A HISTORY OF HOPE: THE ADAMS FAMILY'S STORY
11-year old Hawke Adams loves to draw and build with Legos. He wrestles with his brothers and pretends to throw Spidey webs at his sisters. As his father, Jason, puts it, “He’s full of life”.
This description is particularly salient considering Hawke was born with no heart rate and was resuscitated nearly 20 minutes later.
The complications surrounding Hawke’s birth have paved a difficult road – Hawke has suffered from hydrocephalus and he has cerebral palsy – but not an insurmountable one.
“There was a lot of uncertainty on how much progress Hawke might make,” said Jason. “But he’s made phenomenal progress. I’m confident that technological breakthroughs could provide an even better quality of life for him and other children and adults with hydrocephalus.”
A CHANCE AGAINST ALL ODDS
When Hawke and his twin brother, Jaxon, were still in the womb, Carmen, their mother, was warned the pregnancy may be challenging.
Around 18 weeks, doctors discovered Hawke and Jaxon had twin-to-twin transfusion syndrome, meaning their shared placenta had connecting blood vessels, resulting in one twin receiving most of their necessary nutrients and putting both brothers at risk for heart failure.
“At the time, there were only 12 doctors in the nation performing the necessary surgery our twins needed,” said Jason. “We were blessed one of them was nearby.”
Doctors prepared for fetoscopic laser ablation surgery. They would use guided ultrasound technology to see into Carmen’s uterus and use just one tiny instrument – a fetoscope – to locate the abnormal blood vessel connections and cauterize them with laser energy.
Hawke’s and Jaxon’s surgery went well but eventually sent Carmen into premature labor. Doctors admitted and treated Carmen with medication for six weeks but were then forced perform an emergency C-section 12 weeks before Hawke’s and Jaxon’s due date.
“It was the best and worst day of our lives,” said Jason. “Both of our sons were born dead. One graduated to heaven, and one came back to life after being dead for 20 minutes. It was really surreal.”
As Jason and Carmen were adjusting to their new reality – one baby, Hawke, instead of two – they were faced with more bad news. Hawke’s traumatic birth had caused a brain bleed resulting in obstructive hydrocephalus.
A MEDTECH INTERVENTION
Huddled together in the NICU, Jason and Carmen digested the diagnosis: Hawke was experiencing hydrocephalus, meaning too much cerebrospinal fluid had built up in the ventricles deep within his brain. That buildup was causing pressure on his brain, and, if left untreated, could damage his brain tissue permanently.
“First, the doctors were just going to monitor Hawke and see if we needed to consider surgery to drain the fluid,” said Jason. “Our primary doctor stayed overnight, past the end of his shift to make sure Hawke made it through the night.”
Hawke did survive the night, and his 3-month hospital stay, but it was clear he would need eventual intervention. Hawke’s doctors suggested ventriculoperitoneal (VP) shunting. This requires brain surgery to implant a catheter and a pressure valve to drain the excess fluid from the brain.
At nine months old, Hawke underwent VP shunt placement surgery. His first shunt revision occurred merely 3 months later, and he spent his first birthday in the hospital.
“The prognosis was very vague at that point,” said Jason. “I look back, and remember the doctor telling us that, even with the surgery, he might not be able to live like we hoped. There was a lot of uncertainty.”
As Hawke was recovering from his traumatic birth and surgery, he began intensive therapy to spur developmental progress.
“He was attending up to twelve appointments a week,” said Jason. “It was very slow, and the milestones that others might not consider to be milestones became milestones for us. Simple movements, even smiles, were just huge for Hawke.”
Although Hawke was advancing in therapy, his shunting required frequent revisits. By the time he was four, he had endured nine additional surgeries to fix or replace his shunt.
“Shunting is the leading way to treat hydrocephalus, but it’s still incredibly risky and failure-prone,” said Jason. “Our story, and the stories I hear from other families navigating this disease, are emotionally moving and frustrating at the same time.”
Still, despite every obstacle, Hawke continued to set and surpass many goals.
“We didn’t know if he was going to be able to eat on his own,” said Jason. “And now he loves to eat nachos and pizza. We didn’t know if he was going to be able to crawl or even turn over on his own. Three years ago, he started to walk without crutches, and he navigates the house on his own.”
Hawke’s personal growth was just as impressive as his physical growth.
“He’s had the best attitude since he was born,” said Jason. “He has a good sense of humor. He’s really friendly, personable, and optimistic.”
Today, Hawke’s life looks somewhat similar to other kids’, with the exception of occasional therapy, disease monitoring, learning delays and challenges, such as headaches, he encounters as result of his hydrocephalus. His therapy focuses on addressing the learning delays and evolving his fine motor skills and mobility. Disease monitoring requires MRIs annually.
“It’s been so long since he’s had a brain surgery. I am very concerned about a potential shunt failure or hydrocephalus complication requiring brain surgery as the current primary treatment has a high failure rate,” said Jason. “That’s why it’s so important to me that we’re pursuing a breakthrough, a cure for hydrocephalus.”
Jason himself is on the frontlines of the charge toward a cure, serving as a co-Director at the Pediatric Hydrocephalus Foundation in his spare time.
ADVOCATING FOR A NEW ANSWER
The Pediatric Hydrocephalus Foundation (PHF) helps educate the public about hydrocephalus and raise awareness among policymakers to push for more research and funding into the disease. PHF also serves as a support network for the hydrocephalus patient community.
“There are so many people who don’t know about the condition,” said Jason. “Without everyone’s support, progress is going to be difficult. We need to increase awareness and we need funds to invest in reserach.”
Hydrocephalus shunting has improved incrementally over the last 60 years, but more research and development is desperately needed, especially considering the significant risks associated with the procedure and its maintenance.
Today, pressure valves used to drain fluid can be fixed or adjustable, meaning the rate of draining can be changed in real time after implantation, potentially avoiding additional revision surgeries. Catheters are coated with antibiotics to help reduce site infections. Innovative neuronavigational technology allows doctors access to the brain without a major incision and can pinpoint the best place for shunt implantation.
Still, especially in children, there is a high rate of complication. The management of hydrocephalus has challenged doctors and innovators alike because of the unique nature of the disease in each patient.
“I work as a manufacturing engineer, so I’m always applying my problem solving here,” said Jason. “With collaboration and resources we can make progress in how we treat hydrocephalus. We need to collaboratively be pursuing a breakthrough.”
Jason shares this message with whoever will listen. He organizes fundraisers, reviews and provides input on what research grants are worthy to invest into, and draws people into the PHF’s social media channels, even persuading his congressional representative to join the Congressional Pediatric and Adult Hydrocephalus Caucus. Jason also succeeded in passing a Resolution by the Michigan House of Representatives recognizing September as Hydrocephalus Awareness Month.
“This work is really personal to me,” Jason said. “It impacts my family personally. Beyond that, I want everyone in the hydrocephalus community to live the highest quality of life as possible. The more we advocate and increase awareness the better.”
“There are so many stories within the hydrocephalus community, Hawke is just one of countless,” said Jason. “That’s why I keep raising awareness. I know we have the ability to find them a better treatment or cure.”