Skip to main content
SpringerLink
Log in

Psychosocial, Cognitive, and Quality of Life Considerations in the Child with Liver Disease and Their Family

  • Chapter
  • First Online:
Diseases of the Liver in Children

Abstract

Morbidity associated with chronic liver disease affects the psychosocial and health-related quality of life (HRQOL) of patients and their families. Pediatric patients with liver disease demonstrate deficits in IQ that typically persist even after liver transplantation. This may be related to prolonged hospitalizations and malnutrition that often occur in this population. Research on other cognitive domains are ongoing but have demonstrated similar deficits. The chronicity of liver disease negatively affects both patients and their families through frequent hospitalizations and procedures as well as emotional and behavioral problems at home. HRQOL remains below that of the general population with school function of particular concern.

Use of different tests based on age, small sample sizes, and heterogeneous study designs has made the accurate assessment of these outcomes very challenging. In this chapter, we present a summary of what is known regarding psychosocial, cognitive, and HRQOL outcomes of children with liver disease and describe approaches to better measure, study, and improve these outcomes.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 139.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 179.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 249.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

References

  1. Simon NB, Smith D. Living with chronic pediatric liver disease: the parents’ experience. Pediatr Nurs. 1992;18(5):453–8, 489.

    PubMed  CAS  Google Scholar 

  2. Mastroyannopoulou K, et al. Psychological effects of liver disease and transplantation. Eur J Pediatr. 1998;157(10):856–60.

    Article  PubMed  CAS  Google Scholar 

  3. Garrison WT, McQuiston S. Chronic illness during childhood and adolescence: psychological aspects. Newbury Park: Sage Publications, Inc.; 1989.

    Google Scholar 

  4. Foerster BR, et al. Minimal hepatic encephalopathy in children: evaluation with proton MR spectroscopy. Am J Neuroradiol. 2009;30:1610–3.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  5. Yadav SK, et al. Brain MR imaging and 1H-MR spectroscopy changes in patients with extrahepatic portal vein obstruction from early childhood to adulthood. Am J Neuroradiol. 2010;31:1337–42.

    Article  PubMed  CAS  Google Scholar 

  6. Yadav SK, et al. Encephalopathy assessment in children with extra-hepatic portal vein obstruction with MR, psychometry, and critical flicker frequency. J Hepatol. 2010;52:348–54.

    Article  PubMed  Google Scholar 

  7. Srivastava A, et al. Pro-inflammatory cytokines are raised in extrahepatic portal venous obstruction, with minimal hepatic encephalopathy. J Gastroenterol Hepatol. 2011;26:979–86.

    Article  PubMed  CAS  Google Scholar 

  8. Eroglu Y, et al. Improved neurocognitive function after radiologic closure of congenital portosystemic shunts. J Pediatr Gastroenterol Nutr. 2004;39:410–7.

    Article  PubMed  Google Scholar 

  9. Mack CL, et al. Surgically restoring portal blood flow to the liver in children with primary extrahepatic portal vein thrombosis improves fluid neurocognitive ability. Pediatrics. 2006;117(3):e405–12.

    Article  PubMed  Google Scholar 

  10. Lautz TB, et al. Management and classification of type II congenital portosystemic shunts. J Pediatr Surg. 2011;46:308–14.

    Article  PubMed  Google Scholar 

  11. Rodrigue JR, et al. Impact of Hepatitis C virus infection on children and their caregivers: quality of life, cognitive, and emotional outcomes. J Pediatr Gastroenterol Nutr. 2009;48:341–7.

    Article  PubMed  PubMed Central  Google Scholar 

  12. Rodrigue JR, et al. Peginterferon with or without ribavirin has minimal effect on quality of life, behavioral/emotional, and cognitive outcomes in children. Hepatology. 2011;53(5):1468–75.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  13. Sorensen LG, et al. Cognitive and academic outcomes after pediatric liver transplantation: Functional Outcomes Group (FOG) results. Am J Transplant. 2011;11:303–11.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  14. Amodio P, et al. Characteristics of minimal hepatic encephalopathy. Metab Brain Dis. 2004;19(3–4):253–67.

