Making sense of Jaundice
What does the term jaundice mean?
Jaundice
describes a yellow discolouration of the skin, (plate 1) mucous membranes
(plate 2) and sclera of the eyes (plate 3) caused by greater than normal amounts
of bilirubin in the blood, (Anderson et al 1994). The discolouration is usually
first seen in the sclera and is often accompanied by pruritus.
Jaundice is sometimes referred to as icterus which
means yellow.
What is bilirubin?
When red
blood cells reach the end of their life they are broken down, largely in the
spleen. Bilirubin is the yellow pigment left after the breakdown of the
haemoglobin molecules. Used bilirubin is not recycled to make new red cells but
is excreted from the body. Liver cells take up bilirubin (together with its
oxygenation - product biliverdin), from the blood and
pass it into the bile ducts. Once in the bile ducts, bilirubin and biliverdin are referred to as bile pigments. After storage
and concentration in the gall bladder they pass into the duodenum and are
ultimately excreted in the faeces. Some bilirubin is reabsorbed from the gut
after being slightly altered by microbial action and excreted via the urine as
urobilinogen. In the gut the bile emulsifies fat, it also colours and partly
deodorises faeces, (Green 1978). This means that despite being an excretory
product, bile and the pigments it contains, also fulfils a useful physiological
function. (Fig.1)
What may cause Jaundice?
Any failure
of the bilirubin excretory pathway will result in an accumulation of bile
pigment in the blood causing the patient to turn yellow. Jaundice may also
occur if the capacity of the liver cells to remove bilirubin from the blood is
overloaded as when there is increased breakdown of red blood cells, ie.
increased haemolysis, (Fig.2). Shortly after birth there may be a physiological
jaundice owing to the relative immaturity of the liver coupled with the
breakdown of red cells which occurs after birth, (Campbell and Glasper, 1995). This physiological jaundice should not be
confused with the much more severe condition which is caused by a Rhesus factor
mismatch in haemolytic disease of the new-born.
What pathophysiological
changes are involved in jaundice?
This varies
depending on the aetiology of the condition. A useful way to classify jaundice
is as prehepatic, hepatic and posthepatic.
In pre-hepatic the cause is before the bilirubin reaches the liver, with
hepatic jaundice the pathology is in the liver itself and in post-hepatic there
is obstruction of the bile flow after it has left the liver.
Pre -
hepatic
This is
sometimes referred to as haemolytic jaundice, (
Clinically
the skin is usually a pale lemon yellow due to a combination of anaemia and
jaundice. There may also be splenomegaly as most
haemolysis occurs in the spleen. As there is no hepatic or post hepatic
obstruction bile is able to enter the gut normally so the stools appear normal.
In addition urobilinogen will be present in the urine as it will be reabsorbed
from the gut as normal. As the amounts of bile pigments entering the gut are
greater than normal, the amount of urobilinogen reabsorbed from the gut is also
increased, this raises levels in the urine. Blood tests will show an increase
in the number of immature red cells (reticulocytes)
as the body increases production to replace the haemolysed cells.
The only
causes of pre-hepatic jaundice other than increased haemolysis are some genetic
conditions causing congenital hyperbilirubinaemias.
Hepatic
Damaged
liver cells are less able to transfer bilirubin from the blood into the bile.
This form of the condition is usually referred to as hepatocellular jaundice.
Normal levels of bilirubin are produced from the breakdown of red cells, but
due to reduced liver cell function these accumulate in the blood. Liver cell function may be embarrassed
in infections of the liver such as viral hepatitis (Wilairatana
1996) . Primary liver cancer (hepatocellular carcinoma) may be another cause of
failure of liver cell function, (Lau et al. 1997). Another possible cause is
cell damage caused by poisons or drugs, (Richards, Boyter
and Nathwani 1996).
In addition
to reduced function of liver cells, hepatic jaundice may be caused by
conditions which cause swelling of the hepatocytes
leads to obstruction of the small bile ducts or canaliculi.
