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A Preventable Tragedy Kernicterus :

When Untreated Jaundice Causes Brain Damage

Miriam Iliff and her husband, John, were over the moon when their first child, a baby boy they named Blue, was born in the summer of 2005. Though three weeks early, Blue was a healthy seven pounds and had a hearty appetite. Like most typical new parents, the Iliffs snapped as many photos as they could of their precious baby and settled into their happy new life as a family of three.

Within days of leaving the hospital, however, things began to change. Blue had developed a large bruise on his head and his skin and eyes had begun to turn yellow. He was also extremely lethargic. Nonetheless, at two doctor’s visits, Blue’s parents were assured that all was fine and there was no need to worry.

But on Blue’s ninth day, when he awoke from a nap with a blood-curdling, high-pitched scream and began projectile vomiting, it was clear that something was extremely wrong. Miriam dialed the hospital’s 24-hour advice line and reached a triage nurse who dismissed her concerns one by one and assured her that her baby’s behavior was normal. By then, Blue’s eyes were as yellow as chicken soup and fixed in a downward gaze like two setting suns.

Despite the nurse’s lack of concern, the Iliffs rushed their son to the emergency department, where a blood test revealed that Blue’s bilirubin was 45.6 mg/dL — a dangerously high level that can cause catastrophic brain damage.

As the baby’s oxygen levels plummeted, the hospital scrambled to perform an invasive emergency procedure known as a double blood exchange transfusion. The doctor threaded two catheters, one on each side, into the blood vessels running from his groin to his heart and with two 60cc syringes drained and replaced all the blood in Blue’s tiny body twice over.

Blue, his parents would later learn, had suffered a devastating brain injury called kernicterus, caused by excessive, untreated jaundice. And while the blood exchange that saved his life halted any further brain damage from occurring, it would not undo the harm already inflicted. Blue, they were told, would probably never walk, talk, or even hold a pencil.

When jaundice isn’t normal

Jaundice is one of the most common medical problems encountered in newborns. Sixty to 80% of infants experience some degree of jaundice, or yellow discoloration of the skin, eyes and other tissues caused by a bile pigment in the bloodstream called bilirubin, which is a by-product of the normal breakdown of old red blood cells. Newborns’ immature livers often can’t process and eliminate the bilirubin quickly enough. The resulting buildup of bilirubin in tissues causes the orange-yellow coloring we recognize as jaundice, or hyperbilirubinemia.

Usually, jaundice is a transient and benign condition that resolves on its own with no long-term consequences as the infant’s liver matures. This sort of benign jaundice is often called physiologic jaundice. Research shows that at low levels, bilirubin can have an almost protective effect on the body, acting as an antioxidant and reducing inflammation.In fact, it can even benefit newborns suffering from mild asphyxia, or lack of oxygen. But at higher levels, the yellow compound becomes a potent neurotoxin that can seep outof the baby’s bloodstream and into brain tissue causing significant, permanent neurologic injury. This type of jaundice is referred to as pathologic jaundice and requires immediate medical intervention to prevent complications.

Signs/Symptoms of Newborn Jaundice

    • Yellowing of the skin (including the abdomen, arms, legs). In babies with darker complexions, yellowing may be observed inside the mouth, on the soles of the feet or on the palms of the hands.
    • Yellowing of the whites of the eyes, or sclera
    • Jaundice (yellowing of the tissues) that exceeds more than three weeks
    • Poor sucking or feeding
    • Inability to gain weight
    • Dark urine
    • Pale stool
    • Rapidly increasing bilirubin levels

Signs of Acute Bilirubin Toxicity in a Jaundiced Baby

  • Extreme sleepiness and lethargy
  • High-pitched, shrill crying
  • Stiff, limp or floppy body
  • Backward arching of the back or neck
  • Fever
There are several telltale signs that can help clinicians differentiate between so-called “normal” jaundice and the more severe form that can lead to brain damage. Unlike physiologic jaundice, pathologic jaundice often appears within the first 24 hours of life and warrants testing of the baby’s total serum bilirubin, via a simple blood test, or a non-invasive skin test using what’s known as a transcutaneous bilirubinometer

Bilirubin levels should always be interpreted according to the infant’s age in hours by plotting them on a special chart called a nomogram. A widely adopted tool, the nomogram helps clinicians determine the baby’s risk for significant hyperbilirubinemia and determine appropriate assessment, intervention and follow-up strategies. Relying on visual inspection alone, experts agree, is inadequate and can result in errors and missed diagnoses of hyperbilirubinemia.