    Article  PubMed  Google Scholar 

  15. Bao ZJ, et al. Assessment of health-related quality of life in Chinese patients with minimal hepatic encephalopathy. World J Gastroenterol. 2007;13(21):3003–8.

    PubMed  Google Scholar 

  16. Wunsch E, et al. Minimal hepatic encephalopathy does not impair health-related quality of life in patients with cirrhosis: a prospective study. Liver Int. 2011;31(7):980–4.

    Article  PubMed  Google Scholar 

  17. Stewart SM, et al. Mental and motor development, social competence, and growth one year after successful pediatric liver transplantation. J Pediatr. 1989;114(4 Pt 1):574–81.

    Article  PubMed  CAS  Google Scholar 

  18. Zitelli BJ, et al. Changes in life-style after liver transplantation. Pediatrics. 1988;82(2):173–80.

    PubMed  CAS  PubMed Central  Google Scholar 

  19. Hoffmann 3rd RG, et al. Moderating effects of family functioning on the social adjustment of children with liver disease. Child Health Care. 1995;24(2):107–17.

    Article  PubMed  Google Scholar 

  20. Varni JW, et al. The PedsQL Infant Scales: feasibility, internal consistency reliability, and validity in healthy and ill infants. Qual Life Res. 2011;20(1):45–55.

    Article  PubMed  Google Scholar 

  21. Cole CR, et al. Impact of liver transplantation on HRQOL in children less than 5 years old. Pediatr Transplant. 2004;8(3):222–7.

    Article  PubMed  Google Scholar 

  22. Stewart SM, et al. Mental and motor development correlates in patients with end-stage biliary atresia. Pediatrics. 1987;79(6):882–8.

    PubMed  CAS  Google Scholar 

  23. Caudle SE, et al. Language and motor skills are impaired in infants with biliary atresia before transplantation. J Pediatr. 2010;156:936–40.

    Article  PubMed  Google Scholar 

  24. Stewart SM, et al. Mental development and growth in children with chronic liver disease of early and late onset. Pediatrics. 1988;82(2):167–72.

    PubMed  CAS  Google Scholar 

  25. Stewart SM, et al. Cognitive patterns in school-age children with end-stage liver disease. J Dev Behav Pediatr. 1992;13(5):331–8.

    PubMed  CAS  Google Scholar 

  26. Howard ER, et al. Survival patterns in biliary atresia and comparison of quality of life of long-term survivors in Japan and England. J Pediatr Surg. 2001;36(6):892–7.

    Article  PubMed  CAS  Google Scholar 

  27. Crosnier C, et al. Alagille syndrome. The widening spectrum of arteriohepatic dysplasia. Clin Liver Dis. 2000;4(4):765–78.

    Article  PubMed  CAS  Google Scholar 

  28. Emerick KM, et al. Features of Alagille syndrome in 92 patients: frequency and relation to prognosis. Hepatology. 1999;29(3):822–9.

    Article  PubMed  CAS  Google Scholar 

  29. Papapetropoulos S, et al. Case of pediatric acquired chronic hepatocerebral degeneration. Pediatr Neurol. 2008;38(1):67–70.

    Article  PubMed  Google Scholar 

  30. Elisofon SA, et al. Health status of patients with Alagille syndrome. J Pediatr Gastroenterol Nutr. 2010;51(6):759–65.

    Article  PubMed  Google Scholar 

  31. Dening TR, Berrios GE. Wilson’s disease: a longitudinal study of psychiatric symptoms. Biol Psychiatry. 1990;28(3):255–65.

    Article  PubMed  CAS  Google Scholar 

  32. El-Karaksy H, et al. A clinical study of Wilson’s disease: the experience of a single Egyptian Paediatric Hepatology Unit. Arab J Gastroenterol. 2011;12(3):125–30.

