By far the most common medical problem in newborn infants is jaundice, typically appreciated as a yellowish discoloration of the skin caused by increased blood levels of a pigment called bilirubin. In my role as a newborn hospitalist, I manage jaundice every day. If I am not treating jaundice, in every single baby I see I am at least determining the risk of the child developing jaundice severe enough to require treatment. I then use that assessment to help guide my recommendations on when the infant should follow up with their primary care pediatrician after discharge home.
Fortunately for the millions of infants who develop jaundice every year, in the vast majority it is a self-limited process and often considered to be just a normal part of the first few days of life. But in a significant minority of them, careful management is required in order to prevent complications. Some infants need treatment to prevent neurological symptoms from developing, and to reverse them when they do occur. And in a very small percentage of babies who develop severe jaundice, permanent brain damage and even death can occur.
Because newborn jaundice tends to resolve without any intervention, and complications are now uncommon, it isn’t surprising that a variety of myths and superstitions have arisen that involve preventing or curing the condition. And naturally there are practitioners of unproven alternative medical modalities that can be found claiming to be able to manage it as well. As expected, if you’ve spent any time reading Science-Based Medicine or researching the nonsensical claims of chiropractors, homeopaths and their ilk, their understanding is limited and their recommendations potentially dangerous.
But first a crash course on newborn jaundice.
What is jaundice?
Jaundice occurs when there is an elevation in the amount of bilirubin in the blood. In older kids and adults, anything above 1 mg/dl is considered abnormal. But virtually all babies have levels higher than this within a day or two of birth, and often considerably higher. I routinely see bilirubin levels in newborns of 15-20 mg/dl and occasionally much higher.
The higher the level of bilirubin in the blood, generally the more jaundiced a baby looks and the lower down on the body it progresses. But using a visual assessment is problematic because it doesn’t reliably predict levels in the blood in all babies. We have all been fooled by little pumpkins with low levels as well as babies with high levels despite little apparent jaundice. It can be particularly challenging in infants with more natural pigment in their skin to begin with.
Of course long before the development of techniques to measure serum bilirubin levels, people noticed that newborn infants often developed a transient yellow discoloration of the skin with no apparent associated symptoms. Historically this bore the name Icterus neonatorum, which is based on ikteros, the Greek work for what we now know as jaundice. The word jaundice is derived from the French word for yellow, jaune .
Also known to physicians of the past was a more severe form of newborn jaundice given the name Icterus gravis. They noted severe anemia, neurological effects and typically death in addition to the yellowish skin. It would be many years before the cause of this condition, as well as the many risk factors associated with jaundice in general, would be understood by medical scientists and incorporated into our care of newborns.
Bilirubin in the human body, at least about 90% of it, is a by-product of the breakdown of hemoglobin from our blood. Once produced, bilirubin is bound by the protein albumin in the blood and transported to the liver where it is prepared for excretion by undergoing conjugation with glucuronic acid by the enzyme UGT1A1. This more water-soluble conjugated bilirubin is excreted into the intestines via bile from our gallbladder and leaves our bodies as metabolic waste in our feces.
In the newborn human, there is a problem at every step in this process, which is why even perfectly healthy babies are at risk of developing jaundice. Newborns make more bilirubin normally than at any other time during the lifespan because they have, on average, more hemoglobin than at any other time. Babies can even be born with too much hemoglobin, leading to a condition called polycythemia where the increased viscosity of their blood can reduce oxygen delivery to vital organs. Fetal red blood cells also have a shorter life span than adult. So more blood, and more breakdown of that blood, leads to more bilirubin in the blood.
The ability of the newborn to conjugate bilirubin in the liver is impaired because of a deficiency of the necessary enzyme. This occurs in all babies but can actually be worse in some populations, particularly in families designated as Eastern Asian. It takes over three months for the liver to reach adult capacity to conjugate bilirubin. So babies produce more bilirbubin, and more of it stays in the blood in the unconjugated form that can cause problems. It’s important to note that patients of all ages can develop a buildup of conjugated bilirubin in the blood as well which is typically related to a variety of liver diseases. That, however, is a horse of a different color. Well, technically it’s still yellow.
