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Are there worldwide implications?
Although neonatal jaundice is usually a self limited condition, in exceptional circumstances, extreme hyperbilirubinaemia, with its devastating potential of irreversible brain damage due to bilirubin encephalopathy or kernicterus, may occur. For the most part, kernicterus should nowadays be a preventable condition; however, cases continue to occur. The reasons for the persistence of kernicterus are undoubtedly multifactorial. One factor may have been an opinion which evolved at the beginning of the 1990s that in some cases higher serum total bilirubin (STB) concentrations could be allowed than were previously acceptable. At about the same time a new phenomenon developed: that of earlier and earlier discharge of the mother-infant dyad. The potential consequences of discharge at or even earlier than 24 hours are that in many instances lactation may not yet be established, jaundice may often not have become manifest, to say nothing of reaching its peak, and associated illnesses may not yet have declared themselves. Additional factors included discharge of borderline premature neonates of 35–37 weeks gestation as if they were born at term, and failure of physicians to abide by the guidelines for the detection of hyperbilirubinaemia and prevention of kernicterus published by the American Academy of Pediatrics (AAP) in 1994.1 During the last 15 years, cases of kernicterus were reported not only from the United States,2 but also, although to a lesser extent, from other industrialised countries, including Canada, Denmark, Holland, and New Zealand.3 The myth that haemolysis was a prerequisite for kernicterus was disproved.4 By the year 2001 the number of cases of kernicterus reported was sufficient for the AAP to publish a warning cautioning paediatricians of the potential of kernicterus and the dangers of hyperbilirubinaemia.5
In response to the continuing occurrence of cases of kernicterus, the AAP has recently issued a revised set of guidelines.6 In this protocol thresholds for starting phototherapy and performing exchange transfusion are lower to an extent than those of the 1994 recommendations. However, the new guidelines are far more comprehensive than simply comprising a manual of when to turn on or off the phototherapy lamps. Rather they offer a wide ranging approach to the detection of hyperbilirubinaemia and the prevention of kernicterus. Granted, as emphasised by the AAP committee members themselves, there is little evidence on which to base many of the AAP’s recommendations, and many are based on experience and consensus between the committee members. Some of the salient features of the new guidelines include:
Strategies for evaluation of the risk of developing hyperbilirubinaemia before discharge.
Stressing the need to assess the risk of developing hyperbilirubinaemia by plotting the specific STB concentration on the hour of life specific nomogram7: the higher the centile, the greater is the risk of developing hyperbilirubinaemia.
Emphasising the value, in cases where the STB concentrations are of concern, or rising, of providing intensive phototherapy by putting the infant under multiple banks of phototherapy lights.
Giving nurses the prerogative of ordering an STB test without requiring a doctor’s order.
Recognising that borderline preterm (35–36 weeks) neonates are at higher risk than term counterparts.
Guidelines are given for the administration of intravenous immunoglobulin in selected cases of hyperbilirubinaemia due to isoimmunisation.
Some doctors may be concerned that, if the new AAP guidelines are indeed followed, the number of neonates requiring phototherapy or exchange transfusion will increase. Others will find that the implications for change may be minimal. For example, in our practice, from 1994 till the present, we started phototherapy at STB concentrations suggested by the 1994 guidelines for considering phototherapy, rather than mandating it. Thus the upper limit set by the AAP in 2004 for an STB concentration of 428 μmol/l (25 mg/dl) will not mean, for us, that we will be performing more exchange transfusions or treating more babies under phototherapy. Certainly, although there is room for individualisation, we do feel that the AAP’s new cut-off points for starting phototherapy and performing exchange transfusion should probably not be exceeded. Should there be an abundance of risk factors or if clinical judgment deems it necessary, there is no reason not to start phototherapy earlier or at lower concentrations of bilirubin than the AAP recommends.
The guidelines are not limited to the hospital stay. The realisation that in most cases the STB peaks at about 3–5 days, which is often long after the baby has been discharged home, has prompted instructions about when follow up appointments should be scheduled. The timing of follow up was planned to coincide with the projected peak in STB.
