Cutting-edge device approved for compassionate use saves leg, life of young girl

When 7-year-old Naomi Adrian took a spill on the school playground earlier this year and a schoolmate fell on top of her left leg, she got up with a slight limp.

After what appeared to be a bruise kept growing, and as Naomi continued to walk awkwardly, her mother, Maria Nino, took her to the doctor.

A subsequent visit to a specialist revealed a tumor – an osteosarcoma, a type of bone cancer — on Naomi’s left distal femur, the area of the leg just above the knee joint.

When a doctor who was supposed to remove the tumor at a hospital near their home was unable to see Naomi on the day of the planned surgery, Maria rushed her to CHOC.

This began a medical journey for Naomi that, among other lessons, would give her and Maria a firsthand look at CHOC’s commitment to outside-the-box thinking when it comes to patient care, including investigational therapies, diagnostics and cutting-edge devices — as well as the benefits of CHOC’s partnership with UC Irvine.

Naomi’s lead physician at CHOC, Dr. Amir Misaghi, an orthopaedic surgeon with specialties in pediatric orthopaedics and musculoskeletal oncology, was able to secure a cutting-edge device — that doesn’t have U.S. Food & Drug Administration approval yet for use in pediatric patients — to help him successfully remove the tumor and reconstruct Naomi’s leg. He had used the cutting-edge device, Onkos Surgical cutting guides, before, but this would be the first time using it on a pediatric patient. These guides help surgeons make extremely precise cuts to remove tumors and surrounding bone.

Naomi with her mom and her CHOC doctoer
Naomi with her mom and Dr. Misaghi

When creating a personalized treatment plan for Naomi, Dr. Misaghi ruled out several surgical options that he deemed less promising and more burdensome on Naomi’s growing body. These included a rotationplasty, which would have resulted in the loss of her knee joint and a large portion of her leg, and required a prosthesis. He also ruled out another technique that likewise would have meant the loss of Naomi’s knee joint and a prosthesis that would have required several more surgeries as Naomi grew.

Dr. Misaghi then applied for a one-time compassionate use trial request with the FDA, which was approved.

In the surgery, Dr. Misaghi, assisted by CHOC orthopaedic surgeon Dr. Carl Weinert, used the Onkos 3D resection guides which had been customized to Naomi’s leg and the growing tumor on it, and removed the baseball-sized tumor and surrounding bone. This allowed them to spare Naomi’s knee joint and the part of her bone that grows through adolescence.

Dr. Raj Vyas, chair of plastic surgery at CHOC and vice chair of plastic surgery at UCI School of Medicine, then began to reconstruct Naomi’s femur. To do this, he dissected free a segment of Naomi’s fibula bone – which runs from the knee to the outside of the ankle, parallel to the tibia or shin bone — along with the artery and vein that supply blood to that segment of bone.

The fibula bone can be mostly removed without affecting someone’s ability to bear weight.

Drs. Misaghi and Vyas then worked together to hollow out a humerus bone – which runs from the shoulder to the elbow — and insert Naomi’s fibula bone into its core. The humerus bone was custom selected from a cadaver to match the size of Naomi’s femur and provide structural support to her leg. Dr. Vyas then connected the artery and vein supplying the fibula bone to a nearby artery and vein in Naomi’s leg using microsurgical techniques. This allowed the vascularized fibula to “bring back to life” the cadaver’s humerus bone so that it can continue to grow with Naomi.

“This was pushing the envelope as far as reconstructive surgeries go for this type of thing,” Dr. Misaghi says.

Dr. Vyas spent 10 hours working on Naomi’s leg.

“In kids, it’s especially an advantage to use their own tissue if possible. If we didn’t have the ability to do this miscrosurgery, then we would have just done an inferior operation,” Dr. Vyas says. “Being able to work together and plan to do this at a children’s hospital with Dr. Misaghi’s expertise and our expertise at UCI Health, we were able to provide Naomi with a state-of-the-art reconstruction.”

He added, “A while ago, maybe at some hospitals they would have just performed an amputation. Because we have Dr. Misaghi, who can do a limb salvage using a cadaver graft, we were able to really perform the best operation possible.”

A very active child, Naomi loved to participate in ballet and gymnastics before her diagnosis.

“Hopefully we can get Naomi back dancing,” Dr. Misaghi says. “That would be the goal.”

Trips to the park

These days, as Naomi continues to undergo chemotherapy, the now-8-year-old enjoys trips to the park where her older sister, Itzel, pushes her in her wheelchair.

Naomi, whose family has since relocated to the Fresno area, also loves arts and crafts, her dolls, and playing with her twin sister, Natalie.

twin sisters
Naomi with her twin sister Natalie, during a pre-pandemic trip to Disneyland

“I felt sad, but I knew that someone was always with me,” Naomi says of her cancer diagnosis and journey. “Thanks to Dr. Misaghi, they saved my leg and I’m feeling happier now.”

So far, Naomi is progressing very well, Dr. Misaghi says, who keeps in regular contact with her. A year out from surgery, Naomi will be back at CHOC for a second surgery to remove screws that were placed near her growth plate.

“It remains to be seen how her growth plate responds,” Dr. Misaghi says. “But the survival part of her prognosis is very good, since she had clear margins and she’s resumed chemotherapy, and so the function of her leg prognosis is very good. She has a normal knee joint. And hopefully when everything heals, she’ll have some metal plates remaining but be able to grow normally with her own growth plate.”

Naomi says her leg feels “good” but that she can’t walk yet – maybe in a year, though.

Itzel says Naomi has been a champ through the entire process.

“She never complained about pain even after the surgery,” Itzel says.

Itzel and her mother are grateful for the excellent care Naomi received at CHOC.

