Palliative Care and End-of-Life Considerations in Children on Chronic Ventilation

Chapter
First Online:
Part of the Respiratory Medicine book series (RM)

Abstract

Despite being assisted on respiratory devices, children on chronic ventilation commonly have conditions that will result in shortened life spans and leave them at risk for acute, critical deterioration. In addition, these children and their families have substantial needs beyond health maintenance and respiratory care. Thus, they require a care paradigm that complements strategies focused on prolongation of life and provides other medical, psychological, emotional, and spiritual supports. These supports should increasingly fill care voids as prolongation of life becomes less possible or prolongation of suffering less favorable. In short, many of these children and families require palliative care to address their end-of-life and other complex needs. However, providing this palliative care and preparing them for the end of life can entail several challenges. Many of these challenges stem from the difficulty of prognostication, but also include the idiosyncrasies of the complex child-family relationship, professional caregivers, and chronic ventilation itself. These challenges can be avoided or minimized by concurrently focusing on non-end-of-life palliative care, as well as good communication, avoiding any suggestion of abandonment, and propitious timing of discussions.

Keywords

Children Chronic ventilation Palliative care End of life Advance care planning 
This is a preview of subscription content, log in to check access.

Notes

Acknowledgments

Special thanks to Drs. Gloria Chiang and Robert Graham for their reading portions of the manuscript and for their suggestions.

