December 6, 2016

12716mechanicalleechpg640Have you ever swam in a lake or trudged through swampy waters? If so, you probably have encountered leeches during your outdoor adventures. These worms have been used in human medicine for centuries because of their anesthetic, anti-inflammatory and tissue healing properties.

Primarily used in plastic surgery to restore blood flow to reattached body parts, leeches are considered natural healers. While removing deoxygenated blood from damaged tissue, their saliva contains hirudin, an anti-blood clotting protein, that allows oxygenated blood to flow to the injured tissue until normal circulation can be restored.

While leech therapy has never been used in gynecology and is rarely used in pediatrics due to the psychological impact it may have on patients and parents, what if there was a way to develop a mechanical device that could perform the same functions as a traditional leech?

Dr. Julie Hakim, a pediatric and adolescent gynecology fellow at Texas Children’s and Baylor College of Medicine, proposed this idea to a group of biomedical engineering students at Texas A&M University (TAMU) who then developed a mechanical leech prototype for potential use in pediatric gynecological surgeries.

“In pediatric gynecology, we often perform vaginal reconstructive surgery for girls who are born with congenital abnormalities,” Hakim said. “A graft of tissue is taken from somewhere else on the patient’s body to line the new vaginal tract. However, there are times when the grafts fail or do not take because of poor blood circulation to that area. Developing a novel mechanism to promote graft and tissue flap survival by increasing neovascularization of the tissue would help in these type of surgeries.”

With support from the Denton Cooley Innovation Award, the mechanical leech project and design requirements were presented to TAMU students on their first day of Senior Design Course. Two teams totaling eight students met every week with Hakim via Skype to provide updates on their progress. They also met with Dr. Jennifer Dietrich, Texas Children’s chief of Pediatric and Adolescent Gynecology, to present their updates once per semester.

The head of the mechanical leech was created with a small, flat circular unit measuring 1×1 cm fitted with an array of microneedles underneath for leech saliva egress and blood extraction that interfaces with the tissue. The head contains an outer reservoir for leech saliva and an inner reservoir that collects the extracted blood.

The leech body contains pumps that control and monitor the outflow of leech saliva and the inflow of blood, saliva and blood reservoirs. A blue tooth chip inside the pumps’ electronic assembly communicates through an iPhone app that runs the mechanical leech. The motor’s speed and the flow rate of the fluids can be recorded in real time and adjusted remotely via the iPhone app. The outer shell of the leech body and the gear mechanisms are connected by standard medical tubing.

“The mechanical leech has the potential to reduce complications associated with the use of live leeches,” Hakim said. “Since uncontrolled amounts of leech saliva can cause prolonged bleeding, controlling how much leech saliva is placed into the tissue can reduce the risk of transfusion. This mechanical novel device can also help reduce infection since live leeches are known to harbor bacteria.”

The mechanical leech head and the outer shell of the leech body and the gear mechanisms were 3-D printed. The project’s next step is to refine and test the feasibility of the prototype.

“I think this innovation could be of tremendous importance to the field of pediatric gynecology as well as pediatric surgery in general,” Dietrich said. “I’m excited to see where the next phase of this project takes us as we explore new avenues for improving the care we provide to our patients.”

12716pediatricpilotawardinside900Dr. Jordan Orange, vice chair of research in the Department of Pediatrics, announced the winners of the 2016 Pediatric Pilot Awards Research Grant Program. Ten research applications were chosen by review committee members to receive grant funding in the amount of up to $50,000 for their projects.

The Pediatric Pilot Awards Research Grant Program provides initial start-up “seed funding” to support research projects. This grant program provides opportunities for new or less established researchers as well as experienced researchers who desire to expand their area of research. The grant projects are awarded based upon their scientific merit and the potential to generate the initial data necessary for a successful grant application submission to the National Institutes of Health or other external, peer-reviewed funding mechanisms.

The pilot award program is a collaborative effort between Texas Children’s Hospital and its academic partner, Baylor College of Medicine.

Congratulations to the following 2016 pilot grant awardees. View the names below to learn more about the research project being funded.

