Patient Stories: Krese Family
One Team, One Miracle, One Daughter
Like so many expecting parents, Amy and Joe Krese went into their 20-week ultrasound with a mix of excitement and apprehension.
They were overjoyed to find they were having a girl but also learned their daughter had a bilateral congenital diaphragmatic hernia, or bilateral CDH. An MRI showed her stomach and liver were both high in the chest, and her lungs were two tiny slivers.
Most diaphragmatic hernias are left-sided, some are right-sided, and very few are bilateral, so their baby’s condition was rare and very serious. If their baby made it to delivery, she would need extensive surgery after birth, and her lungs might not develop enough to allow that to happen. They were told their baby likely would not survive.
Amy, who works as a nurse at Texas Children’s knew that her work home was the best place for her baby.
“We already trusted the doctors, nurses, and staff, and we knew they would do everything possible to get the best outcome for Lydia,” says Amy. “We also had no illusions about the severity of her diagnosis, or what the treatment would entail.”
They read books on neonatal loss, and asked family not to send baby gifts. She also found an organization that would turn her wedding dress into an “angel gown” for Lydia to wear when she passed away.
Then, at 25 weeks, the Krese’s were surprised to get a call from Jayme Molohon, their nurse coordinator at Texas Children’s Fetal Center. Jayme explained that there was an experimental in-utero procedure for congenital diaphragmatic hernias, called fetal endoscopic tracheal occlusion (FETO).
With FETO, a balloon is inserted into the baby’s trachea which prevents the fluid normally produced by the lungs from escaping. This fluid accumulates in the lungs, and hopefully stimulates their growth. Normally FETO was only available for moderate to severe left-sided hernias. However, the Fetal Center team reviewed Lydia’s case and decided to offer it off-protocol as “compassionate treatment.”
“We were ecstatic to have something that might improve her chances after delivery,” remembers Amy. “We knew that as an experimental treatment, there was no guarantee. But we wanted to give our baby any chance available.”
Three weeks later, Amy and Lydia underwent FETO surgery, a procedure that literally requires an operating room full of doctors.
The period between the FETO procedure and delivery was the hardest time, recalls Amy. She moved into an apartment near the hospital, a necessity should she go into early labor. Her husband stayed at home to continue working and caring for their son.
During her twice weekly ultrasounds, the doctors reported that Lydia’s lungs appeared to be growing. Six weeks later, an MRI confirmed it -- her lungs had grown. Amy and Lydia returned to the OR, and the balloon was removed. While her lungs had grown, they wouldn’t know if it had been enough and whether the lungs would function until after she was born. And so, the waiting continued.
“There were times during our pregnancy that if someone had told me we were the only family being seen at the Fetal Center, I would have believed them,” says Amy. “Even though my OB/GYN wasn’t on call when I went into labor, she was there. When I texted Dr. Belfort and Dr. Shamshirsaz on our way to the hospital at three o’clock that morning, they both texted back within a minute.”
On January 8, 2017, Lydia Julianne Krese was born and spent the next 80 days in the Level 4 NICU. At three days old, Dr. Oluyinka Olutoye repaired her diaphragm, putting her abdominal organs back where they belonged, giving her lungs room to grow.
“The neonatologists, pulmonologists, and cardiologists welcomed us into the NICU, included us in rounds, answered all our questions, and asked our thoughts,” says Amy. “The NICU nurses spent hours caring for our daughter, and me, while I sat at her bedside day after long, hard, lonely day.”
After eleven-and-a-half weeks with many ups and downs, the Krese’s took Lydia home where son Colton gets to be a big brother.
“We are so grateful for all the people at Texas Children’s who not only believe in miracles, but find ways to make them happen,” says Amy.
About Congential Diaphragmatic Hernia
Congenital diaphragmatic hernia, or CDH, is a condition in which the diaphragm (the muscle between the chest and the abdomen) does not grow properly as the fetus develops. The diaphragm may have a hole (a hernia) or even be absent. Approximately one in 2,500 to 5,000 babies develops CDH.
Because of this defect, the intestines and other organs in the abdomen can enter the fetal chest area and compress the lungs. This makes it difficult for the lungs to grow and develop properly.
CDH also can cause blood vessel abnormalities, or pulmonary hypertension, or high blood pressure. Babies born with CDH may have problems breathing after birth and other issues because of improper development of various organs, including the heart, brain, kidneys and intestines.
CDH commonly occurs on the left side but can occur on the right side or bilaterally (on both sides).