As midwives we see many babies that become jaundiced. It makes one wonder if it happens so commonly is it ever a problem? We hear of many friends whose babies were born in the hospital, and who must spend a lot of their early life under ‘bili-lights’ and not in their mother’s arms. In our own practices do we ever need to worry about a little jaundice? Or is it something we can just ignore and it will go away?

 

What Causes Newborn Jaundice?

 

Jaundice is a yellow discoloration of the skin or whites of the eyes caused by a buildup of bilirubin. Bilirubin is a by-product of the breakdown of red blood cells. Newborns normally have a high amount of bilirubin in their blood on about day three. This is because when they were fetuses, they needed more red blood cells so that they could attract oxygen from the maternal blood stream. Once they are born and their lungs are working well, they do not need so many red blood cells because it is easier to get oxygen from the air. So by about day three it is common to see some jaundice in white babies due to a build-up of bilirubin. Newborns are often not able to eliminate bilirubin as efficiently as older children or adults.

 

When Can Jaundice Be Dangerous?

Jaundice can be dangerous if the level of bilirubin gets so high in the blood stream that it can cross over into the brain and stain the basal ganglion of the brain. This can result in a condition called kernicterus. In the long run, kernicterus can result in severe brain damage such as cerebral palsy, or more minimal effects such as hearing loss, behavioral disorders, or perceptual problems. As many as 50% of infants with classic kernicterus may die.1

 

Jaundice will become dangerous if you begin to see it early, especially if is evident before 24 hours of age. This means that the baby has some disease state or abnormal situation that is causing more than the normal amount of bilirubin to be in the bloodstream. The baby will not develop irreversible problems until the level gets high, but if the bilirubin level is rising quickly, it is very likely to get too high. For the home birth midwife this situation needs aggressive treatment while trying to find a very good pediatrician in a tertiary hospital.

 

The information and procedures contained on this web page are based on the research and professional experience of Midwifery Institute of America staff. The publisher and the authors are not responsible for any adverse effects or consequences resulting from the use of any of the suggestions, preparations, or procedures discussed on this web page

 

In the hospitals bilirubin is usually monitored by blood testing, but how is the home-birth midwife to monitor this? In white babies you can estimate the level of hemoglobin by looking at the baby to see how much of the baby’s body is jaundiced. If it is just seen in the face and whites of the eyes, the level is 6-8 mg/dL. Extending down to the shoulders and chest corresponds to a bilirubin level of 8-10 mg/dL and if the lower body is also involved, from 10-12 mg/dL. If the palms of the baby’s hands are jaundiced, you should already have gotten medical help. This corresponds to a bilirubin level of 12-15 mg/dL.

 

To check for this get the baby in good lighting and press with your finger on the baby’s forehead, or belly or leg, then let up your finger. This will momentarily cause the skin to blanch so that you can see the underlying color. Remember that this only works for white babies. In dark skinned babies, one can look at the whites of the eyes, or blanch the gums.

 

Visual Estimation of Jaundice in White Babies
Jaundice Evident Bilirubin Level
Face and Whites of Eyes 6 - 8 mg/dL
Shoulders and Chest 8 - 10 mg/dL
Lower Body 12 - 15 mg/dL

 

Early signs of kernicterus are lethargy, poor feeding, and vomiting. If the condition goes untreated these early signs can be followed by high pitched cry, tremors, twitching, convulsions, severe spasms of the muscles where the head bends back and the heels bend back on the legs and the arms and hands flex rigidly (opisthotonos), absence of moro and sucking reflexes, low muscle tone, and diminished deep tendon reflexes.2

 

So if you have an alert, normal acting baby that looks pretty yellow, and you didn’t begin to see jaundice until after 36 hours, you probably do not have a problem, at least not yet. With such a baby, make sure that the mother is able to nurse as much as possible and the baby can get into sunlight. The easiest way to do this is find window in the house where sun shines in. Whenever the baby is not nursing, place the mostly naked baby on the floor in the sunlight with the baby’s eyes in the shadow, and just move the baby around the room as the location of the spot of sunlight changes. Or if it is sunny and warm, sit outside and nurse the baby. Sunlight, or more specifically the blue wavelengths of light3 will break up bilirubin. But in severe cases, it is important to use triple-banked bili-lights placed as close to the baby as possible, which means hospitalization. Even the home phototherapy blankets do not provide enough light to treat very serious cases.

 

Because it also takes energy in the form of glucose to break down the bilirubin, and bilirubin is removed from the body through the bowels, it is important that the baby be fed often. The baby should get one ounce of milk every two hours. Feeding a normally alert baby like this is very effective at reducing bilirubin quickly, if the mother’s milk has already come in.