12716drsaurabhagarwal175Saurabh Agarwal, Ph.D.
Pediatrics – Hematology/Oncology
Epigenetic targeting of neuroblastoma cancer stem cells

More than half of the patients with high-risk neuroblastoma (NB) will relapse despite intensive multimodal therapy. Treatments for these patients are challenging due to disease heterogeneity, drug resistance, and toxicity. Thus, novel effective therapies are urgently required to specifically target those tumor cells which escape initial treatment and regenerate chemotherapy resistant recurrent disease.

We identified a G-CSF receptor expressing (CD114+) neuroblastoma cancer stem cell (CSC) subpopulation that is drug resistant, drives metastasis and may cause drug resistant relapse. These highly tumorigenic CSCs are distinguished by specific epigenetic alterations that lead to the expression of specific stem cell genes and maintenance of neuroblastoma CSCs.

We found that epigenetic modifiers MLL1 and JMJD3 increase the expression of G-CSF receptor gene (CSF3R) in NB CSCs by maintaining active histone modifications. Our pre-clinical studies show that blocking these epigenetic modifiers with specific small molecule inhibitors leads to neuroblastoma tumor regression and blockage of metastasis in vivo.

This pilot award will further enable us to test novel dual therapeutic approach by combining epigenetic inhibitors with standard chemotherapy for targeting both stem and non-stem neuroblastoma subpopulations. These studies will define specific epigenetic mechanisms contributing to the maintenance and tumorigenicity of NB CSCs, and pave the way for further clinical translation of our findings to block NB CSC-driven relapse and to advance a novel curative approach to neuroblastoma.
12716drwendyallenrhoades175Wendy Allen-Rhoades, M.D.
Pediatrics – Hematology/Oncology
Validation of a plasma microRNA panel as a biomarker for osteosarcoma

Osteosarcoma is the primary bone cancer in children and young adults. Currently, there are no reliable, non-invasive biological markers to detect the presence or progression of disease, assess therapy response or provide upfront prognostic insights. MicroRNAs (miRNAs) are evolutionarily conserved, stable, small non-coding RNA molecules that are key post-transcriptional regulators and are ideal candidates for circulating biomarker development due to their stability in plasma, ease of isolation and the unique expressions associated with specific disease states.

In our previous work, we analyzed more than 750 plasma miRNAs from a genetically engineered mouse model of osteosarcoma and identified a diagnostic panel of four plasma miRNAs. This diagnostic panel was able discriminate healthy from diseased animals. Subsequent analysis of 70 human patient samples corroborated these results and the diagnostic panel could discriminate healthy patients from patients with osteosarcoma. Furthermore, low plasma levels of miRNA-214 in metastatic patients at time of diagnosis were prognostic and conveyed a significantly better overall survival.

With the funding from the Pediatric Pilot Award, we will continue the necessary steps to fully validate this novel biomarker by completing validation of the diagnostic and prognostic miRNA biomarkers in 200 additional human samples. The long-term goal of this project is to test these new biomarkers in a prospective clinical trial.
12716drsaraanvari175Sara Anvari, M.D.
Pediatrics – Immunology, Allergy and Rheumatology
Defining biomarkers of successful peanut oral immunotherapy

Peanut allergy is one of the most common causes of severe and fatal allergic reactions related to food. The prevalence of peanut allergy has nearly tripled in the last 20 years and current standard of care for peanut allergy is strict avoidance of peanuts and ready access to emergency medications. While recent research has demonstrated that early introduction of peanuts, instead of avoidance, during infancy can greatly reduce the risk of a peanut allergy, this strategy is not applicable to individuals who have already developed an allergy.

For older children, teens, and adults, peanut oral immunotherapy (pOIT) is one method by which peanut allergies can be treated through step-wise introduction of peanut protein. This introduction is an effort to manage and reduce the allergic reactions in patients. However, how pOIT alters patients’ immune systems to recognize peanut protein as benign instead of “dangerous” (the nature of severe allergy) is poorly understood. Additionally, biomarkers, or testable indicators of efficacy, for pOIT success in a given patient are also still unknown at this time.

Most current research is focused on how pOIT modifies a white blood cell population, called T regulatory cells, which help control the severity of inflammation caused by immune reactions inside the human body. But T regulatory cells are just one end point of a larger set of immune reactions to pOIT. My research program will focus upstream of T regulatory cells on another population of white blood cells, called dendritic cells, which can communicate with and modify T regulatory cell, as well as several other types of white blood cells.