Finally, there are even problems with the bilirubin that does make it into the gut in the conjugated form. Older kids and adults further break conjugated bilirubin down with the help of bacterial enzymes, but it takes time for the newborn gut to become colonized. As conjugated bilirubin sits in their intestines, a human-produced enzyme deconjugates it, thus allowing it to be reabsorbed back into the body in a process called enterohepatic circulation.
We call the end result of these normal barriers to removing bilirubin from the body “physiologic jaundice” to differentiate it from the many disease states and environmental factors that can also lead to severe elevations in bilirubin. Again, this occurs in every single baby to varying degrees. It can, even without any pathological assistance, lead to jaundice bad enough to cause symptoms or require treatment, although alone it would be extremely unlikely to lead to permanent injury or death. Physiologic jaundice tends to peak at around the fourth day of life, although that peak can be higher and further out in premature and Eastern Asian infants.
What is pathologic jaundice?
There are a variety of diseases and environmental insults which can exacerbate physiologic jaundice in newborns or lead to severely elevated levels of bilirubin all by themselves. Rather than give an exhaustive review, I’ll focus on the two most important. Increased production of bilirubin from excessive destruction of red blood cells is the most common cause of pathologic jaundice. This typically occurs when the blood type of the mother and baby are mismatched, usually with the mother having O+ blood while the baby is A+ or B+, leading to the production of maternal antibodies. These antibodies can cross the placenta and signal baby red blood cells for destruction in the spleen.
ABO incompatibility can be severe, but often isn’t. But there is an extremely severe and often deadly form of this kind of mismatch which I’ve never seen thanks to advances in modern medicine. Rh incompatibility, when a mother with negative blood gives birth to a baby with positive blood, used to kill a lot of babies prior to the development of Rho(D) immune globulin (RhoGAM) because the maternal immune response is so intense. The first use of Rho(D) immune globulin in a mother, which facilitates destruction of fetal red blood cells before an immune response can occur, was in 1968. Research and treatment of Rh disease in the three decades leading up to that year is credited as the impetus for the development of neonatology as a unique specialty within pediatrics.
The second most common cause of pathologic jaundice is known as “breastfeeding jaundice”. Unfortunately, it is a well-established fact that breastfeeding is a major risk factor for the development of a severe elevation of bilirubin, which is why pediatricians tend to want earlier follow up visits after discharge from the nursery with breastfed babies. The process by which breastfeeding can negatively impact bilirubin levels is by increasing the enterohepatic circulation I mentioned earlier. If things are going well with breastfeeding, the risk is actually not much higher than in babies receiving formula. But when breastfeeding is going poorly during the pivotal first few days of life, the inadequate intake of calories and fluid leads to more unconjugated bilirubin being reabsorbed from the gut back into the blood.
The possibility of breastfeeding-failure jaundice is something we take very seriously in the newborn nursery. It isn’t the only reason why there is such a push for improving breastfeeding support of course, but it is a big part of it. We provide extensive education on lactation, document successful latching, track weight loss and the number of wet and dirty diapers, and troubleshoot a variety of potential roadblocks such as nipple pain and maternal fatigue. We are especially careful with breastfeeding premature infants, who are at increased risk of breastfeeding failure and physiologic jaundice already tends to be more pronounced.
A related but benign cause of jaundice in young infants, typically occurring during the 2nd week of life, is “breast milk jaundice.” It occurs after breastfeeding has been well established in exclusively-breastfed babies when physiologic jaundice persists after the usual rapid decline of bilirubin around the fifth day of life, and it can last for several weeks without intervention. Although the exact cause is unknown, it is believed that there is a component within breast milk that promotes intestinal absorption of bilirubin. A brief interruption in breastfeeding, usually just one to two days, leads to rapid resolution, although many pediatricians do not recommend this as there is no harm from the elevated bilirubin at that point.
