Microcephaly and Encephalocele: Michelle’s Story

By Allyson Duran, mother of CHOC patient Michelle

When I was 21 weeks pregnant with our little girl, my fiancé and I received devastating news. Michelle’s head was not measuring large enough for how far along I was in my pregnancy. We were scared and didn’t know what this would mean for our little girl. My OB-GYN referred us to a high-risk maternal-fetal specialist, where we received a shocking diagnosis: our baby had microcephaly, a rare condition where a child’s brain does not fully develop, resulting in an abnormally small head size. They also diagnosed her with another condition called encephalocele, where brain tissue protrudes out to the skin from an abnormal opening in the skull. Although very rare – only 340 babies in the U.S. are born with this each year- this is one of the most common neural tube defects, a birth defect involving incomplete development of the brain and spinal cord.

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During the rest of my pregnancy it was a little emotional because we didn’t know if Michelle would survive the pregnancy or even worse, pass away shortly after birth. We didn’t know if she would come out breathing or what to expect. I had ultrasounds every week to monitor the growth of the encephalocele. When I was six months pregnant, my high-risk OB-GYN sent me to meet with Dr. Michael Muhonen, a pediatric neurosurgeon at CHOC. I didn’t know what to expect, but I couldn’t handle any more bad news. He told me the mass growing outside of her brain could either be tissue or fluid, but they wouldn’t be able to tell for sure until after she was born.

He told us that the prenatal ultrasounds showed a giant encephalocele with severe concerning brain anomalies. I was terrified about what was going to happen to my baby, but I felt reassured that she would be in good hands.

Michelle was born October 10, 2016 at 10:34 a.m. at University of California, Irvine. She thankfully came out breathing on her own, and my fiancé and I were an emotional wreck. I couldn’t hold her or kiss her right away because she was whisked away to the neonatal intensive care unit (NICU). Four long hours later, I was finally able to see her and give her a kiss, but I couldn’t hold her yet.

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Dr. Muhonen came to UCI to see Michelle and review her MRI so they could get a better idea of what was inside her encephalocele. While we waited for the results in the NICU, Michelle stopped breathing right in front of our eyes. The NICU nurses rushed to her aide and resuscitated her. She started breathing again on her own, but I can’t begin to describe how scary those two minutes were. My heart broke and fell into my stomach. At two days old, her condition was declining, but I knew in my heart that she was going to be ok.

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After that, a nurse approached us and handed us a little sack and said, “Someone who heard about your situation wanted to give this to you and remain anonymous.” As we opened the little sack we pulled out a key that said HOPE on it. I broke down in happy tears.

Michelle’s MRI results came back literally seconds later. Dr. Muhonen said that he had never seen an encephalocele this big before, but that it was mostly made up of brain fluid, and he was confident that he could successfully operate on her. Later that day, Michelle was transferred via ambulance to CHOC, where she would be prepped for surgery.

The next day, we put our three-day-old baby in the hands of Dr. Muhonen and prayed that her surgery would go well. Three hours later, he came out of the operating room to where we had been anxiously waiting in the lobby, and told us that the surgery gone well.

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“We were able to successfully put the exposed brain back into the skull and remove the outer sac of the encephalocele. The post-operative results were superb, and Michelle had no difficulties with the complicated surgery,” Dr. Muhonen told us.

Michelle stayed at CHOC in the NICU for three weeks to recover and get strong enough to finally go home. During that time, my fiancé and I stayed just down the street at Ronald McDonald House. When our baby was hospitalized, we were grateful to have an incredible team of nurses caring for her. This was my first baby and I had never been through anything like this before, and Michelle’s nurses were very patient with me and explained everything. They taught me things like how to feed her. They spent so much time with her and knew tiny little nuances about her. They were there to help and support not only their patient, but her parents as well.

Our favorite nurse, Maria, told me that one of her favorite parts of being a NICU nurse is empowering parents so that they can eventually play the role of their baby’s biggest advocate.

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Michelle’s favorite nurse, Maria, feeding her a bottle in the NICU.

For a while, we had weekly appointments with Dr. Muhonen to monitor the shunt (a device that relieves pressure on the brain) he implanted a few months after surgery. During surgery. Michelle was doing so well that our appointments were changed to once every three months!

Dr. Muhonen told us, “While Michelle is still growing, and has challenges ahead, I am optimistic that she will love and enjoy life, bring joy to her family, and will always be an inspiration to others.”

Today, Michelle is a happy baby. We are busy with physical therapy, occupational therapy, and infant stimulation appointments, but Michelle laughs and smiles all the time. She loves when people pretend to sneeze. She loves to jump. She is trying to walk, but she doesn’t like to crawl.

