Amanda Wendorff and three of her four children have Chiari malformation, a neurological disorder
Amanda Wendorff remembers clearly the otherwise beautiful fall day in 2009 when her family’s life went from storybook charm to a medical hell that won’t end.
It began when her son, Brayden, collapsed in debilitating pain when he was just four years old.
“He came home from playing with friends and just dropped onto the floor, crying with an awful headache,” Wendorff, 34, of Middleburg, Florida, tells PEOPLE.
“Our pediatrician said, ‘Well, sometimes kids have migraines but we’re going to order a CT scan just in case,’ ” she says. “She called back a few days later and said, ‘I need you to come in.’ “
Brayden, it turns out, had Chiari malformation, a statistically uncommon neurological disorder where the cerebellum, the bottom, back portion of the brain that regulates muscular activity, descends out of the skull into the spinal area, compressing parts of the brain and spinal cord and disrupting the normal flow of cerebrospinal fluid.
It got worse.
By that December, her son Skyler (now 13) began showing signs of the disorder, too. Zachary (now 9) was next, diagnosed in May 2011 and undergoing surgery the next month followed by Amanda herself in September 2012.
“When Zach first showed symptoms, I think I was in denial,” says Wendorff, a former Jacksonville sheriff’s office dispatcher who left work to raise her children. [He had] “severe pain, headaches, he got dizzy playing. I knew in my heart of hearts, ‘This kid has Chiari.’ “
Chiari is relatively uncommon, affecting one in 1,000 to one in 5,000 people living in the U.S., according to the C&S Patient Education Foundation, informally known as Conquer Chiari.
It’s even less common for so many members of one family to have the condition.
“It is indeed rare,” says Dr. Heger, chief of pediatric neurosurgery for Georgia’s Augusta University Health System. “Most cases of Chiari malformation are sporadic, although it is clear that there are family clusters. This does suggest that there is a genetic basis for the malformation, although the gene or genes responsible have not been identified as of yet.”
In the years since, the family has battled not only their varied conditions, but the constant financial stress of extended out-of-state hospital stays, six-figure medical debts, limited income and criticism from many who doubt illness claims and question the validity of the crowdfunding campaigns that the family often depends upon to stay afloat.
“We don’t make any long-term plans,” Wendorff says. “We just live day by day.”
A Painful Disorder
In addition to the multiple Chiari diagnoses, she and her sons also have Ehlers-Danlos syndrome, a connective tissue disorder marked by hypermobility of joints, and daughter Kami, 9, has several kidney issues.
“We are a medically complex family,” Amanda says.
But while other family members’ cases are relatively mild, Brayden takes the brunt. Thus far, he has undergone 33 brain and spine surgeries, suffered four strokes and learned that he also deals with a number of Chiari-related issues.
Chiari experiences vary greatly among patients. Some never show symptoms or require little treatment beyond pain management, while those whose symptoms interfere with quality of life may undergo multiple decompression surgeries or placement of a shunt (a tube-like device) to channel the flow of cerebrospinal fluid and relieve pressure on the brain.
Symptoms can include muscle stiffness and weakness, problems with coordination, overactive reflexes, blurred or double vision, tingling or burning sensations, difficulty swallowing, numbness and vertigo, among others.
But the symptom most characteristic of Chiari malformation is debilitating headache that can last for days, often accompanied by nausea and vomiting. These headaches manifest as intense pressure in the back of the head and are brought on by the simplest of activities – exercising, bending over, coughing, sneezing or even laughing.
Brayden describes the pain as “like getting hit with a hammer, except with a real hammer, you have to pull back it back to hit again. Instead, this feels like the hit all the time.”
The disorder is still tough to diagnose. Amanda recounts conversations with multiple medical students who say they recall spending no more than 30 minutes, if any time at all, learning about the condition. Improvements in imaging testing over the past several years, however, are resulting in more frequent diagnoses.
But hope may be on the way thanks to Brayden himself.
Over nearly a decade of treating Brayden and coming to know him almost as well as he knows his own children, Dr. Heger tells PEOPLE he’s already changed his approach to treatment of several common Chiari symptoms based on what the boy’s case has taught him.
One example involves a shunt run from the base of the skull to the belly to drain fluid downward and minimize pressure on the brain. Brayden complained of pain in his belly and X-rays showed that trouble spots coincided with the location of the tip of the shunt tube.
“In the past, we would have pulled the tubing out of the belly and put new tubing in near the lungs,” says Dr Heger, who has treated Brayden for eight years.
“Instead, we went in with a laparoscope, took the tip of the catheter and attached it to the wall on the inside of the belly cavity so that it would stop flipping around. It worked and we’ve subsequently done the same for other patients.”
More changes may be in store.
Though he won’t yet expound upon his developing theory, Dr. Heger is exploring ways to potentially predict which Chiari patients will be asymptomatic and which will have more complicated or intense issues.
“I’ve probably learned more from Brayden than any other patient,” he says, noting that he hopes his theory ultimately will be tested, published in medical journals and, if proven, implemented by other physicians. “I keep hoping that I’m going to find that one thing that’s going to get him to turn the corner for good.”
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While the physical and emotional struggles are tough enough, financial burdens weigh heavily on the family as well. Amanda occasionally works as a tax preparer, but day-to-day care for Brayden and frequent out-of-state hospital stays that can last weeks or even months makes regular employment impossible.
Husband, Dan, 39, works for the Florida Department of Corrections and has insurance with Blue Cross Blue Shield with a $6,000 annual deductible.
Brayden gets some Social Security funds and Florida’s Children’s Medical Services helps with certain related costs, but excludes coverage of out-of-state expenses. He’s been treated at hospitals in Georgia, New York and Boston.
“Travel is what kills us,” Amanda says. “The Ronald McDonald House is a $20-per-night donation but sometimes they’re full or the food pantry is empty. Hotel rooms, meals, paying whoever is taking care of my other kids and making sure the bills are paid is difficult. A few times, I’ve had to leave town spur-of-the-moment with just the clothes on my back because they were loading up a jet to transport Brayden.”
To help lighten the financial burden, the family has launched upward of a dozen crowdfunding campaigns over the years and, when treatment schedules allow, often hold fundraising events such as poker runs, bake sales, raffles and car washes.
[IMAGE “3” “” “std” ]Meanwhile, Brayden goes about life as normally as possible.
Days-long headache episodes, along with frequent extended hospital stays mean homeschooling, though he hopes to return to public school and his friends by ninth grade.
Sports are out of the question, so he fills his days playing video games, Legos and the occasional Nerf ball battle with his siblings, though he knows that his condition means he must “pay to play.”
After participating in a recent Chiari fundraising walk, “We had to pull over seven times for him to throw up on the way home and he’ll be in bed for the next few days,” Amanda says. “Still, he chooses to live life to the fullest and do everything he possibly can.”
While he understands that his aspirations of joining the Marines and becoming a police officer like his dad, Dan, are unlikely, Brayden is considering a culinary career, learning to cook from a family friend who is a professional chef.
“One time, we cooked steak, twice-baked potatoes, salad and cheesecake,” he said, excitedly. “I love to cook. Cheesecake is so much fun.”
Such moments are fleeting, but Brayden takes it all in stride and hopes that his case ultimately will help others.
“God only chooses the people who can handle it and I feel thankful and blessed for what I have – still being alive, my family, my doctors, my supporters, everyone.”