    Article  PubMed  Google Scholar 

  33. Medalia A, Scheinberg H. Intellectual functioning in treated Wilson’s disease. Ann Neurol. 1991;29(5):573–4.

    Article  PubMed  CAS  Google Scholar 

  34. Hegde S, et al. Cognitive profile and structural findings in Wilson’s disease: a neuropsychological and MRI-based study. Neurol India. 2010;58(5):708–13.

    Article  PubMed  Google Scholar 

  35. Xu P, et al. Category and perceptual learning in subjects with treated Wilson’s disease. PLoS One. 2010;5(3):e9635.

    Article  PubMed  PubMed Central  Google Scholar 

  36. Lorincz MT. Neurologic Wilson’s disease. Ann N Y Acad Sci. 2010;1184:173–87.

    Article  PubMed  CAS  Google Scholar 

  37. Lin JJ, et al. Psychological presentations without hepatic involvement in Wilson disease. Pediatr Neurol. 2006;35(4):284–6.

    Article  PubMed  Google Scholar 

  38. Carta MG, et al. Bipolar disorders and Wilson’s disease. BMC Psychiatry. 2012;12:52.

    Article  PubMed  PubMed Central  Google Scholar 

  39. Svetel M, et al. Quality of life in patients with treated and clinically stable Wilson’s disease. Mov Disord. 2011;26(8):1503–8.

    Article  PubMed  Google Scholar 

  40. Thimm E, et al. Neurocognitive outcome in patients with hypertyrosinemia type I after long-term treatment with NTBC. J Inherit Metab Dis. 2012;35(2):263–8.

    Article  PubMed  CAS  Google Scholar 

  41. Krivitzky L, et al. Intellectual, adaptive, and behavioral functioning in children with urea cycle disorders. Pediatr Res. 2009;66(1):96–101.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  42. Nicolaides P, et al. Neurological outcome of patients with ornithine carbamoyltransferase deficiency. Arch Dis Child. 2002;86(1):54–6.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  43. Campeau PM, et al. Early orthotopic liver transplantation in urea cycle defects: follow up of a developmental outcome study. Mol Genet Metab. 2010;100 Suppl 1:S84–7.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  44. Bachmann C. Long-term outcome of patients with urea cycle disorders and the question of neonatal screening. Eur J Pediatr. 2003;162 Suppl 1:S29–33.

    Article  PubMed  CAS  Google Scholar 

  45. Cederbaum JA, et al. Psychosocial issues and coping strategies in families affected by urea cycle disorders. J Pediatr. 2001;138(1 Suppl):S72–80.

    Article  PubMed  CAS  Google Scholar 

  46. Lee WS, Sokol RJ. Liver disease in mitochondrial disorders. Semin Liver Dis. 2007;27(3):259–73.

    Article  PubMed  CAS  Google Scholar 

  47. Salviati L, et al. Mitochondrial DNA depletion and dGK gene mutations. Ann Neurol. 2002;52(3):311–7.

    Article  PubMed  CAS  Google Scholar 

  48. Forton DM, et al. Hepatitis C and cognitive impairment in a cohort of patients with mild liver disease. Hepatology. 2002;35(2):433–9.

    Article  PubMed  Google Scholar 

  49. Lim JK, et al. The impact of chronic hepatitis C and comorbid psychiatric illnesses on health-related quality of life. J Clin Gastroenterol. 2006;40(6):528–34.

    Article  PubMed  Google Scholar 

  50. Nydegger A, et al. Health-related quality of life in children with hepatitis C acquired in the first year of life. J Gastroenterol Hepatol. 2008;23(2):226–30.

    Article  PubMed  Google Scholar 

  51. Ng VL, et al. Outcomes of 5-year survivors of pediatric liver transplantation: report on 461 children from a north american multicenter registry. Pediatrics. 2008;122(6):e1128–35.

