Category Archives: Technology and innovation

Hearing aids expand 9-week-old boy’s world

Clockwise from bottom: Elijah Cook, born Jan. 2, mother Ahavah, sister Evelyn and father Jason (Photo courtesy of the Cook family)

Clockwise from bottom: Elijah Cook with mother Ahavah, sister Evelyn, 7, and father Jason. Elijah was born Jan. 2 with severe-profound sensorineural hearing loss. (Photo courtesy of the Cook family)

Jimmy Bellamy

After Ahavah Cook’s baby, Elijah, was diagnosed with profound hearing loss shortly after his birth, the Andover, Minn., mom thought she’d never hear sweet coos and sounds produced by her beautiful newborn.

Elijah Cook was born Jan. 2 at Mercy Hospital in Coon Rapids. Twelve hours later, he failed his newborn hearing screening. Nurses downplayed the result, reassuring Ahavah and husband Jason that it was common for babies to fail the first hearing test only to pass the 24-hour follow-up exam.

But that, and a third test with an audiologist, yielded the same result.

“We could see there was a good chance that he wouldn’t pass additional tests,” Ahavah said. Though she and Jason had been optimistic, the couple knew that hearing challenges were a possibility with their son. Both of their mothers and fathers are deaf, and Jason has no hearing in his left ear. “When Elijah came back (from the first test), we were expecting good news because we have almost a dozen nieces and nephews that have no hearing issues.”

The hearing aid Elijah wears in one of his ears, with a quarter to show scale

The hearing aid Elijah wears in and around one of his ears, with a quarter to show scale

After the three failed tests, the Cooks were referred to the developmental and rehabilitation program at Children’s Specialty Center, connected to Children’s – Minneapolis. There the Cooks met with Lori Johnson, AuD, for another hearing exam.

“We had a lot of time to ask questions,” Ahavah said, “and from that moment all of our worries went away.” Though the Cooks didn’t get the results they wanted, Ahavah said, they felt more knowledgeable about Elijah’s diagnosis, which is severe-profound sensorineural hearing loss. While it is likely genetic, upcoming tests will confirm that.

“Lori has been great. Overall, I give her a 20 out of 10,” Jason said. “She answered all of our questions and gave us more time than was allotted.”

On March 5, Johnson fit then-9-week-old Elijah with tiny hearing aids that allowed him to hear his mother — and the world — for the first time. The moment was captured on video.

“The first time I saw him blink, I had a lot of emotions,” Ahavah said. “I was trying really hard not to cry. I didn’t want him to see me cry. I was trying hard to keep it together and just talk to him.”

Since then, Elijah has been cooing, smiling and responding to the sounds made by his family, including big sister Evelyn, 7.

Elijah with his mother, Ahavah, shortly after his birth (Photo by Tres Belle Studio)

Elijah with his mother, Ahavah, shortly after his birth (Photo by Tres Belle Studio)

“The cooing is the big thing,” Jason said. “He wasn’t very talkative, but ever since he was fitted for his hearing aids he’s cooing more, he pays more attention.”

The goal for audiologists is to have infants with hearing loss fit with hearing aids when they’re a couple weeks or months old. If babies don’t pass their initial and follow-up screenings, and get referred to Children’s immediately, it’s common to be fit at 2 or 2½ months. Elijah was fitted early because of his family history and parents’ timely follow-up, Johnson said. The youngest child she has fit with hearing aids was 2 weeks old.

“The hard part with Elijah and the severity of his hearing loss is you really don’t know (if he can hear) until he can give you cues — looking around for sound, searching with his eyes,” Johnson said. “Computer technology lets us know if he should be able to hear sounds.

“Once he’s sitting up, at around 7-month age, we’ll know for certain what he’s hearing with the hearing aids on as he will be able to respond for a behavioral hearing test, and that’s true for any child with any degree of hearing loss.”

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USA TODAY / KARE 11: Baby hears mom for first time

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Elijah with hearing aids

Elijah was fit for hearing aids at a young age because of his family history and parents’ timely follow-up.

Early diagnosis and fitting combined with the technology and medical procedures available today — hearing aids, cochlear implants and bone conduction hearing aids — allows children with hearing loss a life with limitless possibilities.

Johnson said any child diagnosed with hearing loss and fit with hearing aids before 3 or 6 months can do anything as long as hearing-aid use is consistent.

“The big thing to take away in the case of Elijah and every other case is the earlier we can get diagnosis and family into early intervention and get started, you can have some really great outcomes,” she said. “But it’s really about the early detection piece of it.”