References

  1. 1.
    Srinivasan S, Doty SM, White TR, Segura VH, Jansen MT, Davidson Ward SL, Keens TG. Frequency, causes, and outcome of home ventilator failure. Chest. 1998;114(5):1363–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Chatwin M, Heather S, Hanak A, Polkey MI, Simonds AK. Analysis of home support and ventilator malfunction in 1,211 ventilator-dependent patients. Eur Respir J. 2010;35(2):310–6.CrossRefPubMedGoogle Scholar
  3. 3.
    Boroughs D, Dougherty JA. Decreasing accidental mortality of ventilator-dependent children at home: a call to action. Home Healthc Nurse. 2012;30(2):103–11.PubMedGoogle Scholar
  4. 4.
    Gregoretti C, Ottonello G, Chiarini Testa MB, Mastella C, Ravà L, Bignamini E, Veljkovic A, Cutrera R. Survival of patients with spinal muscular Atrophy type 1. Pediatrics. 2013;131(5):e1509–14. doi: 10.1542/peds.2012-2278.CrossRefPubMedGoogle Scholar
  5. 5.
    Ishikawa Y, Miura T, Ishikawa Y, Aoyagi T, Ogata H, Hamada S, Minami R. Duchenne muscular dystrophy: survival by cardio-respiratory interventions. Neuromuscul Disord. 2011;21(1):47–51.CrossRefPubMedGoogle Scholar
  6. 6.
    Brooks JC, Strauss DJ, Shavelle RM, Tran LM, Rosenbloom L, Wu YW. Recent trends in cerebral palsy survival. Part II: individual survival prognosis. Dev Med Child Neurol. 2014;56(11):1065–71.CrossRefPubMedGoogle Scholar
  7. 7.
    Bittles AH, Glasson EJ. Clinical, social, and ethical implications of changing life expectancy in Down syndrome. Dev Med Child Neurol. 2004;46(4):282–6.CrossRefPubMedGoogle Scholar
  8. 8.
    Edwards JD, Kun SS, Keens TG. Outcomes and causes of death in children on home mechanical ventilation via tracheostomy: an institutional and literature review. J Pediatr. 2010;157(6):955–9.CrossRefPubMedGoogle Scholar
  9. 9.
    Edwards JD, Kun SS, Keens TG, Khemani RG, Moromisato DY. Children with corrected or palliated congenital heart disease on home mechanical ventilation. Pediatr Pulmonol. 2010;45(7):645–9.CrossRefPubMedGoogle Scholar
  10. 10.
    Jernigan SC, Berry JG, Graham DA, Bauer SB, Karlin LI, Hobbs NM, Scott RM, Warf BC. Risk factors of sudden death in young adult patients with myelomeningocele. J Neurosurg Pediatr. 2012;9(2):149–55.CrossRefPubMedGoogle Scholar
  11. 11.
    Parker D, Maddocks I, Stern LM. The role of palliative care in advanced muscular dystrophy and spinal muscular atrophy. J Paediatr Child Health. 1999;35(3):245–50.CrossRefPubMedGoogle Scholar
  12. 12.
    Steele RG. Trajectory of certain death at an unknown time: children with neurodegenerative life-threatening illnesses. Can J Nurs Res. 2000;32(3):49–67.PubMedGoogle Scholar
  13. 13.
    Carnevale FA, Alexander E, Davis M, Rennick J, Troini R. Daily living with distress and enrichment: the moral experience of families with ventilator-assisted children at home. Pediatrics. 2006;117:e48–60.CrossRefPubMedGoogle Scholar
  14. 14.
    Blucker RT, Elliott TR, Warren RH, Warren AM. Psychological adjustment of family caregivers of children who have severe neurodisabilities that require chronic respiratory management. Fam Syst Health. 2011;29(3):215–31.CrossRefPubMedGoogle Scholar
  15. 15.
    Toly VB, Musil CM, Carl JC. A longitudinal study of families with technology-dependent children. Res Nurs Health. 2012;35(1):40–54.CrossRefPubMedGoogle Scholar
  16. 16.
    Noyes J, Hartmann H, Samuels M, Southall D. The experiences and views of parents who care for ventilator-dependent children. J Clin Nurs. 1999;8(4):440–50.CrossRefPubMedGoogle Scholar
  17. 17.
    Heaton J, Noyes J, Sloper P, Shah R. Families’ experiences of caring for technology-dependent children: a temporal perspective. Health Soc Care Community. 2005;13:441–50.CrossRefPubMedGoogle Scholar
  18. 18.
    O’Brien ME, Wegner CB. Rearing the child who is technology dependent: perceptions of parents and home care nurses. J Spec Pediatr Nurs. 2002;7:7–15.CrossRefPubMedGoogle Scholar
