Livia's Story - Congenital Diaphragmatic Hernia (CDH)

We found out we were pregnant the year after we married. Most people’s idea of the dream. We were living it! I was so impatient that I twisted Jamie's arm to go have a gender scan at 17 weeks. So in October 2016, we found out we were having a girl! We were overjoyed, but I was convinced I was carrying a boy, so was a little shocked. Our 20-week scan was mid-November. I went with a feeling that they were going to say it actually was a boy I was carrying, and the gender scan was wrong. Nope. They confirmed she was a girl! So she carried on with the scan, and at the end, said she was having trouble viewing her stomach due to position. We were asked to come back for rescan in 2 weeks. We were fine with that, as we had no reason to feel concerned. Fast forward 2 weeks, we were back to the hospital and scanned again. This time she said, “I want a second opinion,” and she called in another nurse. They were talking in medical jargon, which back then meant nothing to us. She said, “We want you to be scanned by a fetal medicine specialist.” We asked why, and she said, “We think your baby may have a diaphragmatic hernia.” Okay, that was fine, I knew what a hernia was - my dad had his fixed 2 days prior. However, on our way home, Jamie started googling diaphragmatic hernias, and at the traffic lights, he started reading out the information in front of him. We felt broken. We went back the following day where I was scanned in fetal medicine department. She called a consultant in to scan me, and the silence spoke a thousand words. I just burst into uncontrollable sobbing and he said, "get her in a room.” When we got to a room, he said, “Your baby has a congenital diaphragmatic hernia,” and he explained what it was and that she only had a 50% chance of survival. They kept mentioning termination and how it was important I decided now, as I was 23 weeks pregnant. They told me I had to have an amniocentesis, and IF there were any genetic or chromosome-related problems, no doctor would operate, so I need the amnio to see if it’s WORTH continuing the pregnancy. I obliged and had an amnio 3 days later, which eventually came back all clear. In the meantime, I was referred to the hospital I would now be delivering at, as that hospital didn't have a NICU.

I was also sent to another hospital for FETO discussions. At this specialist hospital, they told us that Livia’s chance of survival was 27% according to lung size. We were given 2 weeks to decide if we wanted to be put forward for FETO trial. In those 2 weeks, we met with surgeons where her repair would be done and had more scans, and they told us her head to lung ratio was 35%. They made us feel at ease, as they were used to dealing with CDH, so we decided against FETO and prayed we had done the right thing. I had fortnightly scans for the rest of my pregnancy, and her lungs improved each time. At our last scan, the lung they could see was 65%.

I was brought in for induction at 38 weeks on the 13th March 2017. It was a long process. 3 days later, it was classed as failed induction. I was offered a C-section, so I agreed. I was ready, they were ready. She could be here within an hour, let's do this. She was born at 10:58 a.m. on the 16th March. She didn't cry. She was whisked away and intubated. My husband was called into the side room and got to cut down her cord and take photos for me. 4 hours later, I was brought up to the NICU to see her. She was so fragile. She was on everything but ECMO. Her whole body was wobbling because of the oscillating ventilator. It's all a bit of a blur to be honest, but the NICU staff explained what everything was for, and we just sat and watched her. At 5 days old, she was ready to be transferred to Great Ormond Street Hospital, but they had no beds available! We had to wait till she was 9 days old to be transferred. It doesn't seem like much, but it felt like a lifetime! She had to go back on blood pressure medication, plus she needed extra support from the ventilator once transferred, so she needed time to settle before they could operate. At 12 days old, we got a call early in the morning asking us to come and sign paperwork for surgery. 4 hours later, she was repaired, and all went well. We met her surgeon who said she had everything up, including liver which we were told from scans they thought was down. He said she had very little diaphragm on her left, so has a large patch repair. Every day there was less medication and less oxygen. She eventually came home at 51 days old. No oxygen and feeding orally. SHE DID IT!

They were concerned about Livia gaining weight, as her reflux was terrible, and she vomited up most of her feeds. They asked us to come back for a weight check, in 3 days. We went back to hospital 3 days later (our first outing!) And when they undressed her to check temp and sats, I pointed out that it looked like she was working hard to breathe. So, they recommended an x-ray just in case. 1 hour later, they came and told us her patch had failed and she had reherniated. Our hearts broke. 3 days she was home. She was admitted straight away and had surgery 2 days later. They also did a Nissen Fundoplication to help with reflux and put a g-tube in for venting, as she was so young still. 7 hours after she fell asleep in Daddy’s arms under gas, she was back in the PICU. Oh, our little girl was all swollen and looked like a different baby. This hurt more than the first time around. Why, I don't know. Once out of PICU and on a post-op ward, Livia started breath-holding, which was the scariest thing for me. She was in so much pain, she was doing this. My heart was breaking, and I couldn't help her. They took her back to PICU for a night just to keep an eye on her, but she was okay the next day. 2 weeks later, she came home. Livia has thrived since coming home. She has been at the top end of weight charts since having her Nissen. It really made the world of difference for us.

We found out just after Livia turned 1 that she has reherniated again. She isn't symptomatic, so we are waiting as long as possible to put her through surgery again. She is feeding, developing, and growing just as any almost 2-year-old, and her surgeon sees no need to take her in right now.

Livia is our absolute world, and we are so blessed. We have had the roughest few years, but they have been the best of our lives.

I hope we can bring hope to a family going through this journey.