Neurosurgeon Offers Hope to Family with Chiari Malformation
Before he was old enough to talk, Brandon Newcomb suffered severe headaches. His parents knew this was no ordinary pain. "He would take his fist and pound himself in the back of his head. He would pass out, unconscious. He would turn completely blue and my husband and I would have to start giving him CPR," said Brandon's mother Lisa. For Lisa, Brandon's headaches weren't just worrisome--they were familiar. When the Newcombs took their son, at 5 years old, to the University of Chicago Comer Children's Hospital, she found out why.
Expert Diagnosis
David Frim, MD, PhD, chief of pediatric neurosurgery and an expert in congenital anomalies of the nervous system, diagnosed Brandon with Chiari malformation, a herniation of brain out the opening of the skull that obstructs the flow of spinal fluid to the brain. When Dr. Frim explained the symptoms of Chiari, Lisa knew she and her mother needed to be checked. Sure enough, both Lisa and her mother were found to have the condition, making the Newcombs one of only 17 known families to carry Chiari across three generations. When Brandon's brother Noah was born, he, too, was eventually diagnosed with Chiari.
"Lisa had headaches from when she was quite young. Brandon was falling down and he was having headaches. Noah, I met when he was having fainting spells while exercising," said Dr. Frim. "It's been a very complex situation for them."
Unique Approach to Treatment
Combining accepted knowledge with innovative treatment options, Dr. Frim performed decompression surgery on both Newcomb boys. "The approach is to decompress--to remove the bone--and what we also advocate is to enlarge the lining of the brain to give more room for the fluid to flow around the brain structures," said Dr. Frim. This two-fold technique can induce brain structures to spread out into extra room that the surgery creates, ending pain and improving brain functions.
These operations alleviated much of both boys' pain, but failed to end their headaches. "We're a little bit in uncharted territory with Noah and Brandon and their genetic syndrome," said Dr. Frim, "but we do have patients who have failed the Chiari surgery before and many of them respond to spinal fluid drainaige with an implanted shunting device." So, Dr. Frim and his team performed a series of operations in which he inserted a shunt into each boy's spine, redirecting the flow of spinal fluid. Both surgeries required follow-up operations and adjustments, but today, the boys' headaches, backaches, and blackouts are much decreased.
Living with Chiari Malformation
Chiari malformation is a painful life-long condition without a cure, but the Newcombs find hope, support, and relief through each other and their doctor. "I've been there as a mother, I've been there as a patient. I live this, I eat it, I sleep it, I drink it," said Lisa, who has been a volunteer in the pediatric neurosurgery clinic for six years, started a Chiari support group through the clinic and works with Dr. Frim as a research assistant on several projects related to Chiari malformation. "Dr. Frim and I are doing some very promising research on Chiari," says Lisa. "I hope we can find better ways to improve the quality of life for these children."
The gene for Chiari malformation has not been found, but researchers across the country are investigating genetic foundations of the disease--with the help of the Newcombs' DNA.
"Dr. Frim's always been very, very positive," said Lisa. "He always comes up with something new for my kids and for me." Today, Brandon and Noah still deal with some of the symptoms of Chiari, including low pressure headaches, severe leg pain, and nausea. But they are able to participate in basketball and soccer, play with kids in their neighborhood, and attend school--and, with Dr. Frim's help, work towards a life free from pain.