 

Sugar water should not be given; it does not help and wastes the baby’s energy and everyone’s time. Breastmilk is definitely best, but some elemental (amino acid) type of milk substitute should be used if the mother’s milk has not yet come in and the baby’s bilirubin level is rising rapidly.4 If the jaundice gets significant to the point of producing lethargy in the baby, and the mother’s milk has not yet come in, the jaundice is probably due to some abnormal, disease process, so again, hospitalization looks like the medically-correct solution. Unfortunately, this is not to say that the baby will always get the best possible care in the hospital, so be ready to advocate for this baby.

 

There is a scoring system to judge the Bilirubin-Induced Neurologic Disfunction (BIND) of a baby5 These scores represent levels of Acute Bilirubin Encephelopathy (ABE) or brain damage. Peak scores of 1-3 represent reversible classic kernicterus, but they may also be linked to permanent impairment that is more subtle.

 

Bind Score: 0 1 2 3
Mental Status Normal Sleepy but
arousable;
decreased feeding
Lethargy, poor suck
and/or irritable/jittery
with strong suck
Semi-coma,
apnea, unable
to feed,
seizures, coma
Muscle Tone Normal Persistent mild to
moderate
hypotonia
Mild to moderate
hypertonia alternating
with hypotonia,
beginning arching of
neck and trunk on
Persistent
arching of head,
neck and body,
bicycling or
twitching of
hands and feet
Cry Pattern Normal High pitched
when aroused
Shrill, difficult
to console
Inconsolable
crying or cry
weak or absent

Abbreviations:
BIND, bilirubin-induced neurological dysfunction
ABE, acute bilirubin encephalopathy
TSB, total serum bilirubin
ABR, auditory brainstem response

Score of 7–9 represent advanced ABE: urgent, prompt and individualized intervention are recommended to prevent further brain damage, minimize severity of sequelae and possibly reverse acute damage.

Score of 4–6: represent moderate ABE and are likely to be reversible with urgent and prompt bilirubin reduction strategies.

Score of 1–3: are consistent with subtle signs of ABE in infants with hyperbilirubinemia. An abnormal ABR or ‘referred’ automated ABR is indicative of likely bilirubin neurotoxicity and would be suggestive of moderate ABE. In infants with these non-specific signs (score 1–3), a failed ABR hearing screen supports a diagnosis of moderate ABE. Serial ABR may be used as an objective measure of progression, stabilization or reversal of acute auditory damage and could interpret effectiveness of bilirubin reduction strategies.5

 

For the home birth midwife it is very important to notice the timing of the first appearance of jaundice. If the baby begins to get jaundiced before 24 hours of age, you have a problem that is due to some abnormal condition and not just physiologic jaundice. You will need to get the baby to an experienced, highly trained pediatrician as soon as possible. Do not necessarily depend on your friendly, family practice back up doctor for this situation. You need someone to take this problem very seriously, begin treatment as soon as possible, and find the reason for the jaundice.

 

When the baby was in the womb the mother’s body was clearing the excess bilirubin from the baby’s system if the baby has some pathological condition. Once the baby is born, the baby has to clear his own bilirubin. In normal jaundice the buildup of excess bilirubin will be slow so that jaundice doesn’t appear until day three, but if some abnormal condition is present causing a greater breakdown of red blood cells, the jaundice will manifest sooner, and in these cases the bilirubin level is likely to get high enough to be dangerous.

 

There is a graph called the Bhutani Nomogram that depicts the risk for a near-term baby given the bilirubin level against the hours of age.6 For example, a 12 hour-old baby with a bilirubin level of seven is in the high risk zone, but a 72 hour-old baby with a bilirubin level of ten is in the low risk zone. Basically this means that a 12 hour old baby with a bilirubin above 7 is very likely to get such a high level of bilirubin that kernicterus will occur without aggressive intervention. The sooner the intervention can begin the better for the long term prognosis of the child.

 

Data from a Bhutani Nomogram units in mg/dL
Age Low Risk
Zone
Low Intermediate
Risk Zone
High Intermediate
Risk Zone
High Risk
Zone
12 hours less than
4
between
4 and 5
between
7 and 5
greater than
7
24 hours less than
5
between
5 and 6
between
6 and 7.5
greater than
7.5
36 hours less than
7
between
7 and 9
between
9 and 11
greater than
11
48 hours less than
8.5
between
8.5 and 11
between
11 and 13
greater than
13
60 hrs
2 1/2 days
less than
9.5
between
9.5 and 12.5
between
12.5 and 15.2
greater than
15.2
72 hrs
3 days
less than
11
between
11 and 13.5
between
13.5 and 16
greater than
16
84 hrs
3 1/2 days
less than
11.5
between
11.5 and 14.5
between
14.5 and 16.7
greater than
16.7
96 hours
4 days
less than
12.5
between
12.5 and 15.2
between
15.2 and 17.4
greater than
17.4
108 hours
4 1/2 days
less than
12.8
between
12.8 and 15.5
between
15.5 and 17.5
greater than
17.5
120 hours
5 days
less than
13.2
between
13.2 and 15.8
between
15.8 and 17.6
greater than
176
132 hours
5 1/2 days
less than
13.2
between
13.2 and 15.5
between
15.5 and 17.4
greater than
17.4
144 hours
6 days
less than
13.2
between
13.2 and 15.2
between
15.2 and 17.2
greater than
17.2