This is sometimes referred to as intrahepatic
obstructive (cholestatic) jaundice, (Houston Joiner
and Trounce 1987) and may be caused when there is swelling and inflammation of
liver cells in infections, after some drugs, or due to liver trauma (Nowak and Handford 1994). (Table 2)
Post
hepatic
This is due
to obstruction of the bile ducts after they have left the liver, sometimes
referred to as extrahepatic cholestatic
jaundice. The bile ducts may be obstructed due to the presence of gall stones
or other calculi. Alternatively, they may be blocked by pressure from outside
the ducts, for example cancer of the head of pancreas may obstruct the flow of
bile. In all forms of post hepatic obstructive jaundice the bile dams back into
the liver and then back into the blood to cause the jaundice. Because little or
no bile reaches the gut the stools become a pale colour due to the absence of
bile pigment. As there is no bilirubin in the gut none is reabsorbed into the
blood to be excreted by the kidneys as urobilinogen. This means the urine
contains little or none of this pigment. However as the levels of bile pigment
in the blood continue to rise it is excreted in the urine, once a renal
threshold is reached, causing dark coloured urine. (Table 3) Prolonged
obstruction may lead to damage of the liver cells leading to fibrosis and
ultimately cirrhosis.
What do the terms conjugated and unconjugated refer to?
Bilirubin is
released from the phagocytes of the reticuloendothelial
system. Red cells are broken down by phagocytosis which is the process of `cell
eating`, although this process takes place in various parts of the reticuloendothelial system the spleen is the primary site.
After this the `freed` bilirubin becomes attached to albumin in the blood which
facilitates its transport to the liver. This is the so called free or unconjugated form of the pigment. In this form bilirubin is
relatively insoluble and cannot pass into the glomerular
filtrate. Inside the liver cells the bilirubin is conjugated with glucuronide which makes it soluble in water, (conjugate
means `to join together`). It is in this soluble conjugated form that it is
passed into the bile ducts. The application of this is that in haemolytic and
hepatocellular disease most of the excess bilirubin is unconjugated
as it has not yet been processed by liver cells. In obstructive disease the
bilirubin has been processed through the liver cells and conjugated to make it
soluble. The cause of jaundice in obstruction is that the bile has dammed back
into the liver and reabsorbed back into the blood in conjugated form. As the
conjugated bile is water soluble it can pass into the urine.
What specific nursing care is required in
Jaundiced patients?
General
principles
The primary
management is aimed at treatment of the underlying condition which gave rise to
the jaundice. The management an individual receives will therefore be largely
determined by the cause of the condition. Regular observation of skin and sclereal tone should be made as indicators of improvement
or worsening of the condition. Urine should be monitored for the presence of
urobilinogen and bile pigments as these will vary with the forms of jaundice
described. Stools may also be observed as a guide to the presence or absence of
bile in the faeces. The patients should be asked if the skin is itchy and
informed that this is part of the condition. Nurses need to appreciate that itching may be severe and leading to
self mutilation due to scratching. Clearly unecessary
damage to the skin should be prevented where possible. A further complication
of itching is an inability to sleep. Oral chlorpheniramine
may sometimes reduce pruritus. The patients general
systemic condition should be maintained by attention to diet, activity and rest
as the underlying pathology allows. It should be remembered all patients with
jaundice may complain of malaise.
In hepatic
and post hepatic jaundice there may be inhibition of clotting factor production
so patients should be observed for bruising or other signs of haemorrhagic tendencies. Blood will
usually be tested for INR (International Normalisation Ration) which may be
raised. Injections of vitamin K will be given to improve blood clotting if
required. As there will be reduced
volumes of bile entering the gut in hepatic and posthepatic
jaundice the patient may feel better on a low fat diet.
Patients
should be fully informed and prepared for any investigations which may be carried
out such as ultrasound scanning or ERCP, (endoscopic
retrograde cholangiopancreatography in which the
biliary system is illuminated with contrast medium inserted during endoscopy).
Clearly
jaundice may be a distressing symptom for the patient and the full range of
psychological and interpersonal support is crucial. Nurses need to be aware that jaundice
will lead to altered body image which may alter a persons self - concept. This
means the condition may affect all aspects of their life including relationships
with others which in turn may lead to feelings of isolation. Nurses can help
the individual to maintain a realistic perception of the condition and by
working with and through the individuals coping strategies improve their self -
image. Taking time to listen and communicate clearly may significantly help any
person with altered body image.
Jaundice
can lead to feeling of lethargy and depression. It is important to realise
depression can complicate any physical illness. While nurses should not lie to
their patients hope should be maintained where recovery may occur. The quality
of the relationship between the nurse and the individual can often prevent
depression and help if depression is already established. If clinical
depression is recognised and a psychiatric opinion should be sought.
