Isla was born in Christchurch With Prader Willi Syndrome, and when she was two months old, we decided to move back to Cornwall, UK, to be nearer family and away from the ongoing Earthquakes which made us feel uneasy.
We flew when Isla was still NG tube feeding, so I was still expressing which made that long haul flight all the more exhausting and challenging. The trip overall went well, and we started settling back into our little Cornish village once again.
We have family spread all around Europe, and all over the world, but still felt like we’re a little near to them been back there. With it being, where I grew up, we’re happy to reconnect with our old friends once again.
We settled in, Lola started the local playgroup, which she very much enjoyed, and having some normality back in our lives was good for us all. Life is quite routine with a baby, at the best of times, so at first, we spent a lot of time at home. I was regularly expressing – I can still hear that pump in the distance if I think about it!
For those Mum’s now expressing this is how we made it work –
I had one express machine permanently set up at home, running off the mains with double cups to save time. Also, a second compact one for on the move which allowed us much needed days out, battery-operated, with a single cup and sets of sterilized bottles ready to fill as we went. I remember If I couldn’t cuddle Isla, due to her sleeping when I needed to express, I’d look at her, or at a photo, this would help with the milk supply. Sometimes to pass the time I’d get online or read – all rigged up to the cups! LOL – nice look! Anyhow in private, it’s fine – it was for Isla, not myself.
Even though I had done my best, I felt like I had let her down, or mother nature had let us down. I felt I needed to give her all the nutrients she needed, helping her to become stronger, and fight any illness that might have come her way.
Isla was NG tube fed for 4 months; we spent the last month of that doing a changeover. From NG tube feeding to soft bottle feed – we’d offer her the bottle of expressed milk at the beginning of a feed until she was too weak to suck or swallow anymore, then we’d top her feed up with the NG tube. We could clearly see on the graph, as time passed taking more from the bottle and less from the tube, we were so excited at her progress – we’d always shear the news of how well the last feed had gone.
One of the bonuses to tube, or bottle feeding, it that Paul could also be part of the feeds from time to time, even Lola would like to help with assistance, as Isla became stronger. Eventually, at 4 months old she was strong enough to take her whole feed from the soft bottle, allowing the NG tube to come out permanently. Golly, it was so nice to see her beautiful face without the plaster, and the inconvenience of the tube during dressing and bathing was now gone. She then moved onto a normal bottle with – still expressed milk. I dreamed of the day I might be able to feed her naturally in my arms, although I was told the prospect wasn’t good, I still hoped.
Isla was also having extra, super-duper, nutritious, milk to help her gain weight, being so tiny she needed all the extras she could get. I can’t remember exactly when she started pureed foods, but we started balancing her nutritional intake with homemade baby foods, and expressed milk, along with the super-duper baby milk, so the dietician was happy that she was getting all she needed.
I had always had skin to skin cuddles regardless of how we fed, so when Isla was 8 months she was strong enough to feed naturally, and the all bottles could go, along with the expressing and the machines, and all the dishes that go along with that! It all suddenly all felt right – and how it’s meant to be.
She was still a very weak baby in comparison, but now able to feed and communicate as babies do. The nurse was concerned about the breastfeeding – that Isla wasn’t getting enough fluid, as there wasn’t a way to measure the feed anymore. Her nappies were wet and changed regularly, so all was well on that front. Along with healthy purees and mashes and, teaching her to use a sippy cup, we were on track. I was able to breastfeed until Isla was 19 months which was amazing for us both.
Isla had to sit up to eat but found it hard for even short periods of time, so we’d wedge her in with little cushions, and quickly let her rest if she tiered, which would often happen, as food digestion would zap her energy fast. As time went by, we made the most of what we had, we addressed any complication or challenge as we approached them. Isla’s developed is slower than most children but she always gets there in the end. The first hurdle was feeding, and we eventually got there.
Then sitting and gaining inner core strength to do so was another big hurdle. As a family, we’d slowly show Isla, going through the broken-down movements of how to sit, she also had a special chair to help her at first, but in time she eventually got there. Crawling was also delayed, as her back would slump, but with extra input from the physical development team, she was once again achieving her goals, and in time crawling. Therefore, able to start exploring the world, learning more about her surroundings, and interacting with Lola.
We had support visits weekly from various specialists, directing us to support Isla to achieve her goals – one at a time we worked through each challenge. We’d also have a speech and language input as Isla was very behind with her speech, we started working on simple sign language when she was only one to help her communicate.
Most children develop naturally – be it in their own time through play and interaction, but with Isla and most children with PWS, each step of the development needs breaking down into tinny, more achievable steps with extra support along the way. Isla was walking by the time she was one and a half, which is wonderful for a child with PWS. She had upper hypertonia, so her legs were stronger than her back and arms, so therefore very unsteady, and wavered all around, so often falling or bumped herself. But as time went by she became stronger, and then able to run! – now that was worrying! – No fear, strong legs, at high speed, with little upper muscle tone, kept us -well – on our toes.
By the time Isla was two, she was receiving growth hormone to help with her muscle tone, development, and growth. She’s always been the smallest, and wore clothes years and years bellow her age, but over time she’s caught up with her pears. This has made a big difference to her overall strength and growth to this day. Isla went to a part time playgroup at 2 and a half and loved every minute. Loa was also going to school too so both girls were enjoying their own things.
She can now do what the other kids all do – she’s not as strong as others and gets fatigued quicker but can participate which is wonderful. We’ve always pushed Isla to her limits, helping her to keep the muscle tone she gains. We try and keep fit by doing what I call invisible exercise – well, Isla she hates walking – she just thinks about how tired she is ! but if we’re having fun like an outside ball game, swimming (with her lifejacket on as she still doesn’t have the upper strength to swim yet), Horse riding for the disabled, or can now ride her bike, well this is all good fun, and she forgets all the negatives – So the invisible excessive works well for us – Keeping us all fit – then we all sleep well!
