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Liver Failure

Lyn Crellin

Paediatric Liver Transplant Coordinator


Victorian Liver Transplant Unit
Royal Children’s Hospital
Liver Physiology

Major functions of the liver


o Regulates blood glucose, stores energy

o Regulates blood clotting

o Produces & eliminates bile

o Neutralises and destroys toxins, drugs

o Detoxifies/eliminates – hormones, endotoxins etc

o Resists infection

o Regenerates its own damaged tissue

o Stores glucose, iron, vitamins, minerals, blood

Guyton Arthur C. & Hall John E. (2006)


Textbook of Medical Physiology 11th.
Ed., Elsevier, Philadelphia
Chronic Liver Disease 2008
Liver Disease
Arises from
o Congenital disorders

o Genetic disorders

o Medications/Drugs

o Toxins

o Infections

Inflammatory process Cirrhosis

Chronic Liver Disease 2008


Cirrhosis
Distorted architecture of the liver
o Necrosis
• Inflammation & cytokines release
o Fibrosis
• Formation basement membrane &
channels
o Regeneration
• Node formation

Hardy, Stephen, & Kleinman Ronald. (2007) Cirrhosis and


Chronic Liver Failure In: (Eds.) Suchy Frederick J., Sokol
Ronald J. & Balistreri William F. Liver Diseases in Children.
3rd. Ed., University Press, Cambridge
Chronic Liver Disease 2008
Cirrhosis

Injury→
formation
Cirrhosis collagen, &
basement
membrane

Regeneration,
Ischaemia & node
cell death formation →
↑pressure &
ischaemia

Chronic Liver Disease 2008


Cirrhosis

Portal Hypertension
Chronic Liver Disease 2008
Portal Hypertension
Reduced blood flow
through liver

Ascites
Splenomegaly
Collateral
circulation
Varices and
haemorrhage
(With thanks to Associate Professor Winita Hardikar
who supplied this slide)

Chronic Liver Disease 2008


Clinical Features of Liver Disease

o Malabsorption/diarrhoea o Coagulopathy
o ↓ Growth o Osteopaenia
o ↓ Development o Fluid & electrolyte
derangement
o Ascites o Hormone derangement
o Vomiting o Jaundice
o Gastro oesophageal reflux o Itching

Hardy, Stephen, & Kleinman Ronald,. (2007) Cirrhosis and Chronic Liver
Failure In : (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri
William F. Liver Diseases in Children. 3rd. Ed., University Press,
Cambridge

Chronic Liver Disease 2008


Liver Disease – causes in children
Most causes are uncommon:

Chronic Acute
o Biliary atresia o Medications

o Alagille’s syndrome o Some Homeopathic

o A-1-antitrypsin deficiency remedies


o Wilson’s Disease o Poisons

o Autoimmune Hepatitis o Toxins

o others… o unknown - ?viral

Chronic Liver Disease 2008


Chronic Liver Disease 2008
Gastrointestinal Tract: obstructed
bile flow

 Absorption of fats

Absorption
Steatorrhea Weight loss  Growth
of vitamin ADEK

Chronic Liver Disease 2008


Gastrointestinal Tract: obstructed
bile flow

Management
o Formula with pre-digested fats
o Maximal calories
o Fat soluble vitamin supplements

Chronic Liver Disease 2008


Gastrointestinal Tract:
catabolic/anabolic

Glycolsis
Glucogenosis
Glucogeogenesis
Ketogemesis

Energy Weight Loss Growth Hypoglycaemia

Chronic Liver Disease 2008


Gastrointestinal Tract:
catabolic/anabolic

Management
o Monitoring of growth
o High calorie formula & diet
o Smaller & more frequent volumes
o N/G feeds (continuous)
o Control of ascites
o Conserve calories – keep well covered

o I/V Dextrose & BSL’s when fasting

Chronic Liver Disease 2008


Gastrointestinal Tract: portal
hypertension

o Gastropathy
o ↑ gastric secretion
o Varices (+/- Bleeding)

Varices - Gastroscopy
Hardy, Stephen, & Kleinman Ronald . (2007)Cirrhosis and Chronic Liver
Failure In: (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri
William F. Liver Diseases in Children. 3rd. Ed., University Press,
Cambridge