How does jaundice affect newborns?
In most newborns with jaundice and even moderately elevated levels of bilirubin in the blood, there are likely no adverse acute or long term effects. The standard approach to screening and treatment of the condition, which admittedly can seem overly aggressive at times, is primarily geared towards preventing a very rare outcome known as kernicterus. This involves permanent damage to specific regions of the brain and can result in death in some cases. We believe that it should never happen, that it should be preventable virtually 100% of the time. In fact, The Joint Commission, who certifies health care institutions and programs, designates it as a sentinel, or “never” event.
As with many medical conditions, there is a spectrum of severity when it comes to symptomatic jaundice. At a certain point in any baby, the bilirubin in the blood can reach such a level that it is able to cross the blood-brain barrier and begin binding to neural tissue, which leads to cell death. The more brain tissue involved and the longer a child goes untreated, the more severe the symptoms. Although we can never perfectly predict at what level this will begin to occur in for any individual child, we tend to see subtle symptoms when levels hit around 20 mg/dl, with the more overt injury evident at 25-30 mg/dl. Anything that can impact the permeability of the blood-brain barrier, such as prematurity or infection, can lower the level that causes brain injury.
Bilirubin-induced neurologic dysfunction (BIND) can be broken down into acute encephalopathy, which is reversible with appropriate treatment, and kernicterus, which isn’t. At first, babies with increasing bilirubin levels become sleepy and begin to feed poorly. They may seem floppy or have a different sounding, high-pitched cry. As things progress, a baby can develop fever and extreme lethargy, or they may become extremely irritable and jittery. They may have no suck at all or develop an aggressive suck. Their cry may become more high pitched and urgent sounding and they may be very difficult to console. Stiffness can set in, the first sign of which is often arching of the neck and body when touched.
Without emergency treatment at this point, the damage will become irreversible. They will begin to have periods without respiratory effort and will stop feeding altogether. Fever and seizures are common as they become more persistently stiff and arched at the neck and back and progress into a comatose state. They will ultimately die from either respiratory failure or prolonged seizure activity.
Kernicterus is diagnosed when signs of brain injury become evident during the first year of life. Because bilirubin deposition in the brain tends to occur predominantly in the areas that regulate movement (basal ganglia) and hearing, cerebral palsy and hearing loss are common, as are difficulties with eye movement. The development of the teeth can also be affected. Cognition tends to be unaffected but there is some controversy regarding the possibility that even moderate levels of bilirubin elevation are a risk factor for neurologic problems later in life. At this time, the evidence is mixed but trending against any association with autism, learning disabilities, ADHD or any other psychological disorders.
How is newborn jaundice evaluated?
The key to managing newborn jaundice is awareness of risk factors, such as prematurity, genetics and whether or not the baby is being breastfed. Most hospitals incorporate universal pre-discharge bilirubin screening into our decision-making process and always attempt to establish appropriate follow up post-discharge. Our goal is the prevention of severe elevations of bilirubin in order to prevent the development of symptoms and ultimately of kernicterus.
Since the early 1990s, a systematic approach to newborn jaundice has been recommended. Every baby goes through the process of screening, risk assessment, breastfeeding evaluation (if breastfeeding), and use of an established treatment algorithm based on the bilirubin level and the infant’s hours of age. This has led to a decrease in the number of babies that develop severe elevations of bilirubin. The rarity of kernicterus has made it difficult to assess whether our interventions have successfully decreased its incidence, but it is reasonable to use severe bilirubin elevation as a surrogate outcome for kernicterus. The US Preventive Services Task Force disagrees with the AAP on this, and does not recommend universal screening, which I believe is a good example of the difference between evidence-based and science-based medicine.
