Midway through their pregnancy, Dan and Dorothy Olds learned that their baby Lincoln’s heart was located in the extreme left of his chest, a possible indication of a rare condition known as congenital diaphragmatic hernia (CDH).
More tests confirmed their fears. “We received a confirmed diagnosis of Lincoln’s right-sided CDH at 23 weeks gestation,” says Lincoln’s mom, Dorothy Olds. “The doctors could tell that Lincoln’s heart was pushed over as far as it could be, but we had to wait until he was born to see how serious the CDH was and what other complications may exist.”
CDH is a birth defect in which a fetus’ diaphragm or the tendon in the middle of the diaphragm does not develop properly. This results in some of the organs normally found in the abdomen moving up into the chest cavity through the abnormal opening. The fetus’ lungs are unable to develop properly.
In Lincoln’s case, he was diagnosed with a Morgagni hernia. This CDH involves an opening on the right side of the diaphragm and occurs when the tendon that should develop in the middle of the diaphragm does not grow properly. Only 2 percent of all babies with CDH have this type of hernia, compared with 80 to 90 percent of cases that develop the left-sided Bochdalek hernia.
The Olds were referred to the Fetal Care Center where a team of maternal fetal medicine and pediatric specialists began caring for baby Lincoln.
Specialized Care with ECMO at St. Louis Children's Hospital
Once Lincoln was born in May 2011, imaging scans and other testing confirmed his Morgagni hernia and that he had mild heart complications. No other major anomalies were identified. Although he remained relatively stable on a ventilator his first night, within 24 hours Lincoln developed pulmonary hypertension. For the next five days the newborn intensive care unit (NICU) team attempted to regulate his condition with oxygen and blood pressure medicines, but by June 1 his progress had slowed to the point of his physicians recommending he be placed on extracorporeal membrane oxygenation (ECMO).
“We were told that survival without ECMO was around 10 percent, but with ECMO the survival rate was 55 to 60 percent,” says Dorothy. “It was an easy decision that was still difficult to make since the blood thinners he needed to be on with ECMO could possibly cause internal bleeding or stroke.”
His first nine days on ECMO, Lincoln remained stable but didn’t improve. Then, finally, he began breathing better and by day 12 was weaned off ECMO.
“Our nurse called him the ‘perfect ECMO patient,’ meaning he was on for about two weeks, and he was able to wean from it without any issues,” says Dorothy. “His medical team only had to add one medicine to help Lincoln’s heart work more efficiently in order to wean, compared to the nine medications he was on before ECMO support.”
Surgery to Repair the CDH
Lincoln was well enough to undergo his hernia repair in June, which was performed by Kathryn Bernabe, MD, Washington University pediatric surgeon at St. Louis Children’s Hospital and the Fetal Care Center. Lincoln’s entire small intestine, part of his large intestine and liver were herniated into his chest. Dr. Bernabe was able to reposition his organs and repair the hernia using Lincoln’s native diaphragm rather than a patch, which helps reduce infection and the chance of the hernia recurring.
Lincoln recovered well from his surgery and was able to go home after 83 days in the NICU. Now 10 months old, he continues to need oxygen and an inhaled medication for asthma, but he no longer takes any oral medication.
“We are turning down his oxygen and hope to have him off it soon. He hasn’t had any problems with oral aversion like some babies who begin life on an ET tube,” says Dorothy. “His first hearing test was normal, and he responds and carries on little conversations when we talk to him. He continues to have some physical and occupational therapy, but he’s pretty much on target for reaching and picking up toys.”
Reassured by Excellent Care
Despite the long days that bounded between worry to hope, Dorothy and Dan are grateful for the advanced care Lincoln received from the NICU and pediatric surgery teams at SLCH, as well as the ancillary services vital to Lincoln’s recovery.
“When I was first at home taking care of Lincoln, it was always reassuring to know I could call the hospital with any concerns I had. I got to the point where I was totally comfortable telling the nurses and doctors everything, and I still have no reservations asking any questions I have,” says Dorothy. “Now it’s comforting to take him in and have everyone so happy to see him and how well he’s doing. It’s a great relationship for which Dan and I are grateful.”