A case of kernicterus and resultant neuro injury

At that age (>72hrs), a bilirubin greater than 22 is in the high risk level. This child's bilirubin never dropped below that until exchange was finally started. A child with a bili in the 30s should have been exchanged immediately.

Kernicterus and resultant neuro injury are well known complications from hyperbilirubinemia

The bilirubin level at the time of admission to the ED was well above the general threshold for consideration of exchange transfusion. Although details of the case are important, for example, degee of dehydration at the time of admission, more details of exam and neurologic status, etc., rapid institution of exchange transfusion then seems indicated.

Based on comments in case summary of neurologic injury.

I refer to the Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation1 (attached), as that was the standard of care in place at the time. Since that time, Guidelines were updated and liberalized slightly. 8/08-36 6/7 weeks EGA birth G6PD diagnosis came from testing much later, so while in differential diagnosis, this adds an element of risk not known at time of birth or at PICU admission as these results may take a week to return unless available in hospital at time or from a close reference lab. Clearly available here as results back next day. 8/10(Fri) Serum TB 9 DC w/ ‘F/U in 48h’ -which would be a Sunday-no one open, could have a prescription for Total Bili lab to be drawn & called to nursery/NICU. Presuming the 12 was a transcutaneous bilirubin and the 9 is the accurate measure and gold standard. 8/13 (Mon) Serum TB 25 sent to ED & 38.6 at 1546. Triple lights (presumably) and Intravenous fluids? Repeat 4 hours later (standard measure interval) TB (Total bili) 29 at 1930 (730 pm). 2200 (10 pm) TB at 26.4. 8/14 at 0131 TB 18.6 direct 1.7, 0537 TB 24.0 direct 2.4. “Wait & See option” given at 0131 result. Consents signed at time of PICU admission hours earlier is common for critically ill children to prevent delays in care in middle of the night. How much weight had the baby lost since birth? “Lethargic?” This can mean many things-often misused term. Was baby not responsive to tactile stimuli? Was baby nursing/feeding? Was baby stooling? What was character/color of stools? Was baby wetting diapers? Was the fontanel (soft spot) sunken? Eyes sunken? Skin turgor decreased? NICU (Neonatologist) consulted merely for placing lines or for assistance with exchange transfusion? Was PICU attending knowledgeable/experienced with doing an exchange transfusion? When was last time s/he did an exchange? How many has s/he done in last few years? Was this included in their medical staff privileges? Were PICU nurses qualified to participate in exchange transfusion or were NICU nurses used in the PICU to help with this procedure? (This is not a common procedure in PICU and if staff not trained/completed training competencies-then NICU (where more common-but still not very common) staff should be engaged for support in care of this neonate in PICU. THIS is NOT uncommon to do in such situations. All of these factors and decision point can delay the commencement of an exchange transfusion. If the baby truly lethargic (in true medical exam) and not feeding or stooling and exam findings of severe dehydration by presence of documented exam findings above-then this is a sick neonate for whom sepsis is also a severe concern. That would also necessitate that workup which was not addressed in the vignette. Initial discharge at 9 from NICU nursery reasonable at 48 hours of life based on the nomogram assuming the rate of rise from any prior measure (if done) does not exceed acceptable standard. If truly concerned, then a repeat measure sooner than 48hours and “see a PED,” which would have been a Sunday. Recognize this would be pushed off to Monday. It is not necessarily a departure, but could not predict a less likely underlying risk factor of G6PD deficiency. Questions to be addressed: 1. Was it a departure to take the wait and see position when the bili levels began to decrease while the child was receiving phototherapy? On 8/13 (Monday) when level was 25 at pediatrician’s office-appropriate prompt referral for hospital admission appropriate. The serum total bili was already at the highest point on the nomogram. Unclear why no direct admission to hospital as this would have been appropriate and unclear how long this baby was in ED awaiting care-potential for valuable time lost of IV fluids and phototherapy. Very great increase by almost 14 more points may have been averted here, potentially preventing need for exchange transfusion. I would carefully examine these timelines. In response to the query, however, the PICU attending physician got a neonate who was quite ill and did consult with the Neonatologist. The decision to wait was not within the accepted standard of care at the time. A total bili of 38.6 at that time of admission should have warranted aggressive intervention as that result far exceeded the nomogram highest levels and would be off the page in plotting. One would anticipate the levels to decrease somewhat with institution of therapies as done but the level still was 29 and far above the top of the nomogram. Since lines placed and other interventions already helping, even a partial exchange could have been considered at this point. But no intervention is not congruent with the accepted standard of the nomogram guidelines. 2. Was it a departure to leave the decision to commence the exchange transfusion up to the parents at midnight? Consulting with parents is absolutely appropriate. That being said, at the end of the day it is our role to ensure we give parents all the information they need to participate in sound medical decision making. This decision should not be solely theirs to make in an instance such as a critical illness. So leaving the decision solely up to them was not appropriate if that is what was done. 3. Given the elevated bilirubin level of 38.6 at 15:46 when the child was rested in the ER having increased from 25 earlier in the day was it a departure for the PICU not to have the commenced the exchange transfusion on a stat basis as soon as the child was admitted and the lines were placed? In my best medical judgement, I would have commenced the exchange transfusion in this critically ill neonate as soon as lines were placed, as recommended by the Neonatologist. I would explore why the PICU Intensivist Attending chose not to do this at this time. There may have been some other factors weighing in that are not provided. There is absolutely no guarantee that the earlier high levels of 25 and 29 were not already contributing to the kernicterus this baby ultimately suffered leading to neurologic devastation, but there was an opportunity to ameliorate the damage at that time. It will never be known to what degree this could have been diminished. Reference: 1. Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation, PEDIATRICS Vol. 114 No. 1 July 2004, pp.297-316. (http://publications.aap.org/pediatrics/article-pdf/114/1/297/1005960/zpe00704000297.pdf)

