For those like Gabby with hydrocephalus, a condition also known as “water on the brain,” it’s hard to know if a headache is just a headache. It could be a sign a patient’s shunt, a medical device implanted in the brain to drain built-up fluid, is failing. Garzon rushed Gabby to the hospital. Sure enough, she was in shunt failure. Garzon couldn’t believe it was happening, again. For the 16th time, Garzon waited helplessly as her teenage daughter went through brain surgery. “Dozens of surgeries in a lifetime is no way to live,” says Garzon, who is now the national director of program services and communications at the Hydrocephalus Association. “No more BS!” she thought, then decided to take her frustration public. The No More Brain Surgeries (#NoMoreBS) campaign — which relaunches this June through the Hydrocephalus Association — will educate the public about the condition, and advocate for research into new treatments through community events across the country. More than 36,000 shunt surgeries are performed each year, and 10,000 pediatric hospital admissions are due to shunt failure, according to research published in the Journal of Neurosurgery: Pediatrics. Researchers, including Benjamin Warf, MD, director of neonatal and congenital neurosurgery at the Boston Children’s Hospital in Massachusetts, say shunts save lives, but they also regularly clog and cause infections. A study published in 2013 in the journal Surgical Neurology International reported that nearly all shunts will fail within 10 years of implant. “I think there was a perception we had a cure for hydrocephalus,” says Dr. Warf. “People generally thought that with hydrocephalus, you put a shunt in and that’s the end of it.”
The History of the Brain Shunt
Children’s author Roald Dahl is one to thank for the modern day shunt, which was invented in 1962. A few years prior, Dahl’s 4-year-old son, Theo, was struck by a taxi in New York and developed hydrocephalus. Dahl teamed up with a neurosurgeon and toymaker who specialized in pumps for model airplanes, and the new shunt model saved Theo’s life. Before the shunt, the survival rate for hydrocephalus was only 20 percent. Some children are born with it — a tiny tear in the brain is a common cause in newborns — but a brain injury can cause hydrocephalus at any age. When injured, the brain swells, trapping fluid that normally circulates through the brain and spinal cord. With nowhere for the fluid to drain, the brain essentially drowns. Pat McAllister, PhD, a professor of neurosurgery at the Washington University School of Medicine in St. Louis, is able to study new treatments for hydrocephalus through grants funded by the Hydrocephalus Association. In a perfect world, he says, the pharmaceutical treatments he’s experimenting with would eliminate the need for a shunt altogether. “We weren’t making much progress at all with treatments until the Hydrocephalus Association starting funding basic research,” Dr. McAllister says. “It’s really allowed us and other labs to explore some of these treatments in detail.” The most promising research is on decorin, a protein found in the body. McAllister and other researchers in the field discovered decorin prevents the immune system from fighting against hydrocephalus as it starts to form. It may seem counterintuitive to stop your body’s natural response to trauma, but McAllister explains the immune system doesn’t know when to stop fighting, which can cause scars that form blockages in the brain. Those blockages leave no room for fluid to escape. “The animal studies have been really promising,” he says, citing his work from a study published in May 2016 in the journal Fluids and Barriers of the CNS. “There are at least three publications now in good journals showing that decorin works. We still have more work to do, but I honestly think we could be two or three years away from a clinical trial.” McAllister says decorin also has the potential to reverse inflammation in the brain, which can cause shunts to malfunction. “Most of the cells that clog catheters are inflammatory cells,” he says. “People forget that shunts are still a foreign body. If decorin is on board, it could minimize that response.”
Spreading the Word About Hydrocephalus
Olivia Maccoux, a 22-year-old college student in Minneapolis, is one of many hydrocephalus patients sharing her story for the #NoMoreBS campaign. She’s had 140 brain surgeries, most due to shunt malfunctions. She spent 167 days in the hospital when she was a toddler, and her mother Cathy Maccoux says she remembers watching in surreal horror as doctors did all types of experimental procedures on her 2-year-old, including removing her frontal bone. “They removed her forehead bone and put it in a freezer for a week,” Maccoux recalls. “If you touched her forehead, it would have just been her brain right there. They put this pink helmet on her, and there was Olivia, this happy toddler wandering around the hospital, and the whole time I’m totally freaking out.” As #NoMoreBS relaunches this month, researchers, advocates, and families share stories about how hydrocephalus affects their lives. Some, like Jennifer Bechard, lost out on just being a teenager. Bechard missed 100 days of high school, and underwent more than 40 brain surgeries to fix continuously malfunctioning shunts. Others, like Olivia Maccoux’s parents, were forced to refinance their home to cover hundreds of thousands in out-of-pocket medical expenses, despite having private insurance. Most, including Mia Padron, who just watched her 16-year-old son Tyler have his 34th brain surgery in April, suffer from medical PTSD and fear the future. “It’s the uncertainness that’s really hard. I have a deep sadness for my daughter’s future,” Garzon says. Upcoming events to support the cause include the National Conference on Hydrocephalus on June 28, 2018, in California, and Hydrocephalus Education Day with the Johns Hopkins All Children’s Hospital July 14, 2018, in Florida. Supporters can join the national campaign at the No More BS website, and connect with local community networks on the Hydrocephalus Association website.