Certain conditions can also predispose an infant to neurotoxic jaundice and physicians should assess every baby for such risk factors. These include: fetal-maternal blood type incompatibility, being exclusively breastfed, prematurity, and having a sibling who was afflicted with severe jaundice requiring phototherapy. Significant bruising, including bruises caused by the trauma of labor and delivery, and certain types of infections can increase a baby’s chances of developing hyperbilirubinemia. A number of rare inherited disorders, including Glucose-6-phosphate dehydrogenase deficiency (G6PD) and Crigler-Najjar Syndrome, can also cause severe jaundice that results in kernicterus.

Ethnicity can play a role. Infants with East Asian, Middle Eastern or Mediterranean ethnicity have a higher risk of developing pathologic jaundice. Gender is also important, as baby boys are more likely than girls to develop severe jaundice.

Fortunately, kernicterus is almost always preventable if potentially toxic bilirubin levels are caught and treated in time. Physicians can and should take swift action to lower bilirubin and prevent permanent neurological damage by placing a jaundiced baby under a special blue light, or in more advanced cases, by replacing the child’s blood through an exchange transfusion like the one Blue received.

But despite the ability to detect and treat severe neonatal jaundice, cases of kernicterus, or chronic bilirubin encephalopathy, continue to occur throughout the world. In developing countries, untreated jaundice is among the top five causes of infant death. And while catastrophes related to severe jaundice are usually avoided here in the U.S. thanks to rigorous screening and proper and timely treatment, dozens of children are still slipping through the cracks each and every year and suffering severe brain damage that is completely preventable.

In most cases, this failure to diagnose, monitor and treat the severe jaundice that leads to kernicterus can be attributed to negligence or medical malpractice — and these appalling failures can have devastating, lifelong consequences.

A pattern of damage

While French pediatrician Jacques François Édouard Hervieux was among the first to describe neonatal jaundice of the brain in 1847, it was German pathologist Christian George Schmorl who coined the term “kernicterus” in 1904 after observing a pattern of intense yellow staining in the brains of 120 jaundiced infants who had died. Notably, in six of the autopsied infants, the yellow staining was isolated to a very specific section of the brain — the basal ganglia, or basal nuclei — so Schmorl called this pathological finding “kernicterus,” the German word for jaundice (icterus) of the nuclei (kern).

Today, the term kernicterus is commonly used to describe not just the pathology of bilirubin-induced brain injury, but the condition itself.

“There’s a pattern of damage,” explains Dr. Steven Shapiro, MD explained in a recent interview. Shapiro is the , director of neurology at Children’s Mercy hospital in Kansas City, Missouri and one of the leading physicians specializing in the treatment of children with kernicterus. “It’s many of the areas of the brain that are affected selectively by bilirubin when it gets into the brain, but there are just a couple that really have a major impact on causing disability.”

One of those areas, as Schmorl correctly observed, is a region deep within the base of the brain called the basal ganglia — and more specifically a section of the basal ganglia called the globus pallidus, which regulates voluntary muscle movements. “The other is in the brain stem auditory nuclei, which controls sound getting in and up to the brain and it can cause distortion of sound or deafness,” says Shapiro. As a result, many children with kernicterus suffer from movement disorders and hearing loss.

Because the degree of damage differs from child to child, individuals with kernicterus can exhibit a wide range of symptoms and disabilities, much in the same way that children on the autism spectrum can vary widely.

