NPA too short? End of Feb 2022

We started to notice that Rosie is gradually rejecting her bottle which is upsetting as she was enjoying it a lot and we had hoping to gradually increase the amount she would take from it and have less going down via the NG. It seemed that she would get upset and reject it when it came to the first or second swallow so we thought it may be the NPA becoming too short and causing irritation/discomfort when she goes to swallow the milk. 

On advice from a cleft nurse at Broomfield who specializes in NPAs, we took out her NPA for the day and offered her a bottle at her feeds. She took to it straight away and would have 20ml (the usual volume she had got up to previously) very easily. This confirmed that the NPA must be the cause of her rejecting the bottle and it may be due to it being too small. So the cleft nurse was happy for us to insert an NPA at 7.5cm. 

Of course we had to take advantage and have a mini photo shoot where we got to see more of her beautiful face:

Since the increase in length of NPA she has seemed to be a lot more happy and smiling. Which has been lovely to see. She has been very reluctant to take her bottle again however which is upsetting. She will take it for a few sips which is more than what she was doing when it was 7cm but no where near as much or as easy as she did without her NPA. 

We are now waiting to hear if we will have her follow up sleep study brought forward so they can check the position and length of the NPA/test if she even still needs it. While it would be so so lovely for her to not have her NPA anymore I am holding my hope back as I do realize it is early days. But it does seem that the NPA will be a barrier to getting her to increased her orally feeds so as soon as it can come out (and she is safe) the better!

Reflux and medication drama

Apparently, reflux is very common in PRS babies and we have struggled with it very much with Rosie. She has had a lot of vomiting and a lot of classic reflux discomfort movements (throwing head back and arching her body back into a banana shape). When she first came home it was awful trying to manage it and seeing her in so much discomfort every time we fed her .

We have finally got it under control with taking omeprazole 2.5ml twice a day and having Gaviscon infant 2 sachets with every feed (she is fed 6 times a day)

Unfortunately, that made her very constipated and she now needs lactulose 2.5ml twice a day too... 

It is a lot to manage and keep up with but she if finally a lot more comfortable and keeping her food down. 

There have been a lot of difficulty with managing her prescriptions with the pharmacy - they initially wouldn't make the omeprazole up despite that being advised to do in the prescription. Her dr also didn't want to give her that much Gaviscon without speaking with her dietician, but instead of calling us to discuss they just cancelled the prescription so we didn't know until we went to pick it up when she was almost out of it!

Video showing the reflux discomfort prior to correct medication allowing it to be under control - this was every feed:

Video showing the amount she would vomit multiple times in the day:

Now we are dealing with challenges over how long her omeprazole and lactulose should last her. The pharmacist calculates that we are requesting it a week earlier than we should based on the amount given, but they aren't accounting for wastage when pouring the liquids into the lid to then syringe up. It definitely does feel like a constant battle and often Rosie is the one losing out and being left without medication she needs. My husband had to go to her doctor and wait over an hour just to bring her syringes to demonstrate that what they are prescribing doesn't last - they finally authorized an increased amount to account for wastage, but it's just another thing we didn't need to have to battle over with everything else.

Jaw growth

I will be using this page to update and document her jaw growth.

Collage of jaw and chin when newborn:

Collage of jaw/jaw at 2-3 months old. Already a huge difference which is great news for her airway!

 

Development (Holding head up, smiles and growth!)

Since coming home I was worried for her development after spending a month of her life in hospital and also arriving slightly earlier than expected. (37 weeks is considered full term so she only just made it to being considered full term - 3 days earlier she would have been considered late-premature.)

I am happy to share that Rosie is making amazing progress. She is doing wonders with her tummy time and loves holding her head up. She has a very strong neck! She loves her play gym too!

She started smiling in her sleep very early at around 4 weeks But didn't do her first social smile until about 10 weeks and now she won't stop. She loves playing peekaboo and having her bottom lip tickled. 

At about 11.5 weeks she has started smiling at me and my husband in the morning and in response to just us smiling and we are not having to work as hard as we previously did to get he rot smile. It is amazing seeing this feedback from her!

She is growing well and putting on weight! I cannot wait to see the updates here each month compared to the previous. We are using her dolly which she got for Christmas from grandparents for scale each time and you can really see the difference!

 

Tapes

Rosie and her tapes have been a journey themselves... While in the NICU where there is limited staff to responded to plasters coming up and while she had her temporary NBPA which was not very secure she had many problems with her tapes. They would often come up and she would pull tubes out. It was devastating as her parents to see and we desperately wanted control to mange her tapes and make them secure and to respond to them when they started to peel. This was hard to do in the NICU but once we were home we were finally able to have control over her tapes to ensure she was comfy and everything was in place and not going to move. 

