There is a very good reason to monitor and treat newborn jaundice.
It’s called Kernicterus (KSD).
No one is born with KI. This preventable malady can occur when jaundice (hyperbilirubinemia) is mismanaged in the first days or weeks of life. Athetoid cerebral palsy, auditory neuropathy, cortical visual impairment, dental enamel hypoplasia, and gaze abnormalities are the common neurological sequelae. In 2001, the National Quality Forum issued a “Never Event” ranking kernicterus among 27 medical errors that should never happen.
The word kernicterus comes from the root “kern,” meaning core and “icterus,” meaning yellow. When bilirubin reaches a toxic level it crosses the blood-brain barrier in an infant and literally stains a certain part of the brain (the globus pallidus in the basal ganglia) yellow, causing the irreparable damage known as kernicterus, or KSD, Kernicterus Spectrum Disorder.
The bilirubin level at which this occurs is not a magic number. The amount of bilirubin that is safe in an infant is determined by physicians based upon the infant’s gestational age, general health, and risk factors, thus we strongly advocate for screening of newborns to best identify those at risk.
With proper testing, screening and treatment kernicterus is a preventable malady.
Individuals living with kernicterus are affected to varying degrees. Some live with mild hearing loss, behavioral challenges, and/or clumsiness while other might be mistaken for someone with spastic quadriplegia. Kernicterus is a spectrum.
“My doctor acted as if jaundice was a common cold, a little nuisance that couldn’t harm my baby. What he didn’t tell me nearly killed my son. Now my bright and happy boy, this same boy, rides in a wheelchair and cannot speak to us or control his constant movements. I tell all moms to be sure to get their babies tested and charted. Preventing KI isn’t hard or costly, but living with it is.”
An otherwise healthy infant that suffers this injury can suddenly have an altered future, a future that can include impairments to vision, hearing, movement, digestion…essentially every area of life – the ability to interact with others and care for self.
For lack of one blood test, a child’s future can change.
Jaundice can do that.
Kernicterus can include all or some of the following (remember, it’s a spectrum):
- MOVEMENT DISORDERS
- Athetoid Cerebral Palsy
- Dystonia
- Myoclonus that impairs ability to sleep
- (Vestibular Instability a BIG issue that seems to worsen these movement disorders for our children. This at it’s worst during the first year, as many infants with kernicterus constantly experience the falling sensation, flinging arms out and crying constantly when not being held.)
- SEIZURES
- These sometimes are only in the neonatal period, although with some they re-emerge later in life.
- VISUAL IMPAIRMENT (these can improve over time)
- Gaze Abnormalities
- Nystagmus
- Strabismus
- Cortical Visual Impairment
- DIGESTIVE IMPAIRMENT
- GERD – reflux
- Impaired Digestion
- Impaired ability to swallow or eat orally
- DENTAL
- Dental Enamel Dysplasia or Hypoplasia
- HEARING IMPAIRMENT
- Auditory Neuropathy Spectrum Disorder (ANSD)
- This is a form of hearing loss which is difficult to detect and can often only be diagnosed by an experienced audiologist using a sedated hearing test called an ABR
- Given the physical impairments and the undetected hearing loss, in the past kernicterus was associated with MR. Those that cannot communicate and hear are easily misunderstood. We now know that it is NOT associated with any degree of cognitive impairment (mental disability).
- Auditory Neuropathy Spectrum Disorder (ANSD)
For further information on treating kernicterus, read here.
IF NEWBORN JAUNDICE IS LEFT UNTREATED IT CAN CAUSE KERNICTERUS.
Help spread the word. Make sure every child gets a TEST! Make sure your hospital and doctors educate parents with these materials: http://www.cdc.gov/ncbddd/jaundice/freematerials.html .
Has your child been diagnosed? GET ON THE MAP.
Awareness is the precursor to change!!!
https://www.diseasemaps.org/en/kernicterus/api/7c2ca204f153d626/
Do you have a child who has been diagnosed with kernicterus? Or do you yourself have kernicterus? Stand up and be counted. http://www.diseasemaps.org/en/kernicterus/Also, be sure to join our family group: Affected by Kernicterus.
HOLA,SOY DE COLOMBIA TENGO UNA HIJA DE 8 AÑOS QUE TUVO KERNICTERUS,Y AHORA ESTA TENIENDO MUCHOS PROBLEMAS EN EL COLEGIO TANTO ASI QUE LA RECTORA YA ME DIJO QUE NO LA RECIBIRIA MAS EL OTRO AÑO,Y ME SIENTO MAL POR ELLO, PUES PIENSO QUE NO DEBERIA HABER SEGREGACION,NECESITO ALGUN TIPO DE AYUDA QUE ME DIGAN QUE PUEDO HACER,GRACIAS
Janeli, muy apesadumbrado de oír hablar de los problemas de su daugher en la escuela. Por favor únase a nuestro grupo de habla español para hablar con otras personas pasando por pruebas similares. – Miriam (a google traductor)
https://www.facebook.com/groups/afectadosKI/
Janeli, Very sorry to hear about your daugher’s problems in school. Please join our spanish speaking group to speak with others going through similar trials. – miriam (via google translator)
I believe this is what happened to my son at 3wks of age. He was jaundice and it went unchecked. They checked it 1time at 10 days old. When he was 3wks he was still jaundice and had a bili of 7.7 and was still pretty yellow. We found him blue and not breathing in the crib. He suffered a near miss SIDS. Would love more info to help our Dr get a better diagnosis. Charles is 16 mos. And yes the delays show up much later. He doesn’t sit up or feed himself. He is visually impaired, has some e hearing loss. He has bowel trouble and digestion problems. He is also the light of my life and lights up a room when he enters. Its his magic personality. 🙂 thanks for your time
Your son sounds like one wonderful little guy! Have you joined our parent group? I highly encourage you to post your questions there and maybe find some resources in your area to help figure out your son’s diagnosis. Our group is here: https://www.facebook.com/groups/Kernicterus
My sincere apologies for the delayed response!
