Virtual pediatric lecture series: COVID-19 in children

CHOC Children’s virtual pediatric lecture series continues with a session on COVID-19 in children.

This online discussion will be held Thursday, Oct. 15 from 12:30 p.m. to 1:30 p.m. and is designed for general practitioners, family practitioners and other healthcare providers.

Dr. Antonio Arrieta, medical director of pediatric infectious disease at CHOC, and Dr. Jasjit Singh, assistant medical director and medical director of infection prevention and control, will present information on COVID-19 in children and the world of infectious disease. Particular focus will be given to counseling patients about the importance of influenza and other routine vaccines this fall. Given the current pandemic and concerns that flu season may exacerbate it, this timely lecture is ideal for providers looking to address preventative matters with patients.

This virtual lecture is part of a series provided by CHOC that aims to bring the latest, most relevant news to community providers. You can register here.

CHOC is accredited by the California Medical Association (CMA) to provide continuing medical education for physicians and has designated this live activity for a maximum of one AMA PRA Category 1 Credit™.

Please contact CHOC Business Development at 714-509-4291 or BDINFO@choc.org with any questions.

Artificial intelligence seen as critical tool in helping to diagnose rare diseases

Machine learning algorithms could make a dramatic difference when it comes to diagnosing children with rare diseases, two CHOC Children’s doctors said in a recent webinar.

Although the use of artificial intelligence (AI) in diagnosing medical conditions is in its infancy stages, the potential is huge, said Dr. Jose Abdenur and Dr. Terence Sanger, speaking on a panel during a two-week summit on rare diseases hosted by Global Genes, an Aliso Viejo-based non-profit that advocates for the rare disease community.

“Human decision making is very, very good,” said Dr. Sanger, vice president for research and chief scientific officer at CHOC. “But we’re not very good at incorporating tens of thousands of pieces of information into making these decisions.”

That’s where machine learning could be of immense value, he and Dr. Abdenur said in the one-hour discussion on Sept. 22, which can be viewed in its entirety here.

Machine learning involves the use of computer algorithms that improve automatically by building mathematical models based on reams of data. This makes AI particularly valuable for improving the rare disease diagnosis process, which remains far from perfect, says Abdenur, chief of the division of metabolic disorders at CHOC and director of CHOC’s metabolic laboratory.

Although great strides are being made in diagnosing rare diseases through such processes as rapid whole genome sequencing, 40 percent of families with sick children still do not have diagnoses, Dr. Abdenur said.

“We’re doing better, but we’re definitely not good enough,” he said. “We hope in the future that artificial intelligence and machine learning will help us (reach diagnoses faster).”

In diagnosing patients, clinicians consider a list of possible conditions or diseases that could be causing symptoms – what’s known as making a differential diagnosis. They consider such things as a patient’s symptoms, his or her medical history, basic lab results, and a physical examination.

With AI, a virtually limitless amount of information beyond that – such as similar symptoms that have occurred in patients around the world, the environment they live in, etc. – could be factored into helping make differential diagnoses.

Dr. Sanger compared the benefits of using AI in diagnosing patients to a standard camera – what’s used now – to a wide-angel lens that can take in much more information, which machine learning would provide.

“If you have an avalanche of information, (physicians) can’t take all of it in themselves,” Dr. Abdenur noted.

But a sophisticated machine-learning program could, he and other panelists said.

An algorithm that gets smarter over time would lead to faster, simpler, accurate, and earlier diagnoses, said panel member Annastasiah Mhaka, co-founder of the Alliance for AI in Healthcare.

“Data is at the heart of (learning more about rare childhood diseases), and AI would help along every step of the way,” said another panelist, Sebastien Lefebvre, an analyst with Alexion Pharmaceuticals.

Both Dr. Abdenur and Dr. Sanger agreed that AI could be used to augment a clinician’s decision, but never replace it.

“(AI) never makes a decision for you,” Sanger said. “It just assists in the decision making.”

Newly emerging technologies such as machine learning in healthcare could lead to lower healthcare costs and better treatment, Mhaka said.

“Diagnosis needs are huge and unmet in the (rare disease) population,” she noted.

Learn more about rare disease research at CHOC.

Wired for hope: deep brain stimulation for dystonia

Every morning when she awakes, Sydney Amato begins her daily battle with her body.

If she’s lucky, the 16-year-old will have gotten a handful of hours of uninterrupted sleep – dreaming, perhaps, of doing what most healthy kids her age take for granted:

Hanging out with friends. Going to school. Learning to drive.

