GREENFIELD — With a quick glance at the Niece boys — Lincoln, 10, Norrin, 5, and Remley, 2 — one would see three happy and rambunctious boys playing games on their iPads, with Legos and little toy cars that feature Yoshi and Toad from the Mario games.
However, over the last few years, not all the boys’ time has been fun and games. What started out as complaints of headaches turned into all three boys being diagnosed with Chiari Malformation.
Whitney said Remley was sick in June of 2022 and had no urine output for 24 hours. He was taken to the hospital and diagnosed, but surgery was not planned at the time.
Lincoln started having headaches when he was around age five but, due to his congenital heart defects, the headaches were passed off as due to that. It wasn’t until after a CAT scan in February 2023 that Lincoln was diagnosed with Chiari Malformation and scheduled to have surgery in March of 2023.
That same month, Remley was showing signs of not progressing developmentally and was scheduled for surgery in August of 2023. In the same month of Remley’s surgery, Norrin started to complain of headaches and was scheduled for the same surgery in January.
Laurie Ackerman, Kalsbeck professor of clinical neurosurgery at Riley Hospital for Children, performed surgeries on all three of the boys and said that Chiari Malformation is when the back part of the cerebellum drops through the opening of the bottom of the skull. When the cerebellum tonsils press into the brain stem or spinal cord, it can cause a variety of symptoms such as headaches, numbness in arms and legs and more.
The boys experienced headaches and vomiting. The youngest, Remley, would randomly fall from his legs giving out.
Whitney said, with the boys at their ages, there are some things you just don’t explain about the condition. Her husband, Ron, agrees.
“You don’t want to scare them. It’s already a scary thing to go through,” Whitney said. “It’s not an easy thing to explain to your kids, that’s for sure.”
Whitney said that Lincoln had already been to Riley Hospital for Children and was used to the visits due to his heart condition, having three open heart surgeries and being under anesthesia close to 30 times in 10 years.
Norrin had always been the visitor to Riley Hospital for Children for his older brother’s surgeries, never the one going in to be operated on. In the past, Riley Hospital for Children was a place for Norrin to get chocolate milk and play in the playroom, but this time they explained he would go to sleep for a while and wake up with a couple “owies.”
With Remley being only two-years-old, they didn’t feel it was necessary to explain details on what was happening, but rather just making sure he was comforted.
The surgery all the boys experienced was called Chiari Malformation Decompression. Ackerman said the purpose of the operation is to remove a little piece of the back part of the skull and take out a three by three centimeter piece of bone. Ackerman said she also opens the cover over the brain called the dura mater and sews a patch in there.
“The goal of all of these things is to sort of disimpact the area,” Ackerman said. “To allow fluid to move around there normally.”
Norrin and Remley experienced complications afterwards, creating hydrocephalus or water on the brain. Both boys had an extra ventricular drain placed for 10 days post-op, and also a ventriculoperitoneal shunt placed which takes the extra fluid on the brain and puts it into the belly to be dispersed. Ron said that Dr. Ackerman said she always tries to avoid putting in a shunt if it is not necessary, but after monitoring the boys for a few days and still producing hydrocephalus, they went ahead with the procedures.
Whitney said that when they found out that all three boys had the same condition, they were shocked to find out that all three boys needed intervention.
Despite Riley Hospital for Children making sure to explain what was happening and taking extra care of the boys, the transition of handing the boys over to the doctors or surgeons was still hard for Whitney and Ron.
“It doesn’t get any easier, even after multiple procedures and surgeries,” Ron said.
Whitney said that she can remember with Lincoln’s first surgeries she would sit in the hallway and cry, making sure the boys wouldn’t see her.
“Cause you don’t want to cry in front of them, but you also can’t hide your emotions very well when it’s going on multiple times,” Whitney said.
Lincoln said that when having surgery he was afraid and nervous but he was able to have his iPad and his Grover with him — a stuffed animal he had since he was a year old. Also, the chapstick they put on with the oxygen mask for surgery was “stinky.” For Norrin, surgery was both “good and bad” — the good part being the food, and the bad part being the external ventricular drain and the mask because “the smell stinks.”