Teresa, have you connected with us in our facebook group? http://www.facebook.com/groups/kernicterus
Many families travel to see experts to get evaluated for a diagnosis. One such expert is Dr. Steven Shapiro in Kansas City.
Dear Miriam,
This is good to learn from each other experience on facebook. My 5 months old baby has KI.
Hi there,
If you have not already, please join our parents group: https://www.facebook.com/groups/Kernicterus
Very sorry to hear that you baby also has kerniceterus, and my apologies for the delay in seeing and responding to this!
Miriam
hi how was your child developing? my daughter also has KI and she is 2months old now,just finished ABR and it was normal..please give me some info thanks
Hi, I wonder if anyone could help me with information. My daughter is 8 weeks old. She was treated for jaundice with phototherapy at 3 days old. She was born 3 weeks early. We just found out that she has mild hearing loss in one ear. That’s how I started researching whether her jaundice caused it. I’m very concerned if she is going to develop other things like cerebral palsy or the condition of upward gaze, etc. When do these conditions develop? Or would I be able to notice them already? Thank you in advance.
Hi there,
Please refer to our response on facebook. Also, be sure to address your concerns with your doc and pursue early childhood intervention with your state’s early intervention program for therapies etc. I would also look at your child’s bilirubin levels, risk factors and the nomogram. Once you have this info it would be good to talk it over with your child’s doctor, and a neurologist if there is cause for concern.
It really is a great thing that you had testing and treatment. So many of our kids with kernicterus were not so lucky. Also it’s important to determine the type of hearing loss when determining the cause. Auditory neuropathy is a less common and neurological form of hearing loss that can come from kernicterus and can only be found with a specific hearing test called an ABR.
At lower levels, bilirubin is actually an antioxidant
My son who is now 3 was born at 36 weeks and was a low birth weight, within 72 hrs of birth was in a special care baby unit in an incubator unit under quadruple phototherapy and a billi blanket, his levels reached almost 300. He now has speech and communication difficulties and has issues with his teeth. Here in the UK won’t diagnose Ki unless you have cerable palsy, they don’t recognise the milder effects. We started to notice the effects at around 6 months old, but her did Sutter from reflux which is a symptom, at 2 months old. Try and enjoy your little one x
Dear Miriam, thanks once again for your replies. As it’s all new to me, I will look not all the points you’ve mentioned. We live in the UK but I’m sure jaundice testing/treatment is similar to the US. Kind regards, Nat
hi my daughter also had KI and put on early intervention she is 2months old now and just finished the ABR test and it came up perfectly normal,kindly email me to have an exchange knowltdge about KI thanks
Following up. April, did you get our email?
no i have’nt recieve any emails
Hi April! I’m sorry about the mix up!!! The best way to get info is within our facebook group http://www.facebook.com/groups/kernicterus. No matter the hour there are always families awake and willing to listen and help brainstorm treatment and “where do we go from here” kind of stuff.
My son is now 5 years old and is showing many signs associated with bilirubin induced neurological dysfunction. He has low muscle tone, clumsy, teachers convinced he has a hearing loss but tests show normal hearing, speech and language impairment, lack of understanding or signs of processing disorder, attention problems, difficultly potty training number 2. As a newborn 1 day old he was jaundice and treated with light therapy and sent home. At his 3 day visit he was at 22 my/dl. His pediatrician did not present or treat this as an emergency case. Based on my research it should have been. They let me breastfeed without oral feeding, no transfusion. I am still unsure of the exact measures as I am waiting for his medical records. However, he has never been diagnosed for BIND because his pediatrician never referred to a specialist. Now with a new doctor he was referred to a GI pediatrician and a developmental pediatrician, but the wait for developmental is 1 1/2 years. This is along time to not have answers or the correct tests to officially diagnose him so he properly treated and educational plan developed. I feel that there was a significant neglect in his initial care and follow up thereafter. I recently discovered this doing my own research, but very upset not being able to do anything about it for over a year. Can anyone give me some advice on how to proceed to get him care.
Oh Carrie! So sorry to hear all that has gone on!!!! Let’s get you connected to some families in your area that may have additional info I navigating the system. So join our main group www. Facebook.com/groups/Kernicterus and if you happen to be in the UK or South America, let me know and We can get you in those too.
Thanks Miriam! I put in the request for Facebook and looking forward to connecting with other families. Hopefully, there are a few in my area.
Hi my son has juandice.at birth which reaches at 36mg he got blood transfusion now he is two and half he walks he eats by himself he says mama Baba only but doesn’t response when I ask him to clap or ,point your nose etc it seems that he copies me but doesn’t understand my words will he able to speak ? Will he move forward in life
Hi Bush,
These are tough questions and ones really best brought into our group where you can see a variety of experiences.
You can find is at facebook.com/groups/kernicterus
Hi my son has jaundice of 35mg at eight day of birth he got blood transfusion now he is two and half he walks plays responds to his name but doesn’t say anything except mama Baba he doesn’t respond when I ask to clap or such other things will his mind work does he live normal life
Bush, we’d love to have you over in our facebook Group. It’s a tremendous resource for our families mapping out their course with this rare disease where few outsiders understand. http://www.facebook.com/groups/kernicterus
All the best to your family!
Miriam