Prehepatic
Nurses
should monitor the degree of anaemia by observation of skin tone, the degree of
lethargy, and shortness of breath on exertion. Good diet should be encouraged and folic
acid supplement may be required as this is essential for formation of new red
blood cells. Blood transfusions may be prescribed and will require the usual
nursing care. Splenectomy may be indicated in
symptomatic spherocytosis.
Hepatic
There is no
specific treatment for viral hepatitis, for this reason support is symptomatic.
Analgesics may be given if liver pain is a problem. Alcohol must be avoided. In hepatitis A there is a
risk of cross infection from the gastrointestinal tract. In hepatitis B and C
the condition may be transmitted by body fluids or sexually. Primary liver tumours may sometimes be
surgically resected. Patients with hepatocellular
disease are at increased risk from intercurrent
infections so ideally should be nursed in a separate room. They are also at an
increased risk of suffering from anaemia.
The liver pathology may also lead to fluid and electrolyte alterations,
especially if there is vomiting, reduced dietary intake, haemorrhage or
diarrhoea (Long, Phipps and Cassmeyer 1995).
Haemoglobin, urea and electrolytes should therefore be monitored and an
accurate fluid balance chart maintained.
Posthepatic
Pain may be
a feature in obstructive jaundice, often associated with billiary
colic. This must be monitored and will usually be treated with antispasmodics and/or
analgesics. As patients with posthepatic jaundice are
able to excrete bile salts in the urine they should be kept well hydrated as
this will help to flush out some of the excess bile salts. Intravenous therapy
may be given to aid this process. Temperature should be monitored as there is a
risk of biliary tract infection (cholangitis).
1. Red
blood cells are broken down at the end of their life span. Bilirubin is
released into the blood.
2. The liver
cells take up the bilirubin and pass it into the bile ducts. The formed bile is
drained via the canaliculi into the hepatic ducts.
3. The bile
salts pass out of the liver in the hepatic ducts which merge into the common
hepatic duct. Bile enters the gall bladder via the cystic duct for storage and
concentration. Bile enters the duodenum via the common bile duct.
4. Much
bile pigment remains in the gut where it colours and deodorises faeces.
5. Some
bilirubin is reabsorbed from the gut in a slightly altered state and excreted
via the kidneys as urobilinogen
Figure 1. Physiology of bile pigments
1. Prehepatic -
increased haemolysis
2. Hepatic -
poor liver cell function or swollen liver cells blocking small bile channels in the liver
3. Posthepatic -
any obstruction to the flow of bile once it has left the liver
Figure 2 Causes of Jaundice
Red cell membrane defects
These conditions result in
abnormally shaped red cells, eg.
Hereditary spherocytosis
Hereditary elliptocytosis
Sickle syndromes
These conditions result in
abnormal shaped red cells, eg.
Sickle cell anaemia
Sickle cell trait
Haemoglobin abnormalities
In these conditions
haemolysis is increased due to the presence of abnormal haemoglobin, eg.
Alpha and Beta Thalassaemia
Metabolic disorders of the red cell
Here haemolysis is increased
because part of the red cell metabolic processes are abnormal, eg.
Glucose - 6 - phosphate
deficiency
Pyruvate kinaes deficiency
Neonatal conditions
Physiological jaundice
Haemolytic disease of the
new-born
Other causes
Autoimmune haemolytic anaemia
Drug induced haemolytic
anaemia
Table 1. Possible causes of increased haemolysis
Hepatocellular
carcinoma
Adverse drug reactions
Excessive drug use
Poisons
Viral hepatitis (any form)
Leptospirosis
Yellow fever
Liver trauma
Liver abscess
Recurrent jaundice of
pregnancy
Inflammatory bowel disease
Primary biliary cirrhosis
Alcoholic cirrhosis
Alcoholic
hepatitis
Table 2. Possible causes of hepatic jaundice, hepatocellular
and/or intrahepatic obstructive
Gall stone
Pancreatitis
Inflammatory disease of billiary system
Stricture of biliary system
Malignancy of biliary system
Carcinoma of the head of the
pancreas
Secondary
tumour deposits
Table 3. Possible causes of posthepatic
obstruction
References
Anderson
KN Anderson LE Glanze WD (1994) Mosby`s
Medical, Nursing and Allied Health Dictionary, (4th ed), Mosby, St. Louis
Campbell
S Glasper
EA (1995), Whaley and Wong`s
Childrens Nursing, Mosby, London
Green
JH (1978) An Introduction to Human Physiology,
Kumar
P.