Specifically, the research award will help (1) identify biomarkers to predict a patient’s success (i.e. peanut tolerance) or failure (i.e. persistent peanut allergy) early in the course of pOIT, without waiting to complete three years of therapy; (2) development of targeted therapies for peanut allergic individuals aimed at altering dendritic cell populations to better modulate T regulatory populations which aid in the reduction of severe inflammatory reactions which make peanut allergies so life threatening.
12716drevelinebarbieri175Eveline Barbieri, M.D.
Pediatrics – Hematology/Oncology
Targeting MYCN-amplified neuroblastoma through RORa activation

The MYCN oncogene is a transcription factor frequently upregulated in high-risk neuroblastoma, which is profoundly involved in neuroblastoma initiation and progression. Thus, strategies antagonizing MYCN activity are a vital need in neuroblastoma therapy and the focus of this proposal.

Our laboratory has discovered that MYCN-driven neuroblastoma has an increased dependence on glutamine and lipid metabolism. Recent findings in other tumor types suggest an important link between these metabolic pathways and the circadian clock, which is disrupted in aggressive malignancies.

This led us to investigate how MYCN oncogenic signaling, circadian clock, and neuroblastoma metabolic tumor reprogramming are interrelated. Intriguingly, we have found that RORα signaling, a central component of circadian clock, is lost in MYCN-amplified neuroblastoma and this contributes to aberrant tumor proliferation.

Our specific aims will: 1) determine the metabolic programs activated by RORα in MYCN-driven tumors, and 2) determine the in vivo anti-tumor effects of RORα reactivation in pre-clinical neuroblastoma models. These studies will offer insights into critical molecular and metabolic alterations, which will provide new and more sensitive targets that could be strategically deployed with currently available therapies to treat this highly aggressive disease. Moreover, many enzymes in this pathway are amenable to small molecule inhibitors and therapeutic targeting of RORα-mediated metabolism is moving to the clinic.
12716drjennydespotovic175Jenny Despotovic, D.O.
Pediatrics – Hematology/Oncology
Genetic variants and gene expression patterns in acute and chronic immune thrombocytopenia

Immune thrombocytopenia (ITP) is an autoimmune disorder and one of the most common causes of low platelets in children. Twenty-five percent of affected children develop chronic ITP and some have significant morbidity and mortality. Currently, it is impossible to predict an individual patient’s clinical course and likelihood of spontaneous remission at the time of diagnosis.

Identification of children more likely to develop chronic ITP at diagnosis would improve treatment decisions and could also help identify important mechanisms of disease that could lead to more tailored treatment. Based on strong preliminary data produced in our laboratory, we believe that acute and chronic ITP are distinct diseases that can be distinguished at diagnosis; and specific genetic changes and gene expression differences influence the development of chronic ITP.

In our study, we are collecting DNA at enrollment on all patients with ITP, as well as RNA on patients with acute ITP at the time of diagnosis and at the time of disease resolution. For patients with chronic ITP, we are obtaining RNA at several time points. We will use the most current sequencing technologies to look for changes that may help explain differences between these two disorders with the eventual goal of identifying markers that could be used to distinguish the two disorders at diagnosis so that we could determine how to best approach each patient.
12716drjohnhollier175John Hollier, M.D.
Pediatrics – Gastroenterology
Efficacy of pre-recorded guided imagery session on pediatric gastrointestinal pain disorders managed in primary care

Up to 20 percent of school-age children and adolescents throughout the world are afflicted by recurring abdominal pain that cannot be explained by routine medical laboratory tests or procedures. These children miss more school and rank their general well-being much lower than their healthy counterparts. These disorders also may be associated with psychological distress like anxiety and depression.

One of the most effective treatments for these “functional gastrointestinal pain disorders” (FGIDs) fall under the category of cognitive behavioral therapy. However, access to this type of therapy often is not available due to lack of insurance coverage and/or scarcity of trained healthcare professionals.

