What are jaundice and kernicterus?

The National Institute for Health and Care Excellence (NICE) guideline, Jaundice in Newborn Babies under 28 days, has recently been amended to clarify the type and extent of phototherapy treatment that should be given to newborn babies with jaundice.

The NICE guideline acknowledges that although neonatal jaundice is one of the most common conditions affecting newborn babies, it can be difficult to recognise and assess. Even after diagnosis there is widespread variation in the way this common neonatal condition is treated. The guideline aims to provide evidence-based and consensus-based guidance for clinicians involved in neonatal care on how to recognise, assess and treat jaundice in newborn babies.

Around 60% of full term babies and 80% of pre-term babies develop jaundice in the first week of life. 10% of breast-fed babies will still be jaundiced at the age of one month. For most, this early jaundice will be transient and harmless. So why is it such a cause for concern?

What is jaundice?

Jaundice is caused by raised levels of bilirubin, a yellow pigment that is in everyone’s blood. As the body breaks down old red blood cells – a totally normal and healthy process – bilirubin travels in the blood circulation to the liver, where it is processed before moving to the bile duct and gallbladder (for storage) then into the small intestine, as bile, where it helps digest fats before being excreted with the rest of the body’s waste. When the liver struggles to process the bilirubin, maybe because there is too much of it, an obstruction or an inflamed liver, the excess bilirubin shows up as yellow discolouration of the skin and the whites of the eyes. This condition is known as jaundice.

Other signs of jaundice include dark urine which stains the nappy or pale, chalky stools.

What are the effects of jaundice?

If diagnosed early and correctly treated, neonatal jaundice is usually harmless but it can also be an indication of other more serious problems, such as:

• Liver disease
• Infection or sepsis
• Blood group incompatibility between the mother and baby’s blood
• Bruising and metabolic disorders
• Sickle cell anaemia
• Enzyme deficiencies

At Boyes Turner, we specialise in helping families whose babies have suffered severe brain injury, either through avoidable birth trauma or medical negligence during their neonatal care. The jaundice-related injuries that we see therefore arise from damage to the brain in newborn babies whose high bilirubin levels were not correctly monitored and treated, leading to serious, long term, neurological damage from a condition called kernicterus.

What is kernicterus?

Kernicterus occurs when unconjugated bilirubin (which has not been metabolised by the liver) penetrates the blood-brain barrier. This type of bilirubin is potentially toxic to the brain and spinal cord, causing neurological dysfunction known as bilirubin encephalopathy. The clinical features of bilirubin encephalopathy, together with the yellow staining which occurs in the brain, are known as kernicterus.

The risk of kernicterus is increased in premature babies and in full term babies with hyperbilirunbinaemia (excessive bilirubin levels) or bilirubin levels over the recommended threshold for treatment which are rising rapidly. Babies who develop jaundice within the first 24 hours of life are more likely to go on to suffer hyperbilirubinaemia, as are those with siblings who suffered the condition and babies who are exclusively breast-fed.

The NICE guideline, Jaundice in Newborn Babies under 28 days, gives specific guidance for monitoring newborn babies and provides treatment thresholds for phototherapy, intense phototherapy and exchange transfusion, which take into account the gestation of the baby at birth (full term or pre-term), the bilirubin levels, the known risk factors and response to treatment. The guidelines also take into account the natural anxiety and concerns suffered by parents of newborn babies, and sets out guidance for sharing information with parents, alongside allowing time within essential treatment sessions for breast feeding and cuddle breaks when safe to do so.

How Boyes Turner can help?

At Boyes Turner we are currently representing several families whose babies have suffered severe neurological injury as a result of medical negligence in treating their jaundice.

We recently settled a case for a boy with dyskinetic cerebral palsy who was born prematurely 25 weeks into his mother’s pregnancy. Judgment on liability was awarded on the basis that the defendant failed to recognise his risk of developing kernicterus, failed to monitor his serum bilirubin levels and so failed to treat his hyperbilirubinaemia with phototherapy in the neonatal period. Although ultimately successful, the case was more complex, making it more difficult to prove, owing to the wide variation in standards of treatment of jaundice at the time of the baby’s birth. We welcome the clearer guidance about acceptable standards of care for newborn babies with jaundice in the hope that fewer children will suffer the severe neurological damage and lifelong disability that can be caused by this common condition.