SUMMARY: Permanent brain damage from severe neonatal jaundice has been largely eliminated by prevention of the Rhesus problem, light therapy and timely exchange transfusion. The last decade of the twentieth century saw a resurgence of brain damage caused by jaundice in newborn infants. Some of the increase in brain-damage occurred because more relaxed treatment guidelines were misapplied.
In the 1990s a previous downward trend reversed and more newborn infants than previously were brain-damaged by jaundice.
Parents of some of the damaged children are now enquiring about Actions for medical malpractice.
Part at least of the temporary increase in numbers of compromised babies was caused by more relaxed criteria for phototherapy and exchange transfusion for jaundiced newborns.
When Red Blood Cells (RBC) are old, they are trapped and destroyed by the spleen.
The yellow pigment bilirubin is a waste-product of the destruction of the pigment hemoglobin that carries oxygen in the RBC.
The bilirubin is passed by the liver into the feces, giving them their brown colour.
During pregnancy, the mother’s liver performs this detoxifying role for the fetus, and there is commonly a few days’ delay after birth before the newborn infant’s own liver gets up to speed.
Yellow pigmentation of the skin is usually caused by this so-called physiological jaundice and, in the vast majority of babies, the phenomenon is harmless.
However, in a minority, the jaundice is more severe and bilirubin can, in extreme cases, damage the basal ganglia of the brain.
The possible outcomes include death, the immediate neurological condition of kernicterus and the long-term consequences of mental retardation, deafness and Cerebral Palsy (CP).
This is a relatively rare form of CP that is known as athetoid (Greek athetos, without position or place) because of the involuntary writhing movements that accompany the muscular paralysis.
Very rarely, these tragic injuries are caused by exaggerated physiological jaundice.
More frequently, other pathological processes cause the newborn brain to be damaged at levels of serum bilirubin that are not usually toxic, or amplify retention or production of bilirubin.
The commonest contributory mechanism is excessive RBC destruction or hemolysis.
In various dark-skinned peoples in particular, such hemolysis may result from inherited genetic errors.
The most rapid destruction of RBC is, however, caused by antibodies produced in the mother’s blood in response to leakage of fetal RBC during a previous pregnancy.
These antibodies are produced only if mother’s and fetus’s blood groups are incompatible, and the most prolific antibody production occurs when the mother is Rhesus negative and the fetus is Rhesus positive.
In subsequent pregnancies, these Rhesus antibodies, that circulate in her blood and are harmless to the mother, pass readily through the placenta into the circulation of another Rhesus positive fetus whose RBC are thereby destroyed.
Consequent Rhesus-incompatible Hemolytic Disease of the NewBorn (HDNB) would still be relatively common, were it not for the spectacular success of routine Rhesus immunoglobulin injections after abortion and delivery in "mopping up" the leaked fetal RBCs before they can provoke a Rhesus antibody sensitisation in the mother.
Using intense light to break down bilirubin in the skin is, perhaps surprisingly, both effective and the mainstay of treatment in the majority of jaundiced newborns that require any intervention.
The traditional1 threshold for phototherapy treatment has been refined over the last few decades and was previously noncontentious2.
Investigation
The last decade of the twentieth century saw a resurgence of brain damage caused by jaundice in newborn infants.
Practice PointChildren who suffered brain damage from neonatal jaundice may have a viable Cause of medical malpractice Action, particularly if they were born in the 1990s |
In the early 1990s, a number of clinical researchers questioned whether the threshold for investigation of newborn jaundice was too low and proposed a "kinder" hands-off approach for the majority of affected babies.
This more relaxed approached was to some extent endorsed by the American Academy of Pediatrics3.
However, raising the traditional1 serum bilirubin threshold level for exchange transfusion did most of the harm.
This procedure involves replacing the newborn infant’s jaundiced blood with fresh donated blood, as a means of rapidly reducing the body’s content of bilirubin.
Some of the increase in brain-damage occurred because more relaxed treatment guidelines were misapplied.
Two major consequences contributed to the documented4 increase in cases of brain damage during the 1990s.
Practice PointThe more relaxed jaundice guidelines did not apply with these risk factors: 1. Need for resuscitation at birth 2. Preterm (premature) or small 3. Hemolytic disease 4. Jaundice within 24 hours of birth 5. Sepsis |
First, some pediatricians inappropriately applied the more relaxed guidelines to infants who had risk factors for brain-damage, including Rhesus-incompatible HDNB.
Both the major initiating clinical research paper5 and the US Clinical Practice Guideline3 addressed healthy, term infants and explicitly warned against applying the proposed changes to infants with risk factors, and particularly those with hemolysis.
Second, it was found that very high serum bilirubins in the absence of risk factors were not as benign as had originally been thought.
The Canadian Pediatric Society subsequently proposed 6 a return to a more traditional approach to physiological jaundice, and reiterated that the traditional1 lower thresholds for exchange transfusion in HDNB - that had been developed over many decades - should remain unchanged.
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