Lau W. Leung K. Leung TW.
Long BC.
Phipps WJ. Cassmeyer
VL. (1995) Adult Nursing, Mosby,
Nowak
TJ. Handford
AE. (1994), Essentials of Pathophysiology, Wm. C. Brown,
Richards
JM. Boyter
AC. Nathwani
D. (1996) A jaundiced farmer. Scottish Medical Journal. 41(6):176-7, Dec.
Wilairatana P. (1996), Acute viral hepatitis A: a cause
of jaundice in typhoid fever. Southeast Asian Journal of Tropical Medicine
& Public Health.
27(2):406-7, 1996 Jun.
Jaundice
Yellow
discolouration of the skin, mucous
membranes, sclera of the eyes
Greater
than normal amounts of bilirubin in the blood
Usually
first seen in the sclera
Pruritus
Icterus
Bilirubin - yellow pigment left after breakdown of haemoglobin
Liver cells
take up bilirubin and biliverdin ------ bile ducts ------ gall bladder ---------
duodenum ------- faeces
Reabsorbed
from gut -------------- urobilinogen
Pathophysiology
Increased
haemolysis
Reduced capacity
of liver cells to remove bilirubin from blood
Blockage of
the bile ducts
Physiological
jaundice
Haemolytic
disease of the new-born.
Prehepatic, hepatic, posthepatic
Pre -
hepatic
Haemolytic
Usually a
pale lemon yellow colour
Splenomegaly
Stools
appear normal
Urobilinogen
Reticulocytes
Hepatic
Hepatocellular
Normal
levels of bilirubin produced ------ accumulate in the blood
Infections,
primary liver cancer, poisons,
drugs
Swelling of
hepatocytes ------ obstruction of the small bile ducts or canaliculi This is sometimes referred to as intrahepatic obstructive
Intrahepatic cholestatic
- inflammation of liver cells
- infections, drugs, trauma
Post
hepatic
Obstruction
of bile ducts
Extrahepatic cholestatic
jaundice
Calculi
Pressure
from outside ducts
Bile dams
back into liver -------- blood
Stools -
pale
Urobilinogen
Bile salts
in urine
Damage of
liver cells
Conjugated and unconjugated
Bilirubin released from the phagocytes
`Freed`
bilirubin attaches to albumin
Unconjugated
Bilirubin is conjugated with glucuronide ------ bile ducts
Haemolytic
and hepatocellular - unconjugated
Obstructive
disease ---- conjugated
Conjugated
form ---------- urine.
Nursing care
General principles
Treatment
of underlying condition
Regular
observation of skin and sclereal tone
Urine -
urobilinogen, bile pigments
Stools
Itchy skin
- self mutilation, sleep deprivation - chlorpheniramine
Diet,
activity and rest
Malaise
Psychological
and interpersonal support
Hepatic and
post hepatic - inhibition of
clotting factor production
INR -
vitamin K
Low fat
diet.
Ultrasound
scanning
ERCP -
retrograde cholangiopancreatography
Altered
body image
Depression
Lethargy
Feelings of
isolation
Prehepatic
Degree of
anaemia
Diet -
folic acid
Blood
transfusions
Splenectomy
Hepatic
Viral
hepatitis - symptomatic
Analgesics
Alcohol
Cross
infection
Surgery
Intercurrent infections
Anaemia
Electrolyte
alterations
Posthepatic
Pain -
colic
Keep well hydrated
Temperature
Haemolytic causes
Red cell
membrane defects Sickle
syndromes
Haemoglobin abnormalities Metabolic
disorders of the red cell
Neonatal conditions
Hepatic causes
Hepatocellular
carcinoma Adverse
drug reactions
Excessive drug use Poisons
Viral hepatitis (any form) Leptospirosis
Yellow fever Liver
trauma
Liver abscess Recurrent
jaundice of pregnancy
Inflammatory bowel disease Primary
biliary cirrhosis
Alcoholic cirrhosis Alcoholic
hepatitis
Posthepatic causes
Gall stone Pancreatitis
Inflammatory disease of billiary system Stricture
of biliary system
Malignancy of biliary system
Carcinoma of the head of the
pancreas Secondary
tumour deposits