communication challenges in neonatal encephalopathy. Pediatrics. 2016;138(3):1–9.PubMedPubMedCentralGoogle Scholar
  15. 15.
    Janvier A, Lantos J. Ethics and etiquette in neonatal intensive care-reply. JAMA Pediatr. 2015;169(2):190–1.PubMedGoogle Scholar
  16. 16.
    Kruser JM, Taylor LJ, Campbell TC, et al. “Best case/worst case: training surgeons to use a novel communication tool for high-risk acute surgical problems. J Pain Symptom Manag. 2017;53(4):711–9.PubMedPubMedCentralGoogle Scholar
  17. 17.
    Taylor LJ, Nabozny MJ, Steffens NM, et al. A framework to improve surgeon communication in high-stakes surgical decisions: best case/worst case. JAMA Surg. 2017;152(6):531–8.PubMedPubMedCentralGoogle Scholar
  18. 18.
    American Academy of Pediatrics Committee on F, Newborn, American Academy of Pediatrics Section on S, et al. Prevention and management of pain in the neonate: an update. Pediatrics. 2006;118(5):2231–41.Google Scholar
  19. 19.
    Breau LM, McGrath PJ, Stevens B, et al. Judgments of pain in the neonatal intensive care setting: a survey of direct care staffs’ perceptions of pain in infants at risk for neurological impairment. Clin J Pain. 2006;22(2):122–9.PubMedGoogle Scholar
  20. 20.
    Stevens B, Johnston C, Petryshen P, Taddio A. Premature Infant Pain Profile: development and initial validation. Clin J Pain. 1996;12(1):13–22.PubMedGoogle Scholar
  21. 21.
    Krechel SW, Bildner J. CRIES: a new neonatal postoperative pain measurement score. Initial testing of validity and reliability. Paediatr Anaesth. 1995;5(1):53–61.PubMedGoogle Scholar
  22. 22.
    van Dijk M, de Boer JB, Koot HM, Tibboel D, Passchier J, Duivenvoorden HJ. The reliability and validity of the COMFORT scale as a postoperative pain instrument in 0 to 3-year-old infants. Pain. 2000;84(2–3):367–77.PubMedGoogle Scholar
  23. 23.
    Hummel P, Lawlor-Klean P, Weiss MG. Validity and reliability of the N-PASS assessment tool with acute pain. J Perinatol: Off J Calif Perinatal Assoc. 2010;30(7):474–8.Google Scholar
  24. 24.
    Hall RW, Anand KJ. Pain management in newborns. Clin Perinatol. 2014;41(4):895–924.PubMedPubMedCentralGoogle Scholar
  25. 25.
    Vinall J, Grunau RE. Impact of repeated procedural pain-related stress in infants born very preterm. Pediatr Res. 2014;75(5):584–7.PubMedPubMedCentralGoogle Scholar
  26. 26.
    Davidson A, Flick RP. Neurodevelopmental implications of the use of sedation and analgesia in neonates. Clin Perinatol. 2013;40(3):559–73.PubMedGoogle Scholar
  27. 27.
    Edwards L, DeMeo S, Hornik CD, et al. Gabapentin use in the Neonatal Intensive Care Unit. J Pediatr. 2016;169:310–2.PubMedGoogle Scholar
  28. 28.
    Gok G, Cox N, Bajwa J, Christodoulou D, Moody A, Howlett DC. Ultrasound-guided injection of botulinum toxin A into the submandibular gland in children and young adults with sialorrhoea. Br J Oral Maxillofac Surg. 2013;51(3):231–3.PubMedGoogle Scholar
  29. 29.
    Macario A, Weinger M, Carney S, Kim A. Which clinical anesthesia outcomes are important to avoid? The perspective of patients. Anesth Analg. 1999;89(3):652–8.PubMedGoogle Scholar
  30. 30.
    Sessler DI, Rubinstein EH, Moayeri A. Physiologic responses to mild perianesthetic hypothermia in humans. Anesthesiology. 1991;75(4):594–610.PubMedGoogle Scholar
  31. 31.
    Alfonsi P. Postanaesthetic shivering: epidemiology, pathophysiology, and approaches to prevention and management. Drugs. 2001;61(15):2193–205.PubMedGoogle Scholar
  32. 32.
    Roka A, Melinda KT, Vasarhelyi B, Machay T, Azzopardi D, Szabo M. Elevated morphine concentrations in neonates treated with morphine and prolonged hypothermia for hypoxic ischemic encephalopathy. Pediatrics. 2008;121(4):e844–9.PubMedGoogle Scholar
  33. 33.
    Zanelli S, Buck M, Fairchild K. Physiologic and pharmacologic considerations for hypothermia therapy in neonates. J Perinatol: Off J Calif Perinatal Assoc. 2011;31(6):377–86.Google Scholar
  34. 34.