Researchers have previously demonstrated the success of guided imagery, a type of cognitive behavioral therapy in treating FGIDs. Guided imagery can be delivered via using compact disc players so that patients can receive therapy at home. Our goal is to find out if audio-recorded guided imagery can be used to treat FGIDs when children are seen in the primary care setting (i.e., by their pediatrician or nurse practitioner). If so, we would be able to get treatment to these children sooner and likely decrease the need for them to be referred to a specialist (gastroenterologist). Our long term research goal is to use mobile cost effective technologies to improve the clinical care of patients with FGIDs and other pediatric diseases.
12716drandrewlandstrom175Andrew Landstrom, M.D.
Pediatrics – Cardiology
The role of junctophilin-2 in the regulation of cardiac nodal tissue

Diseases that impact the nodal tissue of the heart, such as the heart’s pacemaker, can be life-threatening. Children can suffer from these arrhythmias following surgery, through inheritance within families or for no identifiable reason. These arrhythmias can cause fainting, inability to play with the same energy as other children, or even death from collapse of the circulatory system. Despite how serious nodal disease can be, little is known about how these cells beat and how misbeats can occur. Since basic science has limited understanding of this specialized tissue, the therapies levied against nodal disease are toxic and can be ineffective.

A major reason for the lack of specialized therapies is the absence of experimental models which accurately reflect the arrhythmia. We have created an unparalleled mouse model with cardiac nodal disease that can be molecularly triggered to have arrhythmias from the nodal tissue of the heart. This mouse hosts a molecular switch which allows exposure to a pharmacological trigger to decrease the amount of a protein named junctophilin-2 (Jph2) specifically in the heart.

We have previously shown that reduction in the normal amount of Jph2 in the muscle cells of the heart causes calcium to leak into the cell. This causes a loss of contractile force and cardiac failure. We have also found that human mutations in the gene which encodes Jph2 can lead to cardiac hypertrophy as well as atrial fibrillation. All of these diseases are associated with early, and sometimes sudden, death. We have recently found that expression silencing of Jph2 specifically in the nodal tissue results in a rapid resting heart rate and an arrhythmia known as accelerated junctional rhythm. Our early studies have given strong evidence that this mouse has nodal disease that is very similar to many of the children which suffer from nodal dysrhythmias.

With support from pilot research grant, we hope to delve into the physiology of the cardiac pacemaker and to discover the molecular causes of nodal arrhythmias. We believe that the same calcium signaling that becomes perturbed in the muscle cells of the heart may be to blame for these arrhythmias. Careful interrogation of this possibility, and dissection of the molecular underpinnings of this mouse’s arrhythmias, will offer the first insights into the nodal diseases which remain unexplained and ineffectively treated.
12716drjennettemoreno175Jennette Moreno, Ph.D.
Pediatrics – Nutrition
Assessment of differences in children’s circadian rhythms during the school year and summer vacation

Consistent evidence indicates that school age children demonstrate improvements in their weight status during the school year, yet gain substantial weight during summer. These summertime increases in body mass index (BMI) increase children’s risk for becoming overweight or obese. Further, children at risk for developing chronic health conditions associated with obesity are more likely to demonstrate increases in BMI during summer. Preventing increases in children’s weight during summer may be an important opportunity to address the obesity epidemic in children.

Obesity is conventionally considered a problem of imbalance in energy intake (diet) and expenditure (physical activity/sedentary behavior). There is growing awareness of the role of sleep and circadian rhythms in the development of obesity, yet differences in children’s sleep and circadian rhythms during the school year and summer have not been examined.

Circadian rhythms are internal processes present in all living things that operate on a roughly 24 hour cycle. Behavioral rhythms such as the timing of meals and going to bed and waking up at a consistent time are some of the behaviors known to promote stable circadian rhythms. Changes in sleep and behavioral rhythms may result in disruption of circadian rhythms. Because summer vacation is associated with changes in children’s sleep and behavioral rhythms, the school year and summer vacation paradigm offer an important opportunity to expand our scientific understanding of the role of disruptions in sleep and circadian rhythms on the development of obesity in children.

Little is known about differences in children’s sleep and circadian rhythms during the school year and summer. With the current proposal, we plan to address this gap in scientific knowledge by measuring differences in children’s sleep and circadian rhythms during the school year and summer. We will assess whether differences in sleep and circadian rhythms are related to changes in children’s weight during the school year and summer. These data will may lead to novel approaches to the prevention of obesity in children.
12716drrobinparihar175Robin Parihar, M.D.
Pediatrics –Hematology/Oncology
Testing a novel non-invasive method to assess efficacy of tumor microenvironment-directed immune therapy

Some children with cancer have solid tumors, or collections of abnormally growing cells, within their organs. These collections are made up of mostly cancer cells, but also of accessory cells that help the tumor hide from the body’s immune system and grow – collectively called the tumor microenvironment. Our laboratory created a new type of immune therapy to specifically target and destroy these accessory cells found within the tumor microenvironment so that they can’t help the cancer grow.