Jason and Ahavah said their experience growing up in the deaf community and seeing struggles will provide their son advantages. Many of their family members are fluent in American Sign Language (ASL) and are helping educate and sign to Elijah.

“Every parent wants the best for their kid. I just want to be able to equip him to handle life’s challenges,” Jason said. “I want to make sure he can overcome them and succeed.”

The Cooks encourage others with children born with hearing loss not to wait to do early invention and evaluation.

“Let other people help you because it’s overwhelming,” Ahavah said. “There are a lot of additional appointments. Get the help that you need.”

Elijah and big sister Evelyn

Elijah and big sister Evelyn

subscribe_blogSo how has that cooing been since Elijah received his hearing aids? It has been music to his mom’s ears.

“I kind of lost hope that I would get that milestone,” Ahavah said, “but it’s pretty cool that the hearing aid is able to help us bring it back.”

“That’s really the reason why I do my job; it’s for the parents who have children with a hearing loss and want their kids to develop speech and language,” Johnson said. “My goal is to give that to parents whenever possible.”

Children’s Hospitals and Clinics of Minnesota has audiology services through ear, nose, throat (ENT) and facial plastic surgery as well as the developmental rehabilitation program. Children’s has comprehensive care for hearing loss, from screenings to hearing-aid fittings to cochlear-implant surgery through follow-up audiology and speech pathology.

Raising kids with the Internet as a co-parent

Every generation faces unique challenges in life, this generation is no exception. The main difference for new parents in 2015 is the Internet — offering a mixed bag of benefits and burdens to the ancient art of parenting. (Pimonova / iStock illustration)

Every generation faces unique challenges in life, this generation is no exception. The main difference for new parents in 2015 is the Internet — offering a mixed bag of benefits and burdens to the ancient art of parenting. (Pimonova / iStock illustration)

Jeri Kayser

While visiting my marvelous niece and her exceptionally marvelous newborn baby, she mentioned that women who have already raised their children don’t know what it’s like to be a parent in today’s world. This struck me as interesting on many different levels.

I remember having that same exact thought after having my first child; it’s only after that baby has grown a bit or you’ve had your second or third child that you can look back on this phase of your parental evolution and see that the change is less about the world and more about you. You have changed. Your priorities, your worries, your decreased expectation that sleep is something you get to choose. The world is now more demanding but infinitely more fascinating and filled with an indescribable love. Plus, you get to learn some awesome multitasking skills!

subscribe_blogSo, is it different to raise a child now?

Every generation faces unique challenges in life, this generation is no exception. I would argue that the main difference for new parents in 2015 is the Internet — offering a mixed bag of benefits and burdens to the ancient art of parenting. It’s a great place to find bargains for the stroller you want or show you how to install the car seat. But it’s equally a never-ending source of unsolicited advice and distorted parent bragging that can make any rock-solid parent feel insecure, questioning if it’s true: “Should I really only feed my child blue foods?”

When I was raising kids and standing in the checkout lane at the grocery store, the magazines would shout from their rack all of the things I could be doing to be a better parent: “How to create the perfect birthday party!” “Fun and easy Halloween costumes you can make at home!” “Teach your child 12 languages before they enter kindergarten.” Every title offering a suggestion came with the subliminal message that failure to follow the advice was evidence that you weren’t up to this whole parenting thing. It’s hard not to feel insecure when you’re so motivated to be perfect for your obviously perfect child while residing in the imperfect package of a human being.

I could step away from the parenting magazines in the checkout line, maybe read up on what alien has married what celebrity, but you can’t really avoid the Internet. Those photos of your friends and relatives in gorgeously orchestrated family bliss are still going to pop up in your feed.

Sigh.

Mining the Internet for truly helpful information that empowers your parenting mojo instead of inviting in trolls who create chaos with your self- esteem requires some thoughtful navigation.  The Internet is great for advice about things that have easily verifiable facts, like “where can I find an indoor playground?” Questions that have long-term consequences like “how do I get my kids to get along with each other?” are best answered by the posse of people closest to you — your friends and family as well as professionals educated in the field of question.

Important parenting advice should be gathered from people important to you, people who are invested in you for the foreseeable future who will be around to be held accountable for their advice. Sift through that advice and take from it what seems right to you. Trust yourself. Yes, others have sailed the parenting seas, but this is your journey and you are the captain. Respecting yourself and recognizing there is no perfect parent smoothes the waters and makes the trip so much more fun!