    Pellock JM. Understanding co-morbidities affecting children with epilepsy. Neurology. 2004;62(5 Suppl 2):S17–23.PubMedGoogle Scholar
  35. 35.
    Gelfand AA. Psychiatric comorbidity and paediatric migraine: examining the evidence. Curr Opin Neurol. 2015;28(3):261–4.PubMedGoogle Scholar
  36. 36.
    Qubty W, Gelfand AA. Psychological and behavioral issues in the management of migraine in children and adolescents. Curr Pain Headache Rep. 2016;20(12):69.PubMedGoogle Scholar
  37. 37.
    Chong L, Jamieson NJ, Gill D, et al. Children’s experiences of epilepsy: a systematic review of qualitative studies. Pediatrics. 2016;138(3):1–18.PubMedGoogle Scholar
  38. 38.
    Carney T, Murphy S, McClure J, et al. Children’s views of hospitalization: an exploratory study of data collection. J Child Health Care. 2003;7(1):27–40.PubMedGoogle Scholar
  39. 39.
    Carnevale FA, Gaudreault J. The experience of critically ill children: a phenomenological study of discomfort and comfort. Dynamics. 2013;24(1):19–27.PubMedGoogle Scholar
  40. 40.
    Nelson JE, Mercado AF, Camhi SL, et al. Communication about chronic critical illness. Arch Intern Med. 2007;167(22):2509–15.PubMedPubMedCentralGoogle Scholar
  41. 41.
    Shapiro MC, Henderson CM, Hutton N, Boss RD. Defining pediatric chronic critical illness for clinical care, research, and policy. Hosp Pediatr. 2017;7(4):236–44.PubMedGoogle Scholar
  42. 42.
    Abdel-Latif ME, Boswell D, Broom M, Smith J, Davis D. Parental presence on neonatal intensive care unit clinical bedside rounds: randomised trial and focus group discussion. Arch Dis Child Fetal Neonatal Ed. 2015;100(3):F203–9.PubMedPubMedCentralGoogle Scholar
  43. 43.
    Lilly CM, De Meo DL, Sonna LA, et al. An intensive communication intervention for the critically ill. Am J Med. 2000;109(6):469–75.PubMedGoogle Scholar
  44. 44.
    Lilly CM, Sonna LA, Haley KJ, Massaro AF. Intensive communication: four-year follow-up from a clinical practice study. Crit Care Med. 2003;31(5 Suppl):S394–9.PubMedGoogle Scholar
  45. 45.
    Aslakson R, Cheng J, Vollenweider D, Galusca D, Smith TJ, Pronovost PJ. Evidence-based palliative care in the intensive care unit: a systematic review of interventions. J Palliat Med. 2014;17(2):219–35.PubMedPubMedCentralGoogle Scholar
  46. 46.
    Boss RD, Hutton N, Sulpar LJ, West AM, Donohue PK. Values parents apply to decision-making regarding delivery room resuscitation for high-risk newborns. Pediatrics. 2008;122(3):583–9.PubMedGoogle Scholar
  47. 47.
    Cavinder C. The relationship between providing neonatal palliative care and nurses’ moral distress: an integrative review. Adv Neonatal Care: Off J Natl Assoc Neonatal Nurses. 2014;14(5):322–8.Google Scholar
  48. 48.
    Sauerland J, Marotta K, Peinemann MA, Berndt A, Robichaux C. Assessing and addressing moral distress and ethical climate Part II: neonatal and pediatric perspectives. Dimens Crit Care Nurs: DCCN. 2015;34(1):33–46.PubMedGoogle Scholar
  49. 49.
    Hays RM, Valentine J, Haynes G, et al. The Seattle Pediatric Palliative Care Project: effects on family satisfaction and health-related quality of life. J Palliat Med. 2006;9(3):716–28.PubMedGoogle Scholar
  50. 50.
    Ahmed R, McCaffery KJ, Aslani P. Development and validation of a question prompt list for parents of children with attention-deficit/hyperactivity disorder: a Delphi study. Health Expect: Int J Public Particip Health Care Health Policy. 2015;19(2):234–52.PubMedPubMedCentralGoogle Scholar
  51. 51.
    Clayton JM, Butow PN, Tattersall MH, et al. Randomized controlled trial of a prompt list to help advanced cancer patients and their caregivers to ask questions about prognosis and end-of-life care. J Clin Oncol: Off J Am Soc Clin Oncol. 2007;25(6):715–23.Google Scholar
  52. 52.
    Dimoska A, Butow PN, Lynch J, et al. Implementing patient question-prompt lists into routine cancer care. Patient Educ Couns. 2012;86(2):252–8.PubMedGoogle Scholar
  53. 53.