1. Was it a departure to take the wait and see position when the bili levels began to decrease while the child was receiving phototherapy? On 8/13 (Monday) when level was 25 at pediatrician’s office-appropriate prompt referral for hospital admission appropriate. The serum total bili was already at the highest point on the nomogram. Unclear why no direct admission to hospital as this would have been appropriate and unclear how long this baby was in ED awaiting care-potential for valuable time lost of IV fluids and phototherapy. Very great increase by almost 14 more points may have been averted here, potentially preventing need for exchange transfusion. I would carefully examine these timelines. In response to the query, however, the PICU attending physician got a neonate who was quite ill and did consult with the Neonatologist. The decision to wait was not within the accepted standard of care at the time. A total bili of 38.6 at that time of admission should have warranted aggressive intervention as that result far exceeded the nomogram highest levels and would be off the page in plotting. One would anticipate the levels to decrease somewhat with institution of therapies as done but the level still was 29 and far above the top of the nomogram. Since lines placed and other interventions already helping, even a partial exchange could have been considered at this point. But no intervention is not congruent with the accepted standard of the nomogram guidelines. 2. Was it a departure to leave the decision to commence the exchange transfusion up to the parents at midnight? Consulting with parents is absolutely appropriate. That being said, at the end of the day it is our role to ensure we give parents all the information they need to participate in sound medical decision making. This decision should not be solely theirs to make in an instance such as a critical illness. So leaving the decision solely up to them was not appropriate if that is what was done. 3. Given the elevated bilirubin level of 38.6 at 15:46 when the child was rested in the ER having increased from 25 earlier in the day was it a departure for the PICU not to have the commenced the exchange transfusion on a stat basis as soon as the child was admitted and the lines were placed? In my best medical judgement, I would have commenced the exchange transfusion in this critically ill neonate as soon as lines were placed, as recommended by the Neonatologist. I would explore why the PICU Intensivist Attending chose not to do this at this time. There may have been some other factors weighing in that are not provided. There is absolutely no guarantee that the earlier high levels of 25 and 29 were not already contributing to the kernicterus this baby ultimately suffered leading to neurologic devastation, but there was an opportunity to ameliorate the damage at that time. It will never be known to what degree this could have been diminished. Reference: 1. Management of Hyperbilirubinemia in the Newborn Infant 35 or More Weeks of Gestation, PEDIATRICS Vol. 114 No. 1 July 2004, pp.297-316. (http://publications.aap.org/pediatrics/article-pdf/114/1/297/1005960/zpe00704000297.pdf)