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Earlier this year, we participated in CHOC Walk in the Park in honor of our daughter, and to show our support for the wonderful neurosurgeon and all the other CHOC staff members who helped save her life! We are forever grateful and feel blessed for this wonderful hospital for keeping our family whole.

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Learn more about neurosurgery at CHOC

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My Journey from CHOC Patient to CHOC Volunteer

Written by Daniel Boucher, cancer survivor and current CHOC volunteer

My name is Daniel, and ever since I can remember, I have always wanted to play football for the University of Notre Dame. I had many motivations: my dad went there, they put academics before football, and I consider it almost a holy place, where people
“surrender to excellence” in their personal life, academics and community. And I had an athletic build too. At 10 years old, I was already 5 feet 2 inches and well on my way to achieving, if not surpassing, my dad’s height of 6 feet 3 inches. I had a competitive, determined drive in any situation. I was only satisfied if I gave my all.

I was ten years old when everything changed. It was a November afternoon and I was doing what I loved most― playing football. Trying to tackle my little brother, I followed the advice so often quoted in sports: keep your eye on the ball. As a consequence, I ran into a tree face first. I came home and threw up once or twice, but otherwise seemed unharmed. I didn’t really lose consciousness, but my dad took me to the emergency room just to be safe. That visit changed my life. It was a slow day, and there was an available CAT scan machine. Figuring that it would be no harm, the doctor ordered a scan. That scan revealed a cancerous mass sitting on the edge of my spinal cord.

Within hours, I was diagnosed with medulloblastoma, a type of cancer that often spreads to other parts of the brain and spinal cord. I met my pediatric neurosurgeon, Dr. William Loudon, and was scheduled for brain surgery. I remember watching “Honey I Shrunk the Kids” before surgery and telling my younger brother (whether out of innocence or the sheer confidence and determination I applied to every situation) that I’d be home in a week. If my parents heard me say this, they never tried to tell me otherwise. My dad later confided in me that this was one time where he truly thought I wasn’t going to make it. I didn’t fulfill my promise to my brother- after surgery the doctors kept me unconscious for two days, giving my brain the best chance to recover from the surgery, and stayed in the hospital for two weeks before I got to go home. It felt so good to be in my own bed again that I slept for 21 hours straight.

My immune system was weak, and when I came down with a fever I had to go back to the hospital. Unfortunately, it turned out to be a symptom of pressure buildup in my spine. I remember that the hospital was getting in the Christmas spirit at that time. There was holiday music playing and I even got to attend a party while I was admitted, but I got to go home in time for Christmas. After the holidays, I started on my cancer’s treatment regimen under the careful eye of my oncologist, Dr. Lilibeth Torno.

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For the entire month of January, Monday through Friday, I would go to the CHOC Outpatient Infusion Center (OPI) cancer center from 8 a.m. – 12 p.m. There, I received chemotherapy treatments delivered intravenously through my portacath, which connected to an artery on my chest and made access to a blood vessel less painful and more practical than a needle in my arm. I usually slept for the first hour of my infusion. Later in the session, my mom would feed me ramen and read me “The BFG” by Ronal Dahl. Eventually my appetite increased so that I went back to eating my favorite food of corn dogs, and socializing to the point of befriending many of the nurses and asking if they had any ketchup. You can’t eat a corndog without ketchup. The nurses were so kind and hated to see me uncomfortable and they would bend over backward to help. A tall nurse named Ron took especially great care of me, and once I had asked for it, would bring me ketchup every day.

After chemotherapy, my mom and I would walk the CHOC hallways to the radiology department. My radiation treatment was twofold, one dose to the tumor site and one dose to the general brain. When I laid on the table to receive radiation, a special mask molded to my face and tattooed pinpoints on my back helped me line up in the exact right spot. Technicians would line me up and then I would lie for what seemed like hours (it was never that long) until they returned to help me up and send me home.

After I completed this first part of my strict but successful treatment plan, I came into the hospital for one weekend every three weeks. John was my favorite nurse and used to play practical jokes on me to cheer me up. I had a tricky vein but he could always get my IV inserted without hurting me. My dad came with me on these trips, and if I was feeling well enough, I got to visit the play room and receive visitors. My siblings would often come and we loved to play on the X-men arcade console. The machine had been modified to work without quarters, and we fully exploited this.