Characteristics of Kernicterus Injury (Chronic Bilirubin Encephalopathy)

  • Spasticity and movement disorder
  • Hearing loss or deafness
  • Impaired eye movement /gaze abnormalities (often impairment of upward gaze)
  • Abnormal discoloration of teeth
  • Cognitive defects (minor intellectual deficits may but do not always occur)
Some children with milder forms of kernicterus, for instance, might deal primarily with hearing disorders or exhibit subtle neurodevelopmental disorders such as learning disabilities. At the other end of the spectrum, severely affected children often end up wheelchair bound, suffering from a type of cerebral palsy that renders them unable to move and speak and dependent on caretakers for every aspect of daily living.
Their intellect, meanwhile, is usually spared, leaving them in a “locked-in”state, unable to control their muscles or communicate easily with the outside world, but understanding everything going on around them. Trapped, in essence, in their own bodies.Shapiro sees a number of children with these more severe cases of kernicterus at his clinic.“Most of these kids have very

severe movement problems, with abnormal movements and abnormal contractions of muscles” that they can’t voluntarily control, he explains. “When they want to move something, their muscles fire off in a very non-coordinated, abnormal way… It can be very difficult to talk, use a keyboard, communicate, even though you have normal intelligence.”

That’s the reality for Lexi Haas, a 15-year-old North Carolina girl who suffered a kernicterus injury shortly after she was born in 2002.

Her mother, Susan Haas, recalls how as a newborn, Lexi turned a deep orangey bronze from her head to her toes “like she’d been at the beach” and did nothing but sleep after they brought her home from the hospital. At follow-up appointments, the pediatrician dismissed Susan’s concerns about her yellow baby as the unfounded worries of an overly anxious mom. He assured her that Lexi’s jaundice would resolve on its own — and it finally did, three weeks later. But as several months went by, and Lexi failed to reach her developmental milestones, her parents realized something else was going on.

“We were dragging her around to children’s hospitals, trying to figure out what was wrong with her. They told me they thought she might have a brain tumor,” her mother recalls. Eventually, her parents learned that Lexi was suffering from kernicterus, caused by excessive, untreated jaundice.

As a result, Lexi is wheelchair bound and can’t control her muscles or speak. Her intellect is unaffected. She has an I.Q. of 160, understands French and has been working with her mom on a book about her life.

Under the care of Dr. Shapiro, she’s had two surgeries to have electrodes implanted in her brain so that she can receive a type of therapy known as Deep Brain Stimulation (DBS). While she still can’t speak or move unassisted, her mother says the treatments have improved her motor control and made her somewhat calmer. Nonetheless, Lexi will require round-the-clock care for the rest of her life to cope with disabilities from brain damage that was completely avoidable.

That’s the part that haunts her mother — that what happened to Lexi could have been prevented. “You think, ‘This is so simple. How could we not have known?’ … Even if you know intellectually it’s not your fault, it is so hard to let go of that. There’s just an immense guilt that doesn’t go away.”

Slipping through the cracks

In 2001, the Agency for Healthcare Research and Quality (AHRQ) labeled kernicterus a “never event” — a medical error so egregious that just like wrong-site surgeries or medication errors, it should never occur. That move, along with the American Academy of Pediatrics’ (AAP) issuance in 2004 of new conservative clinical practice guidelines for managing hyperbilirubinemia in infants, has led to heightened vigilance among many clinicians in treating newborn jaundice, according to those on the front lines of the kernicterus battle.

Nonetheless, kernicterus cases continue to occur.

While there’s no surveillance system in place to track cases of kernicterus in the U.S., Shapiro, who sees more of them than probably any doctor in the country, estimates about 90 babies born each year are afflicted with “classic,” severe kernicterus. Although proper screening prevents upwards of 99 to 99.9% of cases, he says, “some kids slip through” the preventive safety nets.

That’s in part because bilirubin screening isn’t perfect — and one can’t always predict with complete accuracy when brain damage might occur. Laboratory errors also happen, he says, and bilirubin encephalopathy can develop quickly and unexpectedly if a child develops sudden hemolysis, or rupturing of red blood cells from blood-type incompatibilities and other conditions.

By and far, though, Shapiro says the vast majority of cases he sees are the result of medical errors. “I’m pretty much the only neurologist who specializes in this, so I get to see all the screw-ups or worst cases. Most of them are from failure to follow guidelines or mistakes that healthcare providers have made, or sometimes parents who don’t bring them to doctors,” he says.

Indeed, a 2009 study that tracked 125 infants who suffered kernicterus injuries between 1992 and 2004 attributed 95% of cases to “multi-factorial failure of the post-partum and newborn healthcare delivery system.” The study concluded that “virtually all” of the cases reported could have been prevented by timely identification and treatment of the infants’ severe jaundice. The study also surmised that the 125 cases examined were likely just the “tip of the iceberg” and the actual number of infants in the U.S. suffering from bilirubin poisoning is higher.