I can certainly understand the hesitancy in initiating universal screening of pre-discharge bilirubin levels. Historically this has required a heel stick to obtain a few drops of blood from the baby. Heel sticks hurt without proper procedural pain measures such as use of a pacifier and sucrose, and probably aren’t that comfortable even with them. Labs aren’t free either. A fairly recent development in the management of newborn jaundice has significantly ameliorated these concerns, however.
Rather than the testing of a baby’s blood for elevated bilirubin, an increasing number of hospital nurseries are now using transcutaneous bilirubinometers (TcB). These handheld devices use multiwavelength spectral reflectance from the skin to estimate the level of bilirubin in the blood. A number of large studies have demonstrated their accuracy in various ethnic groups, but they aren’t perfect. They are unreliable once a baby has started phototherapy, possibly even if they’ve just been exposed to direct sunlight, and they have decreased accuracy at high bilirubin levels.
The typical approach to use of TcB is to confirm the bilirubin level with a blood test if the result is greater than the 75th percentile on the standard bilirubin nomogram. My personal rule of thumb is to add 3 to the result, and if that changes my management I confirm the level with a heel stick. Use of these devices significantly decreases the number of heel sticks performed, and they save money in the hospital, but they have not been recommended for use in the outpatient setting yet.
There are a variety of additional tests often incorporated into the evaluation of risk for severe jaundice, such as the infant blood type and presence of maternal antibodies when there is a mismatch. This is often performed whenever a mother has the O blood type. And naturally there are diagnostic tests when other pathology is suspected, such as red blood cell membrane defects, enzyme abnormalities, or genetic/metabolic conditions. We sometimes get even fancier. Some facilities are able to measure the amount of carbon monoxide, another break down product of hemoglobin, exhaled through the nose as a very sensitive test for abnormal destruction of red blood cells.
How is newborn jaundice treated?
Historically the treatment of jaundice was time and perhaps prayer. Infants who developed “icterus gravis” either died or had severe lifelong disability. As medical science advanced and we determined that elevated bilirubin was the underlying cause of symptomatic jaundice, a means of removing it from the blood was developed. The exchange transfusion, where blood is removed from the baby in small aliquots, dumped and then replaced with suitable donor blood, became the treatment of choice. Unfortunately the procedure was, and is, risky although its safety has improved greatly.
Fortunately, as is often the case in science, hard work, keen observation and a measure of luck led to the discovery and development of means to prevent many severe cases and safer treatment alternatives that have become the gold standard. As I’ve already mentioned, the implementation of screening of maternal blood type and use of Rho(D) immune globulin has rendered Rh disease almost nonexistent in developed nations. Again, in ten years of practice I’ve never seen a case of it.
The second advance was the realization, as infant formula was introduced and became a common alternative to breastfeeding, that breastfeeding difficulty increased the risk and severity of jaundice. Greater focus on improving breastfeeding, and at times strategic use of infant formula and mechanical breast pumping, has prevented many cases of severe jaundice. The final significant advance was phototherapy. This combination of interventions has drastically reduced the need for exchange transfusion. I’ve participated in only one. As the intern on the team it was my duty to sit in sterile gown and gloves at the bedside for 6 hours while performing the transfusion manually.
What is phototherapy?
While the practice of exchange transfusion did revolutionize the treatment of severe jaundice, and certainly improved the outcomes of thousands of newborns, it was risky. And we now know that it was done too often in babies that likely would never have developed kernicterus. This was a time before established risk factors and handy iPhone app algorithms to determine the need for treatment.
But in 1956 a nurse in England, working in the nursery at Rochford General Hospital, noticed that babies placed in sunlight for extended periods of time were less jaundiced. Biochemists in the lab were also perplexed by oddly-low serum bilirubin levels in blood samples left sitting in the sunlight. The rest is history. In 1968, a landmark randomized controlled trial published in Pediatrics by Jerold Lucey showed that phototherapy was a safe and effective treatment for newborn jaundice.
