April 14, 2015

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While Ebola is no longer dominating the headlines, ongoing training and preparations ensure we remain ready to handle highly infectious diseases.

In the months since the Ebola concern, Texas Children’s has stock piled personal protective equipment (PPE) and, most recently, acquired 30 powered air purifying respirators (PAPRs). A PAPR covers the head to protect emergency responders from chemical, biological, radiological and nuclear agents. This addition to our PPE inventory helps ensure the safety of our health care professionals when they are treating patients with highly infectious diseases.

“We were well-prepared before, and we’re even better prepared now,” said Dr. Judith Campbell, medical director of Infection Prevention and Control. “We’ve always been in compliance with the CDC. Now we have the benefit of having visited both Nebraska and Emory where patients with highly contagious infectious diseases are frequently cared for, giving us an added level of knowledge.”

The infectious disease leadership team recently observed a PPE donning and doffing exercise used to successfully contain the disease at Emory Healthcare and Nebraska Medicine. The team also received a tour of their special isolation units (SIU).

The visits to Emory and Nebraska also helped in the design of Texas Children’s first special isolation unit. The unit is designed to care for a broader range of infectious diseases. Campbell said the unit and a special response team will be dedicated to caring for patients with highly contagious infectious diseases.

“When we talk about the SIU, it really is with the intent of taking care of any unusual infectious disease that’s highly contagious,” Campbell said. “There are a lot of infectious diseases emerging. Given we are a global society, it’s certainly possible that there may be another pathogen that could have the kind of impact that Ebola has had.”

Texas Children’s leaders have worked with health care facilities across the country and with the Centers for Disease Control and Prevention to share best practices that will continue to prepare us for the possibility of receiving a patient with highly contagious infectious diseases, such as Ebola. The organization continues to monitor the situation with information from the World Health Organization (WHO) and the Centers for Disease Control and Prevention (CDC).

“We’re confident that, in many aspects, our preparation exceeds that of most other hospitals,” Campbell said. “We want to make sure we have the highest level of protection for our health care professionals.”

April 7, 2015

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4815pharmacyinside640Texas Children’s draws roughly 100 therapeutic drug levels per day to examine the presence and the amount of specific drugs in the bloodstream of children. These levels determine how well a patient’s body breaks down the drug and how it interacts with other medications.

“With most medications, you need a certain level of drug in your bloodstream to obtain the desired effect,” said Brady Moffett, a clinical pharmacy specialist at Texas Children’s. “Some medications are harmful if the level rises too high and some do not work if the levels are too low.”

To promote quality improvement and to continue to ensure the safety of our patients, Texas Children’s Department of Pharmacy and Clinical Chemistry Service in the Department of Pathology have produced an online, comprehensive guide to help clinicians monitor the therapeutic drug levels in a patient’s bloodstream to ensure they are within a safe and effective range.

Drug level testing is especially important in children taking certain medications to treat health problems like abnormal heart rhythms, diabetes, epileptic seizures and bacterial infections, as well as organ transplant patients who must take immunosuppressant drugs during their lifetime.

Produced specifically for pediatrics, Texas Children’s 92-page therapeutic drug monitoring guide is the first of its kind produced at a children’s hospital and represents a multidisciplinary collaboration among our physicians, pharmacists and laboratory personnel.

“We worked extensively with specialists across multiple service lines to gather their input and expertise to bring this project to fruition,” Moffett said.

Moffett co-edited the therapeutic drug monitoring manual with Dr. Sridevi Devaraj, director of Clinical Chemistry and Point of Care Testing at Texas Children’s.

“The online version of this manual will be updated in real time as new drugs and technologies emerge,” Devaraj said. “It will provide us with better guidance on how to safely adjust the proper dosage of medications to meet our patients’ individual medical needs.”

Click here to access Texas Children’s Guide to Therapeutic Drug Monitoring.

March 31, 2015

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One month after their successful separation surgery, Knatalye and Adeline Mata are progressing well as two separate little girls.

Knatalye can breathe on her own and was recently transferred to the Progressive Care Unit where she will resume her recovery. Her sister, Adeline, is still on a ventilator in the Pediatric Intensive Care Unit (PICU), but her doctors remain optimistic that she will breathe on her own just like her twin.

“Knatalye’s progress has been a tiny bit faster than Adeline, but it was just because of where they started out,” said Dr. Darrell Cass, co-director of Texas Children’s Fetal Center. “We’re still in the acute recovery phase of care where we’re working on breathing, sedation and pain management and working on their intestinal function.

Prior to the girls’ separation surgery, Knatalye and Adeline spent the first 10 months of their life in the Neonatal Intensive Care Unit (NICU) where they received daily care from their team of NICU nurses. To adequately prepare them for their separation surgery, the NICU nurses helped engineer a custom bed space for the girls in the NICU and collaborated with Hanger Clinic, to devise a swing that would safely keep the twins upright for a large portion of each day after their tissue expanders were in place.

“We emotionally, physically and intellectually invested a lot of care in the girls,” said Alex Luton, a clinical nurse specialist at Texas Children’s Newborn Center. “I know it was a difficult but necessary transition for them to go to the PICU.”

To ensure a smooth transition of care for the girls and their family, the NICU nurses partnered with the PICU team before and after the separation surgery.

“We sent a couple of nurses per shift for the first few days post operatively to kind of be there as a familiar face for the family as they began to build their relationships with nurses in the PICU,” Luton said.

Each of the babies had a PICU nurse and a NICU nurse for the first few days after separation surgery.

“As they stabilized and their condition improved, one nurse was assigned for each baby,” said Shannon Holland, assistant director of nursing in the PICU. “Now, they have one nurse for the two babies together.”

In addition to receiving around-the-clock care from the PICU nurses, Knatalye and Adeline receive therapy every day – physical therapy in the morning and occupational therapy in the afternoon to optimize their muscle development and motor coordination.

“We are practicing a lot of reaching and grabbing using both hands, bringing their hands together, banging toys together, visual tracking, “said Texas Children’s occupational therapist Chelsea Pierce.

Texas Children’s physical therapist Frank McCormick assists the girls with head control, trunk control, stretching, range of motion and working with their feet.

“The sessions are dictated by kind of what they can tolerate,” McCormick said. “Usually 30 minutes is a window, but if they can go further and are still awake and participative, then it can go upwards of 45 minutes to an hour.”

While Knatalye and Adeline’s recovery will take some time, their doctors are optimistic about their future.

“We would expect their gait, their walking stride, to be somewhat altered initially,” Cass said. “We’ll have to monitor them. With some therapy, we’re optimistic that they will be able to walk and live a normal life.”

Texas Children’s Surgeon-in-Chief Charles D. Fraser also shares that same spirit of optimism.

“This is another example of why a case like this should be done at Texas Children’s because we have the team and expertise to handle complex medical conditions,” Fraser said. “I am very, very confident that we will continue to move them on a positive recovery trajectory.”

Connect videos of the Mata twins:

Voices of Nursing: NICU nurses’ reflection on caring for the Mata twins

Hope, faith and expertise: Surgical team leads historical Mata conjoined twins surgery

3D Model of Mata twins helps surgeons prepare for girls’ separation

Positioning team designs, builds swing to help conjoined twins sit up

Mata conjoined twins take first step toward separation with tissue expansion

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April 2 is World Autism Awareness Day when the international community will “Light It Up Blue” to bring recognition to autism as a rising global health concern. Texas Children’s encourages everyone to wear blue that day or post something blue to one of their personal social media pages to show their support for autism awareness.

Autism spectrum disorder is the fastest-growing developmental disability in the United States, affecting 1 out of every 68 children. It occurs more often among boys than girls. Children with autism often experience significant social, communication and behavioral challenges.

For more than a decade, Natanya Dugall searched for a diagnosis for her son that would explain his vision, speech and movement impairments. She knew something was wrong with Grayson from the moment he was born, but initial genetic testing turned up nothing.

When Grayson turned 13, Dugall received some unexpected news. A laboratory had identified chromosomal deletions on a specific gene in Grayson’s DNA which had been collected years earlier. After the initial find, Grayson participated in a study at the Autism Center at Texas Children’s that examined the effects of people who grew up with either chromosomal deletions or copies on this gene.

When the Dugall family made the 1,120-mile trek from their home in Milwaukee, Wisconsin to Houston in 2011, their quest for answers to Grayson’s puzzling condition was confirmed – he had autism.

“I cannot begin to express my gratitude to the research team from Baylor College of Medicine and Texas Children’s Hospital for funding all of the testing and travel expenses,” Dugall said. “Grayson understands the probability that he can pass this to his children – knowledge that we struggle with but are so grateful to finally know.”

The Autism Center’s multidisciplinary team of specialists from developmental pediatrics, psychology, psychiatry, neurology and social work provide diagnostic evaluations and ongoing clinical care to 2,000 patients with autism each year. Researchers at the Autism Center participate in a wide range of research studies focused on autism spectrum disorders, collaborating with scientists from other renowned centers across the nation.

In conjunction with the groundbreaking work emerging from the The Autism Center, Dr. Ruth Ann Luna at Texas Children’s Microbiome Center focuses much of her research on the link between disturbances in the gut microbiome and gastrointestinal (GI) symptoms in children with autism. One of her biggest motivators is her 6-year-old son, Kellen, who has autism.

With the support of a $1.4 million, three-year-grant from Autism Speaks, Luna and her Baylor and Texas Children’s colleagues are embarking on a study to determine if a biological connection exists between autism and GI disorders.

“I was in awe when I found out we were awarded this funding to advance autism research,” Luna said. “The data gleaned from our research will benefit my son and other families who have children with autism, especially those children with limited verbal abilities.” Click here to read more about Luna’s autism study.

For more information about “Light It Up Blue, click here and to learn more aobut the Autism Center at Texas Children’s click here.

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Dr. Huda Zoghbi received the 2015 Mortimer D. Sackler, M.D. Prize for Distinguished Achievement in Developmental Psychobiology, awarded by Weill Cornell Medical College and Columbia University College of Physicians and Surgeons.

Zoghbi is the director of the Jan and Dan Duncan Neurological Research Institute at Texas Children’s, a professor of molecular and human genetics, pediatrics, neuroscience and neurology at Baylor College of Medicine, and a Howard Hughes Medical Institute investigator.

As a member of the National Academy of Sciences, Zoghbi has pioneered research on Rett syndrome and other rare brain disorders, and has advanced research of more common conditions including autism and adult neurodegenerative diseases.

In one of her most well-known achievements, Zoghbi provided a definitive genetic diagnosis for Rett syndrome and opened the door to a biological understanding and a search for treatment. Rett syndrome is a genetic neurological disease that usually affects young girls. Girls born with the disease develop normally for one or two years, but then begin to show progressive loss of motor skills, speech and other cognitive abilities.

Zoghbi has also found other genes, including Math1, an essential gene governing the development of cells critical for hearing, balance, and breathing, and the genes for spinocerebellar ataxia 1 and 6. Her work has led to a better understanding of neurodevelopmental and neurodegenerative disorders.

“We’re thrilled that Dr. Zoghbi is this year’s recipient,” said Dr. B.J. Casey, director of the Sackler Institute and the Sackler Professor of Developmental Psychobiology at Weill Cornell Medical College. “She’s dedicated herself to this field for decades and has made remarkable achievements. But we’re also recognizing her leadership in the field of neuroscience and her track record of mentoring young scientists as they embark on their own careers.”

The prize is named for the Dr. Mortimer D. Sackler, one of the most creative scientists in the field of developmental psychobiology. The Mortimer D. Sackler Foundation, Inc. endowed the prize in a gift in 2009.

“To have a prize bearing the name of someone who’s made such remarkable achievements in this field is a great honor,” Zoghbi said. “There are so many deserving scientists and physician-scientists out there who have done fantastic work. I feel truly honored to have been selected.”

Zoghbi plans to donate the $100,000 prize money to a mentorship fund that she established to help young female scientists get their work – specifically research on “bold, new ideas” – off the ground.

March 24, 2015

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Have you ever found your fruit bowl teeming with fruit flies that seemed to appear out of nowhere? While pesky at times, these 6-legged, red-eyed insects have endured as a laboratory staple for more than 100 years, helping scientists unlock the genetic causes of human disease.

