September 16, 2019

Nurse research scientist Krisanne Graves shares the many opportunities available to our nurses to be at the forefront of nursing research and impact the care they provide to patients and their families. Read more

The Jan and Dan Duncan Neurological Research Institute (NRI) at Texas Children’s Hospital recently hosted the first-ever family conference for EBF3-HADD (Hypotonia, ataxia and delayed development) syndrome (HADDS). Medical residents, genetics counseling students and research scientists joined more than 20 families from across North America that attended in person and 13 families that participated via live-streaming services from countries around the world – including Ireland and Australia – making it a truly international event.

The conference was organized by the EBF3-HADDS Foundation, a nonprofit organization created in 2018 by families to promote awareness, research and support for this genetic syndrome. The foundation was co-founded by Ashley LeMaire and her husband, Mark. After one of their children was diagnosed with HADDS in 2016, the LeMaires started a Facebook group for HADDS families. In just two years, that group has grown into an international community and was the impetus behind the foundation’s creation.

“Our HADDS community is a motivated and talented group of families dedicated to supporting research, education and advocacy efforts for HADDS patients, and we support each other on this journey,” said LeMaire, who is a clinical neuropsychologist at the Menninger Clinic, assistant professor of Psychiatry & Behavioral Sciences at Baylor College of Medicine, and also board member of the EBF3-HADDS Foundation. “There is still much to learn about HADDS, but when you have such a dedicated team of physicians and researchers collaborating with families to learn about the condition and provide needed support, it fosters so much hope for our families.”

During the conference, guests attended presentations on a variety of HADDS-related topics given by NRI researchers Drs. Hsiao-Tuan Chao and Michael Wangler; pediatric urologist Dr. Irina Stanasel, a former Texas Children’s fellow; Texas Children’s genetic counselor Pilar Magoulas; and Geraldine Bliss, research director of the Phelan-McDermid Foundation. Additionally, Chao and Wangler offered clinical evaluations for patients, and attendees were also able to tour research labs and facilities at the NRI, where one of the first genetic discoveries for HADDS was made in 2016, and the first lab (Chao’s) devoted to understanding the biology of this disorder.

A search for answers

If you’ve never heard of HADDS, there’s a good reason. The rare genetic disorder, caused by a mutation in the EBF3 gene, was only discovered in 2016 by Chao and Wangler when they were training in NRI investigator Dr. Hugo Bellen’s lab, in collaboration with colleagues at the Undiagnosed Diseases Network (UDN) and New York University Langone Health.

The UDN had been stumped by a case in which a child exhibited symptoms including impaired speech and cognition, low muscle tone, balance and gait issues, reduced ability to feel physical pain, and an inability to show facial emotional expressions. Though earlier DNA sequencing had yielded a few candidate genes, there wasn’t a significant patient cohort or research to help determine which gene was responsible for the symptoms.

Researchers selected EBF3 as the most likely candidate gene. Chao then used fruit flies to mimic mutations to better understand EBF3’s role, and within a few months three patients were found who presented with similar symptoms and similar mutations in the EBF3 gene.

As result of those efforts, more than 200 patients with HADDS have been identified to date.

“The gene discovery of EBF3 illustrates the ‘Power of One’ in medicine and biomedical research, how a single patient with an undiagnosed disorder – a ‘medical mystery’ – can lead to the discovery of a gene responsible for a previously unknown disorder,” Chao said. “This becomes the starting point to develop the diagnostic tests and therapies that can transform the lives of many patients and their families.”

Since 2017 Chao and Wangler have offered monthly clinical evaluations for HADDS patients at Texas Children’s and have now seen the largest number of such patients at any single institution worldwide. They are also enrolling patients in a study to better understand the condition and to help translate research into potential clinical interventions in the future.

The conference was a testament to the power of teamwork and collaboration in research, and also shows how dedicated parents and volunteers from across the globe can work together to build a community of support.

The Food Allergy Day at The Health Museum was a huge success recently. The event touched more than 1,100 members of the Houston community who played at the unique exhibits and were educated about food allergy. This event neared the most highly attended Thursday Family Days the Health Museum has experienced!

The Houston Health Museum partnered with the Texas Children’s Hospital Food Allergy Program to host the day, which kicked off with Texas Children’s Food Allergy Program exhibits throughout the museum. These exhibits were designed, staged and manned by Texas Children’s Food Allergy Program team and members of the Texas Children’s Food Allergy Program Teen Advisory Board led by Daisy Tran. This program was coordinated by LaTanya Miles from the Museum and Christina Nance, PhD, from Texas Children’s.

