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Paediatric Liver Disease and Liver Transplant

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Paediatric Liver Disease and Liver Transplant PAEDIATRIC LIVER DISEASE AND LIVER TRANSPLANT A Resource for Nurses in New Zealand 2 Acknowledgements and Disclaimer Appreciable care has been taken in the preparation of Meda Credland (Hawkesbay DHB) developed and obtain further information if required this package which Starship Children’s Health Mary Rutherford (Community Nurse Starship) with the support of their health care team. This resource provides as a tool for nurses and others to use in the will cover the most common causes of liver disease likely Haylee Riddell Social Worker (Starship) nursing of children with liver disease and liver transplant. to lead to transplant within childhood or adolescent Robyn Agnew Social Worker (Starship) years and issues related to liver transplant until transfer However in view of the possibility of human error or Caroline De Luca Pharmacist (Starship) of the patient to adult care. advances in medical knowledge, Starship Children’s Kim Herbison Dietitian (Starship) Health cannot and does not guarantee that the It does not cover the myriad of conditions such as Viral Rebecca Bruce Dietitian (Starship) information contained in this package is in every respect Hepatitis which is unlikely to ultimately lead to transplant accurate or complete. You are encouraged to consult Dr Jon Bishop, Gastroenterologist/ Hepatologist, for during childhood. Nor does it include extremely rare other sources in order to confirm the information painstakingly reviewing the medical information or complex conditions that may lead to transplant for contained in any of this package and in the event that Cate Fraser Irwin, CNS Liver Gastro Starship for believing which very individualised management is required. In medical treatment is required seek advice and input from it would happen and supporting me during the project such cases information and support will continue to be an experienced medical practitioner. in a myriad of ways. provided to the shared care team on a case by case basis. This nursing resource was written by Karyn Sanson Dr Helen Evans, Dr Simon Chin and Dr Stephen Mouat Clinical Nurse Specialist Liver / Gastroenterology Gastroenterologists for their unfailing support The Starship Children’s Health 2012 Immune Deficiency Foundation NZ (IDFNZ) who have provided the substantial funding required for the graphic This resource uses the Liver Disease Learning Package for design and diagram drawing, along with ongoing Nurses written by me during my employment by at the support and encouragement throughout the project. Royal Children’s Hospital, Melbourne, as its base. The Judith Phillipson Scholarship trustees for the I would like to acknowledge the following people scholarship grant which allowed dedicated time for the for their contribution and support whilst writing this project and the means to keep it current into the future document: Layout design, Illustrations and production by Tracey Liu Shared Care Nurses Review Group Purpose and limitations of this Ros Robertson (Canterbury DHB) resource Claire Hagglund (Southern DHB) This resource has been developed to assist nurses Robyn Kelly (Southern DHB) in caring for children and their families through the Wendy Diamond (Waikato DHB) transplant continuum; it has not been designed to Donna Aitken (Waikato DHB) meet the needs of parents and other non health Angela Weekly (Wanganui DHB) professionals. Parents and families should be encouraged Trish Silk (Wanganui DHB) use the parent guide to liver transplant which has been Version 2012 Written by Karyn Sanson © 2012 All rights reserved 3 Contents Part One: Liver Disease Section One: Anatomy Gross Anatomy ....................................................................................................................................................................................................................06 Blood Flow ............................................................................................................................................................................................................................08 Biliary Tract ...........................................................................................................................................................................................................................09 The Gallbladder ...................................................................................................................................................................................................................09 Section Two: Function of the Liver Bilirubin ..................................................................................................................................................................................................................................10 Carbohydrate .......................................................................................................................................................................................................................10 Fats...........................................................................................................................................................................................................................................10 Protein and Amino Acids .................................................................................................................................................................................................10 Coagulation ..........................................................................................................................................................................................................................11 Vitamins and Hormones ..................................................................................................................................................................................................11 Drug Metabolism ...............................................................................................................................................................................................................11 Section Three: Complications of Liver Disease Faltering growth .................................................................................................................................................................................................................11 Glucose control ...................................................................................................................................................................................................................13 Vitamin deficiency..............................................................................................................................................................................................................14 Portal hypertension and varices ...................................................................................................................................................................................15 Ascites ....................................................................................................................................................................................................................................19 Spontaneous bacterial peritonitis ................................................................................................................................................................................21 Hepto- renal syndrome ....................................................................................................................................................................................................21 Hepatic encephalopathy .................................................................................................................................................................................................22 Hepato- pulmonary syndrome ......................................................................................................................................................................................24 4 Development .......................................................................................................................................................................................................................24 Blood tests ............................................................................................................................................................................................................................25 Section Four: Disorders Of The Liver Infantile Cholangiopathies .............................................................................................................................................................................................27 Biliary Atresia .......................................................................................................................................................................................................................28 Kasai Procedure...................................................................................................................................................................................................................29 Alpha 1 Antitrypsin Deficiency ......................................................................................................................................................................................30
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