That June, I finished my last chemo treatment. It was a momentous occasion and my family hosted a party to celebrate. There was a bounce house, a slip and slide, and all my favorite foods. We even made a giant finish line banner across our driveway and t-shirts for the family. My favorite part, however, was visiting with my friends and family who had so generously helped with the behind-the-scenes work. Those who watched my younger siblings while mom was with me in the hospital, who made dinners, and especially who prayed unceasingly for my recovery and for my family.

Was I the same person who had hit that tree almost a year prior? No. Did I still have that physical strength to rush up and down a football field tackling other players? No. But the same attributes I’d always had within me in sports had been used to help me through my struggles. I had developed a different kind of toughness, one that is much more important. I may not have been able to run and throw a football down the field, but with my strong active spirit, I would look for new, less physical, adventures.

Recently I was invited to speak at a fundraiser to benefit neurosurgery at CHOC, so that Dr. Loudon and his colleagues can continue helping more kids the way they helped me when I was a patient. The event was a success, but the best part was getting to hang out with Dr. Loudon.

These days, I’m back at CHOC― this time as a volunteer. I get to entertain kids in the same waiting rooms I used to visit as a patient. I read books, play games, do puppet show, and just be their buddy when they need entertainment or a distraction. I also get to host Turtle Talk, an interactive show in the Disneyland Resort lobby of the Bill Holmes Tower, where patients and siblings can interact and have live conversations with Crush, the animated sea turtle from “Finding Nemo.” Sometimes I even run into Dr. Loudon and Dr. Torno. I really appreciated the efforts of CHOC to make me motivated and happy, (not to mention the medical care to recover from my disease), and am now proud to help make your stay as good as mine.

Learn more about neurosurgery at CHOC

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From CHOC Neurosurgery Parent to CHOC Chaplain

The first time Chaplain Steve came to CHOC Children’s, he had just found out his daughter Catie needed an emergency neurosurgery. Now, he’s back for good― as the newest chaplain on CHOC’s spiritual care team.

Chaplain Steve and Choco Bear
Chaplain Steve is the newest member of CHOC’s spiritual care team. But first, he was a CHOC parent.

Ever since pre-school, Catie had experienced difficulties with body awareness, some motor skills, coordination, attentiveness in school and other challenges that didn’t seem to fit her developmental stage.

For five years, Steve and his wife Claudia explored everything: psychiatry, ophthalmology, behavior modification, medication, coaching, neurofeedback and more. Finally, when she was 8 years old they advocated for a referral to a pediatric neurologist, rule out the possibility that there was something wrong with her brain. Even though Catie didn’t show any of the physical symptoms typically associated with a mass in her brain, such as headaches, seizures, fainting or major motor problems, their neurologist ordered an MRI just to be safe.

After five years of trying new therapies and hitting dead ends, Steve and Claudia didn’t know what to expect from Catie’s MRI results, or if they were finally about to get answers.

Catie’s scans revealed that she had a large arachnoid cyst in her brain. The fluid-filled sac measured 10 centimeters, about the size of a baseball.

“I was in shock” Steve says of the moment his wife called him with the results. “I remember exactly where I was and exactly what I was doing at that time. I remember begging my wife, “Please tell me you’re kidding. Please tell me you’re kidding.”

Dr. William Loudon, a pediatric neurosurgeon at CHOC Children’s came to The Julia and George Argyros Emergency Department to meet the family, who was trying to process a lot of information at once.

Dr. William Loudon CHOC pediatric neurosurgeon
Dr. William Loudon, a pediatric neurosurgeon at CHOC Children’s.

“He told us that this was serious, but that they were going to take care of it right away,” Steve recalls. “He explained very clearly what he was going to do to drain the cyst and how he was going to do it.”

Steve and Claudia didn’t understand how a cyst could have been growing for years inside her skull undetected.

“Although Catie hadn’t yet shown physical side effects, she inevitably would have begun to decompensate, which would’ve greatly increased her risk of injury,” said Dr. Loudon.

Since Catie is the oldest child in her family and the first to undergo a major surgery, her parents were naturally worried, about everything from anesthesia to recovery

“Dr. Loudon told us that he would care for our daughter as if she were his own child,” Steve says. “Since working at CHOC, I’ve heard him tell other families in the emergency department the same thing. I know that he means it every time.”

Dr. Loudon performed a series of surgeries to open the cyst and allow it to drain internally, a procedure known as endoscopic cyst fenestration. He made a small cut in her skull and then punctured a tiny hole on either side of the cyst to allow the fluid to drain internally over a period of time.

Dr. Loudon’s commitment to Catie’s safety was deeply appreciated by her parents.

“I saw the way his team acted, and how they interacted with my daughter,” Steve recalls. “Dr. Loudon takes his job very seriously and he goes after the problem. We knew she was in good hands.”

