Featured Child~ Emery

The Story of Emery Elizabeth (a.k.a. E3)

My husband & I went in for our 18 week sonogram & took our 3 kids with us, so they could find out with us whether they would have a new brother or sister. "I have girl parts" the technician wrote on the screen – my son, and only boy, sobbed in the corner (he REALLY wanted a brother.) As the scan continued, we were told that our newest little girl had a left-sided Congenital Diaphragmatic Hernia or "CDH". Even after a very brief physical explanation of the defect, we had no idea how this diagnosis would so profoundly affect us. It almost seemed unfair that a 10, 8 & 5 year old had to go through this too.

From Womack Army Medical Center, we were referred to UNC Hospital in Chapel Hill where they would take over my care and be able to handle her much needed intensive care after birth. From there we started our journey with CDH.

Emery Elizabeth was born in October 2009 at 7 lbs 12oz and began her fight. Immediately after birth, she made an attempt to cry, but it was only a muffled squeak and was quickly intubated – that would be the last cry we would hear for a very long time. For the first few hours she seemed to be doing well, but all that would change in the overnight hours. During the night the NICU staff was having increased difficulty in controlling her pulmonary hypertension, so at 5 AM it was determined her best chance was to be put on ECMO. Within an hour, she was moved from the NICU to the PICU for the procedure. Fortunately, she was relatively stable at the time, so the transition onto ECMO was manageable. Emery stayed on ECMO for 15 days – she had 1 circuit change and 2 failed trial-off attempts before she was finally able to be successfully taken off ECMO. The following week she had her 1st surgical repair.

The surgery went well, but we were told her defect was very large. 2/3 of her back-left diaphragm wascompletely missing, she showed some indication of potential complications with reflux and her left lung was about 1/3 of normal size. A Gore-Tex patch was placed. She came back from surgery wide-eyed – thatwas the first time we saw her with both of them open – I cannot explain the flood of emotion that brought us!

Then after 37 days, Emery was taken off of the ventilator and we heard her scratchy cry for the first time since she was born. What a beautiful sound – it still brings tears to my eyes when I think about it. Emery was in the PICU for a total of 45 days before transitioning to an intermediate care unit. Unfortunately, it didn’t last and Emery was returned via Rapid Response call to the PICU where it was determined after ultrasound that she had reherniated. She had her 2nd surgical repair on Thanksgiving Day 2009. After recovering from her second repair, she was moved to “the floor” and activities were focused on getting her to eat and gain enough weight to be able to go home. She had another surgical procedure to place a g-tube and 3 days before Christmas, after 76 days in the hospital, we were finally released to go home!

Emery went home on 1 liter of oxygen & continuous pump feeding through her g-tube. She was scheduled for monthly surgical follow-ups, follow-ups with Pulmonology, referred to Child Developmental Services for Speech/Feeding & Physical Therapy evaluations. At our first surgical follow-up, her surgeon was so excited to see her, and said that (clinically) she looked great, but after a close look at her x-ray we learned that Emery had in-fact reherniated again – and had shown none of the obvious signs. This was at the same time as my husband was receiving calls that he was to deploy with his unit to Haiti to help earthquake victims. The following week, while my husband was in-route to Haiti, Emery & I were at the hospital for her 3rd repair. Her hospital stay was only 10 days this time, but because of her previous experiences it was difficult for the medial staff to manage her pain as she had a very high tolerance to the medications she was given. We had a couple minor scares, but once we made it back out of the PICU and up to the floor, we were able to successfully wean her from oxygen. So home we went with one less tube!

She continued to be monitored by her surgeon, now on a 6 month schedule & we continued our monthly visits to the Nutritionist. She “graduated” Pulmonology follow-ups and Feeding Therapy and evaluations for Physical Therapy have shown us that Emery is on-track and age-appropriate! This is truly amazing considering what she went through. This past November, Emery had a cold and ended up having a Febrile Seizure and passed out – her lips were blue and it was incredibly frightening so we went by EMS to the emergency room where they took a chest x-ray looking for lung-collapse or pneumonia. Fortunately she didn’t have any of those issues, but upon review of the x-ray with her pediatrician, we found she had reherniated AGAIN! She was taken back up to UNC, where they confirmed the defect. This 4th repair, was approached by a cross-functional team of Pediatric & Cardio-Thoracic surgeons since her reherniation was in a difficult spot – adjacent to her aorta, esophagus & spinal cord. Fortunately, it went well, even though we spent the majority of the 10 days in the PICU. We’ve had long talks with Emery that this is NOT going to
become a family Thanksgiving tradition!

While everyone hopes that this latest surgery was the last time, we remain guarded and vigilant when it comes to her care. The issue we continue to monitor most is her weight gain and caloric intake, so we can get her weaned from the g-tube. This has been a very difficult and long road, but one we hope will end soon. We recently enrolled her in daycare a couple times a week in the hopes that peer-pressure will get her eating!

CDH has been a long and rocky journey thus far, a tremendous exercise in patience and a test of faith. We cannot explain why Emery got to be here, particularly given the severity of her defect, in fact we were told long after she was “out of the woods” by her surgeon that he many of the staff in the PICU were terrified because they didn’t think she was going to make it. While that is a scary thought, I am somehow comforted in knowing what a tremendous strength she has and that she had so many people fighting the fight & praying for her.

Now, she is a very active toddler (almost 2) and by looking at her you would never know the struggles she had starting out – until she lifts her shirt to show you her “button.” Her numerous scars left over from multiple surgeries, ECMO, chest tubes & central lines serve as a constant reminder, but these battle scars are ones of courage & triumph, not of pain & struggle. For the most part, we haven’t seen CDH slow her down – my bet is this is a sign of things to come. Bring it on – I don’t believe there’s anything this kid cannot do.

-Shannon – Emery’s Mom