All three children in an Indiana family have been diagnosed with a rare brain defect that can cause seizures and paralysis.
The children, ages two, five and 10, suffered seemingly random bouts of dizziness, headaches and nausea that neither doctors nor their parents linked together.
Whitney and Ron Niece began to notice something was wrong in June 2022 when their youngest son, Remley, was not as rambunctious as usual and had not urinated in more than 24 hours.
He was taken to hospital where doctors performed an MRI of the boy’s brain, revealing that he had Chari malformation, a one in 1,000 defect that causes a hernia at the base of the brain that puts pressure on the spinal cord.
Lincoln, Norrin and Remley Niece were diagnosed with Chiari malformation, a rare disorder that causes the brain to extend into the spinal canal.
Each of the children has undergone brain surgery to correct the condition, which only occurs in one in 1,000 people worldwide.
Less than a year later, in February 2023, Lincoln, now 10 years old, began having headaches and was also diagnosed with a Chari malformation.
And just as Remley was about to undergo surgery about six months later, five-year-old Norrin began suffering from the same headaches his siblings suffered from and he, too, was diagnosed.
Since their diagnosis, all three children have undergone “multiple procedures and surgeries” to correct their condition, which is usually caused by a small or misshapen skull that puts pressure on the brain.
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The children’s father, Ron Sobrina, said the Greenfield Daily Reporter: “It doesn’t get any easier, even after multiple procedures and surgeries.”
According to the Mayo Clinic, there is some evidence to suggest that Chiari malformation may be hereditary, but the research is still early.
Chiari malformation often causes problems such as headaches, neck pain, poor coordination, dizziness, and difficulty swallowing.
But some people with this condition may never show symptoms or receive a diagnosis, leading doctors to believe the condition is more common.
There are five types of Chiari malformation, with type 1 being the most common and benign, while type 3 is the rarest and most severe.
This type is associated with debilitating and life-threatening complications.
It is not clear what type the Niece boys have.
All of the Niece brothers experienced frequent headaches and vomiting, and Remley often fell due to his legs failing at random.
However, despite her similar symptoms, the niece initially did not suspect Lincoln’s condition and attributed the symptoms to her congenital heart condition. It is not clear what defect it has.
Lincoln was the first of the brothers to undergo corrective surgery to prevent his brain from putting pressure on his spinal cord in March 2023.
But by the time Lincoln went into surgery, Remley had shown signs of developmental delay, such as trouble walking and talking, so he was scheduled for surgery in August.
That same month, Norrin received his diagnosis after a period of constant headaches. He underwent surgery in January 2024.
The children’s mother, Whitney Niece (pictured), said the family is now focused on raising awareness about the disorder, which can cause headaches that are attributed to more common causes.
Mrs. Niece told the Greenfield Daily Reporter that the family had tried to stay positive around the children, but when Lincoln had his first surgeries, she would sit in the hospital hallway and cry so her children couldn’t see her.
And while she and her husband have tried to be transparent with their children about their symptoms and surgeries, they try to make the whole ordeal seem less scary.
Norrin was told by his parents that he would go to sleep and wake up to a couple of ‘owies.
All three children underwent Chiari malformation decompression, the standard treatment for this condition. The surgery involves removing a three-centimeter piece of bone toward the back of the skull to relieve pressure.
However, Norrin and Remley had complications after the procedure. Both children developed hydrocephalus, a buildup of fluid in the brain, which required drains to be placed to remove excess fluid.
All three children completed their surgeries in January 2024 and have been healthy since then.
According to Weill Cornell Medicine, almost all patients who undergo decompression surgery experience partial or complete improvement in their symptoms.
Despite the restrictions needed to prevent the siblings from hitting their heads, such as not riding their bikes or jumping on the trampoline, the family hopes they can go back to “being typical kids.” It is unclear how long they will face restrictions.
Mrs Niece said she is focused on raising awareness about this disease, which she had never heard of until her children were diagnosed.
She said: “So if we can help another child who may be suffering from headaches that have been overlooked several times, that’s a win in my book.”
“Because a lot of people are looking for something that gives them a headache, and unless you specifically say you’re looking for Chiari, they might pass it off as something else.”
