Published: Tuesday, September 10, 2013 3:38 PM CDT
Imagine you’re ailing. Imagine one of your body’s major organs is failing. Imagine needing a transplant — badly.
Now imagine you haven’t yet turned 2.
All of that should give you a small insight into the plight of Josilyn Mae “Josi” Bourisaw, the daughter of Ste. Genevieve County residents Terry “Turk” Bourisaw and Jennifer Burt-Bourisaw.
“We’re actually in the hospital again,” Burt-Bourisaw reported by phone late last week from SSM Cardinal Glennon Children’s Medical Center in St. Louis. “We came up yesterday. She’s downstairs getting another PICC [peripherally inserted central catheter] line put in.”
Josi, now all of 15 months old, suffers from a liver affliction called biliary atresia, its cause still unknown.
“She has no bile ducts. Her bile ducts just disintegrated when she was like 3 or 4 weeks old,” Burt-Bourisaw said. “Officially diagnosed when she was 8 weeks old and had surgery to correct it.”
Her mother provided background on Josi’s affliction.
“It doesn’t affect her appetite,” Burt-Bourisaw said. “What it affects is, she doesn’t have the ability to absorb the nutrition because she has an impaired bile flow. So she doesn’t absorb the calories, which is hard to gain weight if you’re not absorbing the calories.”
Earlier in her brief life, Josi underwent a bile-drainage surgery called Kasai — pronounced “kuh-sigh” and otherwise known by the term hepatoportoenterostomy.
A few months of steroid treatments followed the Kasai.
“It’s a temporary fix,” Burt-Bourisaw said. “It worked for a while. She had gotten to the point where she just quit gaining weight by eating … so she is getting all of her nutrition I.V. now.”
Kasai, named for the Japanese surgeon who originated the procedure, helped but not nearly enough.
“All the research shows that if it’s done by 8 weeks of age, the chance of success is higher,” Burt-Bourisaw said. “But still, the chance is like two-thirds of the children that have that procedure still end up needing a liver transplant. It is a temporary, long-shot fix. Most kids still end up having the transplant.”
When that time comes, Dr. Betsy Tuttle-Newhall, a pediatric-transplant surgeon, will perform the liver surgery. To date, Dr. Ajay Jain, a specialist in pediatric gastroenterology and hepatology, has been overseeing Josi’s day-to-day health.
At the moment, Josi weighs 20 pounds, just shy of the requisite transplant weight.
“It’s 22 [pounds] — 22 is what we’re shooting for,” Burt-Bourisaw said.
In the meantime, the family has been closely monitoring transplant options and focusing on the optimal option of a liver from a donor of Josi’s age and size.
“That would be the best thing,” Burt-Bourisaw said. “Right now, she’s doing well. I mean, she has an infection right now, but her liver is functioning well enough, it’s not an immediate need for us to have a transplant.
“If it was an immediate thing, they’d find the best match as quick as possible, whether it was an older [liver] or partial or anything like that. But for right now, they really want to find one that’s as close to her size and age and all that stuff — it’s just the best outcome if they do it that way.”
A resolute note entered Burt-Bourisaw’s voice.
“My transplant coordinator, who I speak with a whole lot — it’s one of the nurses up here — she assures me that when the time comes, the liver will come,” she said.
“We’ve had offers. We’ve had livers offered to her that, first of all, she’s not big enough. Second of all, this number was off, or that number was off. There’s a lot that goes into selection and finding a liver for these kids — and adults, for that matter, just finding one in general.”
Even for a patient of Josi’s age and size, Burt-Bourisaw noted that availability ought not pose a problem. “Actually, it’s not that hard for our region — unfortunately for those families,” she said.
Until the transplant, Josi’s affliction heightens her susceptibility to infection.
“That’s what we end up in the hospital with all the time,” Burt-Bourisaw said. “It’s liver infection. And that’s from the surgery that she had to fix the whole thing.
“What sucks, when she gets these infections, we can be in the hospital anywhere from a week to four or five weeks. I mean, we’ve been up here as long as 26 days, like, consecutive. And that’s really hard.”
Josi’s affliction has affected her spirit only to the extent to be expected.
“Only when she has these acute episodes,” Burt-Bourisaw said. “Other than that, she’s happy. She plays. She loves the music, and she loves to sit there and dance and have a good time.”
Also, Burt-Bourisaw’s own professional background has benefited her daughter.
“Fortunately, I am a registered nurse, so that is what has enabled her to stay home as long as she has,” Josi’s mother noted. “When we’re in the hospital and we get to go home and she’s on all these I.V. things, the doctors are comfortable enough with me and confident enough in what I can do to make sure she’ll be all right.”
The professional advantage, ironically, has been accompanied by a personal disadvantage.
“I’m so in the nurse mode that I don’t get to be ‘Mommy’ that much because I’ve got so much to do,” Burt-Bourisaw said.
Cardinal Glennon has impressed her a great deal. “I’m pretty glad we’re here,” Burt-Bourisaw said with a small, weary laugh.
“There’s play rooms, and there’s all these cute little wagons, ‘Rally Squirrel’ wagons, all this stuff,” she added regarding the hospital’s relevant department. “They’re called ‘Child Life,’ and basically, their job is to go out and play with the kids, which would be like an awesome job.
“And then, there’s all the volunteers, which are great up here. There’s the Ronald McDonald Room up here, which is great — it’s just for families to go and get some snacks or a soda or just relax. It’s really nice.”
Those in her own profession also have earned high marks from Burt-Bourisaw.
“I couldn’t ask for a better staff up here,” she said. “The nurses are awesome, and they basically fight over who gets to take care of my daughter.”
The local community likewise has embraced Josi’s cause.
This Saturday, for example, both Bloomsdale’s Dew Drop Inn and the Ste. Genevieve Jaycees will host fund-raisers for the toddler.
To learn more about the Bloomsdale event, call either (573) 483-3256 or (573) 535-8381. To learn more about the Ste. Genevieve event, call (573) 631-2856.
Also, the Ste. Genevieve branch of the Bank of Bloomsdale is accepting donations payable to the Josilyn Bourisaw Benefit.
Even for a nurse, Josi’s ordeal has hammered home for Burt-Bourisaw “how important it is to people just in general to be an organ donor,” she said. “You can save so many lives by being an organ donor, it’s just unreal.”
Her professional demeanor notwithstanding, the pressure may be taking its emotional toll, understandably.
“We have a son — I have a son as well,” Burt-Bourisaw said. “And he’s perfectly happy, healthy, running around. He’ll be 4 in November. And it’s really hard, y’know, with that.”
Her voice quivered as she added, “I just want her to feel good, that’s what I want.”