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.

Explore the Orthopaedics Institute at CHOC 

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For inspiring cancer survivor, it’s 1st and goal

Israel, a standout athlete, spent his junior year of high school fighting cancer and returned home just before the onset of COVID-19. Now a senior, he’s working to get back on the football field and grateful to have his cancer journey behind him.

Every morning, 17-year-old Israel begins his day by lifting weights and, on alternate days, adding in a two-mile run.

Then, it’s time for a hearty breakfast fit for any teen bulking up for football season – four or five eggs, bacon and two slices of toast.

Israel Escobedo, a high school senior, then flips open his laptop for virtual classes.

In the afternoon, he again pumps iron to add muscle to his 6-foot-1 frame.

Israel, a middle linebacker, is preparing himself for a 10-game season that may or may not start in December, depending on COVID-19 guidelines.

That he’s in such great shape – just 15 pounds short of his usual weight of 200 – is something to behold, considering that earlier this year Israel underwent a bone marrow transplant (BMT) after being diagnosed with high-risk B-cell acute lymphoblastic leukemia (ALL).

Israel’s journey has been one of baby steps, hurdles and at times, low points.

But through it all, his clinicians at the Hyundai Cancer Institute at CHOC say Israel has exhibited grace, kindness and a positive attitude, despite the fear and pain that comes with any cancer diagnosis.

Israel saw his grandfather Jose battle prostate cancer for eight years before he passed away several years ago. So, when Israel found out last year that he had leukemia, he was floored.

“Honestly, I was just scared,” Israel says. “I’ve always had big goals and plans. I was always outgoing and healthy. I didn’t know what to expect.”

But how Israel handled his diagnosis and subsequent BMT – a process that can bring with it a grueling recovery process, with a brand-new and vulnerable immune system, especially for the first 100 days – has inspired his team at CHOC.

“My goodness,” says Miranda Wichelns, a social worker who helped care for Israel. “We’re all kind of in awe of him.”

A shining example

Israel was not able to attend the first day of in-person varsity football practice this season, but his pediatric oncologist Dr. Rishikesh Chavan hopes that Israel may be able to practice with his teammates soon.

For Dr. Chavan, who also serves as director of CHOC’s Blood and Marrow Transplant Program, Israel’s excellent recovery from his BMT is a shining example of how most BMT patients eventually resume normal lives.

“A lot of people think of a transplant as a big, bad thing,” Dr. Chavan says.

With the proper medical care, it’s not.

“When you look at Israel today,” Dr. Chavan says, “you would not be able to tell that he had a bone marrow transplant just eight months ago.”

A perfect match in the family

Back in August 2019, Israel wasn’t feeling his usual healthy self. He found it difficult keeping down food.

His dermatologist, who had been checking Israel’s blood monthly because of the acne medication he had been prescribed, noticed an alarmingly low number of white blood cells.

Israel’s primary care physician was also very concerned and referred him to CHOC.

On Israel’s first night at CHOC, his child life specialist Carly helped ease his jitters.

The next day, pediatric oncologist Dr. Carol Lin diagnosed Israel with ALL.

Israel underwent several rounds of chemotherapy before Dr. Chavan and Dr. Lin, along with the leukemia team, determined he needed a BMT.

Israel receiving treatment at CHOC
Israel receiving treatment at CHOC

The actual day of BMT is relatively simple, akin to receiving a blood transfusion – but a lot goes on before and after the procedure.

Israel needed a donor and his sister Genesis turned out to be a perfect match.

“I just didn’t want to sit around and see him suffer,” says Genesis, who was 14 at the time of the transplant. “It was good to know I could actually help him.”

Israel is forever grateful for his sister.

“I love her,” he says, adding with a laugh, “but there are days I can’t stand her.”

Undergoing a bone marrow transplant

 The process of donating her blood-forming cells – or blood stem cells – for transplantation wasn’t too painful, Genesis says.

After a BMT, a recipient’s immune system becomes like that of a newborn baby. The immune system needs to develop and be cared for to keep toxicities from wreaking havoc on the body.

Dr. Chavan uses the metaphor of gardening to describe a BMT.

“It’s like taking a seed from a plant and then replanting it somewhere else,” he says. “Think about the cancer cells as weeds in your garden. Before the transplant, through a combination of chemotherapy and radiation, you are not only able to get rid of the weeds, but you are also preparing the soil. Then you plant the new seed.”

Keeping a positive attitude throughout treatment

 A common complication following a BMT is Graft-versus-host-disease (GVHD), in which the recipient’s new immune system, which is actually the donor’s immune system, may attack the recipient’s body. Symptoms include rash, mouth ulcers, abdominal pain, vomiting and diarrhea. Steroids and other immunosuppressive medications are used to treat the condition while preventing infections.

