Keaton “Jax” Koehler
On April 12, 2011, Kory and Erika Koehler welcomed their first and only child to this world. Keaton Jax Koehler was born 7lbs, 4.8 oz. After spending the first night in the nursery, Keaton joined mom and dad in mom’s recovery room. After spending 4 or 5 wonderful hours with family and friends, Keaton began to show complications. He would stop breathing on multiple occasions. The doctors at San Jacinto Methodist had no answers after having performed several tests including spinal taps and blood work. Keaton was transferred to Texas Children’s Hospital where he was under the direct care of Dr. Jen Arnold of TLC’s The Little Couple.
After extensive brain MRI’s, it was preliminarily determined that Keaton was born with several brain abnormalities. Keaton was missing several key brain anatomy all together and some failed to form completely. No corpus callosum, small cerebellum, very small brain stem, lack of white matter, among many others. Keaton was not given any chance to survive more than an hour once we decided to remove the artificial ventilator. Each time they tried, he would stop breathing. Kory and Erika spent 13 days in the TCH NICU and decided that letting their son pass away and return to our home in the heavens was the right thing to do.
Kory and Erika issued a Do Not Resuscitate order and as family arrived, the ventilator was removed. Keaton spent the day with family and defied the 2 hour odds. While daddy was holding him in his room 7-8 hours later, Keaton finally began to stop breathing. A nurse, against the DNR order, stimulated Keaton. She had a look of shock as to what she just realized she had done. That would be the last time Keaton would struggle for air.
After months of the unknown and several tests, a genetics team found, what they believe, is a diagnosis. Desmosterolosis. A defect in the cholesterol biosynthesis. A rare genetic disorder, so rare that Keaton is only the 4th human being in the history of medical science to be born with it. Even more rare, the other three children showed various bodily mutations including clubbed feet, both genitalia, cleft palate, large foreheads, off set eyes and only one lived more than 24 hours after birth. Keaton has no visible mutations. All of Keaton’s mutations are in his brain.
Keaton is now 2 years old and a very happy child. He has slept through the night since the day mom and dad were able to bring him home. He loves his Paw Paw and Sweetie, music, animals, and wind. He does not crawl, sit up or talk, but he laughs and loves to play from his back. Keaton has several Early Childhood Interventions such as Physical and Occupational Therapy twice a week, vision therapy for cortical visual impairment, and speech therapy. Keaton, until more miracles happen, may never be able to sit, walk or talk on his own, but he has a tremendous family network who love him very much. Keaton is the sweetest child you’ll ever meet. Not one person who has ever heard his story or spent a minute with him, walks away untouched. He is an angel. He shouldn’t be here by all accounts, but our faith in The Lord and his plan has given us two amazing years and many more foreseeable years to come.