Because of a neurological condition called dystonia, Sydney, who is in excellent cognitive health but speaks and walks with some difficulty, suffers from involuntary and near-constant contraction of muscles in her neck, arms, legs and trunk.

Sydney with her father, Louis

Her mind is unable to control the painful jerking that makes most of her body twist and go rigid, her muscles moving out of normal sequence.

Born a right-hander, she can feed herself with some struggle using her left hand. She wants to dress and put on makeup herself, but those normally simple tasks become lengthy ordeals.

“My body fights me all the time,” says Sydney, trying to distract herself in her hospital bed by watching an old episode of “Keeping Up with the Kardashians.” Listening to her favorite music – Ariana Grande, Lauren Daigle, Drake – can only temporarily transport Sydney away from her debilitating condition.

“She knows what she wants to do,” says her father, Louis. “But her body won’t let her.”

Specialists at CHOC Children’s are working hard to change that.

A first for CHOC

On Aug. 14, 2020, a team led by Dr. Terence Sanger, a physician, engineer, and computational neuroscientist who joined CHOC in January 2020 as its first chief scientific officer, and Dr. Joffre E. Olaya, CHOC’s functional restorative neurosurgeon, implanted several temporary electrodes into Sydney’s brain.

Dr. Terence Sanger, a physician, engineer, and computational neuroscientist and CHOC’s chief scientific officer

The surgery marked the first time a patient with a movement disorder at CHOC underwent a procedure called deep brain stimulation (DBS).

Working in perfect harmony as a team, Dr. Sanger and Dr. Olaya performed the first stage of a three-stage surgery on Sydney. As the surgeon, Dr. Olaya placed the leads following advice from Dr. Sanger, the neurologist, where they should go.

In the procedure, millimeter-thick electrodes were precisely positioned into the basal ganglia region of Sydney’s brain – about three inches deep. The surgery involved the use of the ROSA Robot, the same tool that has been used during brain surgery on epilepsy patients at CHOC since 2015.

Dr. Joffre E. Olaya, CHOC pediatric neurosurgeon

Considered one of the most advanced robotized surgical assistants, ROSA includes a computer system and a robotic arm. It’s a minimally invasive surgical tool that improves accuracy and significantly reduces surgery/anesthesia time.

The ROSA Robot helped with implanting and targeting the electrodes, and a portable operating-room CT scanner confirmed their position.

Turning down the volume

DBS is designed to ease Sydney’s condition by sending electrical currents to jam her malfunctioning brain signals.

Think of turning down the volume on your car radio.

“Nobody really understands the cause of dystonia,” Dr. Sanger explains, “but there’s probably too much electrical stimulation going on in the motor areas of the brain. We’re trying to calm down that extra noise.”

Although DBS dates to the 1960s, it wasn’t until the 1980s that the modern era of using it to treat adult patients with tremor and Parkinson’s disease began.

In 2000, Dr. Sanger, working with engineers, data scientists, neurosurgeons, and others, began implanting electrodes in pediatric patients.

Instead of the established method of placing the leads at predetermined sites and hoping they worked, Sanger and his team, just as they did in Sydney’s case, placed temporary leads to best assess where they should go permanently based on patient response.

In 2016, Dr. Sanger began honing DBS to treat children with dystonia. Before the surgery on Sydney, Dr. Sanger had performed DBS on 26 children using the same three-stage technique. He says 80 percent of those children have seen successful results.

Early signs

Sydney began showing symptoms of dystonia – tremors in her hands — when she was 5 ½ years old.

A year later, she was using a wheelchair. She had her first brain surgery at age 7.

Since then, “she’s been all over the U.S.” seeking the right treatment for her condition after several setbacks, says her father.

But her condition was not improving.

Early this year, a neurologist in Kansas City, Mo., recommended that Sydney see Dr. Sanger.

“I asked him, ‘If Sydney was your kid, where would you go?’ Louis Amato recalls. “He said, ‘Hands down, Dr. Sanger.’”

The COVID-19 pandemic pushed Sydney’s surgery to mid-August.

Sydney already had two electrodes in her brain that were only partially working when she came to CHOC in early August for surgery.

After two extensive run-throughs with their team, Dr. Sanger and Dr. Olaya, in a six-hour procedure that at one point had nearly 20 people in the operating room, implanted more electrodes to give her a total of nine.