Catie recovering from neurosurgery at CHOC
Catie recovering from neurosurgery at CHOC

With every hospital stay, Steve found that his own natural instinct was to offer support to other parents, whether it be in the waiting room or the pediatric intensive care unit (PICU). By this point, Steve had been a chaplain in a hospice setting for six years.

“Even while we were the ones receiving care, my first reaction was always to rush to other families in need, but since I was there as a parent, there was only so much I could do,” he says.

Now that Chaplain Steve has officially joined the spiritual care team at CHOC, he is able to offer spiritual and emotional support to patients and families.

“I have my own beliefs and faith traditions, but these come secondary to what a family needs in a time of crisis,” Steve says.

Today, Catie is a high school student who loves science, space and kids. She hasn’t been hospitalized since her last surgery, although a few years ago she came back to CHOC with a broken foot that she got “pretending to be a ninja,” as her dad says. She still treasures the Choco bear that she received when she was a patient, but sometimes loans him to her little brother if he’s feeling under the weather.

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Catie’s family is happy to report that she has not been hospitalized in the last five years.
Learn more about neurosurgery at CHOC

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CHOC Included Among Nation’s Best Children’s Hospitals in U.S. News Survey

CHOC Children’s has been named one of the nation’s best children’s hospitals by U.S. News & World Report in its 2017-18 Best Children’s Hospitals rankings.

CHOC ranked in seven specialties: cancer, diabetes/endocrinology, neonatology, neurology/neurosurgery, orthopedics, pulmonology and urology, which earned a top 20 spot on the coveted list.

According to U.S. News, the Best Children’s Hospitals rankings were introduced in 2007 to help families of children with rare or life-threatening illnesses find the best medical care available.

The 11th annual rankings recognize the top 50 pediatric facilities across the United States in 10 pediatric specialties.

The U.S. News Best Children’s Hospitals rankings rely on clinical data and on an annual survey of pediatric specialists. The rankings methodology considers clinical outcomes, such as mortality and infection rates, efficiency and coordination of care delivery and compliance with “best practices.”

“At CHOC Children’s, we are steadfastly committed to delivering high-quality, safe and reliable health care to our patients,” said Dr. James Cappon, CHOC’s chief quality officer. “Recognition from U.S. News of our excellence in these seven subspecialties validates our efforts, but also provides our patients and families with even more assurance of our commitment to excelling in all areas of care.”

“The pediatric centers we rank in Best Children’s Hospitals deliver exceptionally high-quality care and deserve to be recognized for their commitment,” U.S. News Health Rankings Editor Avery Comarow said. “Children with life-threatening illnesses or rare conditions need the state-of-the-art services and expertise these hospitals provide every day.”

Learn more about survival rates, adequacy of nurse staffing, procedure and patient volume, availability of programs for particular illnesses and conditions and more. 

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CHOC Walk in the Park 2017: Why I Walk – Team Miranda

Seven years ago, Miranda woke up screaming in the middle of the night from unbearable head pain. She couldn’t move or recognize her parents, and then she collapsed.

Miranda’s parents rushed her, a 10-year-old at the time, to a local hospital. Physicians there initially told them to wait three days for observation.

Dr. Jason Knight, a CHOC Children’s pediatric critical care specialist and medical director of CHOC’s emergency transport services, was on call at that hospital that day, and met with Miranda’s parents, Regina and John.

An Emergency Transport to CHOC

“Dr. Knight explained what was happening better than anyone else at the hospital,” Regina remembers. “He suggested we transport her to CHOC so she could be seen by pediatric specialists. We were scared to move her, so we asked Dr. Knight if he would transport Miranda if she were his own daughter. He said yes, and we trusted him.”

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Dr. Jason Knight, critical care specialist, chair of the department of medicine, and medical director of emergency transport services at CHOC Children’s

Dr. Knight recalls, “As the physician covering the local hospital that day, it was my responsibility to assure that Miranda received the appropriate care in a timely manner. As soon as I assessed her condition and reviewed the CT scan of her brain, it was clear to me that Miranda needed to be transferred to CHOC immediately. I contacted Dr. Michael Muhonen, a pediatric neurosurgeon at CHOC, and coordinated Miranda’s transfer to CHOC.”

Scans revealed she had an arteriovenous malformation (AVM), a tangle of weakened blood vessels that had ruptured and started to bleed in her brain. Upon arrival at CHOC, Miranda immediately underwent neurosurgery with Dr. Muhonen, medical director of the neuroscience institute at CHOC.