“Kernicterus is almost always an unacceptable outcome in healthy > 35 weeks gestation infants in the United States.”

Clinical repORt from the pilot USA Kernicterus registry (1992 to 2004)

Miriam Iliff still wonders why Blue’s severe jaundice wasn’t caught sooner. In fact, the Joint Commission, the national organization that accredits hospitals, had sent out two “sentinel event alerts” about kernicterus prior to Blue’s birth. The special alerts are intended to inform clinicians about serious adverse events that can be avoided — but Iliff says the staff at the hospital where her son was born appeared ill informed.

“I think the hospital had just put stuff on the walls to be compliant but they didn’t actually train any of their nurses on the dangers of jaundice, what that looked like, when to use the [bilirubin meter]. Half of them didn’t know where the bilimeter was. The nurse who discharged Blue didn’t think a cephalohematoma was a bruise,” she recalls.

And like the parents of so many children who end up suffering kernicterus injuries, the Iliffs received little to no education about the dangers of severe jaundice. Though Blue had registered a bilirubin level of 15 mg/dL when he’d left the hospital, which placed him in the high-risk category for developing severe hyperbilirubinemia, Iliff says she was simply told to “place him by a sunny window and feed him often.” Later, when she asked the nurses at the hospital why they hadn’t warned her about the dangers of jaundice, they said that they hadn’t wanted to scare her.

Inadequate patient and family education about severe jaundice, it turns out, is common. According to the Centers for Disease Control and Prevention, a national survey of 5,000 Americans found that nearly 72% had never heard of kernicterus and most parents were unaware that untreated jaundice could lead to brain damage.

An overriding problem, however, is the sense of complacency about severe jaundice that has taken ahold in the medical community as kernicterus cases have become a rarer occurrence. While kernicterus was “one of the major pediatric problems in the early 20th century,” cases plummeted during the 1970s and 1980s after the advent of phototherapy treatment and the development of the drug RhoGAM, which prevents the complications of Rh incompatibility, a major cause of hyperbilirubinemia.

By the early 1990s, however, anecdotal reports of a resurgence of kernicterus began to appear in the medical literature.

Some experts, like Dr. Vinny Bhutani, MD, a neonatologist at Stanford and one of the foremost kernicterus experts in the world, believes the early discharge of newborns may be a contributing factor to the reemergence. Bhutani notes a “dramatic increase” in cases as hospitals and insurers moved toward so-called “drive-through deliveries” — the discharge of infants fewer than 48 hours after birth. “I think it’s a high-risk practice to send babies home before 24 hours, or 30 hours after birth. They still need to be under direct medical supervision,” he explains.

In fact, the typical newborn’s bilirubin levels don’t peak until around their third to fifth day of life. That’s why it’s so important that every baby discharged, no matter the age, is seen within 48 hours by a licensed medical practitioner, explains Bhutani — “so there’s not only a pre-discharge assessment, but there’s a safety net.”

It was on the fifth day of Daniel Deya’s life in 2009 that he suffered a devastating brain injury from kernicterus. Deya had multiple, recognizable risk factors for developing severe hyperbilirubinemia. He was born three and a half weeks early, he was male, he was being breastfed and there was a blood type incompatibility — his mother was O+ and Daniel was A+. He also developed visible jaundice within the first 24 hours after he was born.

Nonetheless, the Missouri hospital where Deya was born allegedly never tested his blood bilirubin levels and he was sent home jaundiced just 41 hours after he was born. The only advice the hospital gave his parents, according to court documents, was to place their baby boy in a sunny window and come back for a “well child” visit six days later.

Daniel never made it to that well child visit. Just three days later, the lethargic and inconsolable infant was airlifted to Children’s Mercy Hospital in Kansas City, where the Deyas learned that their son had sustained severe, irreversible bilirubin-induced brain damage.

In 2010, the Deyas sued Hiawatha Community Hospital, where their son was born. Their lawyers argued that their son’s permanent disability might have been avoided had the hospital implemented better policies and patient protocols. Hiawatha Community Hospital settled the case for $4.3 million shortly before it was set to go to trial. That’s just a fraction of Deya’s estimated lifetime care costs of $27 million, but lawyers representing Deya said the hospital was “grossly under-insured” — a troubling problem that compounded the tragedy.