    Ernecoff NC, Witteman HO, Chon K, et al. Key stakeholders’ perceptions of the acceptability and usefulness of a tablet-based tool to improve communication and shared decision making in ICUs. J Crit Care. 2016;33:19–25.PubMedGoogle Scholar
  54. 54.
    Mancini J, Butow PN, Julian-Reynier C, et al. Question prompt list responds to information needs of myelodysplastic syndromes patients and caregivers. Leuk Res. 2015;39(6):599–605.PubMedGoogle Scholar
  55. 55.
    Scherr KA, Fagerlin A, Hofer T, et al. Physician recommendations trump patient preferences in prostate cancer treatment decisions. Med Decis Making: Int J Soc Med Decis Making. 2017;37(1):56–69.Google Scholar
  56. 56.
    Kersting A, Dorsch M, Wesselmann U, et al. Maternal posttraumatic stress response after the birth of a very low-birth-weight infant. J Psychosom Res. 2004;57(5):473–6.PubMedGoogle Scholar
  57. 57.
    Vanderbilt D, Bushley T, Young R, Frank DA. Acute posttraumatic stress symptoms among urban mothers with newborns in the neonatal intensive care unit: a preliminary study. J Dev Behav Pediatr. 2009;30(1):50–6.PubMedGoogle Scholar
  58. 58.
    Shaw RJ, Deblois T, Ikuta L, Ginzburg K, Fleisher B, Koopman C. Acute stress disorder among parents of infants in the neonatal intensive care nursery. Psychosomatics. 2006;47(3):206–12.PubMedGoogle Scholar
  59. 59.
    Cano Gimenez E, Sanchez-Luna M. Providing parents with individualised support in a neonatal intensive care unit reduced stress, anxiety and depression. Acta Paediatr. 2015;104(7):e300–5.PubMedGoogle Scholar
  60. 60.
    Rossman B, Greene MM, Meier PP. The role of peer support in the development of maternal identity for “NICU Moms”. J Obstet, Gynecol, Neonatal Nurs: JOGNN. 2015;44(1):3–16.Google Scholar
  61. 61.
    Meert KL, Thurston CS, Briller SH. The spiritual needs of parents at the time of their child’s death in the pediatric intensive care unit and during bereavement: a qualitative study. Pediatr Crit Care Med: J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc. 2005;6(4):420–7.Google Scholar
  62. 62.
    Gold KJ. Navigating care after a baby dies: a systematic review of parent experiences with health providers. J Perinatol: Off J Calif Perinatal Assoc. 2007;27(4):230–7.Google Scholar
  63. 63.
    Milstein J. A paradigm of integrative care: healing with curing throughout life, “being with” and “doing to”. J Perinatol: Off J Calif Perinatal Assoc. 2005;25(9):563–8.Google Scholar
  64. 64.
    Kusano AS, Kenworthy-Heinige T, Thomas CR Jr. Survey of bereavement practices of cancer care and palliative care physicians in the Pacific Northwest United States. J Oncol Pract Am Soc Clin Oncol. 2012;8(5):275–81.Google Scholar
  65. 65.
    Meert KL, Eggly S, Berger J, et al. Physicians’ experiences and perspectives regarding follow-up meetings with parents after a child’s death in the pediatric intensive care unit. Pediatr Crit Care Med: J Soc Crit Care Med World Fed Pediatr Intensive Crit Care Soc. 2011;12(2):e64–8.Google Scholar
  66. 66.
    Smith AK, White DB, Arnold RM. Uncertainty – the other side of prognosis. N Engl J Med. 2013;368(26):2448–50.PubMedPubMedCentralGoogle Scholar
  67. 67.
    Lemmon ME, Bidegain M, Boss RD. Palliative care in neonatal neurology: robust support for infants, families and clinicians. J Perinatol: Off J Calif Perinatal Assoc. 2015;36(5):331–7.PubMedGoogle Scholar
  68. 68.
    Wilkinson D. MRI and withdrawal of life support from newborn infants with hypoxic-ischemic encephalopathy. Pediatrics. 2010;126(2):e451–8.PubMedGoogle Scholar
  69. 69.
    Hauer J, Houtrow AJ, Section On H, Palliative Medicine COCWD. Pain assessment and treatment in children with significant impairment of the central nervous system. Pediatrics. 2017;139(6):e1–e27.Google Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2019

Authors and Affiliations

  1. 1.Division of Pediatric Neurology and Developmental MedicineDuke University School of MedicineDurhamUSA
  2. 2.Department of PediatricsJohns Hopkins School of MedicineBaltimoreUSA

Personalised recommendations