Israel had a moderate case of GVHD but bounced right back.

His lowest point, he said, happened when he returned home from CHOC just before the COVID-19 pandemic took hold in mid-March. The emotional toll of his journey finally sunk in, and it took time to adjust to a new normal.

“But I got through it and I feel amazing now,” he says. “I put myself to God, and He got me through it.”

Israel near the ocean

Israel credits his close family — Genesis, his father Juan, his mother Marcia and older brother Joshua — and his team at CHOC for helping him get through his medical crisis.

“They have some of the most amazing people there,” Israel said of CHOC. “They made me feel like I was at home. They’re great people.”

CHOC staffers noted how polite Israel was throughout his treatment and BMT recovery.

Monika Benson, a registered nurse, was Israel’s post-transplant case coordinator.

“He’s an incredible young man,” Benson says. “His outlook during this has been amazing. He’s one of the sweetest kids I’ve met. A lot of bone marrow transplant patients get beat down. He had his down moments, but he remained positive through it all.”

Benson noted that BMT patients go through a lot of social isolation. Most can’t even leave their hospital room for 10 to 20 days post-transplant while their new immune system is at its most vulnerable level.

“Going into the procedure positive and coming out of the transplant positive can make a big difference,” Benson says.

Israel says with his positive attitude, he was simply demonstrating lessons he’s been taught since day one.

“My whole life, I’ve always been taught manners and to respect my elders. I must respect others so I can be respected,” Israel says. “And I’m a very positive and energetic person. I’m always moving with a smile on my face every day.”

Israel with a CHOC nurse
Israel with one of his CHOC nurses Alex, in a photo taken before the COVID-19 pandemic

The importance of wearing a mask

 Living through a pandemic – while recovering from cancer treatment and a BMT – has its challenges, but one thing Israel was already used to doing even before COVID-19 hit was wearing a mask.

He recalls one day earlier this year, when he was watching his teammates practice amid his chemotherapy treatment leading up to his transplant. He didn’t wear a face covering that day and got so sick he had to be hospitalized.

“I believe masks are a big help,” he says.

Masks have always been a common recommendation and practice for post-BMT patients at least in initial days post-transplant.

Looking ahead

Before he was diagnosed, Israel was looking forward to playing football in college or possibly joining the military.

Now, he wants to become a nurse.

“A lot of nurses helped me get through this,” he says. “I want to return the favor and help kids that are going through the same thing that I went through.”

Learn more about the Hyundai Cancer Institute at CHOC Children's

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  • 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 ...
  • Superhero Austin fights cancer
    When you arrive to the hematology and oncology unit at CHOC Children’s Hospital, there’s someone you won’t miss — and if you do, he’ll make sure you notice him.  Three-year-old Austin, a CHOC patient, is a ...
  • A gain against pain
    A child, resting in bed, fires up her 7-inch tablet and opens an app. She selects from a variety of cartoon avatars — such as a panda or penguin — and ...

Superhero Austin fights cancer

When you arrive to the hematology and oncology unit at CHOC Childrens Hospital, theres someone you wont miss  and if you do, hell make sure you notice him. 

Three-year-old Austin, a CHOC patient, is a bundle of energy and can often be found running around in a costume, jamming out to his favorite music or yelling out, Hi! to everyone who passes his hospital room. He is your typical toddler, except for one thing — he has cancer. 

Austin at CHOC Children's
Austin at CHOC Children’s

 Austin was diagnosed earlier this year with acute myeloid leukemia (AML) with FLT3-ITD and NPM1 mutations. Before his diagnosis, Austin had constant and unexplained fevers, bone pain, loss of appetite and extreme fatigue. His pediatrician, Dr. John Carruth, ordered a blood test that showed an alarmingly high white blood cell count. 

Austins parents, Kristen and John, were told by Dr. Carruth to bring Austin immediately to CHOC. 

It felt like a surreal nightmare walking onto the fifth floor at CHOC and seeing the words cancer unit,’” says KristenIt was the most afraid Ive ever been in my life.” 

Due to Austins cancer mutations, his parents were told he may need a bone marrow transplant. This kind of transplant can often be a cure for certain oncology diagnoses, but the process is not easy  especially for a young child. Thankfully, his oncologist Dr. Jamie Frediani is committed to staying up-to-date with the latest research and new studies within pediatric oncology, and she knew that children with similar mutations to Austin’s can do much better without having to undergo a transplant. 

Knowledge is constantly evolving, and with it our treatment strategy  giving patients like Austin an improved prognosis, says Dr. Frediani. 

