Patient Stories
Congenital Diaphragmatic Hernia / CDH
Justin Topp Confronts Severe Form of Congenital Hernia and Marshals Inner Strength So He Can Home With His Parents
"He is the most beautiful baby Marianne or I have ever seen," Wayne Topp of Cincinnati wrote just a few days after his wife Marianne had given birth to their son Justin. That was their first entry in their CarePages web chronicle of Justin's journey so far, from birth at 37 weeks, through his first six months of life, spent at Cincinnati Children's Hospital Medical Center, and finally home.
Justin had grown from 6 lbs, 6 oz, on his arrival on January 12, 2006, to 17 lbs, 2 oz, on his discharge from the hospital on July 24. He had learned to live without the heart and lung bypass machine that had sustained him during his first two weeks, had surgery to repair his diaphragm and properly position his abdominal organs, and fought off infections and other problems.
At home, Justin has a tracheal tube to help him breathe and a gastric "button" for feedings. He also has monitors, a machine that provides a constant flow of air, and a "Kid Kart" to hold his equipment.
"We are still trying to get into a routine of sorts that involves measuring seven types of medicines in syringes two times a day, preparing feeds six times a day, and of course the usual diaper changes," the Topps wrote. "It has been great, though. We can't be more excited to have him home!"
'Heart Pushed Over a Little Too Far'
Marianne Topp was 20 years old and 20 weeks into her pregnancy when she had a routine ultrasound that showed, "the baby's heart pushed over a little too far to the left," Wayne recalled.
A repeat ultrasound was inconclusive. The Topps were told their baby could be affected by congenital diaphragmatic hernia or by a condition that causes cysts to form in the lungs. In either case, specialized care would be needed and the Topps were referred to the Fetal Care Center of Cincinnati.
Another ultrasound and magnetic resonance imaging (MRI) confirmed a diagnosis of congenital diaphragmatic hernia (CDH). This is a defect in the developing baby's diaphragm, the muscle that separates the chest and abdominal cavities. The stomach and other organs that would normally be in the abdominal cavity push into the chest cavity (or herniate), compressing the lungs so they cannot develop properly. While the cause of CDH is thought to be failure of the diaphragm to develop at 9 to 10 weeks of gestation, it is not known why that happens.
Closely Monitored Pregnancy
The Topps met with Timothy M. Crombleholme, MD, FACS, FAAP, fetal surgeon at Cincinnati Children's Hospital Medical Center and director of the Fetal Care Center of Cincinnati, to discuss the implications of the diagnosis, including the need to closely monitor the pregnancy. From then on, Marianne had twice weekly appointments with Carol Egner, MD, an obstetrician / gynecologist affiliated with Good Samaritan Hospital in Cincinnati. Cincinnati Children's and Good Samaritan, along with The University Hospital, are partners in the Fetal Care Center of Cincinnati.
The CDH continued to compress the baby's lung and by the 32nd week of gestation, "the lung volume was far less than it would be for a normal baby," Wayne recalled. In fact, it was only about 20% of what would be expected at that stage in the pregnancy.
Five weeks later, Marianne agreed to deliver Justin by an innovative strategy reserved for only those infants at highest risk. Assisted by other members of the Fetal Care Center team, Dr. Crombleholme performed a procedure known as the EXIT-to-ECMO (ex utero intrapartum treatment to extracorporeal membrane oxygenation). Baby Justin was partially delivered through an incision in Marianne's uterus and placed on ECMO, a heart and lung bypass system that circulates oxygenated blood throughout the body. Once that system was functioning, the umbilical cord was cut and the delivery was completed.
Successes and Setbacks
Entries in the Topps' CarePages from then on chronicled, and continued to chronicle, Justin's struggles, successes, and setbacks. Some problems caused delay in the surgery needed to repair Justin's diaphragm and move his abdominal organs where they belonged and to take him off the ECMO system. An unexpected problem with the ECMO system itself resulted in emergency surgery on January 24 to remove Justin from ECMO.
Later that day, Wayne was able to report, "He is stable and his pressures and stats all look great, but by the next day, Justin had suffered a setback. "When the doctors did rounds this afternoon at 1 pm, the news seemed so gloomy that I had to ask, 'What are his chances?' The only thing the doctor could honestly tell me was that, 'He had a chance.' Of course that was not reassuring, but we were able to stay hopeful and the doctors and nurses continued to do incredible work."
As the Topps waited for the surgery that would repair the hernia and reposition the abdominal organs, the cycle of successes and setbacks continued. Justin would gain strength, then lose ground, then progress again.