The history of phototherapy after that publication, and the struggle between two factions of practitioners over the direction of jaundice treatment is fascinating. Led by “princes of light and dark” at dueling hospitals in New York City during the decade after the paper came out, the battle over phototherapy was heated but ultimately the evidence won out and the exchange transfusion has nearly gone the way of the Dodo. And I “light kids up” weekly.
How does phototherapy work?
Phototherapy isn’t just regular light, and despite what you can find written on numerous alt med websites it isn’t ultraviolet light either. Phototherapy is blue-green light, although more recent evidence has supported a shift over to predominantly blue light, specifically between 460 and 490 nanometers. When this light hits bilirubin molecules in the infant’s skin, bilirubin is affected in three ways.
The most important effect is the irreversible conversion of bilirubin into a more soluble form via structural isomerization. The new form, lumirubin, passes into the gut and urine for excretion without the need for conjugation. Furthermore, phototherapy alters some of the bilirubin into a less neurotoxic form via a reversible photoisomerization. This doesn’t result in an appreciable drop in the level but reduces the risk of brain injury. Finally, photo-oxidation of bilirubin converts some to a form that is excreted only in the urine. This likely only accounts for a small percentage of the drop in total levels however. All in all, and please pardon the pun, phototherapy is brilliant. It is a safe, effective and all-natural treatment but it does line the pockets of “Big Light.”
“Alternative” approaches to newborn jaundice
So now you know the science-based approach to newborn jaundice. It is an approach which does “medicalize” some children that would not go on to develop symptomatic jaundice or kernicterus, but it is extremely safe and effective. Now let’s explore what some of our friends in the world of complementary and alternative medicine have to say about this common condition. Just a quick perusal, as an exhaustive discussion would both take up too much of your time and ruin my day, the first day that actually feels like spring up here.
Practitioners of craniosacral therapy get partial credit, describing jaundice as “yellowness of the skin due to the fact that the baby’s young and small liver is unable to process and [sic] excess of blood and so has to use the skin as it’s [sic] organ for release instead of the intestines or urine.” They make the common mistake of describing phototherapy for jaundice as “ultra-violet”, which if true would cause horrendous skin injury during the many hours a baby requiring therapy will typically spend under the lights. Phototherapy does not burn or increase freckling or mole development. And modern versions don’t even produce much heat as they are LED lights, thus the infant is not at risk of dehydration. Despite a lack of any evidence, they claim that CST can improve clearance of bilirubin, which could lead to false reassurance and a delay in seeking out appropriate medical care.
Believers in traditional Chinese medicine have a unique approach to newborn jaundice:
The causative factors of icterus neonatorum are dampness-heat and cold-dampness which are affected from the mother, or during delivery, or after birth. The hypofunctioning organs, particularly the spleen, fail to eliminate dampness-heat, leading to its retention in the liver and gallbladder and its distribution to the skin. Moreover, on the basis of the deficiency of healthy qi which results from congenital insufficiency or longstanding disease, the maladies of dampness-heat and cold-dampness may lead to the deficiency of spleen-yang and then the retention of the evils, and jaundice results.
Unfortunately, phototherapy has yet to be proven effective for “retained evil.”
One practitioner of homeopathy claims to be able to prevent and treat jaundice using a variety of nonexistent remedies. Please do not try to decipher which remedy is appropriate for your baby yourself, however. Only a fully trained homeopath can tell the difference between sluggish bowels and constipation.
This particular practitioner recommends letting your baby “bask” in the sunlight to treat jaundice, but I can’t just pick on homeopaths about this. The question of using natural sunlight for mild jaundice comes up frequently when caring for newborns. I don’t have data on this, but my best guess would be that a recommendation to put a kid in the sun is as common as recommendations to alternate acetaminophen and ibuprofen for fever, with most pediatricians doing it. And similar to alternating fever medicines, it isn’t recommended by experts because of risk and efficacy concerns.