Special recognition goes to Dr. Christina Nance for leading the expert panel, Drs. Sara Anvari and Katherine Anagnostou for providing expert advice on the panel, and Lauren Kronisch for giving dietary tips. The Food Allergy Program is grateful to the Kelly Family (Mercedes, Adriana and Alexander) who shared patient perspectives on living with food allergy and participation in research trials. Christina Cowperthwait was outstanding in helping the museum plan the event.

Special thanks go to several members of the Immunology Allergy Rheumatology team who participated in handing out flyers and engaging with families and special guests, including Theresa Aldape, Chivon McMullen Jackson, Ashley Reiland, Kathy Pitts, Larraine Lyter-Reed, Joel Oyibo, America Lueso, and Dr. Ivan Chinn.

John Arcidiacono, President and CEO of The Health Museum gave an introduction at the event and members of the Board of Directors were in attendance. It was a great day in the community of education and increased awareness about food allergies.

Houston is both the home of significant chemical processing operations and an identified target for terrorism by Homeland Security. As such, Texas Children’s MUST be prepared to provide decontamination for victims of an accidental or intentional release of chemicals in the Houston area.

During September 30 to October 4, “Decon Week” will be held at Texas Children’s Hospital. The department of Organizational Resilience will host a series of Occupational Safety and Health Administration (OSHA) approved decontamination trainings for Texas Children’s staff. These will include an 8-hour first receiver training and a 4-hour awareness training at Texas Children’s Medical Center Campus, Texas Children’s Hospital West Campus and Texas Children’s Hospital The Woodlands.

The 8-hour training is for staff interested in becoming members of the decontamination or “Decon” team. This team is made up of volunteer staff members who attend the annual training, bi-annual meetings, and also participate in an annual decontamination exercise (to be held the last half of October 2019). Both operational and support members are needed on the team.

The team plays a vital role in protecting the safety and security of patients, visitors, and staff by performing decontamination activities prior to entry into our facilities. Further, the decontamination process helps the victim by limiting exposure to the contaminant on their skin and clothing.

The 4-hour awareness training is meant for Emergency Center and Urgent Care Nursing, and other staff who may encounter a “one off” contamination that occurred at a home, school, or industrial setting. These staff are trained to identify potentially contaminated patients, and direct them outside to our built-in decontamination showers (or outdoor decon area at Urgent Cares) to receive instruction to conduct “self-decon” before entering the facility.

Any staff interested in joining the decontamination team and attending the 8-hour training, or staff interested in the 4-hour awareness training, should contact Emergency Management at ext. 4-1237 or use the link below to sign up for a training and exercise that are convenient to their schedule.

Emergency Management Event Sign Up

September 10, 2019

As part of Texas Children’s Patient Access Initiative, the organization has made major progress to ensure our patients easily and conveniently get in the door to receive the care they need, when they need it.

“Our objective has been to lay a solid foundation and layer on additional patient access solutions now that we have a sturdy infrastructure to build from,” said Texas Children’s Surgeon-in-Chief Dr. Larry Hollier and co-chair of the Access Executive Steering Committee. “Because we really care about our families, we have to pay attention to their needs and do more to help them out. We have to keep the doors open to our patients and their families.”

Last year, Texas Children’s Patient Access Committee evaluated 944 provider templates and found additional slots where physicians could see patients for a duration of four hours, which resulted in the organization adding 53,000 new appointments to the system that had not existed before. In addition to this, the 72-hour flip helped the organization maintain flexibility and open schedules. If an appointment slot for a specific patient type was not filled 72 hours out, the slot automatically opened to a broader group of patients.

7-3-1 strategy: Building on our previous successes

After expanding appointment availability with four-hour clinic sessions and 72-hour flips, the Capacity Management Team this year was tasked with deploying a strategy to ensure these slots were filled.

Led by leaders in Ambulatory Operations and Central Scheduling, the team identified the largest area of opportunity was to work to decrease no shows – particularly for appointments scheduled more than 30 days in advance – and to implement a standardized way to recapture patients following a no show.

Focusing efforts on automated solutions, the team partnered with Information Services (IS) to optimize the hospital’s text message reminder system for appointments. Previously, patients only received a three-day reminder message for their appointment, which particularly for appointments scheduled three months – or even a year – in advance, is not enough of a reminder notice to arrange for time off, childcare or transportation. Now, patients receive their first request to confirm or cancel their appointment via text message at seven days, with additional 3-day and 1-day reminders prior to the appointment date.

The impact has been immediate after going live with the “7-3-1 strategy” at the end of May. In July, the hospital hit the system goal of a 10 percent no show rate for the first time ever, down from a three year average of 11.5 percent. While this change may seem small, a 1.5 percent decrease in no shows represents about 1,000 completed appointments a month.

Because we will never be able to completely eliminate patient no shows, the team also implemented an automated text message that will go out to patients the day after their missed appointments, offering the opportunity to be connected with a live scheduler to get another appointment scheduled. Historically, only 20 percent of patients that miss an appointment get rescheduled. Since launching recapture messaging in July, that number has already started to increase steadily.

