NASHVILLE, TN — After watching her son’s health slowly deteriorate over the past eight years, LaKesha Welch is making the difficult decision to disconnect Darian Jones from life support today on his 10th birthday.
Welch’s journey has been long and the choice wasn’t easy, but Darian, who was diagnosed with a rare and fatal cholesterol disease called Niemann Pick Disease Type C, came to his mother in a dream and told her it was time to let him go.
“I never thought I would do what I am about to do,” Welch said, days before Darian’s birthday. “I don’t want to be selfish and he’s tired. We decided that on his birthday, May 30th, that’s when we are going to do it.”
Darian was a healthy, sweet, and active child who could walk, talk, and spell his name. He laughed while watching his favorite show, Mickey Mouse Clubhouse, and his favorite food was pancake on a stick. But that all changed at the age of two when Darian lost his ability to walk and began to show signs of neurological decline. Welch worked closely with Darian’s pediatrician, Dr. Susanne Tropez-Sims at Meharry Medical College in Nashville, Tenn., to find out what was happening to her son.
“At that time it was a puzzle and no one knew,” Welch said. “They told me he would die and I would never know why.”
Over the course of several years, Dr. Tropez-Sims conducted every test imaginable on Darian, but still there was no answer that explained his condition. It wasn’t until she referred him to the National Institutes of Health (NIH) Undiagnosed Diseases Program (UDP) that questions were answered. The program is for patients with longstanding medical conditions that elude diagnosis by the referring physician. It is designed to provide answers to patients with mysterious conditions while advancing medical knowledge of rare and common diseases.
The NIH began testing Darian in 2012. Welch called consistently for updates, but years passed before she had her answer. In 2015, Darian was diagnosed with Niemann Pick Disease Type C; a disease that he’d previously tested negatively for, but now because of a new testing method used by the NIH, a successful diagnosis was possible.
“He’s not the first person to have it, but he’s the first person to find out through this test,” Welch said.
According to Dr. Tropez-Sims, Niemann Pick is a genetic mutation caused by a defect on the Niemann Pick gene. There are four types (A, B, C, and D); and the type determines the symptoms and what stage of life the disease will manifest. Type A shows up early in life and people with Niemann Pick Type A typically have a cherry red spot in one of their eyes. Type B shows up later in childhood or even as late as teenage years, and is characterized by problems with respiratory infections. Both Type A and B can include swelling of the abdomen. Type C and D can occur as early as infancy or as late as adulthood. Patients like Darian who have Type C or D experience symptoms such as an enlarged spleen and liver, seizures, difficulty moving, learning problems, intellectual decline, loss of muscle tone, inability to walk, and slurred speech.
Darian is one of only about 500 people in the world who have Niemann Pick Disease Type C, according to the Addi & Cassi Fund. According to Dr. Tropez-Sims, both of Darian’s parents are carriers, but because the disease is so rare, children and parents are not tested for the mutation unless the child begins to show symptoms. Every person carries the Niemann Pick gene, but it will only cause the symptoms seen in Niemann Pick Disease if there is a mutation.
Throughout the years, Welch has tried to keep Darian’s life as normal as possible. She has taken him roller skating in his wheelchair; she has taken him on a go-cart ride; and thanks to The Dream Factory, a wish-granting organization, Darian was able to visit Walt Disney World.
But the trip to Disney World marked the last time Darian smiled. In 2011, he took a turn for the worse. He stopped blinking his eyes and he was unable to show any type of emotion. Today his condition is grave. Darian is being kept alive by machines and has been in hospice care for several weeks.
Welch said Darian has appeared to her in dreams that were so realistic that she awoke in tears.
When he spoke to Welch in the dream Darian said, “‘I’m just tired. I’m tired of this momma. I’m tired of being like this. I know it’s school time and I can’t even go to school. Why me?’” Welch said as she described her son’s voice in the dream.
The dream continued with Welch crying while trying to change Darian’s diaper as he continued talking to her and said, “Don’t cry. It’s not your fault. You’ve taken good care of me. If it wasn’t for you I probably wouldn’t even be here now.’”
Welch said her journey hasn’t been easy, but she knew she had to fight for her child.
“Sometimes I would cry, and he would cry because I was crying. It was hard to watch because he’s going backward,” Welch said. “As a parent, you have to be strong, period; whether you have a terminally ill child or a normal child. I’ve always been his voice.”
Welch said she has formed an indescribable bond with Dr. Tropez-Sims, who makes house calls to treat Darian, saving his family the inconvenience and expense of calling an ambulance to have him transported to medical appointments.
Dr. Tropez-Sims said Welch is an extraordinary example of a patient advocate. She said Welch meticulously takes care of her son and was on the forefront of trying to find his diagnosis. In fact, Dr. Tropez-Sims said, it was Welch who first brought the NIH’s Undiagnosed Diseases Program to her attention. Dr. Tropez-Sims advises parents who may be in a similar situation not to give up until you know what’s going on with your child.
“Sometimes it’s hard to know how far you should go or when you should stop, but it’s a personal decision,” Dr. Tropez-Sims said. “When you are told that they don’t know what’s wrong with your child, try to seek all avenues until you get your answers.”
Dr. Tropez-Sims said Welch has been a great mom, holding a birthday party for Darian each year and inviting other children with developmental disorders.
On May 30, Darian’s final celebration was a party with everyone dressed in all white. Darian had a cake and balloons. He was surrounded by loved ones including his mother; his father, Darrell Jones; his grandmother, Deborah Welch; and his pediatrician, Dr. Tropez-Sims. His mother let him know that it is his birthday and it’s time to say goodbye.
“I let him know he’s loved and I’ve done all I can do. He’s going to a place where mommy is not going to be. He’s going to see his Nana [great-grandma] and Nana is going to watch him until I get there. I’m at peace. I know that Darian is going to a better place and he doesn’t have to be like he is now.”