On Thursday, Aug. 20, six days after Sydney’s surgery, Dr. Sanger stopped by her room at CHOC Children’s Hospital. The room was decorated in purple, Sydney’s favorite color.

Dr. Sanger greeted her as CHOC staff members, joined by members of Sanger Lab, which conducts research in pediatric movement disorders, prepared to have Sydney walk back and forth down a hallway while connected to electrical equipment programmed to record signals in her brain and muscles.

A thick coil of multicolored wires snaked from under a large white bandage covering Sydney’s head. Extending about 6 feet, the wires were plugged into specialized recording equipment controlled by Jennifer MacLean, a pediatric nurse practitioner whose job was to manipulate the strength of electrical charges affecting the four points of contact on each electrode.

The goal: determine which charges worked best and on which electrodes.

“It could have turned out that the DBS procedure made no difference,” Dr. Sanger says. “But we’ve seen a very good response in Sydney.”

For example, her once mostly useless right hand was working much better.

“It gives you goosebumps,” Louis Amato says.

After taking a bite of a veggie burger and sipping some water, Sydney started to walk.

Following her were seven CHOC and Sanger Lab specialists.

“Go nice and slowly,” Jennifer told Sydney. “You’re going too fast for us!”

Perhaps Sydney was anxious to get back to riding Tigger, a quarter horse, in her hometown of Carthage, Mo. She has been riding him for six months.

Sydney is eager to get back to riding her favorite horse, Tigger.

“Her balance isn’t bad on the horse,” says Louis Amato.

Sydney also loves to tan by her pool and swim.

What she wants most, however, is to be freed from her body so she can return to school and do what most teens enjoy.

“It’s stressful,” says her mother, Angie. “She has a lot of friends her age, but she can’t do a lot of the things they do. She has her days when she can get really upset.”

Now, however, working with Dr. Sanger, Dr. Olaya and the entire team at CHOC, the Amatos are more optimistic than ever.

“We’re hopeful that this is going to be a big life-changer for her,” Angie Amato says. “That would be the best thing that could ever happen – better than winning the lottery.”

‘The A Team’

After crunching numbers for a week to assess which of the nine electrodes proved to be the most effective based on how Sydney responded to varying degrees of electrical currents, Dr. Sanger and his team settled on four electrodes that were permanently used to treat her condition – three new ones, and one existing one.

The team performed this second surgery on Sydney in late August.

In the third and final surgery, successfully completed in early September, a rechargeable generator that powers the DBS leads was implanted in Sydney’s chest.

“As we get better and better at this,” says Dr. Sanger, “and as the technology progresses, we’ll be able to do this on kids who are less sick than Sydney.”

Dr. Sanger and Dr. Olaya are poised to dramatically improve the lives of many more patients like Sydney at CHOC.

“I’m really excited that we will be doing more of these procedures to help pediatric patients with movement disorders and significantly improve their quality of life,” says Dr. Olaya. “I look forward to continuing to provide this type of personalized care.”

Angie and Louis Amato say Sydney has never gotten this much special attention during her 11-year-plus medical journey.

“Here at CHOC,” Louis Amato says, “we feel like we’re with the A Team.”

Says Sydney: “I’ve never felt this much confidence and this good about treatment before.”

Learn more about deep brain stimulation (DBS) surgery at CHOC.

IN THE SPOTLIGHT: Dr. Amir Misaghi

Earlier this year, orthopaedic surgeon Dr. Amir Misaghi joined the CHOC Children’s Orthopaedics Institute. A member of CHOC’s sarcoma team, his special clinical interests range from bone and soft tissue tumors to limb deformities. He shared with us why he decided to become a doctor, new developments in his field, his hobbies outside of work and more.

Dr. Amir Misaghi
Dr. Amirhossein Misaghi, CHOC Children’s orthopaedic surgeon

What is your education and training?

I completed my undergraduate, medical school and orthopaedic surgery residency all at the University of California, San Diego, followed by a pediatric orthopaedic fellowship at Children’s Hospital of Philadelphia and an orthopaedic oncology fellowship at Mayo Clinic in Rochester, Minn.

What are your special clinical interests?

My clinical interests are bone and soft tissue tumors, trauma, hip disorders, limb deformities and other standard pediatric orthopaedic conditions. The vast majority of tumors in kids are benign but can still be complicated. Tumors can be found in difficult-to-operate areas, or be benign but hard to eradicate, so having both a pediatric orthopaedic and an orthopaedic oncology background helps me manage these complexities.