Chronic Liver Disease 2008


Gastrointestinal Tract: portal
hypertension

Management
o Observation re haematemesis, malaena
o Careful placement of N/G tubes – lubricant ++, no
stilettes,
o Omeprazole
o Banding &/or Sclerotherapy
o Propanolol
o Portocaval shunts

Chronic Liver Disease 2008


Haematological System

o Splenomegaly
o Pancytopaenia
o ↓Clotting factors
o ↓Anti-coagulation
factors
o ↓Haemoglobin
o ↓WCC Hardy, Stephen, & Kleinman Ronald . (2007) Cirrhosis and Chronic
Liver Failure In: (Eds) Suchy Frederick J., Sokol Ronald J. & Balistreri
William F. Liver Diseases in Children. 3rd. Ed., University Press,
Cambridge

Chronic Liver Disease 2008


Haematological System

Management
o Observation for pallor, bruising, bleeding
o Careful management of I/V sites, or injections
o No contact sports re splenomegaly
o Vitamin K
o Iron supplements
o Doppler U/S re potential for thrombosed hepatic
vessels

Chronic Liver Disease 2008


Cardiovascular System &
Haemodynamics

Decreased renal
perfusion
o Peripheral
dilatation ADH secretion
o Collateral vessels Renin-
o Hyoalbuminaemia, angiotension,
(leak into aldosterone
peritoneum) activation
Cardens, Andres & Gines̀ Pere, (2005) Management of
complications of cirrhosis in patients awaiting liver
transplantation. Journal of Hepatology 42, S124-S133

Chronic Liver Disease 2008


Cardiovascular System &
Haemodynamics

Management
o Fluid balance, weight, girth, assessment of
ascites
o ↓Sodium diet

o Diuretics

o Albumin replacements

Chronic Liver Disease 2008


Renal System

o ↓Renal perfusion
  ADH
o Water retention &
hyponatraemia
Hepatorenal
Syndrome
Cardens, Andres & Gines̀ Pere, (2005) Management of
complications of cirrhosis in patients awaiting liver
transplantation. Journal of Hepatology 42, S124-S133

Chronic Liver Disease 2008


Renal System

Management
o Fluid balance, weight, girth,

o Fluid restriction

o Diuretics

o Albumin replacement

o Haemofiltration (Hepatorenal Syndrome)

Chronic Liver Disease 2008


Immune System

o Hypersplenism 
WCC
o Shunting → Kuffer
cell activity
o ↑Risk of infection
(Spontaneous
Bacterial
Peritonitis)
Hardy, Stephen, & Kleinman Ronald . (2007) Cirrhosis and Chronic Liver
Failure In: (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri William F.
Liver Diseases in Children. 3rd. Ed., University Press, Cambridge

Chronic Liver Disease 2008


Immune System

Management
o Monitoring temperature

o Prompt attention to fevers

o Immunizations

o Antibiotic prophylaxis

Chronic Liver Disease 2008


Endocrine System

o ↓ Hormone activity: parathyroid,


mineral/gluco-corticosteroids, growth
& sex hormones etc
o ↓Growth & puberty
o ↓Vitamin D & bone mineralization

Hardy, Stephen, & Kleinman Ronald. (2007) Cirrhosis and Chronic Liver Failure In: (Eds.)
Suchy Frederick J., Sokol Ronald J. & Balistreri William F. (Eds.) Liver Diseases in
Children. 3rd. Ed. University Press, Cambridge

Chronic Liver Disease 2008


Endocrine System

Management
o Monitoring of growth & development

o Vitamin D replacement

o Careful handling to avoid fractures

o Maximal nutrition

o ?Post transplant hormone supplements

Chronic Liver Disease 2008


Respiratory System
o Ascites →
respiratory
excursion
o Muscle bulk →
respiratory
excursion
o Clubbing
Hepatopulmonary
o Arteriovenous syndrome (Resolves
shunts post transplant)
Hardy, Stephen, & Kleinman Ronald,. (2007) Cirrhosis and Chronic Liver
Failure In: (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri William F.,
(Eds.)Liver Diseases in Children. 3rd. Ed University Press, Cambridge

