A patient story, Lilou

Lilou was born premature at 7 months… on Saturday the 26th of October.

Prader-Willi syndrome had been evoked by my gynaecologist 3 days before she was born on a Wednesday.

I had an amniodrain where 1.6l of amniotic liquid was drained from my tummy on the Tuesday … This was then tested for various syndromes.

One being PWS.

However, I was told on the Thursday that at 80% Lilou did not have PWS …. I later found out that this was because only PWS by deletion was tested …. And Lilou has UPD.

The Saturday she was born the birth was very quick and she was born vaginally. But she came into the world quietly with hardly any cry, she just didn’t have the strength to cry …. She was then whisked away as quickly as she had come to the reanimation unit.

I finally got to see her and touch over 8 hours later. She had been transferred to the NICU that was to become our home for the next 3 months.

I spent every moment of every day with her for the first 6 days of her life as I was still hospitalised myself. Every day I would hold her, doing skin to skin and sleeping with her.

Lilou slept a lot … and she barely moved ….

The doctors really didn’t know what was wrong and more genetic testing was ordered. PWS was once again evoked but for me I just knew it couldn’t be that as I had already been told that there was 80% chance, she didn’t have PWS!

Just under 3 weeks after her birth I was told that Lilou did in fact have PWS, after a more detailed genetic test had been performed.

This explained to the doctors why my tiny baby girl barely moved, had almost no tone, she was like a rag doll, her arms would just hang … and why she needed an NG tube as she didn’t have the strength to feed. And this failure to thrive (the lack of being able to feed) so common in PWS was only made worse by the fact that Lilou was born so prematurely.

After being released from the hospital myself, Lilou was still in an incubator in the NICU, I spent every single moment of the day and evening with her until I had to go home to rest. During this time, I tried and bought EVERY single bottle and teat available to me to try and get her to feed. It was excruciatingly difficult … she just didn’t have the strength, even with the freest flowing teats available!

I followed every single piece of advice given to me by the experienced nursing staff at the hospital and I researched by myself EVERYTHING I could find that could potentially help her to suckle! Positioning, stroking her cheeks, using a technique taught to me by the psyschomotrician (a specialised OT) of putting some of my breast milk on a Q-tip and tracing it down her cheek towards her lips, then around them and in to her mouth .. trying to get her to react and suck.

But it was insanely hard for her to take even 2 mL of milk!

Due to me holding her, again, Literally every moment I was with her, skin to skin, she seemed to react , ever so slightly, to my breasts … and so after 2 and a half months, I tried to breast feed her. This worked a little better than the bottle, which she was still only able to manage between 2-4 mL per day, not feed!

I believe that because my milk flowed very very easily this facilitated her being able to take slightly more than the bottle … But she would still only be able to take between 5-8 mL …. Before falling asleep.

She slept so much in her first 6 months of life it was insane …  Almost 3 months after arriving in the world Lilou finally came home with me … this wasn’t because she was drinking sufficiently orally but because the hospital staff agreed her being home with me would be a better solution for us both.

Lilou still had her NG tube and the majority of her feeds were taken through this. I had basically given up on her taking the bottle …. It demanded too much strength that she just didn’t have.

She would still suckle my breast and did manage a ridiculously small intake ….


Ruth, Lilou’s mum