Born an identical twin, Josie Dolena’s allergies set her apart from her sister, Violet, at a very young age. While both girls were originally born with dairy allergies, Violet outgrew hers as a toddler; Josie’s lingered.
Their parents, Heather and John, grew to know Josie as their sensitive child; not in the way of her feelings, but rather her sensitive immune system.
Because Josie was prone to reacting to certain foods and products, Heather acted quickly the moment she noticed changes to the then 4-year-old’s appearance.
“[Josie] woke up one morning to go to preschool and her older sister and I noticed that the one side of her face, especially her eye area, was pretty puffy,” Heather recalled.
“If it were one of my other daughters, I probably would have let it go and just kind of kept an eye on it, but my husband and I thought it was odd since it was the first thing in the morning and she hadn’t eaten anything yet to react like that.”
The couple, who both have medical backgrounds, couldn’t figure out a reasoning for the reaction and ultimately decided to take her to the doctor.
By the time the Dolena’s got to the pediatrician, Josie’s swelling went down. Her seemingly ‘normal’ physical appearance stumped her pediatrician, who requested a urine sample as a precaution.
But the swelling came back the next morning with a vengeance and getting the urine sample was next to impossible. That’s when Heather instinctively knew something was not right with her daughter.
“I kept giving her water and lemonade, it took multiple cups to get her to be able to go,” Heather said.
After numerous tests, the lab work results confirmed the worst: Josie had Nephrotic Syndrome.
The suggested treatment plan started with high dose prednisone for roughly six months, but being a child and adolescence psychiatrist, John was very hesitant about his daughter undergoing such strong medication. He has seen first-hand the mental and physical effects prednisone has on young children.
After meeting one-on-one with Josie’s doctor, John reluctantly decided to go forward with the steroids, as there is no other option available to those with Nephrotic Syndrome or other protein-spilling kidney diseases.
They were pleased when Josie’s protein spillage levels considerably dropped within just days of starting the medication.
“The biggest challenge through all these years has always been the prednisone. The nephrologist said, ‘there’s going to be side effects, it’s a tough drug to take.’ I’m a nurse, I knew it was going to be a tough drug to take, but I was not prepared for daily life with prednisone and how it affects everything, every day, all day long. We’re blessed that she reacts to it, but the side effects are just brutal. It’s a double-edged sword,” Heather explained.
After her first round of prednisone, Josie went into remission and stayed in it for 15 months.
“We were thinking maybe she’ll be one of those kids who only has the initial flare up and they never relapse again. I knew it was a small number, but we were almost getting to that,” Heather expressed.
Unfortunately, that hope quickly vanished as Josie experienced her first relapse after eating something that contained and/or touched dairy. She was quickly put back on prednisone again.
“Mentally the prednisone was tough on her. When she was very young, she would say ‘I feel sad and I don’t know why.’ She’d say, ‘I’m worried I’m going to have to start taking thatmedicine again,’” Heather remembered.
Amid the side effects, Josie responded quickly to prednisone. But just as she was weaned off, she started spilling about a month later. That’s when the brigade of relapses began.
“There was a period of time, for about 8 to 12 months, where she just would come off [prednisone] and within 4 to 8 weeks would relapse again. So, we got to the point, with the third relapse, where we started talking about moving to a second line drug,” Heather said.
After her eigth relapse, the Dolena’s added Cytoxan into Josie’s treatment plan. Finally, after some scares during the bad flu season, the now nearly 11-year-old is in remission!
“We’re at about 18 months now. We’re kind of coming up on the period where it seems like the last few relapses happened— that 15 to 22-month mark. I’m hoping to just keep going,” Heather said.
Although Josie is doing well at the moment, Heather knows the drug will cause anxieties to linger, “she associates any medicine with what prednisone has done to her and she tries to avoid medicine at all costs.”
The disease also causes anxieties to surface for Heather as well.
“I wonder, are we trying to make things as normal as they can be for her? I don’t want her to focus on the illness, I don’t want that to define her. I tend to overthink as a parent,” she said.
Turning to dance and swim as physical outlets, Josie recently competed in the county championship for butterfly stroke. As far as Heather and John’s outlets go, they turn to NephCure.
“You’re not alone,” Heather confirmed. She encourages other parents going through the same thing to, “Talk to other parents. Chances are there’s a ton of them that have a ton of experience going through what you are.”
Heather recently became the Patient and Engagement Volunteer Lead for the Pennsylvania and New Jersey region. She hopes to help as many of those affected by Nephrotic Syndrome as she can, making it her mission to remind them to seek treatment from a specialist, as well as get a second opinion.
“With the new patients I come in contact with I try to tell them to make sure they’re seeing a NephCure Specialist. A lot of nephrologists aren’t necessarily a specialist. I refer them to KidneyHealthGateway.com. There might be clinical trials that they’re eligible for,” she advises.