Lily’s Journey

May 18, 2014

Lily was born with Pierre Robin Sequence with a cleft palate and this is Lily’s Journey.

Day one to three

Lily was delivered by caesarean section full term 6Ib 8oz. Immediately after seeing Lily we knew instantly her mouth did not look right but dismissed the thought as the medical team had performed their checks and deemed Lily to be fine.

Our first concern was when Lily was sucking on her first feed but the contents of the bottle were not going down. We started to notice Lily going purple and alerted a doctor who confirmed immediately that Lily had a cleft palate, Lily was immediately taken to the Neo Natal Unit at just one hour old.

We had no idea of the condition or the impact it would have on Lily, it was a very emotional time and all we could do was watch our beautiful baby in the Neo Natal Unit as we were too frightened to pick her up due to her breathing difficulties. She was placed on her front to help her breath and was attached to a monitor which sounded every time she was not breathing correctly, which was quite frequently and we were very worried. Lily also had a feeding tube through her nose directly into her stomach due to her cleft palate as she could not form a vacuum in her mouth to help her suck.

Lily with her NPA Tube

Day three to week three

On day three a cleft nurse came to see Lily which was arranged by the Neo Natal Unit. This was a major turning point as the nurse was a specialist in this area who explained everything and sign posted us to organisations that could help such as CLAPA.

We found lots of information on the CLAPA website and lots of feeding equipment we would need to use. We were relieved as the cleft nurse instantly took the lead with Lily and suggested an NPA (Nasal Pharyngeal Airway) tube would help Lily to breathe and a dummy would help Lily’s tongue to stay forward as it was her tongue that was blocking her airway causing the breathing difficulties.

The Cleft Nurse inserted an NPA tube the next day and we gave Lily a dummy, the result was instant, Lily could be placed on her side instead of her front and we could pick her up more without the alarm sounding. Lily then developed reflux and was bringing some of her feeds back which was an additional worry with maintaining her weight. Over the next couple of weeks we learned more about the condition and how to handle our baby confidently and then the shock question came! Which one of us parents would insert the NPA tube and feeding tube when Lily goes home? I instantly refused to do this as it made me feel very uncomfortable at the thought of doing it, my husband said he would do it and training was scheduled a few days later. On the days coming up to the training I thought of nothing else and my husband seemed very calm about the situation. My main worry was that my husband would not be able to insert the tube and if none of us could do it this would result in Lily having to be taken to a hospital every week or sooner if she pulled the tube out to have it changed, this would mean sitting around hospitals for hours on end and no consistency for Lily.

The day before the training was due to go ahead I stepped up and said I would change the tubes and undertook the training. My husband was relieved and I was amazed at how easy it actually was. The cleft nurse provided all the training and observed me changing tubes until I was comfortable to do this on my own which was some weeks later at home. The community nurse team arranged all the equipment I needed at home such as, a monitor and suction machine and brought these to me at home and provided with training in how to operate them.

Lily feeding herself - with no tubes!
Lily feeding herself – with no tubes!

Week three to eight months

I changed Lily’s tubes every week and sometimes earlier if she managed to pull them out. At three months old we trialed Lily without a tube for a few hours in the day and this built up to overnight and a home sleep study was arranged and all the equipment was available for me to do this. Lily’s NPA tube was no longer required and our beautiful daughter could breathe on her own for the first time, this was a fantastic result and the support was amazing.

We have had on-going issues with Lily’s reflux and maintaining her weight, Lily had very quickly learned that she did not have to drink from a bottle as we would just put it down the tube and this resulted in Lily refusing to orally feed. She started to eat solids at four months old and could drink water, however, she would not drink milk. At eight months old we took the decision to remove her tube for a trial of 3 days to see if Lily would drink her milk. Day one Lily drank half of her bottles and had a restless night, day two she drank most of her bottles and slept all night, by day three Lily was drinking all of her bottle herself.

A baby with Pierre Robin Sequence eating
Lily after her NPS tube

Lily will have surgery when she is 10 months or so old to close her cleft palate and I am sure her journey will continue but this will be with support. From our experience throughout this journey if I could do things differently I would have spoken to someone who’s child had been through the same journey as I am sure it would have made the journey easier instead of learning through my mistakes or undue worry, however, the support from the professionals has been fantastic. I would like to thank the Cleft team at Alder Hey Children’s Hospital, the community nurse team and CLAPA for their amazing support through this journey.

Lily now - with no tubes!
Lily now – with no tubes!

3 responses to “Lily’s Journey”

  1. Hello, you have a wonderful daughter. thank you for your diary. Also for 2 weeks in our lives will come baby with cleft lips and we wonder how it will be. maybe would you want to meet with me and tell me about your experiences and what can I expect. it will be very help full for me. All the best. Thank you

  2. Hey, Did Lily have PRS as well? How is she with it and everything? My daughter is 2 weeks old and she was born with PRS and a cleft palate. I’d love to get in touch with you and hear about you and Lily’s journey! My name is Cynthia