My baby’s heart defects: Amelie’s Story

Amelie’s heart defects, transposition of the great arteries, coarctation of the aorta and a ventricular septal defect, were diagnosed at mum Eleanor’s 20 week scan. This is their story:

At the beginning of February 2020 we found out I was pregnant. We worked out the dates on an app, which told us the baby was the size of a single sprinkle. From then baby was known as sprinkle by all!

Due to COVID-19, I had my 12 week scan alone. This was sad as it was our first baby and I felt awful for my partner, Sam. I was so sure that by 20 weeks the virus would be gone, but I was so, so wrong!

Once again, I was told I should attend my scan alone. We had already decided we weren’t finding out the gender, so this scan, in my head, was just another chance to see Baby Sprinkle!

At the end of May I went to the 20 week scan. I was laying down and the sonographer was laughing at how active the baby was! She told me baby had lovely long legs, their head and brain were growing and formed well and that Sprinkle had such clear features. She then said, ‘Right I’m checking the heart now,’ and that’s where the whole atmosphere changed.

She spent ages scanning me, asking me to turn different ways and wiggle until she finally said she was going to get a second opinion. At that point my own heart sank and I knew something wasn’t right.

The two sonographers told me they thought something wasn’t right with baby’s heart. They suspected the main valves were the wrong way round, but wanted to send me to Southampton for a second opinion. I burst into tears and was taken to a small room to ring my partner.

The following Monday, after an agonising weekend, we drove for an hour and a half to Fetal Medicine for a full scan with a specialist cardiologist. For this scan, Sam was allowed in, which was a small positive. It meant he would see Sprinkle for the first time.

After about 50 minutes of scanning, we were taken to a side room and the cardiologist explained and drew exactly what was wrong. Baby Sprinkle had Transposition of the Great Arteries (TGA), a ventricular septal defect (VSD) and a possible coarctation of the aorta (they would confirm this after birth).

They told us that with all this there was an 85% chance of survival. Then the cardiologist knocked us for six by asking, ‘Would you like to terminate?’ We both broke down, but had already decided prior to the appointment that, no matter what, we would give our baby Sprinkle a chance!

For the rest of the pregnancy we vowed to be positive and as normal as possible. COVID restrictions eased, so we were able to have a small baby shower in the garden. We decorated the baby’s room and got bags ready. Of course we both had days where it felt overwhelming and anxiety hit, but with every kick and movement we had more faith that our baby was strong! The Tiny Tickers website became a key part of our journey by reading stories and sharing it with friends and family.

On the 28th of September we said goodbye to our parents and headed to Southampton for induction. After some delays waiting for beds, at 4:17am on the 30th of September Amelie was born weighing a good 7lb 3.5oz. She didn’t cry, but she was breathing and lovely and pink in colour! She had cuddles and we got to stare at our perfect baby girl for a while before she went off to neonatal.

The next two hours were the worst. We were left in a labour room to rest, but I just couldn’t sleep. I had no baby in my tummy but also no baby in my arms or in a crib next to me. All I wanted to know was that Amelie was okay, how much she weighed and what she was doing.

We were able to go and see her in neonatal around 9am, which was so surreal. She was having episodes of apneas so was placed on the breathing ventilator whilst they got the prostin (medicine for her heart duct) going through correctly.

Later that afternoon, Amelie was transferred over to PICU in the general hospital. Sam went with her and I did my best to drink lots of water and get myself up and moving so I could be discharged! A few hours later I went over to join them in PICU. Walking into intensive care was so hard. It was difficult to see so many poorly children, but I was immediately put at ease by the staff.

Amelie went from strength to strength the next few days. She moved up to the children’s cardiac ward, established breastfeeding and started to put weight on! I started staying on the ward with her to breastfeed through the night, which felt amazing.

On the 7th October, Amelie changed. She wasn’t feeding as well, she was grumpy and her heart rate was over 200 beats per minute. We knew something wasn’t right.

During the night, Amelie deteriorated more. There was blood in her stools and she was transferred back to PICU and ventilated. Doctors thought she had NEC (Necrotising Enterocolitis) and they did an X-ray to see if any air was where it shouldn’t be. The first look at the X-ray showed that it was okay and that she just needed to be rested., but a further check by a specialist found that Amelie had air in her tummy. This meant something was perforated.