We know that sunlight lowers bilirubin levels, that isn’t the issue. The problem is that in order to achieve that end, a child would need to be placed naked in direct sunlight for hours. This would place a baby at risk for hypothermia and/or sunburn. It also may only make the skin appear less jaundiced while not necessarily lowering the level of bilirubin in the blood appreciably, giving a false sense of security and potentially delaying care. This happens with conventional phototherapy as well. Once a kiddo is lit up, we have to rely on blood tests instead of a transcutaneous level.
The advice I hear most frequently given to parents, to put a baby in sunlight through a window for 10-15 minutes 3-4 times a day, would be very unlikely to lower bilirubin levels any faster than doing nothing. But because jaundice so often resolves on its own, it is easy to see why so many people think sunlight or any alternative approach “works.”
Naturopath Dr. Christopher and his son David, who “generated new ideas on the management of high blood pressure, diabetes and auto-immune diseases, and was one of the first to discourage the use of Prozac in favor of natural methods”, provide us an example of a natural approach to infant jaundice. It demonstrates a fundamental lack of any understanding of the concept. Here are a few of their pearls of wisdom:
- Jaundice is caused by drugs given to the mother during the birth process.
- Physiologic jaundice only affects blond babies, Native American babies, drug babies, premature babies, and babies not allowed to breastfeed.
- Catnip, comfrey and dandelion tea are excellent treatments for newborn jaundice.
- Wheat grass juice is also helpful.
- Phototherapy causes irritability and sluggishness, diarrhea, lactase deficiency, intestinal irritation, dehydration, feeding problems, riboflavin deficiency, disturbance of bilirubin-albumin relationship, poor visual orientation with possible diminished responsiveness to parents, DNA-modified effects and it might even make their lungs bleed.
- Severe jaundice can cause brain damage, and the best approach is to follow your maternal intuition.
Here is the scariest chiropractic “case report” I’ve ever read. It involves the chiropractic treatment of an unresponsive and jaundiced 36-hour-old infant with a heart rate in the 70’s (chest compressions are recommended when the heart rate is < 60 beats per minute in a newborn). The baby responded to the adjustments with an increase in heart rate, immediate resolution of his jaundice and a hearty breastfeed. And he was removed from the list of likely “candidates for SIDS.” But that isn’t the best part:
Interestingly, his testicles had also descended within that hour. That may have been a coincidence, but it seems noteworthy given all the other healthy indicators we witnessed as a result of the adjustments.
These examples are scary, and a little funny, but I realize that they don’t represent all practitioners of alternative medicine. Like few other fields of practice, alternative medicine is comprised of so many disparate and mutually-exclusive approaches to health that their ability to hang out under the same tent is a testament to just how much they don’t like conventional medicine. Trust me, if somehow they won and regular medicine faded away into history, it would be a bloodbath as they turned on each other in an effort to become the dominant modality.
Jaundice is the most common medical problem in the newborn, and though typically benign it can result in severe complications with permanent injury to the brain and even death a possibility if mismanaged. The pathophysiology of jaundice in babies is unique, fascinating, and simple enough that almost anyone can get a handle on it. Even more interesting is the history of how we came to understand the cause of jaundice and the progression from being unable to offer any treatment to only having the risky exchange transfusion as an option, and finally to the safe and effective use of phototherapy.
As our understanding of the many risk factors which play a role in the development of severe jaundice has advanced, the management of newborn jaundice has become more nuanced but also systematic and streamlined in such a way that it is essentially a process on autopilot these days. All babies are assessed for risk and, at least with the vast majority of births, universal screening of bilirubin levels is obtained prior to discharge home from the nursery. Even the decision when to treat can be made with the use of handy algorithms that take seconds to master.
Unfortunately, once we step outside the borders of science-based medical practice there are any number of practitioners of so-called alternative medical modalities that have essentially no understanding of how and why jaundice develops, which babies are most at risk and what are the most safe and effective approaches to treatment. Babies subjected to their machinations are at risk of poor outcome because of a delay in appropriate medical care.