“We knew that it would be very hard for Naomi to accept losing her leg,” Itzel says. “She’s so young and she’s such a girly girl; she’s always worrying about how she looks and everything. We were happy that there was a way that that could save not only her life, but her leg.”

Mom Maria echoes that feeling of gratitude.

“We are extremely grateful to God for listening to our prayers and for the amazing doctors, nurses and staff at CHOC. I want to especially thank Dr. Misaghi and Dr. Vyas as they made sure Naomi got the care she needed. They are a great team,” she says.

Naomi already knows what she wants to be when she grows up.

“I want to be a surgeon so I can save other people’s hands and legs,” she says.

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From hotline to surgery – How CHOC guided a family through an emergency during COVID-19

As the parent of a child with gastrointestinal issues, Kris wasn’t immediately concerned when her son Nolan complained of a stomachache while they were watching TV together. When his pain quickly got worse and Nolan started to vomit, Kris knew it was time to call CHOC.

“With COVID-19 going on, I didn’t know if the pediatrician’s office or emergency room was the best place to bring him. I didn’t want to take a spot in the waiting room from someone who needed it more than us,” Kris said.

Nolan_with_Kris
Nolan with his mom Kris

Luckily, Kris had noticed on social media that CHOC had implemented a COVID-19 hotline (1-844-GET-CHOC) staffed by a team of registered nurses. She called the hotline for advice on where to safely seek care for her son during the pandemic.

“As the parent of an immunocompromised child, I knew CHOC could help,” Kris says. “A nurse took our call, and within a few minutes we were on a Zoom video call with a doctor. She gave us very clear parameters of what to watch for and when to call back. Nolan’s symptoms got worse, and when we called back, we were immediately patched through to a video call with a pediatrician.”

Dr. Lori Openshaw, a CHOC Children’s pediatrician, spoke with Kris and Nolan via video chat. After taking a medical history, Dr. Openshaw was able to walk Kris through doing an abdominal examination on Nolan, while the physician closely observed via video. With Kris’ help, Dr. Openshaw better understood the location and intensity of Nolan’s pain, and what might be causing his symptoms.

“Dr. Openshaw told me exactly what to do. She told me where to push on his stomach and where not to push. She paid attention to Nolan’s facial expressions during the exam,” Kris says.

Dr. Openshaw highly suspected that Nolan may have appendicitis and needed an urgent surgical evaluation. She directed Nolan and his mom to get to the Julia and George Argyros Emergency Department at CHOC Children’s Hospital quickly.

“By the time we got to CHOC, they were ready for us,” Kris says. “Dr. Openshaw had called the emergency department and told them we were coming. She gave us instructions to wait in the car at valet, and a staff member would come to the car and ask for our name. It was clear that everyone from valet to security was following protocol implemented to keep people as safe as possible during this pandemic.”

Nolan’s room in the emergency department was ready and waiting for him. He and his mom noted how everyone in the emergency department, and the medical unit to which he was admitted overnight, was calm and collected, which in turn helped them feel calm during a stressful health emergency.

Although Nolan has been a patient at CHOC many times before, he and Kris noticed that a few things were different about this trip to CHOC. To protect the health of patients, families, doctors, nurses and staff during the pandemic, CHOC had implemented a variety of safety measures. Kris and Nolan noticed that everyone underwent a health screening before entering the hospital, people wore masks (and more protective equipment when necessary), social distancing measures were in place with people staying 6 feet apart whenever possible, and additional cleaning measures were carried out on top of CHOC’s already stringent cleaning practices.

“Seeing people go above and beyond to keep patients safe, healthy and comfortable during this time is what makes CHOC so different and so special,” Kris says.

Testing confirmed Nolan did indeed have appendicitis, and his surgery was scheduled for the next morning with  Dr. Mustafa Kabeer, a pediatric general and thoracic surgeon. Surgery was a success.

Nolan_aftersurgery
Nolan was in good spirits while recovering from surgery.

Nolan appreciated that staff made him feel as comfortable as possible during his stay.

“CHOC sees you as a person, not just a patient,” Nolan says. “Everyone explained to me what would happen and what medicine I would get. They made it easy for me to understand what was going on, which made the process feel smooth.”

Having a hospitalized child during a pandemic isn’t something Kris envisioned, but she knew Nolan was in good hands.

“It gave me peace of mind that there was consistency in protocols from each area of the hospital that we interacted with,” Kris says. “Everything was well thought out, and it was clear that everyone had bought into the changes being implemented throughout the hospital.”

Kris noticed that staff were reminding each other of changes in protocol, which further reassured her that the hospital was a safe place.

“Hearing nurses and other staff remind each other of things – whether that be a change in protocol, or where an item had been moved to – reassured me that the decisions CHOC had made to keep people safe were being upheld throughout the hospital.”

Nolan shares in his mom’s appreciation for CHOC.

“You never want to be sick, but if you’re going to be sick, you want to be sick at CHOC,” he says.

Nolan only had to stay at CHOC one night after surgery for observation and went home the next day. Dr. Openshaw, although not Nolan’s primary pediatrician, called several times to check up on him after surgery.

“Dr. Openshaw’s care and follow-up made us feel comfortable and assured,” Kris says.

Today, Nolan is back at home, getting reacclimated to distance learning while his school is closed – he’s also learning how to cook and do laundry before he heads off to college in the fall.

Kris is happy to have this health scare behind them, but grateful she knew who to call when her son was sick.

“CHOC has always known what is best for my child. No matter what is going on around us – even a pandemic – doing what’s best for my child will always be CHOC’s priority,” Kris says. “I knew the only place I would trust information from during this time was CHOC. The fact that they had set up a COVID-19 hotline for parents was just another sign that they are always three steps ahead in every situation.”

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