Why did you decide to become a doctor?

I love working with and helping others, and I was drawn to orthopaedics because I’ve always enjoyed using my hands to build and repair things. I also like working in pediatrics because I find kids fun and fascinating to be around. Oftentimes, especially when working with pediatric cancer patients, some very difficult situations and decisions arise, but I feel privileged to partner with families and navigate these challenges alongside them. Kids tend to do remarkably well and have incredible resilience, and I have encountered very powerful moments working in this field. It is an amazing feeling to witness my patients growing up and thriving.

What are some new programs or developments within your specialty?

CHOC is an amazing place with a fantastic pediatric orthopaedic department and cancer center.

Pediatric orthopaedics and pediatric orthopaedic oncology have changed with advances in growing type prosthesis for limb salvage surgery, as well as advances in lengthening limbs and restoring function after major trauma or cancer treatment. We have access to many exciting new pediatric-focused technologies at CHOC.

CHOC also has a great sarcoma team, and it is incredibly rewarding to be part of this multidisciplinary group. We take care of complex patients with the shared goal of providing the best care and getting them back to living happy, healthy lives. I love being able to work closely with Dr. Elyssa Rubin, medical oncologist, and Dr. Raj Vyas, plastic surgeon, to grow our limb salvage and musculoskeletal oncology/sarcoma program. We also have fantastic pediatric interventional radiology with Dr. Tamman Beydoun, and an amazing pathology department with Dr. Aaron Sassoon and Dr. Ali Nael.

What would you most like referring providers to know about you or your division at CHOC?

I want providers to feel confident referring patients to us. We are set up to take care of all benign and malignant bone and soft tissue tumors with a comprehensive team. The oncology program at CHOC is a dedicated group of multidisciplinary providers, including oncology, pediatric surgery, radiation oncology, interventional radiology, radiology and pathology. We meet weekly at our oncology tumor board meeting to discuss complex cases, ensuring we can provide up-to-date, cutting-edge treatment. We are fully equipped as a sarcoma team to manage all malignant bone and soft tissue tumors, and we all focus specifically on pediatrics.

What inspires you most about the care being delivered here at CHOC?

The various providers and patients inspire me. I am constantly impressed by the dedication of the nurses, physicians, child life specialists and countless other staff who work so hard. Taking care of pediatric patients, seeing their strength and witnessing them overcome their challenges is incredible.

If you weren’t a physician, what would you be and why?

I would open a small restaurant – I have always loved cooking, especially outdoors. One of the things I love most about my current job is talking to and getting to know my patients and their families. I think that the restaurant world might offer similar rewarding interactions with people, like chatting with regulars over the years, all while making delicious food.

What are your hobbies and interests outside of work?

I enjoy outdoor activities like camping and fishing, and have recently been trying to interest my kids, ages 3 and 7, in these hobbies as well. We’ve also taken up gardening as a family, and we are always ready for a good barbecue or soccer match.

What have you learned from your patients?

I’ve learned that life is a marathon, not a sprint. It is a joy to watch kids heal after surgery, and to then see them months or years later recovered and back to life. They have also taught me some practical things to keep me plugged in to what is cool and trendy. Recently I was educated about TikTok and some of its popular personalities – one patient suggested I even make a TikTok profile and start showing off some of my dance moves.

Refer a patient to the CHOC Children’s Orthopaedic Institute.

An innovative internship approach during COVID-19

A year ago, Jenae Vancura joined an elite group of college and high school students for a unique and innovative summer internship program at CHOC Children’s. The interns shadowed physicians, joined doctors on their rounds and attended meetings with a wide range of professionals in the medical field.

The days were long. The work was challenging.

And Jenae, a 21-year-old biology major from UC Santa Barbara, is back again this year, now serving as a lead intern for the Sharon Disney Lund Medical Intelligence and Innovation Institute  (MI3) summer internship program. She now helps guide a new group of students through the rigorous program.

“But this year is a little different,” Jenae says.

Now in its eighth year, the internship has been effectively reimagined as a virtual program, a pivot quickly executed at the onset of the global coronavirus pandemic.

The COVID-19 crisis emerged just as CHOC leaders and physicians were gearing up for this year’s internship program. That meant that the 63 participating students would not be able to work directly with hospital staff or go on rounds to interact with patients as in years past.

A look at the virtual model of the Medical Innovation and Intelligence Institute summer internship program.

