Hi, my grandaughter Ava, who is 7 weeks old has just been diagnosed with PRS and is requiring a nasal airway. She has a cleft palate and is already on high calorie NG feeds. Does anyone have any experience of looking after a nasal airway at home and any idea of how long we can expect the airway to be in? Ava is still in hospital at present but i would like to be prepared when she comes home.

Oma

Hiya, My daughter Niamh was diagnosed with Pierre Robin Sequence at 2 days old.  She is now almost 8 months.  I hope Ava is doing ok.  I’m sorry but i don’t have any experience of the airway.  Niamh has type 2 Pierre robin and was borderline for needing an airway but we did manage to avoid it.  We did however tube feed Niamh. 
I only have limited experience of Pierre robin but one thing i have learnt over the past 8 months is that all PRS babies are so different and i don’t think anyone really knows how babies are going to do.  It was thought that Niamh would need to be tube fed till she was 7-8 months but with a lot of hard work from myself and Niamh we managed to get the tube removed at 10 weeks.
I’m sorry i’m not much help to your origional question but just wanted to offer you and your family some support.
It does get easier each day and now that Niamh is sitting she rarely has any breathing problems.  We have had some feeding/weaning issues because Niamh has a very wide cleft of the hard and soft palate but again as she has got older she is better at manipulating the food and she now only chokes very occasionally.  Niamh had her soft palate repaired 2 weeks ago and has recovered very quickly, in fact the most difficult part was getting rid of her dummy, which was fantastic in the early days at keeping her tongue forward but unfortunetly can damage a rpaired palate so it had to go.

When your granddaughter comes home she will get regular visits/phone calls from the cleft team (we still get them now).  We had visits also from the childrens community nursing team (in fact we saw them often as Niamh was always pulling out her tube!!.  I also had great support from my health visitor.  You need to remember that if your unsure about anything then ask for help, in the beginning i struggled many times because i don’t like bothering people.

Sorry for waffling, hope i’ve helped.  Any other questions i will try to answer for you. Take care Rebecca and Niamh x

Hi Rebecca,

Thank you for taking the time to reply. Your post does help as it is good to know there is light at the end of the tunnel and that things do get better.
Ava is still in hospital as myself and her mum have to be able to put the airway down Ava’s nose before we can bring her home and at present they are still getting the size of the airway correct. Hopefully early next week we should all be home.

Take care
Oma (Lynn)

Glad to hear that you will be home soon.  We found it much better being at home, at least your more comfortable in your own surroundings.

I hope you get to grips with the airway, i’m sure once you’ve done it a few times you will find your way.  Are you ok with the NG feeding?  I hated doing this at first but after a few weeks it just became a part of our life (a part i’d prefer not to have) but as a family we learnt to adapt and i’m sure you and your daughter will.

I’m not going to lie, the last 7 months have been very hard and have been a rollercoaster of emotions. But i promise you there is light at the end of the tunnel and everytime Niamh laughs and gives us a huge gummy smile it makes all the hard work and tears so worth it.

There is a pierre robin group on facebook and also there is a great forum called faceforward. On there you can find several other families who have children with Pierre robin and i’ve had lots of questions answered for me on there.

Hope you get home soon with your beautiful little Ava xx

Hi Rebecca,

Thank you for the information on the other websites i will have a look at them.
I am fine with the NG feeds as Ava has been on them since she was 11 days old and it also helps that i am a nurse.

Again thank you for your replies, they really do help

Hi Oma,

Congratulations on becoming a granny.

Our son Clive was born with Pierre Robin last november. Fortunately for him and for us he hasn’t needed NG feeding or a nasal airway. Sorry I can’t offer any advice on either of those fronts. It has still presented quite a few challenges over the last 8 months but he’s 4 weeks post op now and doing quite well. His jaw grew forward a bit at the start of May and that’s really helped.

As Bex suggested the Face Forward site has quite a few parents of Pierre Robin babies and it’s really helpful. Our cleft team have also been an enormous help. No question too minor.

Hope you get Ava home really soon.

Dee

Hi Dee

I am glad to hear that things are going well. Thank you for replying to my post. Ava was originally planned for surgery to her palate at the end of November but they decided to postpone giving a date until she had increased her weight by a great deal. Fortunately the high calorie feeds appear to be working and she is now putting on an ounce a day.

Lynn

Oh it’s good to hear she’s putting on weight. Clive was only 5lbs 1 when he was born at full term and so there was a lot of pressure to get his weight up. He was meant to have his op at the start of May but came into contact with another child who later came down with chicken pox so his op was postponed for 2 months. But on the upside his jaw grew forward during that time so it made things easier for him post op. These things do seem to have a way of working themselves out.

Was Ava really tiny? How is your daughter doing? Great that you were a nurse I’m sure it’s very reassuring for her.

Dee

Ava was a good weight when she was born, she was 9lb 4oz. But unfortunately she went down to 8lb6oz and had difficulty puttng the weight back on even with the NG feeds so she was started on high calorie feeds which are working.

My daughter Rhian has good days and bad days and at times feels very low. Ava’s dad split with Rhian before she was born and has had no contact so he doesn’t know about Ava’s condition. Rhian is back with us so she has our help and support.

Lynn

Hi, really pleased Ava has started to put weight back on with the help of the high calorie milk.  Niamh was a good weight when she was born 8lb 12oz but lost over a pound in the first few days but we were very lucky that with the NG feeding she put weight on so we were able to stay on regular formula.

It has taken me a long time to get my head round everything thats happened and i know exactly how your daughter feels although she has the added pressure of being a single parent.  But i think its fantastic that she has you there to help and support her.  Its also an added bonus that you are a nurse.

What area are you from?  We are on the border of cheshire but Niamh is under the cleft team at birmingham children’s hospital and the care and support we have had has been fantastic.

When your daughter and Ava come home if she wants to ask anything i will help as much as possible.  I’ve got loads of pictures of Niamh on facebook or i can email some so you can see that it does get easier with time.  In the beginning i didn’t think things would get easier but they do.

Take care becca xx

Hi Lynn,

Hope things with Ava progressed well over the weekend and the weight continues to go up. An once a day is really good.

Great that Rhian has such good family support. It must be really difficult for her at the moment especially as a single parent.

We live just inside the north west side of the M25 and Clive is under the care of Great Ormond Street.

Please do stay in touch and let us know how Ava gets on.

Take care

Dee

Hi Guys,

I can’t thank you enough for your replies, it is good to know there are other people who have been through what we are going through and have come out the other side with good results. To be honest i didn’t realise how much support you have been until i read your replies today and started crying...thank you again.

Ava continues to improve and it looks like she could be home tomorrow. Myself and Rhian can now insert the airway and all the supplies we need are being ordered.

We live in Newcastle and are under the care of the Royal Victoria Infirmary (the childrens part soon to be the Great North Childrens Hospital) and the North East and Yorkshire Cleft Team. Both have given us excellent help and support.

Hiya,

The best of luck tomorrow. How lovely to finally have Ava home. Will be thinking of you all tomorrow.

Great that your cleft team have been so good. Shame not the same area so god bless the internet.

Dee

Good news, Ava coming home this evening
I am going to pick them up at 6pm, can’t wait to have them home.

Lynn

Ava home at last, and boy do we know it. There is so much equipment. Getting her back into a routine away from the hospital and things starting to settle down now.

Lynn

Glad that she’s home and starting to settle down. It must be scary with all the equipment around. Do they have any idea how long you’ll need it all for?

Dee