One of the main problems for testing our immune therapy in patients with solid tumors is that we can’t detect these accessory cells without performing a biopsy procedure of the tumor inside the body. In order to detect the accessory cells in patients at many different times during their therapy, we would have to perform repeated invasive biopsy procedures, which come with additional risks and costs. There is currently no non-invasive method by which to determine the effectiveness of therapies that target the tumor microenvironment. If strategies targeting the tumor microenvironment are to be tested in humans, non-invasive methods will need to be developed to evaluate their effectiveness, thereby circumventing the need for repeated invasive biopsies.

Our project involves the creation and testing of a new type of CAT scan that can indirectly detect the accessory cells of the tumor microenvironment. If successful, our new CAT scan can be used to detect changes in the number of accessory cells in patients receiving our new immune therapy, without the need for repeated invasive, risky, or costly procedures. This new CAT scan can be used in clinical trials of other immune therapies as well and may be applied to both children and adults with cancer. The long term goal of the project is to develop a clinical imaging tool that will allow doctors to follow changes within the tumor microenvironment induced by immune therapies.
12716drsarahsartain175Sarah Sartain, M.D.
Pediatrics – Hematology/Oncology
The linkage between hemostasis-thrombosis, complement, and inflammation in the pathophysiology of thrombotic microangiopathy

The goal of our research is to improve the health of patients with thrombotic microangiopathy, a group of disorders that cause anemia, low platelets, clots in the blood vessels, and blood vessel damage of the brain, heart, and kidneys. The mechanisms of small blood vessel damage in thrombotic microangiopathy are not precisely defined.

We will investigate the means by which thrombotic microangiopathy causes blood vessel injury and organ damage. We believe that the immune system is involved in the process of vessel injury in thrombotic microangiopathy. This is based on previous work showing that components of the immune system known as the “alternative complement pathway” bind to, and become activated on, long and sticky von Willebrand factor (VWF) strings secreted from blood vessel walls. These VWF strings normally attract platelets to initiate blood clot formation. We intend to determine if activated alternative complement components on these strings contribute to blood vessel injury. We will also determine if a powerful molecule produced during inflammation (known as “tumor necrosis factor”) controls activation of the alternative complement pathway on the VWF strings, contributing to heart, brain, and kidney blood vessel injury.

Our proposed research has long-term biomedical significance because determining the mechanisms of blood vessel/organ injury in thrombotic microangiopathy will lead to the development of therapies to improve the outcomes in this disorder and may be applicable to more common types of blood vessel injury in the general population.

12716bellowsconstructioncrewmeetselsa640Whether you’re walking the halls of the hospital at main campus or glancing outside the window, you may have noticed a new addition to the Pediatric Tower construction site – an 8-foot cardboard cutout of Elsa!

Elsa is Texas Children’s new therapy dog. She has been seeing patients across the inpatient population since October and provides goal-oriented therapeutic interventions. The positive response to her arrival has been overwhelming. Since Elsa is unable to see all patients, Child Life recently launched a fun project for patients, families and staff so they can interact with Elsa in other ways.

An 8-foot replica of Elsa wearing a construction hat can be found in a different location every week in the Pediatric Tower. The Bellows construction team is joining in on the fun and they are moving Elsa each week to allow patients, family and staff to enjoy the weekly game of “Where’s Elsa?”.

There are interactive activities to engage patients and families who find Elsa and a weekly event in the Zone on the 16th floor of West Tower for patients and families to have fun searching for Elsa. For those patients who are on isolation, there are specialized activities to ensure they are able to participate in the game too.

Join in on the fun and see if you can find Elsa this week! Engage your patients and families to find Elsa too!

And as always, Elsa is ready to greet you when she sees you in the hall and continues to be incredibly excited to be a part of the Texas Children’s team.

