a Rearranged Life

Before you read this, please understand that I write this from my experience as a parent of a child with Kernicterus. I have no medical training or experience.  This is what I have learned from speaking with other parents, and logging hours in doctors offices.  This is not medical advice, nor should it be taken as such.  You can read my disclaimer for more info.

Kernicterus is the word that rocked our world, BIG TIME.

It’s the reason behind extended hospital stays and bili lights for newborns here in the US, and I really wish I had known what was at risk!

For a more detailed breakdown:

Kernicterus, originates from:

kern – seed (German)

icterus – yellow (Greek) 

It occurs when jaundice (bilirubin levels) becomes extreme and bilirubin crosses the blood-brain barrier and literally stains a very specific area of the brain yellow (it’s the globus pallidus in the basal ganglia), causing damage in almost the same area that Parkinson’s effects. Kernicterus is characterized by:

• Athetoid Cerebral Palsy
• Dystonia
• Auditory Neuropathy/Dysynchrony
• Dental enamel hypoplasia or dysplasia
• Feeding difficulties
• Speech impairments
• Nystagmus -this resolved for my son
• Strabismus -the one thing my son did not have
• Cortical Visual Impairment – not as well documented, but it seems to be a common issue among kids in our support group

Children with Kernicterus are affected to varying degrees and can have some or all of the above listed conditions.  It is difficult to test intelligence in more severe cases, where the hearing and motor issues prevent communication and was years ago thought to be associated with MR, but it is no longer thought to be so.  Those with Kernicterus are said to have normal intelligence.*

IT IS IMPORTANT TO REMEMBER THAT KERNICTERUS CAN BE PREVENTED IF JAUNDICE IS PROPERLY TESTED AND TREATED.

VISUAL INSPECTION IS NEVER SUFFICIENT,

A SIMPLE BLOOD TEST OR USE OF A BILIMETER, SOME BILIRUBIN LIGHTS, OR A PROLONGED STAY IN THE HOSPITAL CAN SAVE A CHILD FROM A LIFETIME OF DISABILITY!

Furthermore, it also important to note that there is no magically safe number when it comes to bilirubin levels. Gestational age, overall health of the newborn, and risk factors are key in determining when to intervene with phototherapy, and in some cases, blood transfusions.

Lasting damage from Jaundice is rare, it isn’t as rare as you would think.

We have new members coming into our support group across the US on a monthly basis, often several per month. While every baby’s story is different there is one thing in common, a casual attitude among practitioners about newborn jaundice. This is considered to be rare, in part, because it is 100% preventable…there is the blood test to determine exact bilirubin levels, photo-therapy to help little livers process and eliminate bilirubin and, in extreme cases, blood transfusions.

It is also important to note that “visual inspection is never sufficient” according to the AAP, this is directed to health professionals that look at babies all day! Everyone told us that B was fine…doctors and nurses, lactation consultants (multiple times)…but they DIDN’T know…and we thought pink and red were good…but they were early indicators of jaundice. These professionals didn’t know that B’s bili was high, because they relied upon their eyes…the only way to really know is to do a blood test!

Not all children affected by Kernicterus are affected as profoundly as B.  I would guess that the majority have much milder symptoms, although still life-altering.

You also may not be aware of all of the risk factors that a child has (as not all of them can be seen). Many risk factors are listed in the CDC’s literature.  I highly recommed that you refer to it, if you have ANY quesitons about Newborn Jaundice or Kernicterus.This information could save a child from a lifetime of disability! If your baby ever had to be kept in the hospital or put under lights, it may have seemed to be a nuisance, but you really should be thankful!

Dr. Bhutani of Stanford (a world-renown neonatologist) developed the Bhutani Nomogram, a graph that helps access a child’s risk of developing severe Hyperbilirubinemia (Jaundice). This is what Pediatricians, Neonatologists, and nurses refer to around the globe.  Additional tools and info can be referred to here. It’s always a good idea to ask the physician caring for your newborn to show you where you’re child plots on the graph and discuss follow-up and treatment options, if appropriate.

The American Academy of Pediatrics and JACHO (the national organization that regulates Hospitals) have each issued multiple alerts over the past few years, and yet new cases of Kernicterus are emerging every month. Please be a part of making sure that this catastrophic and 100% preventable tragedy is not allowed to keep happening.

Spread the word!  Support PICK!

Here are some other great links:

PICK’s VERY INFORMATIVE VIDEO on Kernicterus

PICK’s parent support email group. Really helpful for affected families needing additional information.

PICK’s facebook page (another great place to go..especially to see/share pictures, stories, and videos)

*

1. http://thehearingblog.com/wp-content/uploads/Thursday-Shapiro-Hyperbilirubinemia.pdf
2. http://books.google.com/books?id=h5NMVkpAgVwC&pg=PA231&lpg=PA231&dq=kernicterus+normal+to+superior&source=bl&ots=tBUq7Gh7Ap&sig=9d4BkSRHCH67rcYjf9bIHDd-fjI&hl=en&sa=X&ei=dUW4T7fMCoTJ6gGU6PXLCg&ved=0CHwQ6AEwCA#v=onepage&q=kernicterus%20normal%20to%20superior&f=false

3. In the “athetoid” form of cerebral palsy due to kernicterus, the abnormal muscle tone does not usually lead to fixed postures and contractions, and the sparing of the cortex and subcortical white matter tracts usually results in normal intelligence, however, there may be specific learning disorders and abnormal sensory function or sensorimotor integration. In the most severe cases, individuals may appear to have severe mental retardation but in fact have normal or superior intelligence but are trapped in immobile, dysfunctional bodies and cannot voluntarily move, hear, sign, type or communicate effectively. -Journal of Perinatology