Bilirubin-induced neurologic dysfunction (BIND) or Bilirubin encephalopathy is a rare neurological condition that occurs in some newborns with severe jaundice. BIND may also result from a genetic disorder known as Crigler–Najjar Syndrome (CNS) which is a life-threatening disorder is caused by the mutation in UGT1A1.
In order to understand what is a Bilirubin encephalopathy or BIND let us first under what is bilirubin?
Bilirubin is a yellow pigment that is created when the body gets rid of old red blood cells (RBC). Increase in the levels of bilirubin in the body can cause the skin to look yellow (jaundice). It can be used to diagnose liver and blood diseases and has a complicated metabolism, which is significant in relation to various drug metabolism pathways.
What happens if bilirubin levels cross the laboratory reference range?
When bilirubin levels in plasma or serum crosses the laboratory reference range, it is diagnosed as hyperbilirubinemia, which can be further categorized as conjugated or unconjugated hyperbilirubinemia (UHB). Clinical jaundice that affects about 85% of newborns in their first week of postnatal life, is a commonly occurring, transitional condition caused by neonatal UHB.
Studies show a mildly elevated bilirubin concentrations has a protective antioxidant-like effect on the body while can neutralize reactive oxygen species (ROS), prevent oxidative damage, necessary for newborns.
However, high levels of UCB can pose serious threats, such as severe brain injury, with the possibility of progressive chronic bilirubin encephalopathy or kernicterus ( kernicterus refers to the yellow staining caused by bilirubin) in 1 in every 100,000 cases. If not treated immediately it develops into kernicterus, almost 70% of newborns die within the week and the other 30%, suffer irreversible brain damage.
The signs and symptoms BIND:
The symptoms differs in babies and not all babies with kernicterus have had definite symptoms. It also depend on the stage of BIND. The symptoms are divided into 3 stages: Early stage:, Middle stage and Late stage.
Early stage: In this stage we notice, Extreme jaundice, Poor feeding or sucking, Absent startle reflex, Hypotonia (low muscle tone) and Lethargy (extreme sleepiness).
Middle stage: In this stage we notice, Poor feeding, Irritability, High-pitched cry, Hypertonia (high muscle tone), the baby may also have a neck hyperextention and arched back
Late stage: In this stage we notice, No feeding, Shrill cry, Muscle rigidity, Stupor or coma, Seizures, neck hyperextention and arched back.
BIND occurs when the accumulated UCB in serum cross the blood–brain barrier(BBB), proceeding to deposit in the basal ganglia or cerebellum, therefore, the various clinical indications are related to the respective targets in the brain.
Evidence from observational studies shows a link between the BIND and NDD. The BIND spectrum may encompass multiple overlapping and comorbid neurodevelopmental disorders (NDDs), including attention deficit hyperactivity disorder (ADHD), autism spectrum disorder (ASD), cognitive delay, specific learning disorder (SLD), and/or language disorder (LD).
Investigations and Test includes:
A blood test: it shows an increase in bilirubin level.
BIND score:
The BIND score is a tool used to assess the severity of acute bilirubin encephalopathy (ABE) in newborns with hyperbilirubinemia. It is a scale from 0 to 9 that assesses a newborn's mental state, muscle tone, and crying patterns. A score of 0 indicates a normal newborn, while scores of 1–3 indicates mild, 4–6 indicates moderate, and 7–9 indicates severe ABE.
Clinical significance of BIND Score: Mild ABE is usually reversible, while moderate ABE is potentially reversible, and severe ABE can lead to long-term disability or death.
A modified BIND score, called BIND-M, has been developed to help differentiate bilirubin encephalopathy from other conditions with similar neurological findings. The BIND-M score includes eye characteristics such as divergent gaze, paralysis of upward gaze, anxious appearance, and nystagmus.
Treatment for Bilirubin Encephalopathy :
Treatment depends on how old the baby is (in hours) and if the baby is at risk factors (such as prematurity). It may include: Exchange transfusions (replacing the child's blood with fresh donor blood or plasma), Phototherapy or Light therapy.
Prognosis:
BIND a serious condition which may not only be temporarily disabling but also permanent, and it is usually accompanied by movement disorders as well as hearing loss. Many infants with late-stage nervous system complications die.
Possible Complications: Hearing loss, Permanent brain damage in some cases Death.
Prevention from Bilirubin Encephalopathy :
All newborns have to follow-up for appointment within 2 to 3 days after leaving the hospital. It is very important for early term or late pretermbabies (more than 2 to 3 weeks before their due date). Treating jaundice that may lead to BIND can help prevent it. Infants with the first signs of jaundice have bilirubin level measured within 24 hours. If the level is high, the infant should be screened for diseases that involve the destruction of red blood cells (hemolysis).
How can Cura Advanced Physiotherapy Centers and Home Care Services can Help?
As discussed earlier that BIND spectrum may encompass multiple neurodevelopmental disorders (NDDs), such as Autism, ADHD, Language disorder, Movement disorder, etc. Cura Advanced Physiotherapy Centers provides a long term care which includes multi-disciplinary rehabilitation involving physiotherapy, speech therapy, auditory interventions, occupational, and pharmacological therapy. Cura Advanced Physiotherapy Centers uses the Gross Motor Function Measure (GMFM) a standardized observational instrument and Bayley Scale of Infant and Toddler Development used to assess the gross motor activities such as lying, sitting, crawling, standing, etc and also Cognitive, Expressive Language, etc respectively.