December 5, 2016

12516zoghbi640On Sunday, December 4, Texas Children’s and Baylor College of Medicine’s pioneering neuroscientist Dr. Huda Zoghbi was honored with the Breakthrough Prize in the field of Life Sciences during a star-studded ceremony in San Francisco’s Silicon Valley.

Awarded annually to the world’s top scientists in fundamental physics, mathematics and the life sciences, the Breakthrough Prize is considered Silicon Valley’s most significant science prize for what they cite as paradigm-shifting research. The Prize was founded by Sergey Brin (Google), Anne Wojcicki (23andMe), Mark Zuckerberg (Facebook), Priscilla Chan (Chan Zuckerberg Initiatives), Yuri Milner (DST Global) and Julia Milner.

Presented during a live broadcast on the National Geographic Channel, Zoghbi was recognized for her discoveries of the genetic causes and biochemical mechanisms of spinocerebellar ataxia, a neurodegenerative disorder affecting balance and coordination, and Rett syndrome, a genetic neurological disease characterized by the loss of motor skills, speech and other cognitive abilities affecting girls one or two years after birth. Prior to Zoghbi’s pioneering work in neuroscience, little was understood about the causes of these diseases, let alone how to potentially treat or cure them.

As one of the world’s leading neurogeneticists, Zoghbi, director of the Jan and Dan Duncan Neurological Research Institute (NRI) at Texas Children’s and Baylor, has been instrumental in other important medical breakthroughs in neurological disease research including her most recent discovery of how lowering toxic levels of tau in the brain could potentially lead to new therapies to reverse or prevent Alzheimer’s disease.

“We are tremendously proud of the transformational work Dr. Zoghbi and her team are doing at the NRI. The numerous discoveries coming from Texas Children’s and Baylor will have an immeasurable impact on so many of our families suffering from unexplainable neurological diseases,” said Texas Children’s President and CEO Mark A. Wallace. “Please join me in congratulating Dr. Zoghbi for this well-deserved and prestigious global recognition.”

Zoghbi plans to donate the majority of her $3-million Breakthrough Prize to support education and research initiatives. She wishes to recognize the institutions that impacted her career including Texas Children’s and Baylor. The gifts will help advance genetic and neuroscience research.

Zoghbi thanked her supporters with the following acceptance speech: “As a young doctor, I found it heartbreaking to watch my patients, young and old, lose their lives to neurological diseases. I turned to research for answers, and today, together with collaborators and trainees, we are charting new paths towards viable therapies. It’s thrilling to see we are beginning to understand the language of life and translate it to help mankind. I feel fortunate to have had my career nurtured by two of the finest institutions, Baylor College of Medicine and Texas Children’s Hospital and to have the support of the Howard Hughes Medical Institute. I thank my husband William and our children, Roula and Anthony, for their unwavering support, and they share this honor with me today.”

Click here to learn more about the ceremony and this award including a feature story on Zoghbi from the Houston Chronicle. FOX will air a one-hour version of the ceremony on Sunday, December 18, at 6 p.m.

In addition to this accolade, Zoghbi has earned dozens of honors and awards, including most recently the 2016 Shaw Prize in Medicine and the 2016 Jessie Stevenson Kovalenko Medal for her research.

November 29, 2016

113016surgicaloncologyinside250Texas Children’s Cancer Center is renowned for extraordinary care and outcomes and is ranked No. 2 in the nation by U.S. News & World Report. An important part of the program’s success is the multidisciplinary care children here receive – particularly in the area of surgical oncology. Many solid tumors – such as liver tumors, bone tumors, neuroblastomas and sarcomas – require complete surgical removal. Chemotherapy and radiation may be used to shrink the size of the tumor or to keep it from coming back after surgery, but surgical removal is a critical step for children with these cancers. In these cases, oncologists and surgical oncologists must work together to carefully plot the course of treatment and time the surgery just right to give young patients the very best chance at a cancer-free life.

Patient diagnosed with hepatoblastoma

In September 2015, Dr. Maria Garcia Fernandez, a pediatric infectious disease specialist, and Dr. Fernando Padilla, a family practitioner, discovered a mass in their 17-month-old baby Victoria’s abdomen. Fearing the worst, they immediately contacted the Solid Tumor Program at Texas Children’s Hospital, where Victoria was promptly evaluated and diagnosed with stage 3 hepatoblastoma.