To our knowledge, the only countries with nationally applicable, published guidelines are the United States and Canada.6,8 The new AAP guidelines are naturally focused on US medical practice and its interrelationship with the society of that country. The question therefore arises: are the new AAP guidelines relevant only to the United States, or do they have implications for the rest of the world as well? In our opinion, some of the current recommendations are universally applicable, whereas others should be modified or adapted according to the cultural needs or environmental influences of a specific society or geographical area.
We cannot overemphasise the concept of assessing an STB concentration according to the infant’s age in hours.8 STB concentrations progress dynamically in an upward direction during the first 3–4 days of life. Thus an STB concentration of 188 μmol/l (11.0 mg/dl) may be of no concern on the third day of life, but may indicate haemolysis, with its potential for severe hyperbilirubinaemia and bilirubin encephalopathy, if it is noted during the first 24 hours of life. Hyperbilirubinaemia is now defined as an STB concentration greater than the 95th centile for hour of life.
Another important AAP recommendation is the assessment of the risk of hyperbilirubinaemia before discharge. One method recommended by the AAP includes universal STB determination with assessment of risk according to the hour of life specific nomogram, before discharge. Excellent as this method may be in singling out those neonates who may develop hyperbilirubinaemia after discharge, the method requires a bilirubin test on each and every neonate, many of whom would never develop significant jaundice. An alternative, less invasive option, possibly preferable to the former in countries with limited resources, is the assessment of risk factors for subsequent hyperbilirubinaemia, with actual bilirubin testing reserved for those who do become jaundiced. Risk factors for every neonate should be assessed before discharge: the greater the number of risk factors, the greater the risk of developing hyperbilirubinaemia.
The AAP also emphasises the need to establish successful lactation as a preventive measure against the development of neonatal hyperbilirubinaemia and suggests indicators of successful nutrition and hydration to be monitored. Although it is important to ensure that lactation has been successfully established, this does not necessarily mean that in each and every instance the dyad should remain in hospital until this goal is reached. In societies that provide a visiting nurse service, the nurse may be able to continue the lactation learning process successfully in the comfort of the home while at the same time following up the baby for hyperbilirubinaemia.
When necessary, and especially in cases in which STB concentrations are continuing to rise despite treatment or are approaching the indications for exchange transfusion, phototherapy should be administered as effectively as possible. Several banks of lights may be used, and fluorescent lamps may be placed close to the baby so as to increase the light irradiance intensity.9 We concur that, in most cases of babies readmitted for breast feeding jaundice, in accordance with AAP recommendations, there is no need to discontinue breast feeding. We believe and agree with the AAP that the nurses managing the baby should be allowed to send blood for an STB determination without the delay of obtaining a doctor’s order.
On the other hand, some recommendations should be customised and tailored according to the individual character of different countries and varying population groups. For example, selective, rather than universal, screening before discharge may be an option in high risk cases such as glucose-6-phosphate dehydrogenase deficiency, early discharge by parental request, or infants ⩽37 weeks gestation. Depending on the follow up services available, parental reliability, distances to be travelled in order to obtain medical services, and availability of these services during weekends or holidays, some high risk neonates may benefit from delayed discharge from hospital. Others may be safely discharged if facilities exist for a home visit from a nurse trained in the care of the newborn, when supportive laboratory and medical services can be called on when necessary.
Cultural differences between population groups may either exacerbate jaundice or facilitate identification of jaundice. Thus feeding the baby herbal teas or applying certain umbilical potions or henna applications to the skin may precipitate haemolysis in population groups with a high incidence of glucose-6-phosphate dehydrogenase deficiency. On the other hand, a routine visit of a Jewish ritual circumciser (Mohel) may serve to discover a jaundiced baby in whom the condition was not recognised by the parents.
In countries where formulation of a set of recommendations is envisaged, it may be wise for bilirubin experts of those countries to base their recommendations on the AAP guidelines, while taking into account individualised, region specific factors appropriate to the cultural variations and needs of the specific population groups under their care. It must be appreciated that no set of instructions can be all encompassing and cover each and every possibility. All those involved in the care of the neonate should study the new recommendations. Their use, in combination with individualisation, evaluation of risk factors, and application of common sense, should limit the number of cases of severe hyperbilirubinaemia and kernicterus to the minimum.
Are there worldwide implications?
Competing interests: none declared