We signed the paperwork and sent her off for emergency surgery, with the possibility of her needing some of her gut removed. Three hours later, we were called to hear that Amelie’s appendix had perforated and had been removed. A section of her intestine was very infected, so a stoma was formed to protect her gut. She was made nil by mouth and put on TPN (total parenteral nutrition) for at least two weeks until her gut had time to recover.

Amelie got better over the following week, but then started showing signed that her heart needed some help. When she was 13 days old, doctors decided to fix her coarctation. They felt that doing the full repair would put too much strain on her gut. Instead, they would do a thoracotomy to fix her aortic arch.

This operation was a success and Amelie eventually moved to the ward with the goal of putting some weight on before her full repair. Not even 24 hours after moving to the ward, her SATS dropped and her heart rate rocketed. After an echocardiogram, the doctors thought Amelie’s VSD was beginning to close and at 23 days old Amelie had her 3rd operation.

Her gut still needed protection, so this time they did a balloon septostomy. The plan was to complete this and return straight to the ward. But Amelie decided she wanted to visit PICU again and didn’t come off the ventilator very well.

After a few days we went back to the ward and had such a lovely week/10 days. Amelie was off TPN, upping feeds, all lines out, all meds orally, she had a ride in her pram round the ward and was able to be away from the bed space!

On Wednesday 4th November Amelie was booked in for her full TGA switch and VSD closure. We walked our baby girl down to theatre at 9am and kissed her little face as she went off to sleep. We almost felt relieved that she was finally going to be fixed. What we didn’t expect was the events that did happen!

At about 4pm a consultant came to say that the operation had been successful, but Amelie struggled to come off the bypass machine. They had tried twice but both times she didn’t cope and they put her back on. Her left ventricle was not working properly. It was then decided that she would go into ECMO and go to cath labs to check her coronary arteries.

The surgeon came up a few hours later to say she was in the ECMO (extracorporeal membrane oxygenation) and she was about to be transferred to have the test done. The test was positive and there was nothing wrong with the coronary arteries, which was good news. The surgeon came to us about 9pm to say that her heart function had already improved, which was really positive, but she would remain on the ECMO in intensive care to give her heart a chance to rest and recover.

When we first saw her at nearly 11:30pm it was heart-breaking. There was so many tubes, wires and machines. She just didn’t look like the same baby we had left 14 hours before.

Over the next few days Amelie was showing really positive signs. They planned a low flow trial just 48 hours after she had gone into ECMO. This was brilliant as we were told the average amount of time on the machine was 5-10 days. The trial went well and her heart showed some brilliant signs. The following day another trial was planned and if all went well, she would come off the machine. We sat around her bed with 19 professionals around us- nurses, ECMO specialists, consultants, cardiologists, her surgeon, perfusionists and other interested people! The trial went well and they said we should leave whilst they got her off the machine.

The following days Amelie showed great improvements. Her chest was still opened to allow for fluid to reduce, but her heart function was good. She had some episodes of pulmonary hypertension, but each day she got stronger.

Amelie came off the ECMO on the Saturday and by the Wednesday morning she was ready for chest closure. Her chest was closed Wednesday, she had the drains out Thursday and was extubated on the Friday! We then had a few days of recovery where Amelie began upping feeds, stabilising her blood pressure and slowing coming off lots of IV medicines.

On 17th November, less than two weeks after her operation, we were moved up to the ward. Amelie did amazingly! She came off lots of medicines, upped NG feeds and started oral feeding. To everyone’s amazement, including mine, one morning Amelie decided to do a whole feed orally straight from me! We couldn’t believe it. She went straight back to breastfeeding like a pro! The 9 weeks of pumping had been so worth it!

Amelie continued to go from strength to strength. She was coming off medicines and getting even stronger at feeding orally. It wasn’t long before the feeding tube was out and I was back to staying in the ward with her. Amelie even came off the heart medicines as her function was doing great!

On the 1st of December, during the morning doctors’ ward round, the consultant said those magic words we never thought we would hear: ‘Would you like to go home today?’ After 9 long weeks we were discharged from hospital and finally came home!

Amelie has since had a check and doesn’t need to be seen for another month. She still has her stoma which we plan to keep for now to allow her time to grow and just be at home. One day we will hopefully be able to get it reversed!

For her birthday, Sam’s mum did a fundraiser for Tiny Tickers and raised over £400! We plan to do some more fundraising in the new year to support the charity’s amazing work!

Find out more about different types of congenital heart defects here.