Austins care plan moved forward with five rounds of chemotherapy, but that was just one part of the battle. A few weeks after Austin was admitted, California enacted stay at home orders due to COVID-19 and CHOCs visitor guidelines were revised in the interest of curbing the spread of the pandemic.

During the past few months, there were moments that were especially hard for Austin – but he was never fighting alone. Although he experienced nausea, an allergic reaction, a fungal infection in his lungs, high fevers, painful mouth sores and overall body pain, Austin and his parents had an entire team fighting alongside them.  

The staff, doctors, nurses, child life specialists, music therapists and all our other cancer-fighting friends on our floor have been so loving and friendly,” says Kristen. You never want to hear your child has cancer, but if it means you get to meet wonderful and inspiring people along the way, then this entire experience is made a little easier and a lot less painful.” 

Austin at CHOC Children’s
Austin at CHOC Children’s

As much as CHOCs staff has made an imprint on Austins heart, he has done the same for them in return. While attached to his IV pole, Austin sprints through the halls daily introducing his bright personality to those around him, bravely shows off his superhero-fighting moves to all the nurses and has chosen to be happy every day for the 163 days he has been at CHOC. 

Under the care of Dr. Frediani and the oncology team, Austins chemotherapy treatment has gone well, and he was recently able to return homeGoing forward, hell have regular follow-up appointments with his care team. Austin and his parents are excited for him to start pre-school, take a karate class, play on a baseball team and continue to lift people up wherever he goes. 

As much as were excited to move on with our lives, I hope Austin remembers this experience,” says Kristen. I hope he remembers the hard things he did, the fears he overcame, the people he met. I hope he remembers just how brave he was and how much braver he will be because of this. 

Learn more about the Hyundai Cancer Institute at CHOC Children's

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A gain against pain

A child, resting in bed, fires up her 7-inch tablet and opens an app.

She selects from a variety of cartoon avatars — such as a panda or penguin — and backgrounds that include a colorful ocean floor with fish and other sea creatures.

Game on.

But this isn’t a typical game. It’s a kid-friendly tool that allows the child, who is being treated for cancer, to report the severity and type of pain she’s experiencing from her home — information her doctor can access in real time.

The app, named Pain Buddy, may aid in the reduction of pain severity in children during cancer treatment, according to results of a pilot study recently published in the online journal Pediatric Blood & Cancer.

The study found that Pain Buddy may be especially beneficial in helping children who have high levels of pain.

Pain Buddy is the brainchild of Michelle A. Fortier, a CHOC Children’s pediatric psychologist who is also a faculty member of the UC Irvine Sue & Bill Gross School of Nursing.

A screenshot of the Pain Buddy app
Pain Buddy app

Fortier, who specializes in pain management in children, was principal investigator of the recently published pilot study that was based on clinical studies of CHOC patients monitored by pediatric oncologist Dr. Lilibeth Torno and pediatric oncology nurse practitioner Christine Yun.

“Pain management is an important part of cancer survivorship, and I think Pain Buddy’s potential for use is very broad,” Dr. Torno says.

Most of the 48 children participating in the eight-week study had been diagnosed with leukemia. All were between the ages of 8 and 18. Results of this particular study come amid ongoing studies on the Pain Buddy app at other sites. Results of the comprehensive research effort, which will track 206 children, are expected in three years, Fortier says.

Pain Buddy, Fortier explains, was developed a few years ago to address a gap in pain management of kids at home compared to kids in the hospital, where it’s easier for doctors and nurses to stay on top of patients’ needs. The 48 children who participated in the pilot study spent a lot of time at home.

Tapping the expertise of professional app developers and researchers at UCI in the California Institute for Telecommunication and Information Technology (Calit2), Fortier and several other colleagues came up with a way for children to rate their pain as they were feeling it from home.

“Most kids experience pretty moderate to severe pain throughout their cancer treatment, and this pain just wasn’t sufficiently being addressed when the patients were at home,” Fortier says. “And when we think about pain assessment, we’re really terrible retrospective reporters of our pain experience.”

But with Pain Buddy, users can say how much they’re hurting, and where, as it’s happening.

“Pain can come from the cancer itself, such as a solid tumor, and it can come from treatment procedures,” Fortier says. “For example, lots of skin-breaking procedures occur during cancer treatment. And treatments like chemotherapy can cause nerve pain, inflammation of the gastrointestinal tract and mouth sores.”

In addition to completing a pain and symptom diary twice daily, the app automatically alerted the participants’ medical teams about such symptoms as nausea, itching, sadness and redness.

With a touch of a finger, the patients could select word bubbles to indicate descriptions — such as bad, annoying or terrible — to describe their pain.

A screenshot of the Pain Buddy app
The Pain Buddy app allows users to describe their pain with word bubbles, and can alert the care team.

