There is a gene that is so incredibly rare, it affects just 36 people around the world. This gene, known as the Sonic Hedgehog gene, causes the skull to form in a way that makes people look almost like they have two faces. A little boy in Missouri was born with this gene, which has made his life quite different from other kids his age.
Boy Has “Two Faces” Because He Was Born With Sonic Hedgehog Gene
Tres Johnson from Missouri was born with what’s known as craniofacial duplication. This is a condition in which the skull does not form properly and causes it to appear as though the person almost has two faces. The reason this happens is because of a gene called the Sonic Hedgehog Gene, or the SHH gene. This gene not only deforms the growth of the human skull, but also brings down the person’s life expectancy to just 10 years. Tres, however, has beat the odds. He recently celebrated his 18th birthday. (1)
When Tres was born, he had a cleft, two separate nostrils, and his head had a duplicate shape to it. He suffers from cognitive delays and regular seizures. Because his eyes are so far apart, he sees more peripherally rather than straight ahead. Still, modern medicine has helped Tres greatly. For example, instead of the typical 400 seizures a day, cannabis oil keeps that down to just 40. He also went through surgery to close his cleft and reshape his skull, to the best of the surgeon’s capabilities.
“When he was born he had such a large cleft that it went up into his nasal passage and you could see into his sinus cavity as it was all open.” said his mom Brandy, who takes care of Tres full-time. “His one eye looked like it was bulging out and the other sucked in, because his eyes are further apart he sees peripherally instead.”
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A Fight From The Beginning
Of course, having a baby born with a condition such as the hedgehog gene is not easy. Brandy explains that seeing Tres for the first time was a tough one. His birth wasn’t the same joyous occasion that most are. Rather, she could only touch his leg as he was immediately hooked up to so many monitors. Still, she loved her son just as she would any other baby and so did her husband. They were prepared to do whatever it took to make Tres’ life the best it could possibly be.
“Doctors weren’t going to sustain Tres and planned to let him pass if my husband hadn’t fought for him,” she recalled. “Once I found out he was here and still alive that was all that mattered to us, we were always in it for the long haul.”
Cannabis Saved Him
For more than a decade Tres suffered from an unimaginable number of seizures each day. It had gotten so bad that even the doctors said that they didn’t think there was anything more that they could do for him. It was Brandy who researched and came across studies about cannabis oil. She tried it for the first time in 2017, and within the first week his seizures dropped to less than 40 per day. In two years he had a 90% reduction in seizures.
The reduction in the number of seizures per day wasn’t the only benefit. Both physically and cognitively he has improved. He has put on weight and is getting smarter and stronger than ever before. All of this Brandy credits to the cannabidiol.
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Of course, Brandy and her husband have dealt with many cruel comments about Tres and his condition. Despite his 18 years, he is still at the developmental age of an infant. Finding treatment has been difficult because his condition is so rare. Her biggest goal is to make sure he is alive and comfortable, as well as to find doctors that will treat him as a person and not a case study. She says they’ve been offered “miracle” surgeries that promise to make him look “normal”. His looks, however, are not of big concern to her – his health is more important.
Why Is It Called The Sonic Hedgehog Gene?
There is a pathway that is incredibly important in the development of vertebrates’ faces. This pathway is called the Hedgehog pathway and has three controlling genes: The Sonic Hedgehog (SHH), the Indian Hedgehog (IHH), and the Desert Hedgehog (DHH). The SHH has the most function in the face as well as the rest of the developing embryo. It is crucial for the development of the skull from the formation of the embryo all the way to the fusion of the lip and secondary palate later on in gestation. If these signals are disrupted, it can cause any number of deformations to the face and skull. Doctors still don’t know what causes many of these mutations or disruptions, however, they do know that alcohol, statins, and steroidal alkaloids can cause them. (2)
The SHH gene is also responsible for developing the front part of the brain. It is required to develop both the right and left sides of the brain. It is also involved in the development of the spine and therefore the central nervous system. It is for this reason that people like Tres experience cognitive delays and seizures. (3) There are treatments and surgeries that can be done, but still there is no way to cure or correct for this deformation either inside the womb or after the baby is born.
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