  19. 19.
    Kirk S, Glendinning C, Callery P. Parent or nurse? The experience of being the parent of a technology dependent child. J Adv Nurs. 2005;51:456–64.CrossRefPubMedGoogle Scholar
  20. 20.
    Alexander E, Rennick JE, Carnevale F, Davis M. Daily struggles: living with long-term childhood technology dependence. Can J Nurs Res. 2002;34:7–14.PubMedGoogle Scholar
  21. 21.
    Toly VB, Musil CM, Carl JC. Families with children who are technology dependent: normalization and family functioning. West J Nurs Res. 2012;34(1):52–71.CrossRefPubMedGoogle Scholar
  22. 22.
    Thyen U, Kuhlthau K, Perrin JM. Employment, child care, and mental health of mothers caring for children assisted by technology. Pediatrics. 1999;103(6 Pt 1):1235–42.CrossRefPubMedGoogle Scholar
  23. 23.
    Donnelly JP, Huff SM, Lindsey ML, McMahon KA, Schumacher JD. The needs of children with life-limiting conditions: a healthcare-provider-based model. Am J Hosp Palliat Care. 2005;22(4):259–67.CrossRefPubMedGoogle Scholar
  24. 24.
    American Academy of Pediatrics Policy Statement. Pediatric palliative care and hospice care commitments, guidelines, and recommendations. Pediatrics. 2013;132:966–72.CrossRefGoogle Scholar
  25. 25.
    Dawson S, Kristjanson LJ. Mapping the journey: family carers’ perceptions of issues related to end-stage care of individuals with muscular dystrophy or motor neurone disease. J Palliat Care. 2003;19(1):36–42.PubMedGoogle Scholar
  26. 26.
    Cockett A. Developing a long-term ventilation service in a children’s hospice: an illustrative case study. Int J Palliat Nurs. 2012;18(6):301–6.CrossRefPubMedGoogle Scholar
  27. 27.
    Carroll KW, Mollen CJ, Aldridge S, Hexem KR, Feudtner C. Influences of decision making identified by parents of children receiving pediatric palliative care. Am J Bioeth Prim Res. 2012;3(1):1–7.Google Scholar
  28. 28.
    Friedman SL. Parent resuscitation preferences for young people with severe developmental disabilities. J Am Med Dir Assoc. 2006;7(2):67–72.CrossRefPubMedGoogle Scholar
  29. 29.
    Hammes BJ, Klevan J, Kempf M, Williams MS. Pediatric advance care planning. J Palliat Med. 2005;8(4):766–73.CrossRefPubMedGoogle Scholar
  30. 30.
    Wharton RH, Levine KR, Buka S, Emanuel L. Advance care planning for children with special health care needs: a survey of parental attitudes. Pediatrics. 1996;97(5):682–7.PubMedGoogle Scholar
  31. 31.
    Steinhauser KE, Clipp EC, McNeilly M, Christakis NA, McIntyre LM, Tulsky JA. In search of a good death: observations of patients, families and providers. Ann Intern Med. 2000;132:825–32.CrossRefPubMedGoogle Scholar
  32. 32.
    Liberman DB, Pham PK, Nager AL. Pediatric advance directives: Parents’ knowledge, experience, and preferences. Pediatrics. 2014;134(2):e436–43.CrossRefPubMedGoogle Scholar
  33. 33.
    Hill DL, Miller VA, Hexem KR, Carroll KW, Faerber JA, Kang T, Feudtner C. Problems and hopes perceived by mothers, fathers and physicians of children receiving palliative care. Health Expect. 2013;18(5):1052–62. doi: 10.1111/hex.12078.CrossRefPubMedGoogle Scholar
  34. 34.
    Feudtner C. Hope and the prospects of healing at the end of life. J Altern Complement Med. 2005;11 Suppl 1:S23–30.PubMedGoogle Scholar
  35. 35.
    Reder EA, Serwint JR. Until the last breath: exploring the concept of hope for parents and health care professionals during a child’s serious illness. Arch Pediatr Adolesc Med. 2009;163(7):653–7.CrossRefPubMedGoogle Scholar
  36. 36.
    Miller EG, Laragione G, Kang TI, Feudtner C. Concurrent care for the medically complex child: lessons of implementation. J Palliat Med. 2012;15(11):1281–3.CrossRefPubMedGoogle Scholar
  37. 37.
    American Academy of Pediatrics, Committee on Bioethics and Committee on Hospital Care. Palliative care for children. Pediatrics. 2000;106(2 Pt 1):351–7.Google Scholar