Jeri Kayser is a child life specialist at Children’s Hospitals and Clinics of Minnesota.

The importance of play — for kids and adults

Hands-on play, where a child uses his or her imagination and ideas to self-discover, creates the best learning environment. (iStock photo / Getty Images)

Jeri Kayser

When people try and remember the name of my profession, child life specialist, they often shorten it to “play lady.” That used to bug me when I was a young professional and ready to solve all of the world’s problems, but now I recognize the compliment. We breathe, drink and eat to stay alive – we play to bring forth a reason for all of that effort. Play is how we learn about our world, practice that knowledge and foster our sense of well-being and personal joy; it’s an honor to promote play in the world of health care, but it’s not without its challenges.

One current challenge is tied to the hot topic in popular culture about the value of gaming devices. Is playing a game on a smartphone when you’re 2 years old considered quality play? Short answer: No. The Academy of Pediatrics recommends no screen time for kids 2 and younger and only one to two hours a day for older children. The core aspect of the definition of “play” is that it’s self-directive. You’re deciding what you’re going to do with whatever you’re interacting with. One of the problems with electronic games is that game designers have done most of that for you.

Your toddler recognizes the status that phone holds, and it works for a bit to keep a child distracted from the fact that he or she is in the hospital or in a long checkout line at the grocery store.

subscribe_blogSo what can we use to help guide our decisions to promote healthy play? A great way to look at this is similar to how we all work to promote healthy choices for our diet. Potato chips are fine for an occasional treat, but we wouldn’t want to eat them all the time. If we did, we’d feel awful. Video games kind of are the junk food of play. The more the play requires from the child, the better the value and healthier the choice.

I notice this in the hospital when I come into a room to meet with a family about what to expect with surgery. People often are busy with an electronic device, but as soon as we start to talk, the interest is there to engage and the devices get turned off. When I bring a toy or some arts and crafts activities, kids always gravitate towards that; they want what they need.

I used to work in a summer daycare program for school-aged kids. We would spend the morning on a field trip and the afternoon at a beach. The director wanted us to provide structured activities for the kids in the afternoon, but we quickly learned that the combination of water, sand and friends led to a more-creative, imaginative and enriched play than anything with which we could have come up. Hands-on play, where a child uses his or her imagination and ideas to self-discover, creates the best learning environment.

I heard an interesting story on public radio on my long commute home. At the electronic show in Austin, Texas, at the South by Southwest conference, the big news at the conference was the “Maker Movement,” stressing the importance of hands-on play to promote understanding of how our world works. They interviewed an inventor, Ayah Bdeir, who created a toy of electronic bits that fit together with magnets, creating circuits. With this process, you can make all kinds of fun things. He explained the value of this explorative play by stating, “We need to remember that we are all makers and touching things with our hands is powerful and inspiring.”

In another century, another scientist noted the same thing. Albert Einstein declared, “Play is the highest form of research.”

Self-directed play offers the healthiest value for our play “diet,” and this extends throughout our lives. We all need to play. As I wrote this, I overheard a conversation between two anesthesiologists talking about how they used play to help them cope with life stressors. One likes his guitar, while the other enjoys making remote-control helicopters.

This important fact, one of the highest forms of self-care, needs to be part of the planning of how we provide health care. Play is important for all age groups, not just those adorable preschoolers. We need to incorporate this in everything we do, for teens, parents and staff.

Late Irish playwright George Bernard Shaw said it best: “We don’t stop playing because we grow old; we grow old because we stop playing.”

Jeri Kayser is a child life specialist at Children’s Hospitals and Clinics of Minnesota.

Top 10 apps for pain management

Mobile apps can be used to help distract children dealing with pain. (iStock photo)

Mobile apps can be used to help distract children dealing with pain. (iStock photo)

Stefan Friedrichsdorf, MD

Stefan Friedrichsdorf, MD

Stefan Friedrichsdorf, MD

State-of-the-art pain management in the 21st century demands that pharmacological management must be combined with supportive and integrative (nonpharmacological) therapies to manage a child’s pain. At Children’s, we are using physical methods (e.g. cuddle/hug, massage, comfort positioning, heat, cold, TENS), cognitive behavioral techniques (e.g. guided imagery, hypnosis, abdominal breathing, distraction, biofeedback), as well as acupressure and aromatherapy.

