By Saloni Mathur
THIS BRAVE little girl with a one-in-a-million condition had her skull rearranged like a jigsaw puzzle in a $4M treatment program so she could survive.
Josephine “Josie” Grace Owen was born in 2020 in Tennessee, USA, with a rare musculoskeletal condition called Antley Bixler Syndrome (ABS).
To date, only 30 patients worldwide have ever been diagnosed with ABS. The estimated prevalence for the condition is less than one in 1,000,000.
This condition affected how her bones grew in utero. Children with ABS have deformed skull plates, and their facial bones do not grow in proportion, which gives them a characteristic appearance.
In addition, Josephine suffers from Craniosynostosis, which causes the bones in her skull to fuse together too quickly before it was fully formed. And as she grew, her skull became more misshapen over time.
The combination of these two conditions has left Josie very vulnerable, and she needs to be looked after 24/7 by her parents, content creator Anna Grace Own (37), and 42-year-old father Nathan Owen, who is a financial advisor.
“Even before Josie’s birth, we knew there were some issues, but we didn’t know exactly what they were,” said Anna.
“Right after she was born, on May 29th, 2020, Josie was put in a Neonatal Intensive Care Unit (NICU) for 62 days and received three major surgeries to save her life.
“The latest surgery that Josie had was in June 2023 and it was extremely extensive. Her forehead, the frontal bone, was removed, re-arranged and placed back in her head.
“In the x-ray, her skull almost looks like a jigsaw puzzle that has been taped together.
“They put dissolvable screws to keep the forehead together. Eventually, the bone will grow across the empty spots and become mostly one solid piece, for the most part, over the next couple of years.
“The bill we received for this surgery, including hospital stays and medicines, was for $50K (around £40K).
“The medical bills she accrued were so high that if it hadn’t been for state insurance, we would have been bankrupt before we even got her home from the NICU.
“From May 29, when she was born, to December 31, 2020, her medical bills stood at over four million pounds. If it hadn’t been for state insurance covering us, we would have been penniless.”
Despite the hardships, Josie is a happy girl who is friendly and much loved by her family.
“Josie is a very loving, empathetic kid. If any of her siblings get hurt, she goes to them, hugs them, and showers them with her love,” she said.
“She is also a very fun-loving child. She’s so funny and silly and loves to play with her siblings.
“We started learning sign language with her, and now she is able to communicate with us with much more fluency and express her needs and desires.”
Because Josie was born disabled, her family qualifies for state-run health insurance, which covers most of her medical bills.
“Because of the state, we have not paid much out of our pockets,” Anna said.
“We have private insurance, too, but they pay for absolutely nothing.
“We get letters all the time that they have rejected our insurance claims because they say that her surgeries are not medically necessary.”
Children with ABS also have choanal atresia, a condition where one or both nasal passages are blocked by bone or tissue.
In Josie’s case, both of her nasal pathways are blocked, and she needs a tracheostomy tube or artificial trachea inserted in her windpipe all the time to assist in breathing.
Her legs also have restricted movement because of deformed feet, which makes her prone to tumbling and injuring herself.
“We always have to be on high alert with Josie. Because of her fragile skull, even a single tumble where she hits her head could prove fatal,” Anna said.
“She has 15 medical specialists that check on her regularly. They follow her progress pretty closely.
“These include her Otorhinolaryngology – ears, nose, throat doctor. Her plastic surgery and neurosurgery team work together to take stock of her condition.”
Josie is the youngest of four siblings in the household, and everyone does their part to keep her safe and secure all the time.
Because they live in Tennessee, they often have to deal with tornadoes during spring and take shelter in their house.
They have to prep their bathroom, where they generally crowd in with emergency supplies and medical equipment.
“We have a special tornado bag for Josie, with all her essential medical needs, including a brand new artificial trachea, gastro tube, and a mini oxygen canister,” Anna said.
“We have to be extra cautious with that and also even if we leave the house. The second we start to relax about her safety, something goes wrong.
“We also have a battery generator in case of a blackout. We have enough generators around to do what we need to do in an emergency.
“When we go out of town for the vacation, we check to see where the closest children’s hospital is, average emergency response time for paramedics, things like that. So, we have to be overly cautious and think four steps ahead.
“Josie will undergo at least a dozen major surgeries in her lifetime to lead a semblance of a normal life, but I still hope that one day she becomes independent, have kids of her own and enjoy the joys that life has to offer.”