“Miranda was critically ill upon arrival to CHOC. She reminded me of my own three daughters― innocent, beautiful and vulnerable. It was with this in mind that I approached her operation― do what it takes to keep her brain alive and functional,” recalled Dr. Muhonen.

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Dr. Michael Muhonen, medical director of the neuroscience institute and director of neurosurgery at CHOC Children’s

After a successful four-hour surgery, Miranda was placed in a medically-induced coma for several days. Her parents didn’t know if she would survive.

“When Miranda was in a coma, I told Dr. Knight that I wanted to take a photo of her so that I could show her when she woke up, but that I didn’t want to take it if she wasn’t going to make it,” recalls Regina. “For a few days, he wouldn’t let me take the photo because he wasn’t sure if Miranda was going to survive. Then one day, Dr. Knight told me it was ok to take a photo of her, and I knew that meant she was going to make it.”

John adds, “My family is intact today because of wonderful doctors like Dr. Knight, Dr. Muhonen and their teams. It’s just amazing what CHOC does. We are so blessed.”

The Long Road to Recovery

Miranda’s recovery process included child life specialists, physical therapy and rehabilitation. She spent one month at an inpatient rehabilitation facility followed by an additional three months of outpatient care learning how to walk and talk again.

The first year after surgery was critical. Miranda saw Dr. Muhonen every few months for checkups to monitor her brain, as well as a CHOC ophthalmologist to make sure her sight was progressing.

“The AVM and the clot were removed successfully, and Miranda has recovered with minimal long-term deficit,” says Dr. Muhonen. “My reward is not only seeing a disease-free brain on the post-operative MRI, it is the omnipresent smile and effervescent personality that Miranda still has, despite her near-death experience.”

Everything came back except her sight. She lost 25 percent of her vision in both eyes, but that hasn’t stopped her from dancing, her favorite activity since age 8. Miranda’s parents had to supervise her at all times for the first year after surgery to make sure she didn’t have a seizure (a possible side effect of brain surgery), which meant that her mom went to every dance practice and every pool party to ensure her daughter’s continued safe healing.

Giving Back to CHOC

A few months after Miranda’s surgery, her family was invited to participate in CHOC Walk in the Park by friends.

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Miranda and her family at their first CHOC Walk in the Park, just a few months after her emergency neurosurgery

“After what we went through, we felt closely tied to CHOC. We walked that year and fell in love with the event. We realized this was an affordable way to give back to CHOC,” recalls John. “My favorite memory was our very first CHOC Walk. Miranda walked alongside us, which was a huge accomplishment. A couple months before that, we weren’t sure if she was ever going to walk again. To see her walk on her own was the most memorable moment for me.”

The family’s first CHOC Walk was especially impactful for Miranda herself.

“I will never forget attending my first CHOC Walk one year after my surgery. I remember seeing other patients participating in the walk with their own team just like me, only they needed walkers or wheelchairs to have the capability of walking the entire distance. Seeing other patients that were struggling to do the simple task of walking, something that people take for granted, made me reflect back to being a patient at CHOC, where I once struggled with relearning how to walk during physical therapy,” recalls Miranda. “From that moment on, I promised myself I would always participate in the CHOC Walk and give back as much as I could, not only to represent the patients who couldn’t recover as fast as I did, but also for the patients that don’t make it. I am reminded every day by the scar on the back of my head of how blessed I am to have been a patient at CHOC.”

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Every year since her emergency surgery, Miranda’s family rallies dozens of friends to participate in CHOC Walk in the Park.

Every year since then, Miranda’s parents gather dozens of friends, family and co-workers to join Team Miranda, the CHOC Walk team they formed in honor of their daughter. They formed their team with the help of the Littlest Angel Guild, a philanthropic group that raises funds to support the mission of CHOC.

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Miranda and her family at the 2012 CHOC Walk in the Park

“We encourage people we meet to participate in CHOC Walk to help provide the best medical care for the children in our community. You never know if or when CHOC’s services will be needed for your children, for your friend’s children, for your neighbor’s children, or even a stranger,” says John, whose employer, Hill Brothers Chemical Company also sponsors CHOC Walk, in addition to the other philanthropic events benefiting CHOC. “We learned firsthand how much everyone at CHOC cares – the doctors, the nurses, the administrators, and staff throughout CHOC all genuinely care about the children and their families.”

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Team Miranda at the 2013 CHOC Walk in the Park

Experience at CHOC Inspires Future Career

Now a senior in high school, Miranda is preparing for graduation and heading off to college. Inspired by her experiences at CHOC, she is planning to major in psychiatry and dreams of becoming a pediatric optometrist.

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