For “needle pokes” (vaccinations, blood draws, etc.), for instance, we expect that kids are sitting upright (use sucrose in the age group birth to 12 months), so we numb their skin with topical anesthesia (e.g. EMLA-patch, LMX 4 percent lidocaine patch, or J-tip) and use distraction such as bubble blowing or blowing a pin wheel. Alternatives can be applications on your smartphone or tablet.

My personal favorite technique remains teaching children in self-hypnosis. However, in addition, I’m increasingly using apps on my iPhone or iPad with patients. Here are my top apps for pain and symptom management in kids:

10. Real ChalkBoard: Drawing on a realistic chalkboard with different-color chalk — much more fun than it sounds. ($1.99)

9. Talking Tom CatThis is a cat that repeats everything you say in a funny voice. You then can record and share videos and send them by email, YouTube and Facebook. Kids really enjoy that they can make Tom the cat say funny stuff. (free)

8. Simply Being – Guided Meditation for Relaxation and PresenceProvides relaxation, stress relief and benefits of meditation without prior experience. It allows the child/teenager to choose from four meditation times and gives them the option to listen to the guided meditation with or without music or nature sounds. Kids also can listen to the music or nature sounds alone; more for older children and teenagers. (99 cents)

7. Drums! – A studio-quality drum kit in your pocket As the name implies, this is a drum kit. Great for kids with too much (or too little) energy. Rock on! (99 cents)

subscribe_blog6. Tesla Toy: “Tesla Toy” is a fun, interactive “particle toy.” When the fingers touch the screen, they generate what looks like a powerful electrical field that makes the thousands of particles react beautifully. 
Kids (and adults) often are mesmerized by this app for a long time. ($ 1.99)

5. Fruit NinjaA “juicy” action game with squishy, splatty and satisfying fruit carnage! (99 cents) 

4. Koi PondOne is gazing into a pond of crystal-clear water with fish, turtles and more. Kids run their fingers across the cool surface of the pond and water ripples away from their touch.
The koi fish, disturbed, dart away… only to quickly forget and swim close once more. (99 cents)

3. Easy-Bake Treats!This is a highly recommended app; it let’s children make, bake, decorate, eat and share virtual cakes, cupcakes, cookies, cake pops and pizza. They pick their mix, fill the pan with batter and water, mix it all together and slide the pan into the virtual oven to cook. Then they can add candles to their cakes and cupcakes, too! Candles can be lit and re-lit for even more fun! 
Once they’ve baked and decorated their treats, kids can show off and share their delicious virtual creations with family and friends via email or Facebook. Highly addictive – kids can spend a long time on this app. (free)

2. The Healing Buddies Comfort Kit“The Healing Buddies Comfort Kit” offers an interactive, virtual way for kids and teens at home, in a doctor’s office or in the hospital to learn and immediately benefit from self-care techniques to manage common symptoms like pain, worries, nausea, fatigue and trouble sleeping. 
The app contains several integrative medicine techniques adapted for use by children and teens that are quick and effective for symptom control so kids can get back to doing what they like to do – and need to do each day. The techniques included in this app are designed by leaders in the field of pediatric integrative medicine (physicians, nurses, psychologists and child life specialists) and have been tested for years in several leading children’s hospitals and clinics. (99 cents)

Disclaimer:

The creation of the mobile app has been a collaborative effort of my department at Children’s Hospitals and Clinics of Minnesota with Ridgeview Medical Center and DesignWise Medical.

1. Balloonimals Absolutely groovy; kids love it! This is my favorite app to distract a child in pain or discomfort. Beautifully rendered balloon animals spring to life when you blow into the microphone on your iPhone/iPad or swipe with your iPod Touch. Watch the balloon inflate and then give your phone a shake to start constructing your balloon animal. With each shake, your animal takes greater shape until… voila! Your balloon animal appears and is ready to play. Pet your “balloonimal” to see what tricks it can do. ($1.99)

Apps, which didn’t make the list but I still like a lot: “Art of Glow,” “Sand Garden,” “Build a Zoo,” “Naturespace,” “SkyView” and “Disney’s Small World”

Stefan Friedrichsdorf, MD, is medical director of the Kiran Stordalen and Horst Rechelbacher Pediatric Pain, Palliative and Integrative Medicine Clinic at Children’s – Minneapolis. This post originally appeared at NoNeedlessPain.org. Download the Children’s Hospitals and Clinics of Minnesota app.

Laser in action: See how Gavin’s tumor met its match

Gavin Pierson (left) and Joseph Petronio, MD, visit during a photo shoot at Children's – St. Paul on Monday, June 16, 2014.

