By Shavonne Potts
spotts@salisburypost.com
Emily Hain, a senior at West Rowan High, has missed much of her high school years because of a rare condition she didn’t even know she had. The 17-year-old was diagnosed with Arnold Chiari Malformation, known simply as Chiari (pronounced kee-AR-ee) Malformation, which essentially is when the cerebellum slips or is pushed into a downward or upward position into the top of the spinal column or the base of the skull.
The cerebellum is the part of the brain that controls balance, coordinates walking and other movement.
She was misdiagnosed for years, her mother, Kelly Hain, said.
Chiari Malformation, Kelly said, is often misdiagnosed as multiple sclerosis, chronic fatigue or as fibromyalgia, which is characterized by tenderness in the muscles and joints.
Emily was even accused of making up her symptoms, which included headaches and neck pain. She was diagnosed with mono (mononucleosis), was kept at home for weeks and eventually seemed well enough to return to school.
“She called me confused. She said she ‘was in building 500 and she didn’t know what to do or where to go,’ “ Kelly recalled.
It was determined Emily not only had Chiari Malformation, but a number of accompanying conditions, Ehlers-Danlos Syndrome Type III or hypermobility, when joints stretch farther than normal as well as Cranial Vertebral Instability or brainstem compression. Many people who have Chiari Malformation tend to have hypermobility as well as compression of the brainstem.
Also 25 percent to 50 percent of people who have Chiari Malformation also have hypermobility issues, Kelly said.
Multiple surgeries
In the past year and a half, the teen has undergone five different operations to correct the conditions.
Traci Burleyson, Kelly Hain’s best friend, has planned a spaghetti fundraiser on Friday to help offset some of the family’s medical expenses. World Hope Worship Center, 2203 Mooresville Road, Salisbury is providing a location where people can pick up lunch beginning at 11 a.m. and dinner beginning at 5 p.m. Organizers are accepting donations and will allow lunchtime deliveries.
“Any moment an illness could knock on your door anytime. And so many people here care. She’s such a good kid, it’s not hard to care,” Burleyson said.
Burleyson met Hain, who is a teacher at Hurley Elementary School, about seven years ago and both have three daughters.
“She is the most awesome kid,” Burleyson said of Emily.
During a Chiari race/ walk in Atlanta Kelly met a woman who told her about a doctor in Maryland who may be able to help Emily.
Emily had already had two previous surgeries to correct the problems in her spinal cord and cerebellum, but required further specialized surgery.
“Her skull began to slide forward and was pressing on her brain stem,” Kelly said.
Dr. Fraser Henderson, a neurosurgeon in Bethesda, is one of four surgeons in the country that would be able to perform the specialized surgery that Emily needed.
At first Kelly immediately said, “I can’t go to Maryland,” but once she began to research the doctor and the surgery, she and her family’s minds were changed.
Having the much needed surgery out of network in Maryland meant the family’s insurance would not cover the cost.
Emily’s first surgery took place in August 2010, followed by others in May 2011, December and as recently as 11 days ago.
Dr. Henderson discovered that in essence Emily’s neck was not strong enough to support her head or skull. Her spine was compressing her brain stem. Surgeons removed and used parts of her rib in what is called a rib graft and placed it in the vertebra to her skull via fusion surgery, thus allowing her brain stem to become stable.
The fusion corrected the angle of where her spine met her skull so it wouldn’t be compressed.
The fusion surgery helped with Emily’s headaches and brain fog as well as problems she was having with comprehension. She had short term memory.
“If you wanted to her to remember something you had to text it to her. It’s almost like having a stroke and coming out of that,” Kelly said.
Emily had surgery December 14 in Maryland and returned home to Salisbury a week later. Emily’s most recent surgery, Feb. 24, was so unexpected, Kelly’s husband, Jeff, and their two other daughters, Lily and Baylie, were unable to join Kelly and Emily in Maryland.
Emily was simply seeing her doctor for a check up following the December surgery when he discovered she possibly had tethered cord — when the cord is abnormally attached within the boney spine.
Hain and her daughter just returned home late last week.
“It was worth it to go to Maryland because he really saved our child’s life,” Kelly said of Henderson.
Rallying support
During one of Emily’s surgeries, West Rowan students, and others around the world, were wearing purple — the color that symbolizes Chiari Malformation. The word had spread via the social networking site Facebook and word-of-mouth so much so the Hain family were hearing words of comfort and prayer from as far away as Australia.
“She’s brought awareness to a disease, to a syndrome that is not widely known,” Kelly said of her daughter.
There are about one out of every 1,000 people diagnosed with Chiari Malformation, but Henderson believes the number is closer to one out of 100 and of those one out of 10 are actually diagnosed correctly.
Kelly called Emily “strong, faithful, loving and kind.”
The family can already tell her symptoms are improving. She’s able to recall things and the amount of pain she was in has decreased.
Emily is even looking forward to the day she can have sweet tea and Cheerwine.
The teen is also looking to graduate this year thanks to her homebound teacher.
When Burleyson told her friend she wanted to help them, it was difficult to accept.
“It’s hard to let people help. You want to be able to be strong and independent,” Kelly said.
Kelly ultimately came to the realization that “sometimes you have to be the taker in order to allow others to be givers.”
For more information about Chiari Malformation, visit www.conquerchiari. org.
For more information about the fundraiser or to make an appointment for a delivery, call Traci Burleyson at 704-797-8300.
Contact Shavonne Potts at 704-797-4253