Cancelling not an option

While many internship programs have been halted worldwide due to COVID-19, canceling the MI3 internship was simply not an option, organizers say. The experience was much too valuable and too many young had worked too hard to get this far.

“Many of our interns look to our program to affirm and motivate their decision to apply to medical school,” says Debra Beauregard, director of MI3. “Nearly all of the interns aspire to become physicians.”

So, with just weeks to go, the decision was made to recalibrate the program and put the whole curriculum online.

“The easy thing would have been to postpone or cancel,” says Dr. Anthony Chang, CHOC’s chief intelligence and innovation officer who launched the program eight years ago. “We wanted to give the students the same level of opportunity. To their credit, the team stepped up and made the internship rotation on par with previous years.”

Dr. Chang started the internship because he wanted to give young people an in-depth experience of the medical field.

“I felt like no one was really doing something like this,” he says. “The students were staying with one mentor doing one assignment. That sounds like a research assignment, not an internship. I wanted to give them access to something that gave them access to a hundred mentors.”

A rigorous pace remains

Even though the pandemic has restricted access to the hospital, it hasn’t slowed the  interns’ pace. Their work schedule starts early and, with a few breaks between, doesn’t end until the evening.

“We set up a lot of Zoom meetings,” Debra says. “Our interns have a full schedule. They participated in rounds and shadowed our physicians virtually. They were even able to remotely view multiple surgeries. This was a challenge, but everyone pulled together to provide what turned out to be a great summer program.”

A small group session during the internship

Dr. Chang says that while the interns are receiving the same level of instruction, what’s missing are some of the personal interactions that come with face-to-face contact.

“Not having one-on-one time in person and not having more intimate moments in small groups is difficult for us,” he says. “For instance, in past years they’ve had one-day retreats where they come to my house for breakfast and lunch. I wasn’t able to do that this year.”

But that doesn’t mean there isn’t room for a little fun.

“Our directors and lead interns made sure that all interns felt connected,” Debra says. “We included team-building exercises, interactive small group sessions, and even a virtual graduation ceremony. The leads even organized a virtual talent show and Zoomie Awards, in addition to a competitive team competition. Our leads have gone above and beyond to ensure that our interns had a meaningful and memorable experience.“

Dr. Sharief Taraman, internship co-director and pediatric neurologist at CHOC, has been part of the program almost since it started. He’s confident that this year’s group is better off than students anywhere else.

“They’re way ahead of their peers in terms of experience and what they can get out of the summer,” he says. “We have a lot of moving parts, so we had to pivot very quickly.”

And to ensure the interns get all the experience they can, they are being invited back next year when they are hopefully able to receive hands-on work.

“We have offered guaranteed spots for our interns next year, so they can have an in-person experience,” Debra says. “We are confident that most will be coming back.”

Student gratitude abounds

The interns themselves are grateful for the chance to continue their internships during these difficult times.

“When I thought it wouldn’t happen, I got very upset,” says 20-year-old Julia Keating from the Massachusetts College of Pharmacy and Health Sciences University. “It’s such a competitive internship.”

For Jessica Octavio, a 20-year old San Diego State student majoring in microbiology, going online was positive experience.

‘’We’re lucky,” she says. “They’ve been more than flexible. The biggest thing was learning this online interface, but as far as programming goes, it’s almost advantageous for us.”

While working on site would have been ideal, Luke Arnold says he appreciates all the work the health system staff have put into making this year’s program a success.

“It’s not ideal and obviously we’d like to have this in person,” says the 21-year-old biology major from Chapman University. “But being in quarantine has given us opportunities to work in group settings. We’re all in this together.”

Interns will be invited back for the 2021 session in hopes of a hands-on experience.

For intern Nicole Fraga, working from home has had some surprising benefits.

“It can be a very rigorous process,” says the 22-year-old recent graduate from Brown University. “But ironically, I think they’re getting closer to the interns online because we are meeting in small groups. We are able to communicate on Slack and share memes. We have a smaller community.”

Dr. Chang calls the internship a “circular experience.” The health system staff, he says, learn as much from the students as the students do from the physicians.

“We’re grateful that the interns are even more inspired to go into medicine despite the pandemic,” he says. “It’s very heartwarming to hear. The future of medicine is in good hands. I see the interns push back against the temptation to give up. They have the idealism and no fear of failure.”

Learn more about the Medical Innovation and Intelligence Institute summer internship program.