“Overwhelmingly, our patients and families tell us that they prefer to engage with us via text message and on their phones,” said Grace Karon, Assistant Director of Business Operations and Strategic Planning for the Department of Pediatrics. “By focusing on technology rich solutions, we are giving families the customer-obsessed experience that they have come to expect from other services they receive, while working to ensure that patients who miss appointments do not fall through the cracks.”

Other patient access improvements

Texas Children’s patients and their families continue to benefit from the hospital’s patient access improvements that were implemented over the past year and a half.

Click here to watch this recent video on other patient access solutions we’ve implemented across the system to improve access, care coordination and patient experience at Texas Children’s Hospital.

Texas Children’s Cancer and Hematology Centers is one of the largest cancer and blood disorder centers in the country, diagnosing more than 4,000 new patients a year. Over the past decade, patients from all over the world have traveled to Texas Children’s because they know we are a leader in family-centered care and provide personalized, comprehensive services that address both the physical and emotional aspects of the disease.

Named No. 3 in the nation by U.S. News & World Report 2019-2020 Best Children’s Hospitals rankings, Texas Children’s Cancer Center comprises a multidisciplinary team of dedicated and compassionate physicians, advanced practice providers, nurses, child life specialists, social workers and other subspecialists who care for the physical, emotional and psychosocial needs of young patients.

Our world-renowned staff of nearly 200 faculty and more than 1,000 employees have pioneered many of the now standard protocols for treating and curing pediatric cancer and blood disorders. In addition, we provide a full range of pediatric subspecialty care available in-house 24 hours a day and treat all pediatric cancer and blood disorders, even the rarest disorders.

In addition to providing world-class, comprehensive patient care, our hematologist/oncologists are leading the way in advancing pediatric cancer and hematology research. The Cancer and Hematology Centers have 47 laboratories and more than 350 people who are engaged in research to help revolutionize cutting-edge therapies for treating and ultimately curing all childhood cancers and blood disorders.

“Texas Children’s Cancer and Hematology Centers, world renowned for its clinical care, research and education, offers innovative therapies for all forms of childhood cancer and blood disorders,” said Dr. Susan Blaney, director of Texas Children’s Cancer and Hematology Centers. “In addition, physicians-scientists at Texas Children’s Cancer and Hematology Centers are working relentlessly to improve the outcome for all patients afflicted with these diseases and to develop and perfect new treatment approaches that emanate from some of their most extraordinary scientific insights and discoveries.”

All of this and more is why Texas Children’s Cancer Center is continuously ranked by U.S. News as one of the best places in the nation to receive pediatric care. The rankings use a well-accepted framework for evaluating quality of health care, which factors in patient outcomes, such as mortality and infection rates; available clinical resources, such as specialized clinics and programs and external accreditations; and compliance with best practices. Improved rankings demonstrate a health care organization’s commitment to not only providing high-quality care, but also to identifying gaps where improvements are needed.

Big wins for patients and families

This past year, the Cancer Center built on its legacy of success and implemented new initiatives to increase transparency, reduce risk of infection and improve the quality of care. Here are some of the center’s big wins for patients and families:

  • Made significant improvements to reduce Central line-associated bloodstream infection rate

Texas Children’s Cancer and Hematology Centers’ leadership team identified high-risk central line-associated bloodstream infection (CLABSI) patients and increased rounding with the Centers’ infection control medical director, infection control practitioner and leadership team. Beginning in 2019, the Clinical Operations Team also partnered with nursing, pharmacy and physicians within the Centers, as well as with other departments, including but not limited to, Pediatric Surgery, Infectious Disease, Interventional Radiology and Anesthesia to develop a standardized central line placement and removal practice to decrease variations in line placement and care. These efforts have facilitated a reduction in Texas Children’s Cancer and Hematology Centers CLABSI rates from 1.31 in 2018 to 0.82 in 2019. Leaders anticipate the rate will lower next year.

  • Implemented computerized provider order entry system for chemotherapy orders

In partnership with Information Services, Texas Children’s Cancer and Hematology Centers implemented Epic Beacon, which is the oncology module in Epic that allows physicians to input orders for services, such as chemotherapy, electronically. Adopting such technology is considered best practice as it provides clarity and automated calculations to enhance safety. It also allows these orders to be created and queued up in advance of patient visits for cancer treatment, which should improve patient satisfaction by decreasing wait times.