    Article  PubMed  Google Scholar 

  52. Adeback P, Nemeth A, Fischler B. Cognitive and emotional outcome after pediatric liver transplantation. Pediatr Transplant. 2003;7:385–9.

    Article  PubMed  Google Scholar 

  53. Schulz K-H, et al. Cognitive performance of children who have undergone liver transplantation. Transplantation. 2003;75(8):1236–40.

    Article  PubMed  Google Scholar 

  54. Kaller T, et al. Cognitive abilities in children after liver transplantation. Transplantation. 2005;79(9):1252–6.

    Article  PubMed  Google Scholar 

  55. Kaller T, et al. Cognitive abilities, behaviour, and quality of life in children after liver transplantation. Pediatr Transplant. 2010;14(4):496–503.

    Article  PubMed  CAS  Google Scholar 

  56. Krull K, et al. Neurocognitive outcome in pediatric liver transplant recipients. Pediatr Transplant. 2003;7:111–8.

    Article  PubMed  Google Scholar 

  57. Gilmour S, et al. Assessment of psychoeducational outcomes after pediatric liver transplant. Am J Transplant. 2009;9:294–300.

    Article  PubMed  CAS  Google Scholar 

  58. Kennard BD, et al. Academic outcome in long-term survivors of pediatric liver transplantation. J Dev Behav Pediatr. 1999;20(1):17–23.

    Article  PubMed  CAS  Google Scholar 

  59. Wayman KI, Cox KL, Esquivel CO. Neurodevelopmental outcome of young children with extrahepatic biliary atresia 1 year after liver transplantation. J Pediatr. 1997;131(6):894–8.

    Article  PubMed  CAS  Google Scholar 

  60. Haavisto A, et al. Visuospatial impairment in children and adolescents after liver transplantation. Pediatr Transplant. 2011;15:184–92.

    PubMed  Google Scholar 

  61. Stewart SM, et al. Neuropsychological outcome of pediatric liver transplantation. Pediatrics. 1991;87(3):367–76.

    PubMed  CAS  Google Scholar 

  62. Kaller T, et al. Attention and executive functioning deficits in liver transplanted children. Clin Transl Res. 2010;90(12):1567–73.

    Google Scholar 

  63. Gilmour SM, et al. School outcomes in children registered in the studies for pediatric liver transplant (SPLIT) consortium. Liver Transpl. 2010;16(9):1041–8.

    Article  PubMed  PubMed Central  Google Scholar 

  64. Grabhorn E, et al. Liver transplantation in infants younger than 6 months old. Transplant Proc. 2002;34:1964–5.

    Article  PubMed  Google Scholar 

  65. Schauer R, et al. Treatment of Crigler-Najjar type I disease: relevance of early liver transplantation. J Pediatr Surg. 2003;38(8):1227–31.

    Article  PubMed  Google Scholar 

  66. Shellmer DA, et al. Cognitive and adaptive functioning after liver transplantation for maple syrup urine disease: a case series. Pediatr Transplant. 2011;15:58–64.

    Article  PubMed  CAS  PubMed Central  Google Scholar 

  67. Barshes NR, et al. Evaluation and management of patients with propionic acidemia undergoing liver transplantation: a comprehensive review. Pediatr Transplant. 2006;10:773–81.

    Article  PubMed  Google Scholar 

  68. Yoshitoshi EY, et al. Long-term outcomes for 32 cases of Wilson’s disease after living-related donor liver transplantation. Transplantation. 2009;87(2):261–7.

    Article  PubMed  Google Scholar 

  69. Beath SV, et al. Long term outcome of liver transplantation (LTx) in babies aged less than 12 months. J Pediatr Gastroenterol Nutr. 1997;24(4):485.

    Article  Google Scholar 

  70. van Mourik IDM, et al. Long-term nutritional and neurodevelopmental outcome of liver transplantation in infants aged less than 12 months. J Pediatr Gastroenterol Nutr. 2000;30(3):269–75.

