The CDC says more public health research is urgently needed to find out why the dramatic increase in what the CDC describes as a "serious birth defect of the abdominal wall."

Patrick Thomas, M.D., FACS, a pediatric surgeon at Cook Children’s says gastroschisis results from a defect in the abdominal wall with herniation of the abdominal contents (such as intestines, stomach and often the gonads). The hernia is always to the right of the bellybutton.

Treatment includes starting immediate IV fluids and placing the infant in an organ bag below the chest to prevent heat loss and to protect the bowel. Surgical options can include immediate repair by placing the organs back in the abdomen and closing the abdominal wall defect. For cases not responsive to immediate closure, a protective bandage (called a silo) is placed and the bowel gradually reduced until closure of the defect can be performed about a week later.

Dr. Thomas said ongoing studies are needed to identify the causes of gastroschisis, but the current treatment through surgery has produced “great outcomes.”

Candace Gamble, M.D., a medical geneticist at Cook Children’s, calls the report from the CDC on the increasing prevalence of gastroschisis, significant.

“It is particularly important for health care providers in the prenatal and neonatal setting as we care for these babies,” Dr. Gamble said. “While expecting parents have cause for concern, the occurrence is still relatively rare considering there are well over 3 million babies born in the U.S. each year. However, it’s a reminder that every pregnancy has a 3-5 percent risk of some type of congenital birth defect. Thus good prenatal care is essential in detecting these defects early so that these babies can receive the proper management.”

Both Dr. Thomas and Dr. Gamble state that researchers and clinicians do not fully understand why this is happening. There is no known genetic mutation that causes gastroschisis. It is likely due to a number of genetic and environmental factors, which lead to an error in fetal development. Doctors do know that there are certain factors that increase the risk for having a baby with gastroschisis, including being a pregnant mother under the age of 20, smoking, and taking vasoconstrictive medications.

“Prenatal care is very important,” Dr. Gamble said. “A screening test where we measure the maternal serum alpha fetoprotein around 16 weeks gestation can be used to detect some babies with gastroschisis, other abdominal wall defects, and more specific defects. In addition, the anatomy ultrasound at 20 weeks gestation can also be useful to detect this and other congenital abnormalities. However, neither measure can completely rule out all birth defects. It is important for parents to discuss these risk and screening options with their health care provider.”

*Illustration courtesy of CDC.

On behalf of my family, I would like to congratulate you on your retirement and successful career. I know that you changed the lives of many families but I wanted you to know how much you changed ours.

From the very beginning, I trusted you and you never failed William. Maybe it is experience or maybe it is luck; I have nothing but confidence when I think about all the times you were called to consult on his case or help him through surgery. My first memory is when you put in his PD (Peritoneal Dialysis) catheter. So much was at stake for this boy in renal failure. Not only his life, but a family who wanted him to live. I asked to pray with you and the anesthesiologist before the procedure. I still remember the way you took off your surgical cap and bowed your head. Thank you for praying with me even when I could not understand myself through fearful stuttering.

The standard measuring system for a kidney transplant is your fist. I can still see you holding your fist to Will’s tummy to show me how much more he needed to grow. Every month we would work for each advance in height and weight. I remember showing up for a check-up like a student eager to have her project graded. You were as excited as I was for 10.2 kg and a torso that could hold a kidney the size of your fist. At 20 months old, you and your team transplanted a kidney in my son that “pinked up right away.” I can still see your smile and hear you answers to our rapid questions. Our son went into your OR in renal failure and came out with the first normal renal panel in his life. Thank you for guiding us through those months and thank you for all of the beautiful stitches.

William was your patient, but we are his family. Thank you for repeating your opinion over the phone with my husband when he had to be at work. Thank you for letting grandparents ask how a procedure had changed since they had children. Thank you for letting us interrupt your sleep, schedule, and rounds over and over because Lord knows Will loved to do something interesting at 3:45 on a Friday afternoon. Thank you for listening to me. Thank you for looking me in the eye when the news was not the best. Thank you for rejoicing when the news was momentous.

I have no idea how many lives are changed because of your work. But I know how much better the world for our family is because you were in it.

God bless you and keep you, may His face shine upon you.

Katie, Chuck, Will and John Spiekerman

Warren and Lori Carver

Chris, Kathy and Jacob Carver

Charlie and Vicki Spiekerman

Liam lived from March 25 to Nov. 1, 2013, and spent much of his brief life at Cook Children’s with critical issues ranging from an operation to repair his intestines, to heart problems, to feeding concerns.

He seemed to be constantly fighting until it was too much for his little body to overcome.

Now a little over a year later, Vickie Phathaphone, Liam’s mother, looks back on her time at Cook Children’s with an emotion that may be surprising to some – gratitude.

