Blue light helps Amish children
Monday, 27 June, 2016
Outside, from far across the fields, a strange blue light beckons in the dark. Where horse-drawn buggies clatter along dusty country roads and many families shun electricity, blue light cuts harshly through the night.
Crigler-Najjar syndrome occurs when the enzyme that normally converts bilirubin into a form that can easily be removed from the body does not work correctly. Without this enzyme, bilirubin can build up in the body and lead to jaundice (yellow discoloration of skin and eyes) and damage to the brain, muscles, and nerves.
Crigler-Najjar (type 1) is the form of the disease that starts early in life. Arias syndrome (type 2) starts later in life.
In Lancaster County, the first Amish arrived from Switzerland between 1737 and 1767. The insular communities presented biological risks that only worsened over time as the population expanded but its genetic profile didn't.
Currently, more than 50,000 Lancaster County Amish can trace their lineage to just 80 ancestors. This causes a 'founder effect' that exists at the unlikely intersection of random chance and deliberate community planning: The result of generations of intermarriage, genetic drift and the biological bottleneck those factors have spawned.
The scene is bathed in the glow of a single gas lamp. Upstairs, a baby sleeps in another kind of light, in a very different world.
High-intensity blue electric rays burn down upon his crib, creating an iridescent haze that envelops the room. The lights are suspended from a heavy stainless steel canopy just inches above the child.
The baby wears only a diaper and has no blankets, just starched white sheets. Mirrors are built into one side of the crib. Fans hum loudly to keep him cool.
With his chubby cheeks and bleached blonde hair, 15-month old Bryan Martin looks like an angel in his luminous cocoon.
But Bryan is a very sick child. The whites of his eyes are yellow and his skin is an unnatural gold.
The blue lights are saving his life.
Light treatment (phototherapy) is needed throughout a person's life. In infants, this is done using bilirubin lights (bili or 'blue' lights). Phototherapy does not work as well after age 4, because thickened skin blocks the light.
A liver transplant can be done in some people with type 1 disease.
Blood transfusions may help control the amount of bilirubin in blood. Calcium compounds are sometimes used to remove bilirubin in the gut. The drug phenobarbitol is sometimes used to treat Arias syndrome (type 2).
In the past children diagnosed with the disease would not survive. In the 1970's doctors realized that the wavelength and energy of blue light changes the nature of the bilirubin, allowing it to be excreted from the body.
References
Crigler-Najjar Syndrome. Medline Plus.
Blue light aids sick Amish, Mennonite children. Fox News. 2007.
Crigler-Najjar Syndrome. NY times Health Guide. 2016.
Genetic disease is ravaging Lancaster County's Amish. PennState.
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