  • Increased the percentage of patients who received intravenous treatment of antibiotics within an hour of initial triage

The Texas Children’s Cancer Center maintained >95 percent compliance the administration of intravenous antibiotics within an hour of initial triage for oncology patients who were seen in the Texas Children’s Emergency Center and Texas Children’s Cancer Center’s Urgent Care Bay across Texas Children’s three hospital campuses. The Centers’ data tracking over the past year improved greatly due to the strong work of Warren Boudreau, director of Quality Outcomes and Impact Service, and his team. In partnership and collaboration with the Emergency Department, the Centers were able to decrease its antibiotic administration time by an average of 20 minutes. This timely administration of antibiotics in our pediatric patient population is a national standard for decreasing the incidence of sepsis, morbidity and mortality.

  • Increased five-year survival of patient with neuroblastoma

Neuroblastoma is one of the most common solid tumors in children, and approximately 700 patients are diagnosed each year in the United States. Texas Children’s Cancer Center cares for patients of all ages diagnosed with neuroblastoma, which in the later stages is a highly aggressive tumor. For localized, low stage neuroblastoma Stage L1, the Center’s survival rate was 100 percent. For metastatic neuroblastoma, the survival rate was 53 percent, a 10 percent improvement from the previous year. Metaiodobenzylguanidine or MIBG therapy is likely a contributing factor to the Center’s increased success as well as the availability of varying molecular and targeted therapies, and new treatment protocols.

  • Closed out all structural related metrics

As described in the methodology section of U.S. News & World Report’s Best Children’s Hospitals’ publication, structure refers to hospital resources directly related to patient care and includes the ratio of nurses to patients, the presence of specialized clinics and programs and the certification by recognized external organizations. The Cancer and Hematology Centers were able to successfully demonstrate that each of these services were available, including molecular oncology/targeted therapy programs, an on-site inpatient pediatric rehabilitation unit with individualized dedicated cancer rehabilitation programming, participation in the Solutions for Patient Safety Hematology/Oncology CLABSI Working Group and maintenance of a quality committee with an identified medical director/leader.

Cancer survivor Sophia Sereni took center stage last week at one of the three Going Gold celebrations held at Texas Children’s hospitals in honor of National Childhood Cancer Awareness month.

Standing before a packed conference room in the Pavilion for Women, the curly-haired teen sang “Be Golden,” a gentle but strong song she wrote with Purple Songs Can Fly following treatment for T-cell acute lymphoblastic leukemia at Texas Children’s Cancer Center. Click here to listen to the full song.

“This has been an amazing experience,” Sophia said. “I’m so glad I could be part of such an important cause.”

Following Sophia’s performance and dressed in bright gold shirts and other festive gear, fellow survivors, current patients, families, Texas Children’s Cancer Center employees and others marched for childhood cancer awareness, ending their short trek on The Auxiliary Bridge where they participated in a ribbon tying event and received information from various support organizations.

View photos from the events below.

Sponsored by The Faris Foundation, similar events and parades were held last week at Texas Children’s Hospital West Campus and Texas Children’s Hospital The Woodlands. A celebration will be held this week at Vannie Cook Children’s Cancer and Hematology Clinic in McAllen, Texas.

“Each year, the events get bigger and better,” said Asha Virani, founder of The Faris Foundation and the mother of Faris D. Virani, who lost his battle with Ewing sarcoma and inspired Texas Children’s to “Go Gold.” “It’s a golden opportunity to spread awareness and love. Texas Children’s has been a leader in making this cause so visible.”

Khole Henry, an 8-year-old cancer patient, said she attended the event last year and was so excited to hear that it was going to happen again this year.

“My favorite part is the snacks!” Khole beamed as she grabbed a couple of bite-sized bags of M&Ms for later. “I’m glad I got to come.”

Director of Texas Children’s Cancer and Hematology Centers Dr. Susan Blaney said she is glad so many people turned out to raise awareness for such an important cause and that even though great strides have been made in combatting childhood cancer, there is still work to be done.

“We need a cure for every child diagnosed with cancer,” Blaney said. “That’s why we have to keep doing laboratory and clinical research, keep developing novel treatment approaches and continue raising awareness about childhood cancer.”

Last year alone, almost 600 children were diagnosed with cancer at Texas Children’s. The disease remains the leading cause of non-accidental death in children. Help spread the word that pediatric cancer is a serious disease and that Texas Children’s Cancer Center is here to help. For more information about the Cancer Center, click here.

Click here to view a preview of the next installment of “This is Cancer: Reflections from our patients.” This installment focuses on Owen, who was barely 2 years old when doctors found a mass the size of a grapefruit surrounding his heart and cutting off his airway. Since then, his tiny body has been through a lot. But, as his mom Emily says, not even cancer can slow this energetic toddler down. The “This is Cancer” series documents the journeys of several families receiving care at Texas Children’s Cancer and Hematology Center. Their stories illustrate in intimate detail what they’re experiencing and how to better support them. Click here to learn more.