Gavin Pierson (left) and Joseph Petronio, MD, visit during a photo shoot at Children’s – St. Paul on Monday, June 16, 2014.

In the two years since Gavin Pierson’s brain tumor, which he calls “Joe Bully,” was discovered, he has undergone 17 surgeries. A combination of craniotomies and the Pfizer drug, palbociclib, had been managing the growth of Joe Bully, but not decreasing its size. Gavin and his family were growing tired and frustrated with invasive surgeries, and Gavin wasn’t bouncing back as well as they hoped.

Enter Visualase.

Visualase is a laser used for neurosurgery and is guided by MRI images to precisely target areas of the brain that were previously thought inoperable. After making a 3-centimeter incision, Joseph Petronio, MD, and his team guided a small laser fiber directly to Gavin’s tumor. Children’s is the only pediatric hospital in the Midwest using Visualase, and Gavin is the only patient in the country to use this technology to treat a mature teratoma brain tumor.

Learn more about how Dr. Joseph Petronio used the Visualase laser:

Subscribe to MightyNot only did this technology target and dissolve a significant portion of Gavin’s tumor, it’s also prohibiting re-growth – stopping Joe Bully in its tracks. The laser is so targeted that the brain tissue surrounding the tumor was unharmed, making for a quick recovery. Within 12 hours, Gavin was sitting up, eating and laughing with his siblings and parents. Gavin went home the next day and was back to school within four days.

These types of minimally invasive surgeries have incredible benefits for Children’s patients. Since obtaining Visualase in October 2013, Children’s has treated patients as young as 12 months for epilepsy and other types of brain tumors. Tools like Visualase are making tumors we once thought were inoperable – operable.

Gavin vs. Joe Bully: First-of-its-kind laser surgery shrinks tumor by more than 40 percent

The Piersons (from left), Steve, Gavin, Nicole, Grace and Gage, have been through a lot in the past two years.

The Piersons (from left), Steve, Gavin, Nicole, Grace and Gage, have been through a lot in the past two years.

If you’ve been following 8-year-old Gavin Pierson’s story, you know he and his family have been through a lot. In 2012, Gavin was diagnosed with a mature teratoma brain tumor.

Since then, he has undergone numerous craniotomies and he and his family have dealt with big drug companies to fight his brain tumor, which Gavin refers to as “Joe Bully.” Unfortunately, Joe Bully is a particularly tough tumor, located in an area that is difficult to operate on and comprised of hard, “concrete-like” tissue. While Gavin’s prior treatments made progress, Joe Bully kept growing back.

give_gavin_blogBut it appears that Gavin’s neurosurgeon, Joseph Petronio, MD, may have found Joe Bully’s weak spot. Over the past eight months, Gavin has undergone two Visualase laser surgeries, an MRI-guided procedure designed to incinerate the tumor. He’s the first patient with a mature teratoma to ever use Visualase – and it may have stopped Joe Bully in its tracks.

Children’s and the family also successfully petitioned pharmaceutical company Pfizer to grant Gavin access to an experimental drug, palbociclib, to help control the tumor’s growth. Gavin is the youngest patient to use palbo.

We’re happy to announce that a recent MRI scan showed Gavin’s formerly peach-sized tumor has shrunk more than 40 percent. Even better? There are no signs of regrowth.

Gavin’s courage and strength inspire us every day. Thank you, Gavin, and congratulations.

Learn more about Gavin’s story and surgery:

Tech Spotlight: A look at Visualase, a minimally invasive laser surgery system

Visualase's workstation interfaces with an MRI to allow temperature control and monitoring in real time during a treatment.

Visualase’s workstation interfaces with an MRI to allow temperature control and monitoring in real time during a treatment.

Gavin Pierson, now 8, of Ramsey, Minnesota, underwent two Visualase procedures in October and February.

Gavin Pierson, now 8, of Ramsey, Minnesota, underwent two Visualase procedures in October and February.

The story of Gavin Pierson, the 8-year-old Ramsey, Minnesota, boy battling a brain tumor, includes a number of key players: his doctors, parents, siblings and care team, and the thousands of people who have followed his two-year fight against the aptly nicknamed “Joe Bully.”

But one of Gavin’s most important allies doesn’t have a degree, voice or personal Facebook page.

Visualase, an MRI-guided, minimally invasive laser system, has been the Kryptonite to Gavin’s tumor since he became the first person with a mature teratoma brain tumor to undergo the procedure. His first laser surgery took place on Oct. 29, 2013, at Children’s Hospitals and Clinics of Minnesota and his second four months later on Feb. 20, 2014.