  38. 38.
    Viallard ML. Some general considerations of a human-based medicine’s palliative approach to the vulnerability of the multiply disabled child before the end of life. Cult Med Psychiatry. 2014;38(1):28–34.CrossRefPubMedGoogle Scholar
  39. 39.
    Gillis J. We want everything done. Arch Dis Child. 2008;93(3):192–3.CrossRefPubMedGoogle Scholar
  40. 40.
    Graham RJ, Robinson WM. Integrating palliative care into chronic care for children with severe neurodevelopmental disabilities. J Dev Behav Pediatr. 2005;26(5):361–5.CrossRefPubMedGoogle Scholar
  41. 41.
    Kane JR, Barber RG, Jordan M, Tichenor KT, Camp K. Supportive/palliative care of children suffering from life-threatening and terminal illness. Am J Hosp Palliat Care. 2000;17(3):165–72.CrossRefPubMedGoogle Scholar
  42. 42.
    Schuster MA, Duan N, Regalado M, Klein DJ. Anticipatory guidance: what information do parents receive? What information do they want? Arch Pediatr Adolesc Med. 2000;154(12):1191–8.CrossRefPubMedGoogle Scholar
  43. 43.
    Haque IU, Udassi JP, Zaritsky AL. Outcome following cardiopulmonary arrest. Pediatr Clin N Am. 2008;55(4):969–87.CrossRefGoogle Scholar
  44. 44.
    Edwards JD, Kun SS, Graham RJ, Keens TG. End-of-life discussions and advance care planning for children on long-term assisted ventilation with life-limiting conditions. J Palliat Care. 2012;28(1):21–7.PubMedPubMedCentralGoogle Scholar
  45. 45.
    Matos RI, Watson RS, Nadkarni VM, Huang HH, Berg RA, Meaney PA, Carroll CL, Berens RJ, Praestgaard A, Weissfeld L, Spinella PC, American Heart Association’s Get With The Guidelines–Resuscitation (Formerly the National Registry of Cardiopulmonary Resuscitation) Investigators. Duration of cardiopulmonary resuscitation and illness category impact survival and neurologic outcomes for in-hospital pediatric cardiac arrests. Circulation. 2013;127(4):442–51.CrossRefPubMedGoogle Scholar
  46. 46.
    Girotra S, Spertus JA, Li Y, Berg RA, Nadkarni VM, Chan PS, American Heart Association Get With the Guidelines–Resuscitation Investigators. Survival trends in pediatric in-hospital cardiac arrests: an analysis from get with the Guidelines-Resuscitation. Circ Cardiovasc Qual Outcomes. 2013;6(1):42–9.CrossRefPubMedGoogle Scholar
  47. 47.
    United States, et al. Compilation of Patient Protection and Affordable Care Act (PPACA) as amended through November 1, 2010, including PPACA health-related portions of the Health Care and Education Reconciliation Act (HCERA) of 2010. Washington, DC: U.S. Government Printing Office; 2010.Google Scholar
  48. 48.
    Wilfond BS, Miller PS, Korfiatis C, Diekema DS, Dudzinski DM, Goering S, Seattle Growth Attenuation and Ethics Working Group. Navigating growth attenuation in children with profound disabilities. Children’s interests, family decision-making, and community concerns. Hastings Cent Rep. 2010;40(6):27–40.CrossRefPubMedGoogle Scholar
  49. 49.
    Callahan M, Kelley P. Final gifts: understanding the special awareness, needs, and communications of the dying. New York: Bantam; 1997.Google Scholar
  50. 50.
    Himelstein BP, Hilden JM, Boldt AM, Weissman D. Pediatric palliative care. N Engl J Med. 2004;350(17):1752–62.CrossRefPubMedGoogle Scholar
  51. 51.
    Jecker NS, Schneiderman LJ. Is dying young worse than dying old? Gerontologist. 1994;34(1):66–72.CrossRefPubMedGoogle Scholar
  52. 52.
    Davies B, Sehring SA, Partridge JC, Cooper BA, Hughes A, Philp JC, Amidi-Nouri A, Kramer RF. Barriers to palliative care for children: perceptions of pediatric health care providers. Pediatrics. 2008;121(2):282–8.CrossRefPubMedGoogle Scholar
  53. 53.
    Hynson JL, Gillis J, Collins JJ, Irving H, Trethewie SJ. The dying child: how is care different? Med J Aust. 2003;179(6 Suppl):S20–2.PubMedGoogle Scholar
