Diaphragmatic Eventration and the Technology Dependence Center: Aaron's Story

When he was born, Aaron was given a 50 percent chance of making it through the night. When he succeeded, he was given a 50 percent chance of making it through the weekend.

Over the following six months, there were multiple times when his survival was deemed uncertain.

Today, the baby with so many odds against him is a 5-year-old who has astounded the Technology Dependence Center team and his parents by overcoming every dire prediction for his future.

A new life in crisis

Carolyn, of Bridgewater, NJ, almost never felt well during her first pregnancy. Then she was hospitalized for preterm labor at 25 and 29 weeks.

During her admission, an ultrasound showed a problem with the baby's chest. Another ultrasound at 30 weeks revealed that Aaron was having life-threatening heart decelerations and Carolyn was rushed to the Operating Room for an emergency C-section delivery.

At birth, Aaron did not breathe and required resuscitation.

His diagnosis: diaphragmatic eventration, a congenital defect of the diaphragm or muscle that separates the lungs from the abdominal organs.

In Aaron's case, the thin, underdeveloped diaphragm allowed his right kidney to migrate upward into the chest. This prevented his right lung from developing. The baby was later diagnosed with bowel, kidney and heart disease.

Aaron spent the first 15 weeks of his life in The Children's Hospital of Philadelphia's Harriet and Ronald Lassin Newborn/Infant Intensive Care Unit (N/IICU).

"My husband Neal and I had him home for just 10 days before he needed to be readmitted for aspiration pneumonia," says Carolyn. "Then he returned home for just three weeks before going back to CHOP for a surgical procedure."

Caring for Aaron at home was a challenge fraught with anxiety for the couple.

"It took a long time until I was finally able to sleep at night," recalls Carolyn. "And at one point, we had to keep a 'blue log' to document Aaron's recurring periods of labored breathing and blue-tinged skin."

The Technology Dependence Center: a lifeline for Aaron and his family

At 8 months, Aaron was referred to the Division of Pulmonary and Sleep Medicine's Technology Dependence Center (TDC) for help in managing his chronic edema, oxygenation and diet.

"The Center staff truly treated us as a part of the healthcare team," says Carolyn. "And, even though Aaron's respiratory care took center stage, the TDC staff helped to coordinate his confusing array of medications and treatments, which was tremendously helpful."

In addition, she says, the TDC staff have always respected the family's need to understand Aaron's medical problems.

"Dr. [Julian] Allen knows that I'm a speech therapist and respects the knowledge I have," says Carolyn. "On many occasions, he's taken the time to sit at Aaron's bedside to answer my questions about his care and the reasons for various treatments."

One of the many challenges that parents of special needs children face is learning to use and troubleshoot complicated medical equipment. At one time, Aaron used several devices to help him breathe and monitor his health status.

These included:

  • Oxygen: supplemental oxygen improved functioning of Aaron's organs, helped him grow, and decreased the demands on his heart.
  • Apnea monitor: this monitor alerted Aaron's caregivers when he stopped breathing. Any infection had the potential to change Aaron's heart and respiratory rate and could ultimately cause him to stop breathing.
  • Feeding pump: as a newborn, Aaron did not have an opportunity to learn to eat by mouth. Due to the effort of breathing, he required more calories than a healthy infant. The feeding pump enabled Aaron to receive nutrition all night long and controlled the rate of food delivered during any given time period.
  • Pulse oximeter: this device measured the concentration of oxygen in Aaron's blood, providing an objective indicator of his general health status and an early warning of impending illness. As Aaron grew and was weaned from oxygen, the pulse oximeter helped staff evaluate his response to the reduced level of oxygen.
  • Bi-level positive airway pressure ventilator: this noninvasive device pushed air into Aaron's lungs via a face mask, allowing him to breathe with less effort. The TDC team's goal was to normalize the respiratory system, making it easier to manage his multiple medical issues.

Loving parents and the TDC team help Aaron surmount every obstacle

"The TDC staff always worked with Aaron as a whole little person, not just as a child with breathing difficulties," recalls Carolyn. "When newer and more portable equipment came out, the advanced practice nurses made certain we knew about it. This enabled us to take Aaron out of the house more easily, which encouraged his growth and development. After all, he was in a hospital crib for so long."

Carolyn and Neal admit that it's been a long, difficult road for the three of them, but today, they are thrilled five-year-old Aaron is flourishing.

"Two years ago, we were considering a lung transplant when, for some unknown reason, Aaron began doing remarkably better," says Carolyn. "First the TDC team took him off oxygen in the daytime. One year later, he was off oxygen altogether. Now he only takes one diuretic for control of edema and that dosage has just been cut in half!"

Once diagnosed with failure to thrive, Aaron's growth can now be traced on standard growth charts. His parents and healthcare providers are astounded and proud of his developmental strides.

"He still needs to work on his motor, social and self-care skills, but his condition has stabilized and he's exceeding everybody's expectations," says Carolyn. At school, Aaron has impressed his teachers with his cognitive abilities.

"We are incredibly grateful to the staff of the TDC and hesitate to think of what might have happened to Aaron without their care," says Carolyn. "We also want to thank our family members. Their support and tender loving care has enabled us to fight the fight and keep Aaron at home."

Originally posted: June 2009

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