“There are many things that make this procedure unique. One is the use of MRI, which allows us to monitor the temperature of both the tumor and the normal brain during the laser treatment,” said Joseph Petronio, MD, medical director of pediatric neurosurgery at Children’s and the doctor who led Gavin’s Visualase procedures. “The composition of (Gavin’s) tumor is unusual, including elements of bone and skin that makes it conduct heat very broadly. By being able to monitor temperature that closely, we are able to target the tumor more precisely without damaging other tissue.”

Founded in 2005, Visualase, Inc.’s system is a minimally invasive laser procedure that allows surgeons to pinpoint and treat lesions and tumors with extreme precision.

So, how does Visualase work?

  • Visualase Cooled Laser Applicator System: The system features a disposable fiber optic catheter with a built-in cooling mechanism that prevents overheating near the surface of the applicator. This laser catheter is placed through a small opening in the scalp and skull and into the center of a tumor using advanced MRI technology. Laser energy is then used to heat the tumor carefully. The system was engineered to allow for the use of higher laser powers to destroy tumors with shorter exposure times.
  • Laser generator: The generator produces light energy that is used to thermally ablate, or destroy, soft tissue.
  • Workstation: The workstation interfaces with an MRI to allow temperature control and monitoring in real time during a treatment. It also provides on-screen visuals of the tissue as it turns into a solid or semi-solid state. Because of the in-depth monitoring, the procedure results in a high level of precision and control.
  • Temperature: The time it takes to destroy parts of the tumor depends on the temperature of the laser. When set to 113-140 degrees Fahrenheit, tumor cells eventually get destroyed. Cells and tissue are destroyed immediately when the laser is between 140-212 degrees. Anything above 212 degrees, though, can cause water in the tissue and areas inside a cell to vaporize, and leads to ruptured cells and tissue components.
  • According to Visualase, once soft tissue is destroyed, or ablated, it is considered non-viable and is reabsorbed, leaving little evidence that a tumor or burn existed.

After getting its start in treating liver and prostate problems, the Visualase Thermal Therapy Subscribe to MightySystem was cleared by the Food and Drug Administration in 2007 for the ablation of soft tissue in neurosurgery. Visualase’s first minimally invasive neurosurgical procedures were performed in 2006 in Paris as part of a study for treating brain tumors.

The Visualase laser system is in use at more than 40 hospitals, nationwide, including 15 pediatric hospitals. In pediatric patients, including at Children’s Hospitals and Clinics of Minnesota, Visualase has also been used to address brain lesions that cause epilepsy.

“What’s exciting to me is the path this technology opens to areas of the brain that were closed to us before,” said Petronio. “To think we could reach a day when the term ‘inoperable brain tumor’ in children is obsolete is extraordinary.”

Source: visualaseinc.com

NBC News: Sharing the story of Children’s cancer and blood disorders expertise

Michael and Megan Flynn with sons Andrew, 7 months, and Thomas, 5, and daughter Olivia, 3 (Photo by Julie Ratkovich Simply Bliss Photography)

NBC News shared the story and collective work of Children’s Hospitals and Clinics of Minnesota’s blood and cancer disorders team, including Dr. Kris Ann Schultz, Dr. Yoav Messinger, Gretchen Williams, CCRP, and Anne Harris, MPH, among others, who have led the way in enabling the early detection and effective treatment of children in families affected by rare genetic cancers.

via NBC News: One rare cancer leads to another: Cancer registry saves baby’s life

In 2009, while trying to understand pleuropulmonary blastoma (or PPB, a rare early childhood lung cancer), researchers leveraging data from Children’s International Pleuropulmonary Blastoma (PPB) Registry uncovered an unexpected cause: a mutation in DICER1, a master controller gene that helps regulate other genes. By leveraging those learnings and coupling it with new data from the International Ovarian and Testicular Stromal (OTST) Registry – a “sister” registry of the International PPB Registry – Children’s has recently discovered that the DICER1 gene mutation may underlie many additional rare childhood genetic cancers and could tell us something fundamental about how most cancers arise.

Children’s presented its promising findings this weekend at the American Society of Clinical Oncology (ASCO) Annual Meeting, advancing the potential for early diagnosis and proactive treatment of children in families affected by PPB, as well as other rare genetic cancers such as certain ovarian, nose, eye and thyroid tumors.