    Article  PubMed  Google Scholar 

  71. Ikegami T, et al. Effect of liver transplantation in a twin for biliary atresia on physical development and intellectual performance: report of a case. Surg Today. 2000;30:841–3.

    Article  PubMed  CAS  Google Scholar 

  72. Baron IS, Rey-Casserly C. Pediatric neuropsychology: medical advances and lifespan outcomes. Oxford, New York: Oxford University Press; 2013.

    Google Scholar 

  73. Chin SE, et al. Survival, growth and quality of life in children after orthotopic liver transplantation: a 5 year experience. J Paediatr Child Health. 1991;27(6):380–5.

    Article  PubMed  CAS  Google Scholar 

  74. Tornqvist J, et al. Long-term psychosocial adjustment following pediatric liver transplantation. Pediatr Transplant. 1999;3(2):115–25.

    Article  PubMed  CAS  Google Scholar 

  75. Gritti A, et al. Emotional and behavioral problems after pediatric liver transplantation: a quantitative assessment. Pediatr Transplant. 2006;10(2):205–9.

    Article  PubMed  Google Scholar 

  76. Wise BV. In their own words: the lived experience of pediatric liver transplantation. Qual Health Res. 2002;12(1):74–90.

    PubMed  Google Scholar 

  77. Gritti A, et al. Psychological impact of liver transplantation on children’s inner worlds. Pediatr Transplant. 2001;5(1):37–43.

    Article  PubMed  CAS  Google Scholar 

  78. Sundaram SS, et al. Adolescent health-related quality of life following liver and kidney transplantation. Am J Transplant. 2007;7(4):982–9.

    Article  PubMed  CAS  Google Scholar 

  79. Tarbell SE, Kosmach B. Parental psychosocial outcomes in pediatric liver and/or intestinal transplantation: pretransplantation and the early postoperative period. Liver Transpl Surg. 1998;4(5):378–87.

    Article  PubMed  CAS  Google Scholar 

  80. Mintzer LL, et al. Traumatic stress symptoms in adolescent organ transplant recipients. Pediatrics. 2005;115(6):1640–4.

    Article  PubMed  Google Scholar 

  81. Young GS, et al. Symptoms of posttraumatic stress disorder in parents of transplant recipients: incidence, severity, and related factors. Pediatrics. 2003;111(6 Pt 1):e725–31.

    Article  PubMed  Google Scholar 

  82. Fredericks EM, et al. Psychological functioning, nonadherence and health outcomes after pediatric liver transplantation. Am J Transplant. 2007;7(8):1974–83.

    Article  PubMed  CAS  Google Scholar 

  83. Alonso EM, et al. Health-related quality of life and family function following pediatric liver transplantation. Liver Transpl. 2008;14(4):460–8.

    Article  PubMed  Google Scholar 

  84. Rodrigue JR, et al. Transplantation in children. A longitudinal assessment of mothers’ stress, coping, and perceptions of family functioning. Psychosomatics. 1997;38(5):478–86.

    Article  PubMed  CAS  Google Scholar 

  85. Maynard A, Martini MI. Learning in cultural context: family, peers, and school, International and cultural psychology series. New York: Kluwer Academic/Plenum Publishers; 2005. p. 280, viii.

    Google Scholar 

  86. Schulz K, et al. Comparison of quality of life and family stress in families of children with living-related liver transplants versus families of children who received a cadaveric liver. Transplant Proc. 2001;33(1–2):1496–7.

    Article  PubMed  CAS  Google Scholar 

  87. DeBolt AJ, et al. A survey of psychosocial adaptation in long-term survivors of pediatric liver transplants. Child Health Care. 1995;24(2):79–96.

    Article  PubMed  CAS  Google Scholar 

  88. Limbers CA, et al. Health-related quality of life in pediatric liver transplant recipients compared with other chronic disease groups. Pediatr Transplant. 2011;15(3):245–53.

