TOF part 3 (finale)

The recovery...

12 hours post op, Sophie started spiking temperatures 39C and above. IV antibiotics were increased as she was already on some as a preventitive post surgery.

Bloods were taken.

Sophie was septic.

She fought well though and things gradually started to improve and the neonatal team got on top of the infection.

Her surgeon was happy with her progress and decided he wanted to take her off the vent at 24 hours post op.

Absolute disaster.

Ian and I just happened to walk into the unit as they had taken the tube out and I looked up at the monitor that was alarming and her sats were 68%, 56% 48% and continued to fall until we were ushered outside.

Lots of running in and out from doctors as we waited. I knew they were trying to get her back on the vent and stable but I didn't let on to Ian.

We were called back in after what felt like an eternity and she was stable and back on the vent. The morphine that she was having to keep her pain under control had caused her to have a respiratory arrest and they needed to wean the dose down before they tried again.

The following day, Ian and I were allowed to have our first cuddle with Sophie whilst she was still ventilated and low and behold, little miss naughty nappies decided she was going to wake up.

She was ready.

She yanked out the tube and extubated herself.

From that moment on, she was able to breathe for herself.

Her recovery for the next few weeks was good. She got bad jaundice and needed phototherapy for a few days to reduce her levels. She started to drink orally and we were able to reduce her tube feeds. She wasn't strong enough to breast feed but I continued to express milk for her.

We were eventually sent back to our local hospital after 3 weeks and as we walked onto the ward so happy to be nearer home, we were met by a consultant.

Leicester had tried to catch us before we left. They had results from the guthrie test.

Sophie had Cystic Fibrosis.

I couldn't hold it together anymore, I broke down and cried. It took a long time for this to sink in. She'd already been through so much. It wasn't fair.

I was angry.




Forward ahead to 5 months old.

We found out Sophie was pancreatic sufficient, no enzymes needed, yay!
Reflux had been a terrible problem and Sophie had steadily become oral phobic after needing to have the join (anastomosis) of her oesophagus stretched 4 times.
She had spent most of the last 5 months in hospital due to all the surgeries she had needed.

Decision was made to have a nissen fundoplication done to stop the reflux burning her oesophagus anymore. Operation number 6.

We continued to feed Sophie via NG tube as she wouldnt have anything near her mouth. A year later she had her peg fitted and exactly a year after that it was changed to a mic-key button. Operation number 9 was her port being fitted. 10 if you are pedantic and count her longline insertion :)


Sophs TOF and CF do help and hinder each other. The scar tissue left in her trachea causes her windpipe to be a bit floppy and this causes her to have a croupe sounding cough that is dry and crisp.

It is REALLY loud.

Obviously scar tissue in her trachea isn't great with CF as it can cause her mucous to get trapped and lead to more infections.
The force of her TOF cough does help her to shift mucous at times though, so it is swings and roundabouts.

The odds of being born with both conditions is approximately 1 in a million.
I know a family here in the UK who have a child born with both, they are a good source of support as we are writing the text books with our kids.

Leave you with a couple of pictures.

Her lifeline, her mic-key button.






This is Sophie's TOF scar now at 3 years of age. She was cut from the middle of her chest and round to the middle of her back. They had to go through her rib muscle, move her little lung out of the way and perform the surgery. Amazing really. (ignore the red blotch and scratch, Sophie decided she had an itch just before I took this)

The scar has really faded well but still very much there as a reminder of one of her battles.





I haven't detailed everything as I would have been here until next year blogging about it. If anyone has any questions, please ask. I'll try and answer as best as I can. I'm bound to have missed something really important out!


Sophie continues to improve with her oral aversion (phobia). She is still solely tube fed at the moment but continues to taste foods.



Still loves her ice cream at nannies!




Take care and thanks for reading!!

xx