It sounds like something out of a Grimm’s Brother’s fairy tale; a girl with locks of spun gold. Yet to Lyla Grace Barlow and her family, it is anything but. Lyla Grace is one of an estimated 100 individuals living with Uncombable Hair Syndrome, and to the five-year-old, the touch of a comb through her unruly locks means agony. Here is a look at the strange and rare medical condition and how it affects the little girl’s life.
Lyla and Spun Glass Hair
Uncombable hair syndrome, otherwise known as spun glass hair, or unmanageable hair syndrome. is a condition caused by a mutation that results in heart shaped hair follicles rather than the normal round shape. Lyla’s mom Alex says, ” It’s so hard to get a brush through it and she cries when I try. But we (Alex and Dad, Mark) tell her to embrace her hair because it’s so rare and beautiful.”
Scientists in Germany were able to confirm Lyla’s diagnosis. After analyzing a sample of her DNA, they were able to determine that both parents, Alex and Mark passed a mutated gene, PAD13, onto their daughter, causing this condition. Lyla’s siblings, Emilia, seven and Nancy Rose, two, were not affected. Dr. Andrew Messenger, consultant dermatologist at Royal Hallamshire Hospital in Sheffield reports only seeing three such cases in his thirty-year career and says there is no medical treatment. He describes the condition. “The hair tends to stand out from the scalp. It is usually blond and may have a spangly appearance.”
Uncombable hair syndrome is characterized by kinky, dry hair that cannot be combed flat. The condition usually presents itself in a child between infancy and the age of 3 but can appear in children as old as 12. Children with the disorder have light colored hair, often referred to as blond, or even silvery with a glowing sheen. The hair grows out from the scalp rather than downward. However, despite its appearance, the hair is not brittle, and grows at a normal or slightly slower pace than in those without the condition; the syndrome affects only the hair at the scalp.
For reasons not yet known, the syndrome usually improves over time, and by adolescence, individuals tend to have hair of a normal or close to normal texture.
Uncombable hair syndrome is caused by mutations in the PAD13, TGM3, or TCHH genes, all of which give instructions for the manufacturing of proteins that give the hair shaft structure. These mutations cause the proteins to be produced with little of no activity. As a result, the hair shaft has a heart like or flat cross section, as opposed to the usual cylindrical shape, preventing the hair from lying flat. Hair will also reflect light differently, accounting for its other worldly sheen.
Professor Regina Betz, of the Institute of Human Genetics in Bonn, has been researching the conditions for four years. She says, “Usually children have dry and frizzy hair, and some parents say that it’s uncombable. Today there are 100 cases around the world but there may be more which have not been reported.”
What do you think of Lyla’s locks? A blessing or a curse? Let us know what you think of the girl with spun glass hair.