Clinicians, in turn, could promptly address any symptoms that warranted intervention.

A key component of the Pain Buddy app, which for now only has been used by the pilot study participants, is the incorporation of coping skills shown to be effective in the management of pain, such as deep breathing, progressive muscle relaxation and guided imagery.

During these skills training exercises, patients could accumulate coins and, visiting a virtual store, customize their personal avatar and buy additional background themes.

A screenshot of the Pain Buddy app
The Pain Buddy app can help patients learn coping skills.

Pain Buddy represents an effective partnership between parents, young cancer patients and the health care institutions that treat them, Dr. Torno says.

“Our focus on cancer survivorship begins on the day of diagnosis,” Torno says.

CHOC’s After Cancer Treatment Survivorship (ACTS) program features a multidisciplinary team of clinical experts who monitor the late effects of cancer and develop a plan for long-term surveillance to ensure the best possible outcomes. Every child at CHOC who has gone through cancer therapy eventually lands in the ACTS program.

Fortier said the ultimate goal is to further refine Pain Buddy and license the app to hospitals for widespread use.

“The goal is to have every kid undergoing cancer treatment — from sarcoma patients to those with bone and other cancers — to have the ability to use Pain Buddy.”

Learn more about the Hyundai Cancer Institute at CHOC Children's

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9 ways to protect immunocompromised children from COVID-19

We know how frightening the COVID-19 pandemic may be for parents – especially parents of immunocompromised children.

At this time, the full impact of COVID-19 on children, especially those with compromised immune systems, is unknown. However, limited information so far shows that most healthy children with the virus have done well.

Here are some recommendations for steps to take to help protect immunocompromised children from COVID-19:

  1. Call your provider

Call your child’s care provider first if your child has a runny nose or cough. Go to the emergency department if a cough or runny nose is accompanied by fever, or if you are advised to by your doctor, or you believe the situation is emergent.

  1. Follow through with medical appointments

If you have medical appointments that are important to your child’s care, you should attend them. Many providers are offering telehealth appointments. Call your provider’s office to see if this is available, and if your appointment can be conducted through telehealth.

  1. Practice good handwashing

Everyone in the home should consistently wash their hands with soap and water for at least 20 seconds. This is one of the most effective ways to stop the spread of illness. If you don’t have access to soap and water, hand sanitizer is a good alternative.

  1. Cover coughs and sneezes

Cough and sneeze into your inner elbow – and teach children to do the same. It’s also important for everyone to avoid touching their mouths, noses and eyes.

  1. Disinfect high-touch surfaces

It is important to disinfect surfaces that are commonly touched such as the cell phones, tablets, game controllers, doorknobs, light switches, tables and counters.

  1. Practice social distancing

Orders from the governor of California for all state residents to stay home — except for essential needs, such as if your work supports the continuity of critical infrastructure sectors, or if you need to access essential services like food, pharmacies, banks or laundromats – are in place until further notice.  Read more here.

If you must leave your home, wear a mask and maintain at least 6 feet of distance from other people whenever possible.

  1. Follow guidance around face masks

The Centers for Disease Control and Prevention (CDC) recommends wearing cloth face coverings in public settings like grocery stores and pharmacies where social distancing measures can be difficult to maintain. These face coverings can slow the spread of COVID-19 and help people who may have the virus and do not know it from transmitting it to others. It is not necessary for children under the age of 2 to wear cloth face coverings.

The governor of California has mandated that face coverings be worn by the general public when outside the home. This applies to high-risk situations such as entering public spaces; obtaining medical attention; riding public transit; certain work settings; and while outdoors in public spaces when maintaining a physical distance of 6 feet from persons who are not members of your household is not feasible. Exemptions include children age 2 and younger; persons with a medical condition, mental health condition or disability that prevents them from wearing a face covering; the hearing impaired or those communicating with them; persons seated at restaurants while eating or drinking, provided they maintain physical distancing; and those engaged in outdoor work or recreation alone or with household members while maintaining physical distancing from others. Read the full order here.

The CDC offers guidance on how to properly wear a cloth face covering, as well as tutorials on how to make your own mask. See these resources here.

N-95 or surgical masks are not recommended for public use, as supplies are needed by healthcare workers and first responders.

  1. Avoid non-essential travel

We recommend following the CDC’s guidance for travel.

If someone in your family has recently traveled to an area with high COVID-19 activity and is showing symptoms of respiratory illness, it is best for you and your child, immunocompromised or not, to avoid contact with the person for at least 14 days.

  1. Maintain enough medical supplies

Ensure you have necessary medical supplies and prescription medications on hand, check levels of all your medications and let your provider know if you need refills.

This article was updated on July 16, 2020.

Get more information on Coronavirus (COVID-19)

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