Cincinnati Children’s Goes the Distance for Finley and Her Family

Cincinnati Children’s Goes the Distance for Finley and Her Family

Finley Biddle, age 2, is a firecracker. The curly-haired little girl comes by it honestly. Her mother, Brooke, has had to fight for her daughter ever since she was born in October 2018.

Finley has Hurler syndrome, also known as mucopolysaccharidosis Type I, or MPS1. It’s a lysosomal storage disease characterized by the body’s inability to make alpha-L iduronidase, an enzyme that breaks down sugars. Without this enzyme, the sugars build up in cells and cause damage throughout the body. The condition is life-limiting—there are treatments but no cure.

Many states do newborn screening for MPS1, but Indiana, where the Biddles live, did not require it at the time.

“Finley was a very raspy, noisy breather when she was first born,” says Brooke, a single mother. “She had five double-ear infections in five months and was very sickly. I noticed she had a little hump in her lower spine. When I asked her pediatrician about it, I was told that all kids have a weak area in their lower back, so it was nothing to worry about. As a first-time mother, I was unsure, but it didn’t seem right to me.”

Brooke, who was in nursing school at the time, took advantage of her spring break to drive from her home in Terre Haute to Riley Children’s Hospital in Indianapolis so Finley could be seen by an orthopaedic specialist. They initially diagnosed her with double hip dysplasia, congenital scoliosis, kyphosis (outward curvature of the spine) and a positional club foot. But after some discussion with colleagues, the doctor suspected there could be something more going on and recommended that Finley see a geneticist. Those tests revealed that she had MPS1 and got her a referral to Cincinnati Children’s.

Starting a Long Journey

The drive from Terre Haute to Cincinnati is 3 hours on a good day, according to Brooke. She and Finley wore a path back and forth between the two locations in the spring and summer of 2019 as Finley underwent enzyme replacement therapy (ERT) once a week. But while the treatment helped her body, it did not protect her brain from potential damage. That would require a hematopoietic stem cell transplant. So, along with the weekly ERT infusions, there were appointments with Cardiology, Ophthalmology, Neurology and other specialties for workups that would clear her for that procedure. The transplant was performed in July when she was 9 months old.

Finley was very sick, but she came through it like a rock star, says Brooke. “Finley is very strong-willed, very feisty and fierce. She thinks everything is funny, and she has the kindest heart. Nothing stops this kid.”

But every day brought more challenges.

As Finley grew, the outward curvature of her spine increased, the result of abnormal growth of one of the vertebrae in her lumbar region. It’s called a gibbus malformation. Brooke noticed that, after a 15-minute ride in the car, Finley’s back was beet red where the gibbus was located.

“Finley has a high tolerance for pain, so she wasn’t complaining,” says Brooke, “but she was not content in her car seat. I knew that something had to be done, especially with all the long rides back and forth from our house to Cincinnati and back.

The problem was not easily solved. Because of Finley’s size and weight, it was safer for her to be rear-facing in her seat. Brooke searched for an adaptive seat designed for kids with special needs, but Finley was too small for them, and they were all forward-facing. That’s when she contacted the Perlman Center, who put her in touch with Angela Campos, RN, CPST, an injury prevention specialist in the Comprehensive Children’s Injury Center. Angela and her team help families in the hospital and the community to obtain and properly fit their children for car seats and educate them on how to install them correctly.

“Angela was so accommodating,” says Brooke. “Finley was scheduled to come in for a sedated MRI. Angela gave me her personal cell phone number and told me to call her when Finley came out of surgery so she could measure her and see what our options were.”

Angela turned to Evenflo, a car seat manufacturer and long-time partner of Cincinnati Children’s car seat program. The company had recently introduced a special waiver program that allowed specific modifications to be made to certain traditional car seat models for children with special needs. The waiver states that parents understand the modified car seat has not been crash-tested, that the modifications should not impact its safety or performance and that the parents are accepting the seat in this condition.

When Angela contacted Evenflo, they were already working with a patient in Michigan. But Finley would be the first to actually use the waiver.

Says Angela, “Evenflo helped us identify a suitable, rear-facing car seat and instructed us on what type of foam we should buy to adapt it for Finley. I want to be very clear that no one should ever modify a car seat without the assistance of the car seat manufacturer and a child passenger safety technician.”

Angela collaborated with the Perlman Center to use their pressure-mapping machine. The machine identified Finley’s pressure points so the additional foam could be placed accordingly.

The end result was clear the first time Finley used the new seat—no redness, no pain.

A Taste of Freedom

Says Brooke, “I can’t tell you what a godsend this has been for us. Before getting this adapted seat, I wouldn’t take her anywhere because I knew her back was hurting. But now she falls asleep in her car seat, and that has never happened before.”

Given the multiple challenges Finley and Brooke have faced, the fit of a car seat might not seem to rank high in terms of impact, but Brooke says otherwise.

“It opens up so many possibilities. Finley is only 2, but I’ve always wanted to take her to the beach. She’s lived in a hospital for months, and we’ve lived in a bubble for what feels like forever. Before getting this car seat, it would have been cruel to attempt a 12-hour drive to Florida, but now it feels like it’s feasible.”

Brooke has high praise for everyone she’s encountered at Cincinnati Children’s who has been involved in Finley’s care.

“Cincinnati Children’s saved my kid’s life,” she says. “Every time I’ve called with an issue, they do everything they can to resolve it or get us an appointment as soon as possible. They never make me feel like I’m overreacting or belittle me about my concerns. Since Finley’s transplant, we have not been back to her pediatrician. We come here, even for something as simple as an immunization.

“It’s wonderful to be in a place where I don’t have to second-guess her care. That peace of mind is worth the world to me, and if it means driving 3 hours to get here, that’s okay. I’ll do it every day of the week if I have to.”

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