AMC is a number of rare muscle disorders that can cause stiff joints and abnormal muscle development in children. It occurs in about one in every 3,000 births. Children with AMC are born with a limited range of motion in any or all of the following: wrists, hands, elbows, shoulders, hips, knees or ankles.
As Sophia was born with this condition, she started coming to Shriners Children's Philadelphia when she was 1. Over the years, Sophia has had surgeries on her elbows, knee plates, hips and legs. She has worked with a plethora of surgeons at the hospital, and continues to regularly see Sarah Nossov, M.D. One recent surgery shortened the femur in each leg so the bones can regrow evenly. This will aid in any future walking for Sophia.
Sophia and Dr. Nossov share a special bond that started when Sophia first arrived at Shriners Children’s Philadelphia. They share a “shrineaversary.” Sophia started coming to the hospital when Dr. Nossov started working there. Lacey, Sophia’s grandmother, said, “Dr. Nossov really took Sophia under her wing. They started at the same time, so they kind of grew up together at Shriners.” Dr. Nossov said, “It’s been so wonderful to be a part of her life and watch her grow up. She has had a lot of bravery going into surgeries, and maintains her 'princess-like attitude.' I know she will go far in life.” When Sophia was asked what she would say to Dr. Nossov, she said, “Thank you, thank you for helping me to walk.”
Sophia has had yearly visits from Michigan to our hospital for surgeries and follow-up appointments, but her current stay has been her longest. Sophia has been here for nearly five months because, in addition to surgery, she attends regular physical therapy (PT) and occupational therapy (OT) appointments. In PT, Sophia prepares her body to learn to walk for the first time. She works with Tate Dawson, DPT, on stretching her legs with weights on them to lengthen her muscles, as they are naturally shorter from her hip to her knee.
In occupational therapy, Sophia works with Meghan Gossenberger, OT, MS, OTR/L, on completing daily tasks independently. Lacey explained that Sophia wants to do everything by herself and doesn’t like help from others. In OT Sophia learns to put a shirt on herself, feed herself and do other daily tasks. In OT, they also set goals and work towards them, such as transferring from her wheelchair to her bed or a seat. Sophia has a perseverant personality and works diligently toward her goals, never giving up until she achieves them.
I know she will go far in life.
Another major contributor to Sophia’s independence is Robert Townsend, wheelchair and seating specialist at Shriners Children’s Philadelphia. Originally, Sophia had a manual wheelchair, but she found it was difficult to push herself. Whenever a child has a problem with a wheelchair, Rob finds a solution. He gave Sophia an electric wheelchair, with a joystick that allows her to independently move at varying speeds. One of Sophia’s favorite activities is zooming through the hallways in the hospital in her new wheelchair.
Sophia also visits Pediatric Orthotic and Prosthetic Services (POPS) for custom orthotics that she wears all hours of the day. Sophia’s favorite color is pink, so she chose pink orthotics. Part of POPS’ mission is to allow each child to customize their assistive devices, which supports their self-expression. Lacey said that Calli Clark, director of POPS, is “very patient and listens to what Sophia needs.”
An exciting new development in Sophia’s medical journey is that she was recently cleared to bear weight on her legs for the first time. This brings her closer to taking her first steps. She successfully stood for seven minutes by herself in her first weight-bearing PT session. The smile that lit up across her face was palpable during this momentous occasion.
Lacey and Makenna, Sophia’s mom, cannot express enough appreciation for this hospital and its staff. Lacey said, “We love Dr. Nossov, she’s great. She’ll come up and hug us after surgery, she truly cares about her patients. It’s not just a job, she sees them all the way through. She really cares about them, it’s not just about work.”
At Shriners Children’s Philadelphia, the staff cultivates a positive and friendly environment for patients and families. Lacey said, “Our quality of care has been very good, from the shuttle drivers to activity directors, family services, everybody. I’ve not had one bad interaction with one person. This place has been great, helpful, friendly, supportive. They make sure the caregivers are okay too, because it can be lonely. There are adults to talk to here.”
For fun, Sophia enjoys spending time with her little sister, Isabella. She also loves playing Barbie games on her iPad, and reading her favorite book, 5-Minute Princess Stories. Additionally, she enjoys coloring pictures with her mouth, as she has limited ability to grasp coloring utensils with her hands. In the future, she wants to be a veterinarian because she loves animals, especially horses.
We will be cheering her on as she grows closer to accomplishing her goal of taking her first steps! With her determination and tenacity of spirit, we know she will succeed.
Sophia stands during a physical therapy session.
Sophia and her sister play at the beach.
