Lilian's Story (0-6 months)

Our daughter Lilian was born at King's College Hospital on January 8th 2010 at 1524. She was delivered by emergency c-section. Lilian weighed just 2.14Kg as she had a gestational age of 31 weeks (9 weeks early). Lilian was diagnosed with Costello Syndrome about 2 months after birth. Below is the story of Lilian's first 6 months.

The Pregnancy
Xmas Eve.
We were always told Lilian was in the 95th percentile for her size and just that she was going to be a big baby. No problems were reported at the two routine scans. However not long after the second scan the medical staff suggested a third scan was carried out to check the due date of the 12th March was correct. During Christmas Mette's tummy seemed to increase in size quite a lot but everyone assumed it was because Lilian was going to be a big baby. The additional third scan was booked for Friday the 8th Jan. Two days before the scan, on Wednesday the 6th, Mette reported less foetal movements and went to King's College hospital for a foetal movement check. Mette was put on a monitor for an hour or so. They found nothing wrong so they sent her home.

The Birth
On Friday the 8th we arrived for the scan at King's College hospital. The results showed that the due date of 12th March (the day before Daddies birthday) was correct but found Lillian's bladder was rather full and that there was quite a lot of amniotic fluid. An antenatal Doctor was called and suggested they put Mette on a foetal heart monitor. This lasted an hour or so and they looked happy but suggested Mette goes to the maternal assessment unit for a second run just to make sure. Mette and I were busy talking over what to buy at Sainsburys for dinner that evening when a nurse entered the cubicle, took a look at the monitor and told us both to stay calm. The nurse then pulled the emergency cord and in less than 5 seconds there were medical staff everywhere. The next thing we knew Mette was being wheeled down the corridor on her bed to surgery. I was allowed to hold Mette's hand whilst they administered the anaesthetics but then had to go to the waiting room and wait for the longest hour of my life. Eventually I got tired of waiting and entered the unit again asking random people where Mette was. Finally I found Mette in the recovery room being looked after by a nurse. Lilian had been taken to intensive care.

During the final period Lilian had been getting weaker and weaker. The nurse pulled the emergency cord because Lilian's heart beat was extremely weak. Even though she was 9 weeks early it's far better to treat babies outside of the womb if the condition is life threatening. From the moment the nurse pulled the emergency cord to when Lilian was born was just 20 minutes. That includes running down a corridor and putting a terrified Mette to sleep. Lilian was born without life. Her Apgar score was at 0 for 4 minutes. At 4-6 minutes she got to 1 but that was due to drugs and various apparatus. Lilian was in big trouble and would need to spend some time in hospital.

The first time I saw Lilian. A few hours old.
Afterwards Mette and I were numb with shock. Mette was still raw from the anaesthetic still and didn't know what the hell was going on. She was also in a lot of pain as the Doctors weren't very careful when they'd delivered Lilian due to the state of emergency. I was ringing my parents trying to speak but failing miserably. I did manage to ring the Danes and held it together long enough to let them know what was happening. Later that evening, it must have been 3-4 hours later, the main Doctor came and visited us both in the recovery room. Mette was far too ill to move but I was taken upstairs so I could meet Lilian for the first time. Here is the photo I took with my camera phone so I could show Mette our beautiful little darling girl. I couldn't get access to the incubator as Lilian still had a team of 5 people working on her. She was so tiny. Connected to a vast array of machines that breathed for her, gave her drugs or monitored her life senses. It was very noisy, hot and busy room with four or five other babies. Welcome to the NICU (Neonatal Intensive Care Unit).

The first 10 weeks
Lilian spent a total of 10 weeks at King's College Hospital and even had a weeks holiday at St Thomas' Hospital (on the River Thames opposite the Houses of Parliament) to look at a problem with her kidney.

On ventilator (3 days old)
For the first few days Lilian was fully ventilated on 100% oxygen (we normally need just 20%) at such a pressure they were worried her lungs would burst. They had to take the risk as Lilian's lungs were still immature and hadn't had time necessary to prepare for life outside the womb. They were covered in surfactant and acting much like two pieces of cling film. This meant it took a lot of effort just to get Oxygen into her system. However, in the end Lilian grew stronger and she was slowly moved to CPAP for small periods of time (an hour or so) until eventually she could stay on CPAP full time and then begin to move over to breathing for herself. This whole process took a couple of weeks when she finally moved over to breathing on her own. Mette

Lilian saying "no way!"
As Lilian had such a bad start in life the early days and weeks were particularly difficult for Mette and I to fully comprehend. At first we were told Lilian wasn't going to survive at all and that her lungs couldn't cope. Lilian must have heard this conversation as she proved them wrong. Then we were told that Lilian had suffered massive brain trauma and would be severely disabled. Again Lilian though better than that and whilst various MRI scans have shown white spots on her brain they are nothing compared to what the Doctors first thought. Essentially the Doctors threw all the negative things at us and Lilian said "no way!".

The list of "firsts" for Mette and I is quite different to the typical birth I suppose. Still we get to have a list none the less so here it is:

Day 0
Seen by Dad in real life and on camera phone by Mummy.

Day 1
Seen by Mummy.

Day 2
Touched by Mummy (just on the hand through the port hole)

Day 5
Lilian opens eyes for first time and sees Mummy and Daddy for first time. Not for long though as she's still on sleeping drugs for a while yet.

Day 6
Mummy and Daddy change Lilian's nappy. Also ventilator taken off for first time.

Day 8
Mummy and Daddy get to actually see Lilian's face without the tubes and mask.

***Day 9***
Cuddle with Mummy.

***Day 11***
Cuddle with Daddy.
First cuddle with Daddy on Day 11.

First cuddle with Mummy on Day 9.

Lilian accepted Mette's expressed milk via an NG tube quite early on and the amounts given increased as she tolerated it more and more. I remember being really excited that she managed to take 1ml of milk! At this stage Lilian was still just a premature baby (in that they were still figuring out what caused it all) so she was moved onto breast feeding and despite her age and size we were told she was doing very well. We were chuffed.

During this early period the medical staff were still trying to figure out what happened. They found some white spots on an MRI (due to her tough start and lack of oxygen) but a subsequent MRI showed them to be relatively minor and just advised a check up when she turns four years. Her bladder size in the womb was related to an issue with her kidney which cleared up with drugs and isn't progressing. They also found her heart muscle to be slightly enlarged (Hypertrophic cardiomyopathy) but various echo scans and multiple ultra sound scans have shown this not to be obstructive.

The Doctors were still unsure about why Lilian had issues and called a genealogist. They ran tests and a few weeks later we were told she had been diagnosed with Costello Syndrome. We both went a bit "odd" given the previous few weeks of NICU and life/death situations but found Costello Kids and Contact a Family websites were very helpful. Of course we read everything possible.

When it was discovered Lilian had Costello Syndrome a lot of the jigsaw pieces fell into place. Lilian was moved onto bolus feeding (breast feeding hadn't gone very well and Costello children don't feed orally for the first few years) and we were slowly prepared to make the next step of going home. Lilian wasn't on any extreme drugs and her current goal was to eat/sleep/poo (in that order) until she grew big enough to go out into the big wide world.

Finally at Home:
It made such a difference getting her home. She was a more settled and a happier baby. The doctors and therapist were all very pleased with her progress. Lilian was broadly on track developmentally and generally doing well.
At home!