Recently, Phathaphone reached out to Cook Children’s Facebook page. She wanted to thank the staff who treated her little boy.

“My family and I, including Liam, loved the medical staff at Cook Children’s and you guys gave us the most amazing experience and educated us well,” Phathaphone said. “The staff at Cook Children’s helped my family and me by teaching us how to not be afraid to hold our little Liam.”

Phathaphone said doctors and nurses updated her family every day and Cook Children’s updated her by cellphone as needed. She said the NICU nurses taught her so much, ranging from the importance of breast milk and how to pump it, to how to carefully bathe, feed and hold her fragile baby.

“Although being a parent is natural, with Liam’s condition it was quite frightening,” Phathaphone said. “We did not want to hurt him, but we were reassured by the nurses what not to do and what to do. I loved how the staff treated Liam, as he was a part of their family, and I am thankful that Liam was under such amazing care.”

After letting a group of the physicians know about the letter, they were appreciative of Phathaphone’s kind gesture and began to reflect on the loss of not only Liam, but other children who have died too soon.

Surgeons Vincent Tam, M.D., James Miller, M.D., and Lisa Roten, M.D., a cardiologist, share with us what their life is like after the loss of a child.

Dr.Tam

He tries to stay in control as he fights back the tears.

But on this day, as hard as he tries, Vincent Tam, M.D., a pediatric heart surgeon at Cook Children’s, struggles to keep his composure. He’s thinking about Liam.

Dr. Tam performed two heart operations on Liam. All the procedures were successful, but other underlying problems were simply too much for the baby, who was born premature, to overcome.

In practice for 23 years, Dr. Tam copes with the death of a patient by compartmentalizing the loss. He tries his best not to dwell on it, so it won’t impact his performance in the operating room or his role as husband and father. Then, every once in a while, he receives a note or hears from a parent whose child has died, like the one from Phathaphone. The parents don’t want to express grief or even anger, but simply to say thanks.

“It’s hard because if somebody dies or somebody has a bad complication, I think, ‘Oh gosh, could I have done something differently?” Dr. Tam trails off and after a long pause continues to speak. “There’s all kinds of questions that you can ask as a Monday morning quarterback, but personally I’ve dealt with it in a way that I feel like I’ve … um …”

Again, a long pause as he composes himself. “I do the best I can with every surgery and that’s all I can do.”

Dr. Miller

Shortly after Liam’s birth, James P. Miller, M.D., a pediatric surgeon at Cook Children’s, operated on him to repair gastroschisis, a condition where the baby is born with the intestines on the outside of the body.

Even after 17,000 surgeries and 27 years as a surgeon, it only takes a quick glance at a child’s chart for the memories to come flooding back. He remembers Liam and his mom. He says he knew from being around her for a short period of time and the love and compassion she always showed to her child, Phathaphone would be a great mom.

Dr. Miller has gotten to know some patient families well over the years. Throughout his career, he’s been to many of their weddings, but he’s also seen death.

“It can devastate you for weeks,” Dr. Miller said. “It can be like reopening a wound when you realize it’s the first anniversary of a child’s death. You hope you have somebody in the hospital that you have helped or made a difference in their lives, just to get out of your rut. I don’t think people realize how much doctors do feel. When you do what we do with these kids, you do get attached to them.”

Dr. Roten

For the patient families who are cared for by Lisa Roten, M.D., a cardiologist who specializes in fetal echocardiography at Cook Children’s, they see someone who cares deeply for her patient families. But they also may be surprised to know that she understands Dr. Tam’s need for compartmentalization.

“The truth is, you have to do that,” she said. “You wouldn’t be a physician if you couldn’t do that. We have a work life and we have a personal life. I can’t carry that home with me. I do talk about the loss of my kids, but with the people who can empathize with me. I talk to my colleagues and staff, the other doctors and nurses, who cared for the child.”

Still, Dr. Roten admits to developing a special bond with most of her families and says a gesture like the one from Phathaphone and other parents means a lot to her. As much as the families sometimes need closure by writing a note or speaking to the doctors, Dr. Roten also needs to find a way to say thank you to the parents for entrusting their child’s care to her and goodbye to the children.

Dr. Roten makes every effort to reach out to families who have had a child pass and to go to the funerals whenever possible. It allows her to complete the circle of care.

“At the end of the day, we’re all humans. We are people who have families and we are touched by these families we see,” Dr. Roten said. “Even after the child dies, they never really leave our lives. There are ways that we continue on together. We take our cues from the family. If they ever want to reach out or touch base with us, that’s comforting to us. It is the end of a relationship that sometimes you’ve had for years and years with families and sometimes like in Liam’s case, it’s been shorter.

“But they are all so special to us.”