With the establishment of the International PPB Registry in 1988 and the International OTST Registry in 2011, Children’s, along with our partners, have become the world’s leading experts on how to care for children with PPB and other cancers marked by the DICER1 genetic defect. As a result, PPB could be among the first cancers routinely curable before it progresses to a deadly form.

Children’s work and ability to follow the science continues to be made possibly entirely by philanthropy, including the St. Baldrick’s Foundation and the Pine Tree Apple Tennis Classic.

Congratulations to the Children’s cancer and blood disorders team!  Thank you for your commitment and your amazing, groundbreaking work.

Child free of seizures after laser procedure

Jack Healy, seen with his family, including his mother, Shannon, had a rare, benign tumor called a hypothalamic hamartoma.

By Shannon Healy

Our sweet baby boy, Jack, was born Oct. 18, 2012. When he was only a few weeks old, we started noticing he was having “episodes.” His body would tense up and he would scream like he was in pain. These continued to worsen as he got older and got to the point where he would wake up with them every time he tried to go to sleep. He also began having episodes where he would appear to be laughing but still looked terrified and in pain.

For months, we sought out different specialists to try to diagnose our baby’s episodes. Finally, in April 2013, we were referred to the neuroscience program inside the St. Paul campus of Children’s Hospitals and Clinics of Minnesota. Still unsure of what his episodes were, the staff began following him closely and keeping a close eye on his other symptoms (a large head and low muscle tone).

In August 2013, Jack had an MRI on his brain. Within days, we had a diagnosis: Jack had a tumor on his hypothalamus and his “episodes” were gelastic and dacrystic seizures associated with the tumor. This time was so overwhelming for us, but we finally knew what Jack was battling.

Jack was prescribed a few different seizure medications, but his seizures continued to worsen. At the peak, he was having up to 15 seizures per day; more severe and longer in duration than when he first started having them. They were taking a toll on Jack and our family. He has two older sisters who adore him, and they would get extremely upset every time he would have one of his “fits,” as they called them.

It seemed like the seizures were affecting every aspect of Jack’s life and development. He began refusing all solid foods and would take only bottles of milk. His seizures constantly would interrupt his sleep. He was having severe delays in his gross motor development and also was falling behind in his speech. Defeat and hopelessness were setting in for us.

After watching the tumor for three months, the neurology team at Children’s was able to confirm that Jack had a rare, benign tumor called a hypothalamic hamartoma. We were told that these tumors occur in only one in 1 million people, often appearing in young children. According to Meysam Kebriaei, MD, Children’s neurosurgeon, that number could be underestimated since hypothalamic hamartomas can be missed without high-resolution MRIs.

In December, we met with Dr. Kebriaei, who discussed a new surgical procedure called Visualase, an MRI-guided, minimally invasive laser procedure. He thought Jack would be a great candidate.

Jack would be the first patient at Children’s with a hypothalamic hamartoma to undergo a Visualase procedure. We were thrilled to have this option available to us and made our decision to proceed before we left the clinic that day.

We also learned that Children’s is the only institution in the Midwest to perform Visualase ablation for hypothalamic hamartomas. Dr. Kebriaei explained that Visualase would allow him to treat Jack’s tumor in such a way that will minimize the risk and decrease his recovery time, when compared to the standard, open surgical procedure. 

Jack had seven seizures the morning of his surgery.

Jack’s surgery was scheduled for Jan. 10. He had seven seizures the morning of his surgery; a strange reassurance to us that we had made the right decision. The surgery took the entire day and we were updated by Children’s staff throughout the process. We never left the waiting room.

At the end of the day, Dr. Kebriaei told us that the surgery had gone perfectly. The tumor had been completely disconnected from Jack’s brain. We spent two nights in Children’s ICU with Jack and, amazingly, he was seizure-free.

After going home, Jack experienced an unexpected drop in his sodium level, which required a readmission to Children’s. He spent 2½ weeks in the hospital, where he continued to receive extraordinary care and, in spite of this complication, continued to be free of his debilitating seizures.

It has been almost four months since Jack’s surgery, and he has not had any seizures. It still is hard to believe, considering he had these seizures daily for 15 months. Sometimes, I still expect him to be having one when he starts laughing, but then I realize that he is laughing because he’s so happy!

Jack has been seizure-free since undergoing the Visualase treatment.

With the seizures gone, we now are able to focus on Jack’s development. He is having therapy multiple times each week, and we already are seeing progress in his eating, speech, motor skills and sleep patterns. Just within the past week, Jack has been standing, walking and pushing toys through our kitchen. We are hopeful these parts of Jack’s life only will continue to improve.