  54. 54.
    Grossberg RI, Blackford M, Friebert S, Benore E, Reed MD. Direct care staff and parents’/legal guardians’ perspectives on end-of-life care in a long-term care facility for medically fragile and intellectually disabled pediatric and young adult residents. Palliat Support Care. 2012;10:1–8.CrossRefGoogle Scholar
  55. 55.
    Serwint JR, Nellis ME. Deaths of pediatric patients: relevance to their medical home, an urban primary care clinic. Pediatrics. 2005;115:57–63.PubMedGoogle Scholar
  56. 56.
    Brook L, Hain R. Predicting death in children. Arch Dis Child. 2008;93(12):1067–70.CrossRefPubMedGoogle Scholar
  57. 57.
    Hauer J. Medical treatment and management at the end of life. In: Friedman SL, Helm DT, editors. End-of-life care for children and adults with intellectual and developmental disabilities. Washington, DC: American Association on Intellectual and Developmental Disabilities; 2010. p. 93–120.Google Scholar
  58. 58.
    Michelson KN, Koogler T, Sullivan C, Ortega Mdel P, Hall E, Frader J. Parental views on withdrawing life-sustaining therapies in critically ill children. Arch Pediatr Adolesc Med. 2009;163(11):986–92.CrossRefPubMedPubMedCentralGoogle Scholar
  59. 59.
    Birnkrant DJ, Noritz GH. Is there a role for palliative care in progressive pediatric neuromuscular diseases? The answer is “Yes! J Palliat Care. 2008;24(4):265–9.PubMedGoogle Scholar
  60. 60.
    Hill DL, Miller V, Walter JK, Carroll KW, Morrison WE, Munson DA, Kang TI, Hinds PS, Feudtner C. Regoaling: a conceptual model of how parents of children with serious illness change medical care goals. BMC Palliat Care. 2014;13(1):9.CrossRefPubMedPubMedCentralGoogle Scholar
  61. 61.
    Singer GR, Koch KA. Communicating with our patients: the goal of bioethics. J Fla Med Assoc. 1997;84(8):486–7.PubMedGoogle Scholar
  62. 62.
    Hsiao JL, Evan EE, Zeltzer LK. Parent and child perspectives on physician communication in pediatric palliative care. Palliat Support Care. 2007;5(4):355–65.CrossRefPubMedGoogle Scholar
  63. 63.
    Selph RB, Shiang J, Engelberg R, Curtis JR, White DB. Empathy and life support decisions in intensive care units. J Gen Intern Med. 2008;23(9):1311–7.CrossRefPubMedPubMedCentralGoogle Scholar
  64. 64.
    Coyle N, Peereboom K. Facilitating goals-of-care discussions for patients with life-limiting disease—communication strategies for nurses. J Hosp Palliat Nurs. 2012;14(4):251–8.CrossRefGoogle Scholar
  65. 65.
    McDonagh JR, Elliott TB, Engelberg RA, Treece PD, Shannon SE, Rubenfeld GD, Patrick DL, Curtis JR. Family satisfaction with family conferences about end-of-life care in the intensive care unit: increased proportion of family speech is associated with increased satisfaction. Crit Care Med. 2004;32(7):1484–8.CrossRefPubMedGoogle Scholar
  66. 66.
    Thornton JD, Pham K, Engelberg RA, Jackson JC, Curtis JR. Families with limited English proficiency receive less information and support in interpreted intensive care unit family conferences. Crit Care Med. 2009;37(1):89–95.CrossRefPubMedPubMedCentralGoogle Scholar
  67. 67.
    Hinds PS, Schum L, Baker JN, Wolfe J. Key factors affecting dying children and their families. J Palliat Med. 2005;8 Suppl 1:S70–8.PubMedGoogle Scholar
  68. 68.
    Durall A, Zurakowski D, Wolfe J. Barriers to conducting advance care discussions for children with life-threatening conditions. Pediatrics. 2012;129(4):e975–82.CrossRefPubMedGoogle Scholar
  69. 69.
    Shneerson JM. Home mechanical ventilation in children: techniques, outcomes and ethics. Monaldi Arch Chest Dis. 1996;51(5):426–30.PubMedGoogle Scholar
  70. 70.
    Simonds AK. Respiratory support for the severely handicapped child with neuromuscular disease: ethics and practicality. Semin Respir Crit Care Med. 2007;28(3):342–54.CrossRefPubMedGoogle Scholar