    Article  PubMed  PubMed Central  Google Scholar 

  89. Alonso EM, et al. Cross-sectional analysis of health-related quality of life in pediatric liver transplant recipients. J Pediatr. 2010;156:270–6.e1.

    Article  PubMed  PubMed Central  Google Scholar 

  90. Alonso EM, et al. Functional outcomes of pediatric liver transplantation. J Pediatr Gastroenterol Nutr. 2003;37(2):155–60.

    Article  PubMed  CAS  Google Scholar 

  91. Bucuvalas JC, et al. Health-related quality of life in pediatric liver transplant recipients: a single-center study. Liver Transpl. 2003;9(1):62–71.

    Article  PubMed  Google Scholar 

  92. Bucuvalas JC, Britto M. Health-related quality of life after liver transplantation: it’s not all about the liver. J Pediatr Gastroenterol Nutr. 2003;37(2):106–8.

    Article  PubMed  Google Scholar 

  93. Varni JW, et al. PedsQL Cognitive Functioning Scale in pediatric liver transplant recipients: feasibility, reliability, and validity. Qual Life Res. 2011;20(6):913–21.

    Article  PubMed  PubMed Central  Google Scholar 

  94. Fredericks EM, et al. Adherence and health-related quality of life in adolescent liver transplant recipients. Pediatr Transplant. 2008;12(3):289–99.

    Article  PubMed  Google Scholar 

  95. Prasad S, et al. Lactulose improves cognitive functions and health-related quality of life in patients with cirrhosis who have minimal hepatic encephalopathy. Hepatology. 2007;45(3):549–59.

    Article  PubMed  Google Scholar 

  96. Mohammad S, Alonso EM, Approach to Optimizing Growth, rehabilitation and neurodevelopmental Outcomes in Children After Solid Organ Transplantation. Pediatric Clinics of North America. 2010;57(2).

    Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Pediatrics, Gastroenterology, Hepatology and Nutrition, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Saeed Mohammad MD

  2. Ann and Robert H. Lurie Children’s Hospital of Chicago, 225 East Chicago Avenue, 65, Chicago, IL, 60611-2605, USA

    Saeed Mohammad MD

  3. Department of Psychiatry and Behavioral Sciences, Clinical Psychiatry and Behavioral Sciences, Northwestern University’s Feinberg School of Medicine, Chicago, IL, USA

    Lisa G. Sorensen PhD

  4. Department of Psychiatry and Behavioral Sciences, Ann and Robert H. Lurie Children’s Hospital of Chicago, 225 East Chicago Ave, 10, Chicago, IL, 60611, USA

    Lisa G. Sorensen PhD

  5. Department of Pediatrics, Hepatology and Liver Transplantation, Gastroenterology, Hepatology and Nutrition, Ann and Robert H. Lurie Children’s Hospital of Chicago, 225 East Chicago Avenue, 65, Chicago, IL, 60611-2605, USA

    Estella M. Alonso MD

  6. Department of Pediatrics, Northwestern University Feinberg School of Medicine, Chicago, IL, USA

    Estella M. Alonso MD

Authors
  1. Saeed Mohammad MD
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Lisa G. Sorensen PhD
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Estella M. Alonso MD
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Estella M. Alonso MD .

Editor information

Editors and Affiliations

  1. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Karen F. Murray

  2. University of Washington School of Medicine, Seattle Children’s Hospital, Seattle, Washington, USA

    Simon Horslen

Rights and permissions

Reprints and permissions

Copyright information

© 2014 Springer Science+Business Media New York

About this chapter

Cite this chapter

Mohammad, S., Sorensen, L.G., Alonso, E.M. (2014). Psychosocial, Cognitive, and Quality of Life Considerations in the Child with Liver Disease and Their Family. In: Murray, K., Horslen, S. (eds) Diseases of the Liver in Children. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9005-0_7

Download citation

  • DOI: https://doi.org/10.1007/978-1-4614-9005-0_7

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9004-3

  • Online ISBN: 978-1-4614-9005-0

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation