The award was presented at the Mayor’s International Dinner by Mayor Mattie Parker on Nov. 1. This award recognizes Dr. Thornton’s tenacity and dedication to helping improve the quality of life for children across the globe with this rare disease.

“Born in Ireland, Dr. Thornton is truly world-renowned and one of the leaders in his field,” said Rick W. Merrill, President and CEO of Cook Children’s Health Care System. “We are so glad he settled here in Fort Worth and we are extremely honored to share him with the world.”

Hyperinsulinism occurs when the pancreas produces too much insulin, driving blood sugar levels dangerously low and depriving the brain of important fuels it needs to function. It affects about 1 in every 25,000 to 50,000 newborn babies each year. For those affected by the rare condition, it can be a life-changing event. Without an accurate diagnosis, children face living with seizures and permanent brain damage.

Dr. Thornton has played a pivotal role in establishing the first two hyperinsulinism centers in the United States. First, at Children’s Hospital of Philadelphia and today at Cook Children’s. Our center provides the highest level of multidisciplinary care to congenital hyperinsulinism (CHI) patients and their families, many of whom travel from around the country and the world to receive care from Dr. Thornton and his team.

“This caliber of treatment is not only rare in the United States, but it’s rare all over the world,” said Jonathan Nedrelow, M.D., associate chief medical officer of Cook Children’s. “He does a lot of work with family advocacy groups for rare diseases and is committed to being a part of the journey for these families and patients.”

Dr. Thornton led a team in creating a new international screening that helps other doctors recognize symptoms and manage newborn infants who are at an increased risk of hypoglycemia. He leads HI research to better understand the disease and treatment options and speaks to clinicians around the world about managing this disease.

“Dr. Thornton's tireless dedication has touched lives across the globe,” said Danielle Drachmann, CEO of Ketotic Hypoglycemia International. “His vision, expertise and the collaborative spirit he instills, with the utmost respect for the expertise of affected families' lived experiences, have created ripples of positive change. His work is a testament to the remarkable power of collective effort to make a global impact.” 

Dr. Thornton’s recognition as a leader also earned him one of the first two endowed chairs at Cook Children’s.

“On behalf of all of us at Cook Children’s, we want to say how proud we are to see Fort Worth Sister Cities International recognize our own Dr. Paul Thornton with the Global Impact Award for his very distinguished career,” Mr. Merrill said.

If you want proof, read this diverse list of our most popular stories of 2016. We hope you enjoy our look back:

10. 7 Ear Infection Myths Every Parent Should Know. Parents flocked to this blog from Doc Smitty. Can your child go swimming with an ear infection? Do all ear infections need antibiotics? If your child gets ear tubes, will the infections stop?  All this and more was answered in this blog.

9. 7 Online Challenges This ER Doctor Warns Could Be Deadly Oh the Internet. Where kids do all kinds of things to get noticed and maybe go to hospital in the process. We asked our medical director of the Emergency Department to look at seven really silly, really dangerous dares that kids (or grownups for that matter) shouldn't try at home. 

8. Almond Milk: A Nutty Fad or Healthy Alternative. Almond milk sales have gone through the roof over the past decade. But is it the right drink for your child? Doc Smitty gave the answer in this informative piece. 

7. The Death of Ethan: A Life of Chronic Pain Ends in Tragedy. This is the most recent entrant in our list of top 10 stories. Only the fact that it was published in December kept this story out of the top five. We were so honored that Ethan's parents shared their son's story of a life filled with chronic pain. They want you to read it to learn about their son and hopefully help other children who may be suffering as well.

6. New Hope In Fight Against Deadly Brain-Eating Amoeba. The Lewis family holds a special place in the hearts of everyone involed in the Checkup Newsroom. Following the tragic death of their son Kyle to a brain infection caused by a water-born amoeba, Jeremy and Julie Lewis created Kyle Cares. Read their inspiring story as they fought to bring a drug to Cook Children's and other hospitals around the nation that may save children's lives.

5. 11 Tips That Could Save Your Daughter's Life.  In Janaury 2016, Rylie Whitten made national headlines after suffering one of the worst cases of toxic shock syndrome (TSS)  that her doctors had ever seen. Rylie's story moved us to run this informative piece on TSS that we hope every parent of a daughter reads.

4. A Sign From Heaven: Family Travels Across U.S. For Care at Cook Children's. A family's desperate search for answers on Google led them to Cook Children's. Read the fascinating story of what led this family to travel from Detroit, Mich. to Fort Worth, Texas, to be treated for a rare condition called hyperinsulinism.

3. The Most Common Rash You Never Knew About. Ever heard of molluscum contagiosum? We hadn't when Dr. Diane Arnaout wrote this blog for the newsroom. Dr. Diane's informative blog "that will save you and your child pain, money, time and anxiety" went viral quickly and became our third most read story of the year.

2. RSV On The Rise: 6 Things Every Parent Should Know. Here we are at the beginning of 2017 and we have been lucky in that we haven't seen much flu yet at Cook Children's. But we have seen plenty of RSV (respiratory syncytial virus), which explains the popularity of this great blog from Dr. Lizy Varughese.

1. Let's Learn Something: Bouncers, Jumpers and Walkers. Dr. Diane Arnaout's "Let's Learn Something" series was a big hit this year and none were bigger than this tale of caution. Dr. Diane's blog on how baby bumpers, bouncers and activity centers could play a role in your baby's motor delays is our most read new story of 2017. You've probably read this blog. But if you aren't one of the more than 117,000 readers who took a look, now's the time. And if you have read it, feel free to click again.

Well, that's it for 2016. Thank you all for reading. We'll be back with plenty more stories to tell in 2017.

Paul Thornton, M.D. is the medical director of Cook Children’s Hyperinsulinism Center, one of only two such centers in the U.S. and the only one in the southern portion of the country. He has dedicated his life to researching and treating congenital hyperinsulinsim (HI), and in turn has helped improve the quality of life for countless children.

Dr. Thornton’s work is so well respected he was recently named a Rare Disease Hero by Rare Disease Communications. The award recognizes five physicians each year for groundbreaking research and treatment in the rare disease community.

“Rare Disease Communications is proud to be honoring these real-life heroes,” said Chris Davis, president of Rare Disease Communications. “This is, indeed, a rare opportunity to applaud the silent victories that mean so much to patients and families.”

In addition to the Rare Disease Hero award, Dr. Thornton was also recently honored at the 2016 sugar sHIndig at the Fort Worth Science & History Museum where he was given the Be My Sugar Medical Excellence Award by Congenital Hyperinsulinsim International (CHI).

“It’s an honor to be recognized by the leaders in the congenital hyperinsulinism community,” said Dr. Thornton. “As we continue to treat children from across the country and the world, we’re excited to share information about our program and the excellent team providing quality, family-centered care.”

Why develop a center for clinical excellence for a rare disease?

Getting help for patients with rare diseases can often be a large struggle for families. Finding someone who understands and has the experience to properly diagnose and treat can lead to frustration for families. Often there is no place to go. For long standing issues, this can lead to bouncing to multiple doctors without resolution of symptoms.

For more acute issues, not having an expert available can cause significant morbidity, even mortality. Today on Pediatric Leadership: The New Medicine, we talk to one of our pediatric endocrinologists who set up a center for treating a rare disease, hyperinsulinism.

Dr. Thornton is a pediatric endocrinologist at Cook Children’s and is the founder of the Cook Children’s Hyperinsulinism Center which is one of only two centers in the country focused on hyperinsulinism. The website for the team lists endocrinologists, pediatric surgeons and many other medical specialities as well as coordination between education, social work and even Ralph Lauren, one of Cook Children’s play therapy dogs.

Questions to work through on the topic:

What is hyperinsulinism and when should a clinician expect it?

What motivated you to begin building a center for treating such a rare condition?

How did you get the institution on board? Who did you have to convince?

What were some of those first steps that got the ball rolling?

What has been the hardest step?

How can someone find out more about the center or refer a patient?

What part of your work are you most proud of?

What is the most important quality for a leader in pediatrics?

To listen:

Download on Soundcloud 

Subscribe for series on iTunes

Download/Listen to MP3

For more information:

• Cook Children’s Hyperinsulinism Center
• Dr. Paul Thornton
• Cook Children's Referral Page

At last, after long days plagued with uncertainty, they found their answer on the other end of a phone call from Paul Thornton, M.D., medical director of Cook Children’s Congenital Hyperinsulinism Center and Endocrine Diabetes program.

The family found Dr. Thornton through a Google search and a story posted on checkupnewsroom.com. The article told the story of how Dr. Thornton had led thea team of experts from around the world in creating new screening for physicians to recognize and manage neonates at increased risk for a persistent hypoglycemia disorder. The fact that Dr. Thornton was establishing these guidelines proved to Jake and Tella that he would be the best person to care for their little boy.

Dr. Thornton expected Brantlee had a rare condition called hyperinsulinism. He was right.

While at Cook Children’s, Brantlee had two pancreatectomies and a Gastrostomy Button, or G-Button, installed.

“Brantlee is now the first baby in the United States to undergo this new experimental therapy for hyperinulinism and so far, it is working, allowing him to go home and keep his blood glucose in the safe range,” Dr. Thornton said.

But before they found their answers, the Sanfords were left with only questions.

Brantlee was born a month and a half prior to his due date, weighing a surprising 8 pounds, 6 ounces. He was taken immediately to a Neonatal Intensive Care Unit in Flint, Mich., leaving behind tearful parents Jake and Tella to wonder and worry about the fate of their newborn son. Over the next few days, Brantlee’s insulin levels continued to climb without any explanation.

“We found out Brantlee was having problems shortly after his birth,” Jake said. “We wondered why he was so big for a premature baby and the doctors were curious if we had our due date right.”

However, within a week of Brantlee’s birth, an endocrinologist pulled his parents aside. Noticing his furrowed brow and serious demeanor, Jake and Tella braced themselves for what they could only expect, was bad news. The doctor informed them that Brantlee’s insulin levels were in the 300s when his sugar was 30mg/dL. Typically, a healthy newborn’s insulin level should be less than 2 with low glucose levels.

“In the 32 years he’d practiced, he said he’d never seen a baby at these levels,” Jake said. The doctors initially informed Jake and Tella that their son had a rare form of hypoglycemia, which is when the blood glucose drops below 50 milligrams per deciliter. However, the doctors’ treatments for hypoglycemia were not working on Brantlee. While Jake and Tella continued to rely on each other, both were growing weary and exhausted, continually praying for healing.

“It was a nightmare for us. We were slowly realizing that Brantlee wasn’t a typical premature baby,” Jake said.

After a week of tests and no answers, the doctors recommended Brantlee be sent to a children’s hospital in Detroit. Jake, not about to be away from his son, decided to use all of his vacation days to travel to Detroit with Tella. After extensive tests by multiple doctors, Jake said no one could figure out what was wrong with his baby. It seemed that Jake, Tella and Brantlee had hit another dead end.

But then everything changed.

“My sister had sent me a newsletter from a children’s hospital in Texas called Cook Children’s. I emailed the writer of this article on congenital hyperinsulinism while waiting for a miracle to happen,” Jake said.

Two days later, Jake received a phone call from a Texas area code. Dr. Thornton was on the other line, asking Jake if he and Tella would be willing to bring their newborn son to Fort Worth. “We figured if a doctor took the time to call us, he wanted to care for us,” Jake said.

After hearing from Dr. Thornton, Jake acted on impulse, desperately hoping that this was the moment that could save Brantlee’s life. Two days later, Tella and Brantlee were on a plane headed to Texas.

“They were amazed I called back so fast and how quickly we were able to arrange transport to get them here,” Dr. Thornton said.

After arriving at Cook Children’s and meeting Dr. Thornton, Brantlee was officially diagnosed with hyperinsulinism. This condition causes abnormally high levels of insulin, which can increase the risk for dangerous complications to the brain. However, fortunately for the Sanford family, Cook Children’s is one of only two hospitals in the United States with hyperinsulinism centers.

“It was really great to know that they were able to find us on the web, make contact, and get here fast so we could treat their baby,” Dr. Thornton said. Dr. Thornton is one of the most recognized hyperinsulinism specialists in the world, which made Jake and Tella feel more comfortable placing their son in his care.

“Dr. Thornton’s call was truly a sign from heaven. We put our faith to the wind and it took us to Texas,” Jake said.

After two and a half months, Brantlee was allowed to return home, just in time for the holidays.

“Since being back, we have tried our best to be a normal family, between the octreotide injections, blood sugar checks, continuous feedings from 9 p.m. to 9 a.m., and sirolimus into the G-Button.” To most, this sounds like a nightmare of foreign terminology. However, for the Sanford family, this circumstance is now a blessing.

Today, Jake and Tella can now manageably raise him from a home instead of a hospital.

“The threat of his sugars going low is still a battle for us, but thanks to Cook Children’s and his medication, we are able to raise it to where it needs to be,” Jake said.

Brantlee will return to Cook Children’s in June for a follow up appointment with Dr. Thornton.

More about Cook Children's Hyperinsulinism Center

One of only two such programs in the nation Cook Children's Hyperinsulinism Center uses a specialized team approach to treat this rare disease. Hyperinsulinism affects many areas of the body, so to truly treat the disease, each child is seen by top physicians, nurses, researchers and specialists in the field. These medical professionals have spent additional years of intense study and have dedicated their practice to focusing on hyperinsulinism so that your child has access to the medical care that treats all the symptoms. It is this level of treatment that has helped earn our program a distinguished international reputation for extraordinary care and achieving positive results.

Even skilled physicians may not be on the lookout for something as uncommon as CHI in newborns, which could be the difference in preventing irreversible damage and improving the quality of a child’s life.

That may all change following an article published in the Journal of Pediatrics titled, “Recommendations from the Pediatric Endocrine Society for Evaluation and Management of Persistent Hypoglycemia in Neonates, Infants and Children.”

Paul Thornton, M.D., medical director of Cook Children’s Congenital Hyperinsulinism Center and Endocrine and Diabetes program and an Endowed Chair, led the team of experts from around the world in creating new screening for physicians to recognize and manage neonates at increased risk for a persistent hypoglycemia disorder.

“Infants with uncontrolled hypoglycemia caused by CHI are at risk for seizures or permanent brain damage without early identification and screening,” Dr. Thornton said. “These new guidelines will help physicians who recognize these symptoms shortly after birth and then provide the specialty care which may make the difference on whether this child has a productive and fulfilling life.”

The article states that “failure to investigate a neonate, infant, or child with suspected hypoglycemia increases the risk of delaying a definitive diagnosis and instituting effective treatment.”

But quickly identifying the specific cause of hypoglycemia allows for timely and appropriate treatment to begin and decreases the risk of permanent brain injury from persistent and recurrent severe hyperglycemia.

Dr. Thornton was chosen as the principal author of the article because he’s internationally known in the field of CHI. He leads a highly skilled team of specialized physicians and supporting specialists to treat hyperinsulinism.

Cook Children’s Hyperinsulinism Center is the only one of its kind in the Southern U.S. and the second in the country.

Davelyn Hood, M.D., president of the CHI Board of Directors said her organization “is thrilled beyond words” that long-awaited hypoglycemia guidelines have been published this month in the Journal of Pediatrics.

“We consider this a sentinel moment in time for the entire congenital hyperinsulinism community,” Dr. Hood said. “Many patients born with this congenital HI are discharged from newborn nurseries without adequate study of their hypoglycemia, often with tragic consequences. These guidelines give us hope.”

Dr. Hood said she believes the guidelines will save countless lives and improve the well-being of HI children everywhere. She also praised Dr. Thornton for all of his work for CHI patients.

“He cares for children across Texas and the U.S. with one of only two dedicated Hyperinsulinism Centers in the country,” Dr. Hood said. “He speaks to clinicians around the globe about the proper management of hypoglycemia and leads HI research to better understanding of the disease and best treatment options. Most importantly, Dr. Thornton, brought the HI research community together, took all of their collective knowledge regarding hypoglycemia and its underlying causes, and articulated that into clear guidelines for clinicians of all types that care for neonates, infants and children. Dr. Thornton is a definitely a ‘CHI Super Hero.’”

More about Cook Children's Hyperinsulinism Center

One of only two such programs in the nation Cook Children's Hyperinsulinism Center uses a specialized team approach to treat this rare disease. Hyperinsulinism affects many areas of the body, so to truly treat the disease, each child is seen by top physicians, nurses, researchers and specialists in the field. These medical professionals have spent additional years of intense study and have dedicated their practice to focusing on hyperinsulinism so that your child will has access to the medical care that treats all the symptoms. It is this level of treatment that has helped earn our program a distinguished international reputation for extraordinary care and achieving positive results.