Risk Factors For Severe Hyperbilirubinemia

  • Jaundice that occurs within the first 24 hours of life
  • Ineffective breastfeeding (poor feeding/sucking)
  • Dehydration
  • Having a TSB or TcB score in the high-risk zone
  • Blood type incompatibility
  • Prematurity (< 38 weeks)
  • Having a sibling who was treated for jaundice
  • Infections (such as sepsis)
  • Significant bruising, such as a cephalohematoma, bruising and bleeding of the scalp
  • Asphyxia (Apgar Score of 6 or less)
  • East Asian, Middle Eastern or Mediterranean ethnicity
  • Numerous conditions can also lead to Hyperbilirubinemia. These include:
    • Polycythemia
    • Glucose-6-Polycythemia
    • Glucose-6-phosphate dehydrogenase (G6PD) deficiency
    • Galactosemia
    • Babies of diabetic mothers
    • Hypothyroidism
    • Gilbert’s Syndrome
    • Crigler-Naijar Syndrome

Unfortunately, there are other cases like Daniel Deya — where risk factors are ignored, bilirubin levels go unchecked and children end up suffering catastrophic brain damage.Florida mother Sundari Greene says the midwifery practice that delivered her daughter in 2008 never checked her daughter’s bilirubin levels even though Sundari was Rh negative and her baby, Radhika, was Rh positive and Rh blood incompatibilities are known to cause severe hyperbilirubinemia and kernicterus.The Greenes were sent home with their baby just hours after her birth — and at first everything seemed fine. Radhika was feeding every two hours, and producing a normal number of wet and dirty diapers, but by early the next morning she had begun to turn yellow. By the following morning, she had stopped eating. Sensing something was amiss, Sundari took her newborn to the midwife practice. She says she was told her baby was fine — and that she was just being an “over-worried, first-time mother” — even as her listless infant began to strangely twist her neck.

Unconvinced, Sundari asked for a referral to a pediatrician, but by the time she arrived there, it was clear that Radhika was deteriorating. “She was out of it. Her eyes were rolling up in her head,” recalls Sundari. Radhika’s bilirubin was so high it wouldn’t even register on the doctor’s machine.

The baby was admitted to her local hospital’s pediatric intensive care unit, where her bilirubin level was discovered to be 47 mg/dL, the highest the hospital had ever seen. Shortly thereafter, Radhika began seizing, was intubated and underwent two and half double exchange transfusions that ultimately saved her life but couldn’t undo the damage to her brain. “The first few months of her life, we were just watching to see what degree of brain damage she had,” her mother recalls.

In time, it became clear that Radhika was severely injured. She is a quadriplegic and suffers from athetoid cerebral palsy, which causes her muscles to alternately and unpredictably fluctuate between being stiff and being floppy. Her hearing is unreliable and goes in and out. She also struggles with eye gaze problems and is sensitive to light and prolonged stimulation. “It’s about six safety nets that failed for us to be where we are now, which is horrendous,” says Sundari.

Justice for innocent victims

There is no cure for kernicterus, but there are legal remedies for children like Radhika and Daniel, whose lifetime care costs will reach into the millions.Victims of medical malpractice that leads to kernicterus may be entitled to compensation for past, present and future medical bills resulting from the injury, as well as awards for pain and suffering.

In Arkansas, a jury recently awarded $46.5 million in damages to the family of a toddler who suffered catastrophic brain damage in 2014 when doctors failed to treat her severe jaundice. Kara Smalls, who is now two years old, had several risk factors for hyperbilirubinemia, including a blood type incompatibility and jaundice that developed within the first 24 hours of her life.

However, the Ouachita County Medical Center and her physician, Dr. Jonathan Lewis, allegedly discharged the infant 48 hours after birth without doing a repeat bilirubin test or warning her parents to be on the lookout for signs of hyperbilirunemia. Days later, according to court documents, Kara had all the classic signs of kernicterus: She was profoundly lethargic, had trouble feeding and was arching her back and emitting a high-pitched cry.

According to Smalls’ attorney, the defendants in the case argued that the “standard of care is lower in South Arkansas than the rest of the country” and that the hospital didn’t have to meet national standards of care for hyperbilirubinemia. But jurors flatly rejected that excuse, and handed the Deyas one of the largest awards in Arkansas history.

