A Family’s Struggle Against Time
Donald Francis, affectionately called Bubba by his family, has been fighting a rare genetic disorder that is steadily destroying his body and mind.
Bubba lives in a small home in the rural countryside of Meigs County, Ohio. He is the youngest child of a family that includes two sisters and two half-sisters. Until the age of 8, Bubba had what many would consider a typical childhood, but that is when his parents starting to notice something was amiss.
“The doctor was really blunt out in front of Bub, told him he had a mutation and a mitochondrial disorder and that it was a terminal disease,” said Bubba’s mother, Michele Barley. But he didn’t use such nice words. He said it was deadly, that he would die early. So driving home from Columbus, Bubba says, ‘Am I a mutant?’…this is before, when he could really speak and when he could understand…and I told him, ‘no,’ and he said, ‘I don’t want to die.’ I stopped the car. We sat there for a good half hour, 45 minutes until we were calm again and drove on home.”
But Bubba’s initial diagnosis was incorrect and he was eventually diagnosed with a more aggressive condition: Niemann-Pick Type-C, or NPC.
NPC is a progressive neurological disorder that degrades a patient’s brain functions and muscular movement, including muscular movement required for some organs to function properly. The disorder causes things like: cerebral palsy, slurred speech, difficulty swallowing, epilepsy, altered sleep cycles, limited muscle movement (including moving eyes), psychosis, dementia, seizures, and bipolar disorder. The genetic disorder affects approximately every 1:150,000 people. Patients around Bubba’s age usually only live into their early twenties.
As NPC slowly degrades Bubba’s mind and body his family does what they can to fight back.
Donald Francis, nicknamed Bubba, right, suffers from Niemann–Pick Type C (NPC). NPC is a progressive genetic neurological disorder that degrades a patient’s brain functions and muscular movements required by some organs to function properly. Patients usually only live into their early twenties. Bubba's family prepares his cake as he waits to blow out the candles during his 19th birthday celebration held at his home in rural Meigs County, Ohio.
Symptoms of Bubba's muscular failure began presenting themselves when he was 8 years old. He ran strangely, he couldn’t catch a football, and missed targets more and more with his BB gun. His mother, Michele Barley, takes Bubba’s temperature after his heart rate dropped suddenly. He was given oxygen to help regulate his heart rate.
As Bubba grew older, other symptoms began presenting themselves. This began a cycle of visits and tests at the hospital every three months until he was diagnosed at the age of 14. Bubba brushes his teeth in his bedroom since he cannot stand long enough by himself to use the sink.
His brain function is deteriorating and is one of the many symptoms of his disease. Bubba currently operates at a 3 to 4-year-old level of comprehension. To keep his mind active, his mother and nurses have him play board games at least once a day. Nurse Barbie Williams, right, pulls out a board game to play with Bubba and his stepsister Leah Barley.
Around the age of 12, Bubba began falling in love with Bobaflex, a hard-rock band from Point Pleasant, West Virginia. “He got something out of it," said his mother. "You could just feel it, he was really getting into the groove of this music and it just touched him somewhere. It was something that we didn’t see very often. We’d never see him enjoy something. He was usually in pain, but when he heard Bobaflex he was happy, danced and sang, just like a normal healthy teenage boy.”
In August, Bubba’s primary caretaker and stepfather, Dwayne “Duey” Barley, was incarcerated at the Southeastern Probation Treatment Alternative (SEPTA) Correctional Facility in Nelsonville, Ohio, for breaking his probation by crossing state lines. “It’s an adjustment, how Duey’s been gone for three months,” said Michele. A photograph of Duey taken during his time in the U.S. Army sits in the family’s laundry room.
Approximately two years ago, Bubba's doctors determined he could no longer eat by mouth as a result of the muscles in his throat weakening. Bubba now receives three meals a day, formula, water, and medications through a feeding tube connected directly into his stomach.
"I like to think he’s still in there, because he loves to laugh,” said his mom. Michele kisses her son on the cheek after trying to tie his beard with a hair tie. Bubba has steadily lost the ability to talk since last year due to a combination of neurological and muscular malfunctions, and soon he will be unable to say anything.
Michele helps Bubba into the car to go visit his stepfather, Duey, at the SEPTA Correctional Facility while she handles Emily, their pet and service dog.
Residents at the correctional facility are allowed visits from family once a week for 30 minutes. A security guard enters the room to end the visitation. Duey was released from the correctional facility in February of 2016. Caring for Bubba has created a close relationship between Duey and his stepson. A relationship that doesn’t have much time left, making every day precious.“I’ve taken five months of my child’s life…I’ve robbed myself of that,” said Duey.
Michele checks Bubba's eyes for signs of a seizure while they sit with their extended family for Thanksgiving dinner. Duey, bottom left, was released for eight hours to spend the holiday with his family and stepson before having to return to the correctional facility.
Over the course of the past year, Bubba has gone from speaking regularly to saying only a few words a day. Bubba struggles to read words from a magazine while his mother helps him sound out the words.
After their Thanksgiving dinner, family gathers on the front porch. "Just to know, that if this was his last Thanksgiving, that at least he could share it with the people he loves and people who love him," said his sister Tiffany Francis, left. "It’s worth more to me than anything."