This surgery has been life changing for us. We learned later that Jack is one of only 50 people in the country who have undergone Visualase ablation for a hypothalamic hamartoma. After months of feeling grief and hopelessness, we have a renewed hope that our sweet boy is going to have a healthy and happy life. As Dr. Kebriaei told us at Jack’s last appointment, “He’s about ready to take off.”

The neuroscience program at Children’s Hospitals and Clinics of Minnesota unites hundreds of experts in a common mission. We care for more neurology patients, more brain tumor patients and more epilepsy patients than any other hospital in the region. And we do it with some of the most advanced treatments and technologies available today.

Doctor teaches patient’s third-grade class about science of sound, hearing

Leah Tygum, MD (back row, left), visited the third-grade classroom of patient Sophia Nelson (middle row, second from left) at Redtail Ridge Elementary School in Savage to talk about the science of sound and hearing.

Working together was the spark that started a fire of learning and understanding for a class of third-grade students this winter. A collaborative project between staff at the St. Paul campus of Children’s Hospitals and Clinics of Minnesota and Redtail Ridge Elementary School in Savage helped students learn about the science of sound and hearing while creating a lifelong lesson that went well beyond the typical third-grade science curriculum.

“What’s that thing in your ear?” is a question Redtail Ridge third-grade student Sophia Nelson often has heard from classmates. Diagnosed at age 6 with moderate to severe hearing loss in her left ear, Sophia has worn a hearing aid for the past two years, and it has raised questions from several curious friends during that time.

Third-grade teacher Stacy Kodada (left) takes a turn using a small stethoscope placed to Sophia Nelson’s hearing aid.

This winter, when Sophia’s classroom teacher, Stacy Kodada, introduced a science curriculum that included studying the physics of sound, Kodada and Sophia connected about inviting Sophia’s audiologist, Leah Tygum, MD, to visit with Sophia’s classmates. Dr. Tygum, affectionately referred to as “Dr. Leah” by Sophia, has helped transform Sophia’s hearing-loss journey from one that began at diagnosis with fear and concern to one that now is characterized by empowerment and confidence. Since first meeting Sophia, Dr. Tygum has helped provide Sophia with resources to help her – and fellow students – better understand what some might consider Sophia’s “disability.” When invited by Kodada to visit with the third-graders, Dr. Tygum seized the opportunity to help Sophia better educate her classmates on hearing loss and hearing aids.

Prior to the on-site visit, students shared their questions about hearing loss and hearing aids with Sophia and her teacher. Their questions were honest, at times simple, and revealing in terms of a young child’s understanding of those who suffer hearing loss. Students posed several questions, including:

  • What happens inside the ear when you can’t hear?
  • How do you get the sound from the hearing aid into the eardrum into the brain?
  • How do you make a hearing aid?
  • How do you know if you need a hearing aid?

Sophia Nelson (left) had Leah Tygum, MD, visit her third-grade classroom at Redtail Ridge Elementary School in Savage.

On the day of her visit, Dr. Tygum was greeted by a room of students buzzing with excitement and questions. She facilitated more than 90 minutes of hands-on, interactive learning about hearing. Attempting to address all questions, she talked about the science of hearing, what goes wrong with hearing loss and how hearing aids work for those who need them. Using Sophia as a teaching partner, Dr. Tygum took the lesson well beyond Q&A, leading students through a variety of engaging learning activities. Dr. Tygum made a mold of Sophia’s inner ear while the students watched, just as she would do in her office to properly fit a hearing aid. To increase involvement, she then gave students their own individual balls of molding material so they could feel how it hardens into a shape in minutes.

To help make the hearing aid even more “real world” and understandable, the children were each given the chance to use a small hearing aid stethoscope which allowed them to hear sounds in the classroom the way Sophia does when she wears her aid. The exercise proved popular. After listening, children raced back into line in hopes of getting a second chance.

The classroom learning experience helped students understand the science of sound and hearing, but for Kodada it went beyond that.

“Having Dr. Leah come to the classroom allowed the students a different insight into others with hearing disabilities,” Kodada said. “The hands-on experience and real-life demonstration is a wonderful way for students to learn and then be able to apply it to their own life situations.”

While Sophia will probably continue to get questions throughout her life about her hearing loss, she’s certain that none of this year’s classmates will ever ask again “what’s that thing in your ear?”

Photo gallery: Leah Tygum, MD, visits third-grade class at Redtail Ridge Elementary School in Savage (Photos by Michael Nelson)