In a recently published article in the journal Genetics, fruit fly biologists at the Jan and Duncan Neurological Research Institute at Texas Children’s – Drs. Michael Wangler, Shinya Yamamoto and Hugo Bellen – explore the value of fruit fly research and the need for more funding to accelerate biomedical discoveries using the Drosophila fruit fly.

Unlike mouse models, the authors argue that fruit flies are inexpensive to maintain, they grow quickly from embryo to adult in 12 days, and share many similar genes with humans, making the Drosophila a powerful model organism for the study of genetics.

To study human disease in fruit flies, scientists mutate, or disrupt, the same gene that is known or suspected to cause the disease, and then figure out why mutations with this particular gene leads to disease. However, one can also take an unbiased approach and discover new genes.

Inside their laboratory at the NRI, Bellen and his team have performed a large scale forward unbiased genetic screen which allowed them to isolate 165 fruit fly genes that cause neuro-developmental and neuro-degenerative phenotypes when mutated, 90 percent of which have human counterparts. The human counterparts of one third of the genes cause Mendelian disorders. By exploring the remaining two thirds of the genes in patients with rare disease they could identify a few novel human diseases genes, work that they did in collaboration with Drs. Jim Lupski and Richard Gibbs.

A detailed characterization of some of the 165 genes also permitted them to discover novel mechanisms that underlie a variety of known diseases such as the peripheral neuropathy associated with Charcot-Marie-Tooth (2A) disease, Leigh syndrome and some ataxias, including Spinocerebellar Ataxia 6 and Friedreich ataxia.

“By understanding how a particular gene works in the fruit fly, we can dissect the molecular and genetic events that underlie the pathogenesis of human disease so that effective treatments can be developed in the future,” Bellen said.

“We see fruit fly research as an avenue of tremendous importance,” Wangler said. “Federal agencies need to actively encourage collaborative research between fruit fly researchers and human geneticists and clinicians to make the best possible use of dwindling research dollars to promote public health.”

Click here to read their article titled, “Fruit flies in biomedical research,” in the journal Genetics.

March 3, 2015

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Texas Children’s Hospital began with a promise more than six decades ago. It was a promise to the community that we would be here to take care of their children – all of them, regardless of ability to pay – for many years to come. Today that promise is even stronger and more evident and has broadened to include the women who now come to us for care.

It stands to reason that Texas Children’s next chapter would be ushered in with a bold capital campaign that will help ensure we can respond to the growing need for care. Texas Children’s has launched Promise, a $475 million comprehensive campaign.

“We are so excited about the Promise campaign,” said Laura Shuford, vice president of Development. “Supporting a fundraising effort that has a goal of providing the best possible care to even more children is a cause that speaks to the heart and one that so many people can relate to.”

The Promise campaign focuses on five key initiatives:

  • CareFirst, for the expansion of our Critical Care services, ORs/PACU and Emergency Center at the Main Camp
  • Texas Children’s Hospital The Woodlands, our new 548,000-square-foot dedicated pediatric hospital serving the growing communities north of Houston
  • Endowed Chairs, which are a powerful tool in recruiting and retaining world-class physicians and scientists
  • Divisions and Centers of Excellence to provide ongoing support for specialty care programs and projects
  • Charity Care and hospital priorities, to help alleviate the financial burdens families take on with complex medical care and to provide flexible funds to address the hospital’s most pressing needs at any given time

Of the $475 million campaign total, $350 million of the funds raised will support the CareFirst expansion and renovation projects, scheduled for completion in 2020, and The Woodlands campus, currently under construction and scheduled for completion in 2017. These two initiatives are the hospital’s top priorities.

“This is one of the biggest challenges Texas Children’s has ever faced,” said Texas Children’s President and CEO Mark A. Wallace. “But I know we can meet the challenge head on and keep the promise we made so many years ago – and the promise we make every single day – to serve all the children and families who come to us for help.”

How you can help
You can help us spread the word about the Promise campaign and the impact it will make right here in Houston and beyond. Visit texaschildrens.org/promise and share the page with family and friends who may be interested in supporting our efforts.