After their extensive surgery journey with the last one being Jan 29, all three boys are doing well and back to playing, which Ron said can be hard to do sometimes — keeping the boys from being little boys.
Whitney said that while there aren’t any medical procedures they have to follow after the surgeries, they have to be extra careful to make sure the boys don’t hit their heads, following what they call the “two feet on the ground rule.” This keeps the boys from jumping on trampolines, riding bikes and more — all things playful boys love to do.
“..Being typical kids,” said Whitney when talking about what the boys aren’t allowed to do after their surgeries. “Just like the other day when it was really nice outside, and everybody else could go outside and ride something, Norrin is standing in the garage in tears because he can’t.”
Despite the stricter rules when it comes to playing, Whitney said most children with Chiari Malformation go on to live normal lives after surgery and that their quality of life is better because they don’t have the headaches and other symptoms that come from the condition.
Norrin and Remley will have to go for shunt revisions, having them replaced at some point in the future.
Whitney said she is also in the process of getting an MRI scan done due to her chronic headaches to see if she, too, may have the condition.
An estimated one in 1,000 people have Chiari Malformation — something that they are born with — but many go undiagnosed due to being asymptomatic. Ackerman said that they are part of a large study with Park Reeves Foundation to evaluate care of children with Chiari Malformation.
For the last decade, Ackerman said they’ve been doing research and trying to find more answers. Another group is investigating the genetics of Chiari Malformation, where they take cheek swabs and test them to see if there any commonalities between those with Chiari Malformation.
Whitney said there is testing being done to see if this condition is hereditary, but it could be months before they hear back about any results.
Ackerman also said that it is unusual to have all three children have this condition.
Ackerman said that being able to get to know the Niece family, Ron and Whitney dove right into what they needed to do as parents, and that the boys are great kids.
“These children are really funny little kids who have the grace of having just absolutely spectacular parents,” Ackerman said. “They are such a nice family.”
Before their journey, Whitney said she had never heard of Chiari Malformation, but now that she is involved in the Chiari Malformation world, she’s spoken with other parents who were also not aware of the condition.
“So if we can help one other kid that maybe is struggling with headaches and they’ve been passed off several times, that’s a win in my book,” Whitney said. “Because, a lot of people — like I said – they go in to look for something that is causing a headache and unless you say specifically you’re looking for Chiari, it can get passed off as something else.”
Throughout their journey, advice for other families who also experience Chiari Malformation or any other kind of surgery process is to take it one day at a time.
Ron said that, with these kinds of situations, it’s out of their hands and they rely on the expertise of the doctors and surgeons.
“Our opinion matters, yes, but at the end of the day, it’s important to trust the Riley team,” Ron said.
Whitney also added that “you can’t worry about the unknown,” and without knowing what the future will hold, they focus on the day that they are in.
Lastly, Ron and Whitney said it’s not a bad thing to ask for help. Ron said that, on the other hand, they like to give back with the Extra Life event, a 24-hour gaming marathon, where they can raise money for Riley Hospital for Children where the donations go toward families who experience similar situations or don’t know where to go to ask for help.
This year will be their 12th year for the event, raising so far a total of approximately $15,000. Lincoln showed off their nine medals they have received from the event.
While Ron and Whitney said their friends and family have been there for them to help when needed, they have also had many others rally together — even strangers — that have donated to the family, prayed and joined their medical group on Facebook to show their support.
“You feel like you can’t adequately enough thank everybody, so then it’s overwhelming but then it’s also, not guilt, but you just feel like you can’t reach out personally and thank everybody,” Whitney said. “Especially with how many people who have supported us over this last year. I can write thank you on the internet multiple times but until I can actually thank somebody in person, you just feel like you haven’t conveyed your gratitude enough.”
For more information on Chiari Malformation, visit www.rileychildrens.org/health-info/chiari-malformation or call 317-944-6201.