Chronic Liver Disease 2008


Respiratory System

Management
o Monitoring of respiratory rate, effort &
oximetry
o Lung function tests

o Control of ascites

o Maximal nutrition

o Oxygen therapy (Hepatopulmonary


Syndrome)

Chronic Liver Disease 2008


Muscular/skeletal System

o ↓Muscle weakness
o Peripheral
neuropathy
o Tremors
o Osteopaenia

Hardy, Stephen, & Kleinman Ronald . (2007) Cirrhoisis and Chronic Liver Failure
In: (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri William F. Liver Diseases
in Children. 3rd. Ed. University Press, Cambridge

Chronic Liver Disease 2008


Muscular/skeletal System

Management
o Observe for muscle weakness & tremors

o Observe for fractures

o Nutrition

o Vitamin, electrolytes & mineral


supplements

Chronic Liver Disease 2008


Neurological System

o Encephalopathy:
?↓Deamination of
aminoacids

? ↓urea &
ammonia
levels
Hardy, Stephen, & Kleinman Ronald, . (2007) Cirrhosis and Chronic Liver Failure
In: (Eds.) Suchy Frederick J., Sokol Ronald J. & Balistreri William F. Liver
Diseases in Children. 3rd. Ed., University Press, Cambridge

Chronic Liver Disease 2008


Neurological System

Management
o Monitor conscious state

o ↓Protein intake

o Lactulose

o Neomycin

o Shunt

Chronic Liver Disease 2008


Psychosocial: parents

o Grief o Loss of control


o Guilt o Disruption of life style
o Anger o Social isolation
o Helpless o Social stigma
o Anxiety & uncertainty re o Financial hardship
future o ↑Risk of marriage
o Concerns re managing disintegration
illness o Worry about impact on
o Lack of information siblings
Knafl Kathleen & Santacroce Sheila (2004) Chronic Conditions
and the Family In: (Eds.) Allen, Patricia Jackson & Verrsy
Judith A. PRIMARY CARE OF THE CHILD with a CHRONIC
CONDITION (4th Ed.) Mosby St. Louis

Chronic Liver Disease 2008


Psychosocial: management -
parents
o Partnership with parents
o Communication - open
o Education - verbal & printed
o Information
o Ready access to team
o Counselling
o Social worker – financial & other resources
o Introduction to other parents
o Parents support group

Chronic Liver Disease 2008


Psychosocial: infants & children
o Sadness o Social isolation
o Frustration o Developmental delay
o Anger o ↓Mastery
o Loss of control o ↓Confidence
o Separation (hospital) o ↓Self Esteem
o Fear of pain o ↑Dependence
o Concerns re body image o ↑Risk of emotional &
o Social stigmatization behavioural problems
o Disruption to life style

Knafl Kathleen & Santacroce Sheila (2004) Chronic Conditions


Angström-Brännström Charlotte, Norberg Astrid &
and the Family In: (Eds.) Allen Patricia Jackson & Vessey Judith
Jansson Lillian. August 2008. Narratives of Children
A. PRIMARY CARE OF THE CHILD with a CHRONIC CONDITION.
with Chronic Illness About Being Comforted. Journl of
4th Ed. Mosby St. Louis
Pediatric Nursing 23, (4) 310-316

Chronic Liver Disease 2008


Psychosocial: management -
infants & children
o Maximal parental participation in care
o Facilitation of communication
o Facilitation of choices
o Facilitation of routine
o Play Therapist - medical play & diversional
techniques
o Pain management team
o Counselling
o Music therapy
o Liaison with school

Chronic Liver Disease 2008


Summary: Principals of Nursing Care
o Monitoring vital signs, conscious state, growth, GI
losses
o Monitoring skin, limbs & abdominal distension
o Accurate fluid balance chart, weight & girth
o Optimization & conservation of calories
o I/V Glucose & monitoring BSL’s when fasting
o Careful insertion of N/G tubes
o Gentle handling & careful positioning
o Administration of medications/infusions
o Psychosocial support

Chronic Liver Disease 2008


Liver Disease

o Is a complex and miserable disease

o Requires meticulous medical & nursing care

o Impacts on all the systems

o Often results in liver transplantation

Chronic Liver Disease 2008


Chronic Liver Disease 2008

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