Brisan Stults of Grain Valley celebrated his seventh birthday on August 27 at Children's Mercy Hospital, after going into septic shock due to an infection in his jaw. Brisan and his brother Parker battle several health problems, the worst of which is Niemann-Pick Type C, sometimes called “Childhood Alzheimer's”. Photo submitted | THE POINTE
The Pointe visits with the Stults’, whom we first met in 2009, to see how life has changed as they continue their struggle.
On August 23, less than a week after the school year started, Brisan Stults nearly died after going into septic shock. Doctors still can’t say exactly how things went bad so quickly, speculating that it may have begun with a simple tooth infection.
Somehow, Brisan developed bacterial sepsis in his jawbone, and within 4 hours he was in full-blown shock. When he made it to Children’s Mercy Hospital, his blood pressure was dropping. His body was shutting down.
To make matters worse, Michael Stults was stuck in New York on a business trip, unable to get back to his family. This was the day earthquakes rattled the east coast and kept planes from taking off, leaving Michael’s wife, Jennifer, to deal with the crisis on her own.
She was told they caught it just in time, that if Brisan had gotten the hospital any later, he wouldn’t have made it.
“He hasn’t been the same since,” Jennifer said.
Today, they’re pretty sure that Brisan has an immune deficiency disorder that has to do with his neutrophils, which are the most common type of white blood cells our bodies, and are essential to our immune systems. In the case of a bacterial infection, they are described as “first responders”, which explains why their not working properly could lead to sepsis so quickly.
As mentioned in last week’s installment, Brisan and his brother Parker suffer from Niemann-Pick Type C (NPC), often referred to as “Childhood Alzheimer’s” because of the way it causes similar neurological problems, leading to a premature death. The boys have severe learning disorders and physical limitations. They spend most of their time in wheelchairs. NPC is 100 percent fatal; there is no cure at this time.
Both of them, and Brisan in particular, suffered from another condition last time we visited with them, one which caused them have frequent and profuse nosebleeds. This has stopped, thanks to a medication the boys take.
It should be noted that Brisan’s immune dysfunction and the bleeding disorder are in no way related to Niemann-Pick Type C; they are simply medical conditions he is burdened with on top of the disease, the product of unfortunate combinations in his genetics.
“I’m pretty sure the bleeding disorder came from my side,” Jennifer said.
Considering the day-to-day issues involved with caring for their ill sons and raising a third son, Duncan, who doesn’t have NPC, Jennifer and Michael have reached a point where they can take much of it without batting an eye. Vomit, dirty diapers, seizures, they take in in stride to a degree most of us can only try and imagine.
But they’re just now getting over the near-death experience Brisan had, more than 2 months on.
“When we walked into the ER his blood pressure was 117 over 80; it dropped to 60 over 29,” said Jennifer. “That’s pretty darn bad.”
Brisan’s and Parker’s little brother Duncan is doing well. Now 3 years old, he is never cold and loves to run around in his underwear. He is their perfectly healthy youngest child, but his mom and dad worry sometimes that he gets a little less attention than his older brothers, who need so much help.
He doesn’t seem to mind, running upstairs during the interview to create a “robot” out of a plastic construction toy set, watching a Pixar movie in the living room while his brothers nap, and otherwise entertaining himself better than most kids his age are able to.
Two years ago, the older boys were on a very expensive drug, Zavesca, which purports to slow the symptoms down. They still take the drug, also prescribed for people with Gaucher disease, another lysosomal storage disorder. Although not FDA-approved in the United States, Zavesca is used “off label” for NPC. It works in cases like Brisan’s and Parker’s by reducing liver and spleen size, while increasing hemoglobin content and platelet count.
It is supposed to maintain development, which important because, as Jennifer puts it, “Once you lose a skill, you’re not getting it back.” Without having any ill side effects, she says they wouldn’t want to risk taking the boys off the medication.
“We think it does what it says it does; it’s hard sometimes to be sure. But would we want to risk taking them off and seeing them deteriorate more quickly? No.”
NPC families dream of that magic bullet; a cure or at least a more effective and viable treatment for their loved ones. Many families have found hope in a drug called Cyclodextrin, which in laboratory tests has been found to reverse the effects of NPC in mice. It is supposed to repair damaged cells and restore skills in subjects. No one’s calling it a miracle cure, but some scientists wonder if this could be the drug that one day leads to an effective treatment for NPC.
The Stults’ are happy to see any progress made, but there are a lot of moral, personal and ethical concerns they had which led them to hold off on trying to get access to the medication for their sons.
As of now, it remains virtually untested on humans. There will be an organized study of the drug conducted early next year at the National Institutes of Health, but the Stults’ won’t be a part of the study.
Among other worries, they have concerns about the method of delivery; directly into the brain through a shunt and into a catheter in the brain tissue.