Hepatoblastoma is a relatively rare type of childhood cancer, with approximately 200 cases diagnosed per year in the country. Usually occurring in children under the age of 5, there are often no initial symptoms other than the mass.

“Hepatoblastomas tend to present very large, because the liver is tucked under the ribs so the mass is hard to feel,” said Dr. Sanjeev Vasudevan, Victoria’s surgical oncologist specializing in liver surgery. “You have to remove the side of the liver that the tumor inhabits without damaging the normal side and still get the tumor completely out.”

The stakes for this type of surgery couldn’t be higher.

“If you attempt to remove the mass and wind up leaving some of it behind, the prognosis for the child becomes much more serious,” Vasudevan said. “Basically, if you can’t guarantee a negative-margin resection, it’s safer to skip the attempt and go straight to liver transplantation.”

Aggressive chemotherapy treatment

At the time of diagnosis, Victoria’s tumor was 6 cm in diameter and covered both sides of her liver. She had to undergo an aggressive regimen of chemotherapy to see if resection would be an option, or if transplant would be required.

“We were devastated,” Fernandez said. “We didn’t know if the chemotherapy would work, what kind of toll it would take on her, or if she’d have to have a transplant and deal with that her whole life. But, what we did know was that Texas Children’s was the best possible place for us to be. They had the numbers. They had the best track record for treating this type of cancer, whether it’s from an oncology perspective or surgery or transplant or intensive care.”

Only a handful of major centers in the country are equipped to take a case like Victoria’s. Of the 200 cases diagnosed in the U.S. annually, Texas Children’s treats approximately 10 percent of them.

“In addition to a strong cancer program, you need to have pediatric ICUs and intensivists, surgical expertise, anesthesia and pain services, all for children under the age of 5 – and enough volume to do it well and have good outcomes,” Vasudevan said.

Victoria underwent four intense cycles of chemotherapy. Each time, she was admitted back to the hospital for about a week, fighting fever, neutropenia and RSV. Knowing that four cycles was probably as much as the petite toddler could take, Victoria’s physicians were hoping to take her for surgery after one or two rounds. After the third cycle, she was placed on the transplant list briefly before scans finally showed a glimmer of hope. Victoria underwent a fourth cycle and was scanned again, and the team was delighted to find a margin of healthy tissue that made surgery possible.

“This entire team of oncologists, radiologists, pathologists, surgeons and transplant surgeons met so many times and discussed her case, all diligently trying to figure out what was best for Victoria,” Fernandez said. “It showed tremendous perseverance and dedication, and I will never forget that as long as I live.”

Surgical tumor removal

On January 6, 2016, Victoria went in for surgery, and she didn’t come out for more than nine hours. The vicinity of the tumor to the main portal vein, the primary blood supply to the liver, was close and required special attention to ensure that the tumor was completely removed.

“When operating on the liver, there is a high risk of disrupting the blood vessels and the bile ducts,” Vasudevan said. “What makes it really complicated is the fact that the liver is brown and completely opaque, and you can’t see the tumor. You rely on ultrasound guidance and external cues, the rest is up to feel and experience.”

Victoria’s procedure went smoothly. Vasudevan removed the tumor and the left lobe and was able to preserve about 60 percent of her liver.

There is a 30 percent chance of liver insufficiency post surgery, but after four or five days, the liver begins to regenerate and compensate for its loss. Victoria was stable and extubated by the next morning, and she went on to have two more cycles of chemotherapy to ensure no microscopic seeding had occurred. She has since celebrated her second birthday and returned to her normal, vibrant self.

Although Victoria is still checked regularly for signs of recurrence, overall her prognosis is excellent. She has an approximately 90 percent chance of an event-free, five-year survival.

“This is exactly why I got into this field,” Vasudevan said. “It’s an amazingly rewarding thing to do. Cancer is so devastating, in general, and to see a small 1- or 2-year-old child robbed of her whole life…that’s motivation enough for me.”

For more information about our Surgical Oncology Program, click here.

112916nursingtownhallflyer350The countdown clock is ticking! In less than one week, Texas Children’s Nursing will host its second virtual town hall on Wednesday, December 7, from 1 to 2 p.m. at the Pavilion for Women Conference Center.

Nursing has partnered with the Corporate Communication team to organize this event to engage our team of more than 3,000 dedicated nurses that make up Texas Children’s largest employee population.