 

Doctors such as Steven Shapiro who specialize in the care of children with kernicterus are not certain at what level permanent disability may occur. They suspect that there may be some permanent, but lesser disabilities that result from intermediate levels of bilirubin. There has been evidence of "moderate to severe sensorineural hearing loss and central auditory dysfunction" in children who as infants had elevated bilirubin levels but did not develop the symptoms of classic kernicterus. Both the level of bilirubin and the duration of high bilirubin levels where associated with this damage.11 So these doctors like to put babies under the bili-lights if there is ever any question. Midwives hate to see babies separated from their mothers, so they are more hesitant to take a possibly normal baby and put the parents and baby through all the trauma of separation and hospitalization. And many home-birthing parents are very reluctant to go to the hospital unless it is truly necessary.

 

A regimen of bright sunlight and one-ounce milk feedings every two hours - even if those feedings need to be given with a feeding syringe or feeding tube, is very effective at bringing down jaundice, even from some types of pathological causes. And this is important to remember for midwives working in developing countries if medical facilities are not readily available. If the mother’s milk has not yet come in and if the baby is exhibiting early signs of kernicterus such as lethargy, formula will need to be given, in addition to phototherapy. If formula is given it should be elemental formula.

 

Elemental baby formulas avoid the problem of inducing cow’s milk allergies or soy allergies because the proteins in the formula are completely broken down into amino acids. Unfortunately these formulas are very expensive. As of Aug 2010, Neocate costs $139 for a case of four cans and Nutramigen AA costs $156 for a case of four cans. Both of these are elemental formulas. Not only are they expensive, but they are not found in stores. They can be ordered online and do not require a prescription. The formula needs to be stored at room temperature. It would be helpful for homebirth supply companies to stock sample sizes of these formulas for midwives to have on hand in the event of needing a small quantity.

Offering the baby someone else’s breastmilk sounds very tempting at this point, but if that person happened to have cytomegalovirus (CMV), and the baby did not have immunity, the baby could get very sick. CMV is very prevalent. In the U.S. 50% to 80% of the population carries CMV by 40 years of age.9 In hospitals breastmilk donations are screened for CMV and milk is not accepted from mothers who shed this virus. Even freezing the milk for ten days does not kill this virus.10

 

So if a baby is alert, but yellow, and the mother is successfully nursing 8 to 12 times a day, is this ever a problem? Usually not, but there is one case where there still can be a big problem. A baby with a blocked bile duct or some type of liver damage cannot eliminate the bilirubin that the liver has processed for removal. If jaundice ever is still evident on day 15 it would be best to go to a reputable pediatrician to find out the total bilirubin and the direct (or conjugated) bilirubin level. If the direct bilirubin is greater than 1 mg/dL, there may be some problem with the liver. If this is not diagnosed early enough and treated, the condition may progress to a point where the only hope for the child is a liver transplant. A later sign of a blocked bile duct is the baby passing grey-colored stools.

 

Cautions for parents and midwives seeking help for severe problems

If a home birth midwife discovers a situation where a baby has early onset jaundice or symptoms of kernicterus - certainly anything above a BIND score of 1, and she takes the parents and baby to the hospital the following are areas of care that she should insure the baby gets:7

  • Once a bilirubin is taken it is very important that the danger of this level of bilirubin be correlated to the age of the baby in hours using the Bhutani Nomogram or similar scale.
  • If a bilirubin level is taken and seems high, do not allow the doctor to stall and get a second reading before beginning treatment. They should get the baby under bili-lights immediately and run the re-test while he is getting treatment. Damage may be reversed if treatment is begun promptly.
  • If the bilirubin level is seriously high, phototherapy should not be interrupted for diagnostic testing to determine the risk of an exchange transfusion. The procedures, even echocardiograms need to be done under the lights. If this is not possible, the lights need to be kept on every possible minute.
  • The total serum bilirubin needs to be used to make treatment decisions not the indirect (uncongugated) bilirubin.
  • Make sure the baby is examined for signs of acute kernicterus.
  • If the bilirubin level is rising too fast, but is not yet dangerous, make sure treatment is begun before dangerous levels of bilirubin are reached.