“That’s a pretty invasive surgery for something that we don’t know will work,” Jennifer said.
Michael doesn’t discount the potential in Cyclodextrin.
“There’s definitely some positives out of this, it’s just that we don’t know what they long-term effects might be; there could be pretty bad side effects,” he said.
Despite the mountain of Federal Drug Administration paperwork and hundreds of thousands of dollars required to access the drug and be part of the study, the Stults’ would pursue it in a heartbeat if they could be sure it was in Brisan’s and Parker’s best interests. So far, they haven’t been convinced that the risk is worth the potential reward.
“Quality versus quantity in life comes into play,” Jennifer said. “There are children who are at different stages than we are, older but not as progressed, who are going to be part of the study.”
Michael is quick to add that he doesn’t think anything is wrong with the families that are pursuing the drug trial, that it’s a personal decision for their families and that he and Jennifer reached a different conclusion than some of the others. They do want the studies to continue, and the cure to be found.
To that end, the Stults’ are hosting a fundraiser this Friday evening for the Niemann-Pick Children’s Fund; dubbed “Vegas in Kansas City Casino Night”. It will take place from 7 to 11 p.m. at the Hilliard Gallery in downtown Kansas City.
The event will include Blackjack, Poker, Texas Hold ‘Em, Craps, Roulette, and a Money Wheel. There will be hors d’oeuvres, a DJ / emcee- Bryan Truta, the morning DJ on 105.1 JACK FM, as well as prizes, raffles, and silent auction items. There will even be an appearance by Matthew Lesko, the question mark-clad TV pitchman known for writing books on how to get hold of government grant money.
“I will be in Kansas City Nov. 11 to help my friends Michael and Jennifer Stults raise awareness for one of the world’s rarest diseases,” Lesko writes on his blog.
“I just met him because I’m in marketing,” Michael said. “Over the last few months, we were discussing ways to develop business. Last week, he wanted to fly in to meet with me, and I had told him about our event. He offered to help, and like most folks when they hear about our story, it is an instant Debbie Downer.”
Despite or perhaps because of his wacky approach to marketing, Michael said he respects Lesko.
“He is a smart man. He’s willing to do the things most of us wouldn’t venture to even do.”
The Stults’ are hoping to get as many people as possible out to support the cause this Friday. The website promoting the event points out there is a value to the types of research being done on NPC; it can bring understanding to more common disorders like Stroke, Adult Onset Alzheimer’s, Atherosclerosis, and even HIV/Aids. Each of these disorders has a cholesterol imbalance component.
It’s impossible to be in the Stults’ home, see what Brisan and Parker have to go through, and not see the importance of that research. Michael and Jennifer are both engaging conversationalists, and they don’t spend a lot of time feeling sorry for themselves or their family. They spend a great deal of their time thinking about and fighting with a faceless disease that is robbing them of time with their children.
Each of the boys still has a distinct personality, but sometimes, they catch glimmers of a little more, something they thought was lost over the years.
Brisan and Parker have no short-term memory. They do still have the ability to move memories into long term, but they don ‘t usually remember things from even a few seconds earlier. They will learn, over time, to recognize a new face; their in-home nurse, for instance. They don’t see their grandmother every day, but when they do, they know that she’s their grandmother.
“But some things make us wonder,” Jennifer said. “They can watch a movie, and it’s only certain movies, where they’ll know it’s a sad part, and Brisan will cry or get really upset.”
In Ratatouille, when the crazy old lady is shooting all the rats, Brisan freaks out and starts crying. In Alvin and the Chipmunks, they laugh at the same parts every time. And Bri cries at the sad part, when Dave’s yelling at the chipmunks and kicks them out. Or in Despicable Me, he will cry when Gru gives the girls back to the adoption agency, Jennifer says.
“It’s appropriate, totally appropriate for him to be emotional about it. So, it kind of makes me wonder, if he’s in there, but the part of his brain that’s expressive is just too damaged to get it out. But it’s in there,” she said.
Brisan will also laugh at things that happen in his presence that he thinks are funny- Duncan falling, or someone tickling him, for example.
Listen to Michael and Jennifer when they relate these stories, and you understand why they fight so hard despite the odds. Their joys in life are the same as any other parent- sharing the laughter and tears of their family, just like the rest of us. Their love of their children just as strong- perhaps stronger, for all they’ve been through.
Tickets for Friday’s “Vegas in Kansas City Casino Night” are for sale online at www.NPCFund.org/VegasNight with several options; general admission is $25, which includes entry into the fundraiser, 1 drink ticket, 1 raffle ticket, and a 5,000 chip voucher to start having fun. Tickets and sponsorships go up to $250 with different options.
Christopher Fischer | The Pointe staff