The California jury that heard Aidan Leung’s case in 2007 was similarly outraged by the alleged medical errors that led to his devastating kernicterus injury. Leung was sent home just one day after being born at Verdugo Hills Hospital in Glendale in 2003 — with instructions to visit the pediatrician the following week. But within three days of his discharge, Aidan had stopped feeding and could not be awakened.

Despite his numerous risk factors for developing hyperbilirubinemia — including his East Asian descent, bruising, exclusive breastfeeding and jaundice that developed the first 24 hours of his life — the hospital allegedly failed to perform any risk assessment on Aidan for jaundice and hyperbilirubinemia. Moreover, the Leungs were never educated about the risks of severe jaundice or the need for a timely follow-up appointment. Verdugo Hills Hospital, which denied those claims in court, was found negligent and the Leungs were awarded $78,000 for past medical bills and nearly $83 million for future costs.

Because the family had previously reached a $1 million settlement with the physician in the case and an arcane, 200-year-old “common release” rule required the release of liability of all jointly liable defendants in a tort action if one of them settles, a California appeals court threw out that verdict in 2011 and reduced the hospital’s liability to $100,000. But the California Supreme Court reversed that decision in 2014, reinstating the judgment that will amount to about $96 million over the course of Aidan’s life.

While some kernicterus cases go to trial, many others are settled out of court. In 2017, Mosaic Life Care in St. Joseph, Missouri paid $3.85 million to settle a case involving an infant who developed kernicterus following his discharge from the hospital. Da’Sean Christophel’s parents alleged that the hospital, known at the time as the Heartland Regional Medical Center, discharged their baby prematurely despite evidence he was ill and suffering from hyperbilirubinemia. The hospital and physician named in the case have both denied any wrongdoing.

And in 2011, a California family received a $10 million settlement in a kernicterus case in which a nursing assistant at a hospital, rather than a nurse, allegedly gave the Spanish-speaking family incorrect discharge instructions, which contributed to their child’s signs and symptoms being missed. At the defendant’s request, the names of the hospital and the patient have been kept confidential, according to the lawyers who handled the case.

A review of successful medical malpractices cases involving kernicterus injuries shows a number of common medical errors that often lead to kernicterus. They include: inadequate screening and monitoring of newborn infants’ bilirubin levels; failure to properly assess the baby for signs of toxicity and dismissive attitude toward parents’ concerns; and overreliance on visual inspection for jaundice, as opposed to actual testing of bilirubin levels. Other frequent mistakes involve temporarily stopping phototherapy treatment on a baby with severe jaundice while tending to other medical problems and discounting elevated lab results as erroneous.

Parents of children who suffer from kernicterus also frequently report a reluctance of clinicians to diagnose their children with kernicterus, because it’s usually a red flag for malpractice.

“Every case that is diagnosed, it’s a failure in the medical system, something that the medical system doesn’t want to acknowledge, and it also opens up other members of their profession to a huge lawsuit, because that’s what’s next. When you see kernicterus, the next thing you’re going to see is the legal team involved,” says Haas, who struggled for years to get a correct diagnosis for Lexi. And she’s not alone. Through her involvement in Parents of Infants and Children with Kernicterus (PICK), a parent-run non-profit group dedicated to the education and prevention of kernicterus, Haas says she has met numerous other parents who had to hunt for answers about what was wrong with their children.

Shockingly, it’s also not uncommon for kernicterus sufferers to have their medical records tampered with or changed. One parent said that while kernicterus was listed on their child’s discharge papers from the hospital, the diagnosis disappeared when medical records were subpoenaed. Another says the physician notes regarding her child’s office visits were all typed except for the one visit where she spent the entire time discussing her child’s extreme jaundice with the doctor. That note, when requested as part of a lawsuit, was uncharacteristically handwritten and made no mention at all of jaundice even though it was the focus of the office visit.

Financial costs aside, families coping with kernicterus say that lawsuits appear to be the best catalyst for change in the healthcare arena and preventing more tragedies. One parent says the hospital and healthcare providers who missed her child’s diagnosis have since instituted new protocols. “It’s too late for us, but at least it’s stopping it from happening again. Unfortunately, money talks and unless it’s costing them something, they don’t care.”