 

In 2009 there was a report from the Pilot USA Kernicterus Registry, a voluntary registry that monitors outcomes of children diagnosed with kernicterus. The report examined the reasons that various babies were not treated early and effectively enough to prevent kernicterus and how these adverse outcomes can be prevented in the future. Kernicterus was almost eliminated during the 1970's but with the advent of short hospital stays, it has become much more prevalent. Many of the children developed high bilirubin levels because of birth trauma, hemolysis, or Glucose 6 phosphate dehydrogenase deficiency (G6PD). G6PD largely, but not exclusively, affected black male children. Most of the primary causes of kernicterus were unknown, but often there had been siblings who had also experienced jaundice. Two cases were due to Crigler-Najjar syndrome and one was due to galactosemia. Four of the 119 cases were babies who were born at home.8 Many of the babies in the study who developed severe disability did not receive prompt and aggressive treatment. Some of the babies in the study demonstrated advanced signs of bilirubin toxicity - BIND scores higher than 7 - but they had prompt and aggressive treatment and the prognosis for those babies was very good. How high the bilirubin gets, but also how long a high level of bilirubin is present, relates to the long term prognosis for the baby.

 

In the Pilot USA Kernicterus Registry, four of the 119 listed children who developed kernicterus were born at home. One of the home born babies had a shoulder dystocia birth trauma compounded with sepsis, two of the babies had Crigler-Najjar syndrome, and one had hemolysis due to spherocytosis. All of the home-born babies in the study developed lifelong severe sequelae.

 

Premature or sick babies are more at risk

Babies who are premature or babies who are sick as well as having abnormally high hemoglobin levels are more prone to kernicterus damage than healthy term babies. The Bhutani Nomogram was developed for babies born after 35 weeks gestation. Home birth midwives in the U.S. do not often care for premature babies, but midwives working in developing nations need to remember that smaller, sicker babies, get kernicterus quicker.

 

 

In conclusion, most of the jaundice that home-birth midwives see will not lead to permanent defects, but without prompt and proper care, some of the jaundice seen in home births can lead to serious disability. It is important for the midwife to

  • get help whenever jaundice is first noticed before 24 hours of age, and to

  • carefully monitor all jaundice for signs of bilirubin toxicity, and

  • treat all jaundice that is not yet dangerous with aggressive, and effective breastfeeding, and sunlight.

  • Get babies who are exhibiting even subtle signs of kernicterus promptly to a competent pediatrician who will quickly and effectively treat the baby.

  • If you think the situation is serious, promptly take the baby first to a hospital that is equipped to do an exchange transfusion. Have the mother breastfeed or give expressed milk or formula enroute. Place the car seat and dress the baby so that he can get maximum sunlight. If a baby is already exhibiting signs of kernicterus he may need to be fed with a feeding syringe or feeding tube, because he will be too lethargic to nurse well.

 

For more information about kernicterus the kernicterus.org website is excellent.

 

Notes:

1. Mosby’s Medical, Nursing, and Allied Health Dictionary 4th ed., Kenneth N. Anderson, revision editor, s. v. "hyperbilirubinemia of the newborn."

2. Ibid., p. 764

3. Dr. Steven M. Shapiro MD, MSHA. "Introduction: Jaundice and Kernicterus"

<http://www.kernicterus.org/> (24 October 2010).

4. Ibid.

5. L Johnson, VK Bhutani, K Karp, EM SIvieri, and SM Shapiro, "Clinical report from the Pilot USA Kernicterus Registry (1992-2004)," Journal of Perinatology (2009) 29, S25-S45.

6. Dr. Steven M. Shapiro MD, MSHA. "Introduction: Jaundice and Kernicterus"

<http://www.kernicterus.org/> (24 October 2010).

7. Dr. Steven M. Shapiro MD, MSHA. "Introduction: Jaundice and Kernicterus"

<http://www.kernicterus.org/> (24 October 2010).

8. Op. cit, Johnson, et. al., pp. S40-S45.

9. "Cytomegalovirus (CMV) and Congenital CMV Infection," <http://www.cdc.gov/cmv/oveview.html> (28 July 2010).

10. N Curtis, L Chau, S Garland, S Tabrizi, R Alexander, and C J Morley, "Cytomegalovirus remains viable in naturally infected breast milk despite being frozen for 10 days," Archives of Disease in Childhood Fetal & Neonatal edition 2005;90:F529-F530 doi.1136/adc.2004.067769

11. SM Shapiro, VK Bhutani, and L Johnson, "Hyperbilirubinemia and Kernicterus," Clinics in Perinatology 33 (2006) p. 399.