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

 

Prehepatic

Degree of anaemia

Diet  -  folic acid

Blood transfusions

Splenectomy

 

 

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

 

Hepatic

Viral hepatitis - symptomatic

Analgesics

Alcohol

Cross infection

Surgery

 

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

 

Posthepatic

Pain - colic

Keep  well hydrated

Temperature

 

 

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

 

 

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

 

 

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, (Harrison 1977). Excessive destruction of red blood cells increases bilirubin formation. When the release of bilirubin from haemolysis exceeds the ability of the liver cells to remove it from the blood an accumulation will occur. However, with normal hepatic function, the liver is able to remove the excess bilirubin fairly rapidly so this jaundice is usually mild, (Kumar and Clark 1995). Haemolytic jaundice may therefore be caused by any problem increasing red cell breakdown. This means the causes are the same as those of haemolytic anaemia, (table 1)

 

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).

 

 

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