Elation and Caution
By March 6, Justin had progressed to the point where he was ready for surgery. "It's been a long time coming and finally, tomorrow at 7:30 in the morning, Justin will undergo his second major surgery of his life," the Topps reported. "The doctors have told us that things will get worse after the surgery and that we should expect things to return to the way they were when each day was touch and go."
On March 7, Wayne wrote: "THE SURGERY WAS A GREAT SUCCESS!!! …They were able to move his liver and intestines back to where they belong with very little bleeding. The doctors said that Justin went through it better than they ever expected! He looks just as beautiful as when he went in with only a small cut on his right side under his rib cage." Yet, Wayne added a note of realistic caution, "Although Justin looks great today, the nurses continue to remind us that Justin will get sicker before he gets better." A day later, Wayne reported: "Justin has had an awesome first day post-surgery…the doctors are in absolute awe at how great he's doing."
On March 19, Justin had a "successful trial run" of breathing without a ventilator, although he had to go back on the ventilator afterwards. The Topps' CarePages entry for that day noted that the doctors and nurses "think that the main reason he had such difficulties being off the vent is because he simply got scared. (Of course, who isn't scared making such a huge change in their life?)"
On May 1, the Topps reported: "So, today Justin decided he wanted to slow down a little and take a few steps backward." Concerns about Justin's breathing and pressure within his lungs led doctors to postpone surgery to place a feeding tube that the Topps could use to feed Justin once he got home. That was done later and Justin also needed a tracheal tube to help him breath. "In some ways this is disappointing because it will be one more thing to worry about when he comes home," the Topps wrote, "but, at the same time, it has an upside…Justin will be able to come home sooner with his tube and we are now one step closer to coming home because we know the solution to the problem and we can fix it."
'Nursing Staff Was Amazing'
Caring for Justin at home has made the Topps realize just how much the nurses did for Justin and for them while Justin was in the hospital and to appreciate the nursing staff even more than before.
"The nursing staff was amazing," Wayne said. "The nurses don't just take care of the patient, but of the whole family." He added that one particular nurse, Barb Gannon, "acted as our Mom at the hospital, not just doing what needed to be done for Justin, but showing us how to do it, so we would know how to do everything with Justin when we got home."
Before they left the hospital, the Topps also had a run-through of what it would be like to take care of all Justin's needs by themselves without asking for help from the nurses.
"Justin is doing wonderfully at home and he has gotten himself into a sort of schedule that includes two long naps throughout the day and getting to sleep at a decent hour and sleeping through the night. He is just as happy as ever," the Topps reported just five days after Justin came home. "We do have 12 hours of nursing care every night to allow us to take a break from watching the monitors and making sure he is still asleep."
Justin and his parents will continue to see specialists at the Fetal Care Center of Cincinnati. As Justin ages, the Topps will also have to consult a pediatric cardiologist and pulmonary specialist and a speech pathologist. Wayne said the overall prognosis for Justin is highly favorable. By two to four years, Justin is expected to no longer need his air flow machine.
Patience and Gratitude
The ups and downs the Topps traversed and face still have reminded them of the virtue of patience. "One of the most important things is being patient with the whole system, the whole process," Wayne said. He advised those who might be facing similar situations to remember, "Things don't always go the way you expect them to go, or the way you've been told they will go. Be patient. The medical team will provide the best possible treatment and do the right thing."
Wayne said that Beth. Haberman, MD, one of the neonatologists who treated Justin, was especially helpful. "Even during the hardest times, she was upbeat, but realistic at the same time. She made us realize there was still hope despite what was going on at the time," he said.
"A good support system is always helpful," Wayne continued. "A good support system can carry you through the worst days and be really excited for you on the best days."
For Marianne and Wayne, that support system extended beyond their family and friends to include others in their community and members of their church—Our Lady of Victory Roman Catholic Church in Cincinnati—and "people we didn't even know," but met through Care Pages.
Grateful for all the help and support they receive, the Topps went to Washington, DC, in June for Cincinnati Children's Hospital Advocacy Day. "It was a wonderful opportunity for us to try to give back to Children's Hospital by sharing Justin's story with Congressmen and Senators from Ohio and Kentucky," they noted. "The trip was great and everyone seemed very interested in Justin's story! We really felt like we were making a difference!"
To request an appointment at the Fetal Care Center of Cincinnati or contact us, please use our secure, online appointment form or call 1-888-338-2559 (1-888-FETAL59).