  71. 71.
    Fraser J, Henrichsen T, Mok Q, et al. Prolonged mechanical ventilation as a consequence of acute illness. Arch Dis Child. 1998;78(3):253–6.CrossRefPubMedPubMedCentralGoogle Scholar
  72. 72.
    Sritippayawan S, Kun SS, Keens TG, Davidson Ward SL. Initiation of home mechanical ventilation in children with neuromuscular diseases. J Pediatr. 2003;142:481–5.CrossRefPubMedGoogle Scholar
  73. 73.
    Gillis J, Tibballs J, McEniery J, Heavens J, Hutchins P, Kilham HA, Henning R. Ventilator-dependent children. Med J Aust. 1989;150:10–4.PubMedGoogle Scholar
  74. 74.
    Nelson JE, Hope AA. Integration of palliative care in chronic critical illness management. Respir Care. 2012;57(6):1004–12.CrossRefPubMedGoogle Scholar
  75. 75.
    Wolff A, Browne J, Whitehouse WP. Personal resuscitation plans and end of life planning for children with disability and life-limiting/life-threatening conditions. Arch Dis Child Educ Pract Ed. 2011;96(2):42–8.CrossRefPubMedGoogle Scholar
  76. 76.
    American Academy of Pediatrics Committee on Bioethics. Ethics and the care of critically ill infants and children. Pediatrics. 1996;98(1):149–52.Google Scholar
  77. 77.
    Burns JP, Mitchell C. Do-not-resuscitate orders and redirection of treatment. In: Friedman SL, Helm DT, editors. End-of-life care for children and adults with intellectual and developmental disabilities. Washington, DC: American Association on Intellectual and Developmental Disabilities; 2010. p. 147–59.Google Scholar
  78. 78.
    Clark JD, Dudzinski DM. The culture of dysthanasia: attempting CPR in terminally Ill children. Pediatrics. 2013;131(3):572–80. doi: 10.1542/peds.2012-0393.CrossRefPubMedGoogle Scholar
  79. 79.
    Lustbader DR, Nelson JE, Weissman DE, Hays RM, Mosenthal AC, Mulkerin C, Puntillo KA, Ray DE, Bassett R, Boss RD, Brasel KJ, Campbell ML, Cortez TB, Curtis JR, IPAL-ICU Project. Physician reimbursement for critical care services integrating palliative care for patients who are critically ill. Chest. 2012;141(3):787–92.CrossRefPubMedPubMedCentralGoogle Scholar
  80. 80.
    Simonds AK, Muntoni F, Heather S, et al. Impact of nasal ventilation on survival in hypercapnic Duchenne muscular dystrophy. Thorax. 1998;53(11):949–52.CrossRefPubMedPubMedCentralGoogle Scholar
  81. 81.
    Feudtner C, Carroll KW, Hexem KR, Silberman J, Kang TI, Kazak AE. Parental hopeful patterns of thinking, emotions, and pediatric palliative care decision making: a prospective cohort study. Arch Pediatr Adolesc Med. 2010;164(9):831–9.CrossRefPubMedGoogle Scholar
  82. 82.
    Stein GL. Providing palliative care to people with intellectual disabilities: services, staff knowledge, and challenges. J Palliat Med. 2008;11(9):1241–8.CrossRefPubMedGoogle Scholar
  83. 83.
    Freed MM. Academy presidential address. Quality of life: the physician’s dilemma. Arch Phys Med Rehabil. 1984;65(3):109–11.PubMedGoogle Scholar
  84. 84.
    Levy J, van Stone M. Ethical foundations and legal issues. In: Friedman SL, Helm DT, editors. End-of-life care for children and adults with intellectual and developmental disabilities. Washington, DC: American Association on Intellectual and Developmental Disabilities; 2010. p. 31–49.Google Scholar
  85. 85.
    Meert KL, Eggly S, Pollack M, Anand KJ, Zimmerman J, Carcillo J, Newth CJ, Dean JM, Willson DF, Nicholson C, National Institute of Child Health and Human Development Collaborative Pediatric Critical Care Research Network. Parents’ perspectives on physician-parent communication near the time of a child’s death in the pediatric intensive care unit. Pediatr Crit Care Med. 2008;9(1):2–7.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2016

Authors and Affiliations

  1. 1.Division of Pediatric Critical Care MedicineColumbia University College of Physicians and SurgeonsNew YorkUSA

Personalised recommendations