During the first virtual town hall in June, approximately 600+ nurses participated via live attendance, group viewing of the livestream or watching the livestream via their smartphone, tablet or personal computer. Building on the tremendous success and impressive turnout of the first town hall, the goal this time around is to increase nursing participation significantly through a series of strategic marketing efforts.

“To help our nurses register early for the town hall, we created an electronic flyer with a registration link that was disseminated to nursing leadership via Nursing Congress and which is now available on our Voice of Nursing website,” said Jody Childs, senior project manager and co-organizer of the town hall event. “We helped nurses register as they arrived to their council meetings on Shared Governance Day which resulted in 65 nurses being registered for the town hall, all of whom received registration stickers to remind their peers to sign up as well.”

As a result of feedback from our first town hall, seating capacity has been expanded at the Pavilion for Women Conference Center and nurses are encouraged to attend the face-to-face town hall. For nurses who cannot attend the live event, there will be several gathering locations to view the live stream:

  • Clinical Care Center (for Ambulatory Services) – D.0900.30, ninth floor
  • West Campus – WC.150.10 (first floor)
  • The Woodlands Outpatient Facility (board room, fourth floor)
  • Health Centers – Sugar Land, Cy-Fair, The Woodlands, Kingwood, Clear Lake)
  • The Center for Children and Women (Greenspoint and Southwest)
  • Forming your own huddles? Please submit sign-in sheet to jcchilds@texaschildrens.org.

As always, patient care is our first priority and we know that not all nurses will be able to attend the live event or live-stream. However, those nurses will still be able to participate by viewing the event on-demand at their convenience.

Hosted by Chief Nursing Officer Mary Jo André, the town hall will include a discussion of FY16 nursing accomplishments, FY17 nursing priorities and system updates. Two videos will be presented – a sneak peek of Texas Children’s The Woodlands Hospital which will open spring of 2017 and a time lapse video spotlighting the progress on the pediatric tower which will open in August 2018. Also, the town hall will include extra Q & A time for nurses to submit their questions. Nurses watching the livestream remotely will be able to participate in this session thanks to our use of virtual technology.

“By leveraging new technology at our first town hall, we were able to engage more nurses in a town hall than we ever had before,” André said. “As our team continues to grow, it will be increasingly important for us to see opportunities to make communication easier and more effective. I encourage our nurses to pre-register so they can attend my town hall on December 7.”

Click here to pre-register for the Nursing Town Hall.

November 22, 2016

Simulations test readiness of new Outpatient Facility in The Woodlands

Ready, set, go!

“Are you OK?” a nurse asks a patient who stumbled to the ground after an unsteady walk to an exam room at the new Texas Children’s Hospital The Woodlands Outpatient Facility. “Help!”

A slew of medical staff come rushing to the girl’s aid, some comforting the patient’s mother and others tending to the girl’s lethargic condition. During what looked like controlled chaos, medical staff rolled equipment into the exam room, ran up and down hallways to gather more help and within what was just a few minutes stabilized the patient.

This was one of 11 scenarios played out during a three-day simulation at the Outpatient Facility before its doors opened for business on October 4. The purpose of the simulation was for medical staff to test their new environment, not their clinical abilities. Were there enough supplies? Is medical equipment in the right place? Is the room set up properly?

“Architects are great at creating these beautiful environments but are they friendly to the providers who are actually seeing patients,” said Julie Barrett, director of outpatient and clinical services in The Woodlands. “Testing those environments to see if we’re able to provide high quality patient care is what we hope to learn from simulation.”

Jeanette McMullin, a nurse in the surgery clinic at the Outpatient Facility, participated in the exercise and said it gave her a good feel for her new clinical space.

“It really took me through the process of what we would do for a patient given the situation and the supplies on hand,” McMullin said. “For me, the outcome was clear and that is we are able to function very well in this new environment.”

Barrett said a robust simulation is planned prior to the hospital opening in April. These efforts will be tailored to inpatient providers and will be led by many of the same simulation team leaders, all of whom are based in The Woodlands and have been trained at the Texas Children’s Simulation Center at Main Campus.

“The staff, providers and leaders have done a wonderful job,” she said. “I am amazed at how vitally important this simulation has been. It’s been a great learning opportunity.”

For more information about the Outpatient Facility, click here.