Photos of the tapes in NICU with her temporary NPA:

Once home we got a very good system. We use cavilon film barrier to protect her cheeks, put duoderm on top of that and then tegaderm secures her NG and for her NPA we use a leukoplast tape. Then over all of that we use a pretty medical plaster with a sweet design from https://www.tubiecheeks.co.uk/. This seems to work super well as it lasts the full week of her bringing her hands to her face and rubbing her face and daily baths/spit ups.

We change her NPA each week and her NG should be changed every 90 days (unless she pulls it out herself but she hasn't manage that yet. 

Photos of her plasters at home:

Meeting the family

Once she got discharged home she was finally able to start meeting her family.

Most importantly she met her immediate family - her furry siblings Luna, Jakob and Lumen (our cats):

She got to meet some grandparents:

She got to meet Aunts and Uncles and cousins!

 There are still many more family members yet to meet her who are desperate too - we can't wait to add further photos here of some special and long awaited cuddles!

First 2 months home

We have loved having Rosie home, getting her dressed all pretty, going for walks and having so many cuddles.

Some photo's from the first 2 months where we have had her home:

 

Discharge from GOSH and COVID

After 5 days at GOSH we were finally getting discharged on the 4/01/22. It wasn't easy though as we had a COVID positive test thrown in the mix just as our discharged was being arranged and we didn't now if it would still go ahead. 

My husband's test came back positive while I was on the ward with Rosie so we got moved from a bay bed to a isolated cubical while we await tests to be arranged for us. It was a long night and I wasn't able to leave the room. I didn't have any clothes on me, I didn't have a phone charger, I didn't have a toothbrush or toothpaste. I didn't even have a spare pair of knickers. It felt awful not knowing what was going to happen and felt awful to feel dirty and gross without my things. 

I had to have random bits of food brought to me which was often things I could eat... the only things they had which I could eat was plain crisps, toast and juice and water.

This was the first night in Rosie's life that she didn't have daddy  with her and the first time in a long time I have been apart from my husband who is my rock and support. It was so hard. 

Some videos from my isolation:

Thankfully Rosie and I were negative which was surprising considering our close contact with my husband We were discharged the next day and the hospital provided my husband a heavy duty mask to use public transport to get home:

Disappointingly, Rosie's medical supplied were given to me like this... all jumbled up in a carrier bag. Luckily, I had the sense to try and go through everything and found multiple things missing like PH strips and nasal syringes. Once I alerted staff about this they apologized and quickly added the extra bits - but it's scary and worrying that we could have been sent home without everything we need. 

Once home I made a make shift storage compartment for her medical supplies in her cot. It wasn't for a while that we came up with a more permanent solution:

Her are the photos of us being discharged from GOSH and finally on the way home! Unfortunately, my husband couldn't join as my parents are in a high risk group and it wouldn't have been wise to risk a close contact with someone with covid. But we ended up getting home at the same time with him using public transport as advised by GOSH's infection control

And this is the first photo of Rosie tucked in her own bed at night. It looks like she is smiling doesn't it?!

 Once home, my partner and I isolated from each other as much as we could in a small house. It was hard but thankfully at 

the end of the isolation period me and Rosie remained negative and we could finally start to get into a routine and enjoy family life together.

A bed at GOSH is finally available

On the 30th Jan 2021, a bed at GOSH was finally available for Rosie! The transport team came and packed her up in a special transport incubator to take her via ambulance to GOSH. My husband wen in the ambulance with her and I followed with my parents driving me. 

We were told they would only accommodate one parent but we were hoping that they would have space for us both. 

It was quite nerve racking as we had been warned if an emergency came in while we were on the way to GOSH they may take priority for the free bed and we would have to turn back. I didn't feel fully settled until we got there and knew the bed was really truly hers. 

Transporting to GOSH:

 

The very evening we were there I was hoping to feel relaxing and glad about the end of the hospital journey being insight... That is when I noticed MY CS had an infection. We were in central London... It was evening... and now I needed antibiotics quick as this infection could go to 0-100 very fast. 

So we spent the evening in a and e... Just when we hoped out bad luck was finally over!:

Photos from our stay at GOSH:

Rosie has her very first bath at GOSH - she wasn't impressed:

Rosie having her sleep study at Gosh. They fitted a new NPA which was 7cm (compared to Colchester's temporary one which was 9cm!) The sleep study showed she had no signs of obstruction during the night with this new NPA and X-rays showed it fitted well in the right position:

Our NPA Training manual:

Learning how to stitch up an NPA:

Daddy putting an NPA in himself to see what it feels like for Rosie:

This was our new years eve in the parents accommodation!

Some videos of the parents accommodation - which was very nice!:

Hearing

Rosie had her newborn hearing screen test at 19 days old. She did not pass and the results were inconclusive. This caused another panic over another potential thing to worry about to get added to an already long list. 

We would have to wait until she could have a more in-depth appointment with audiology. 

We did know she was reacting to sounds as she appeared to do so - but after this result I kept doubting myself. Maybe the reaction was a fluke and nothing to do with the sound she may have or may have not heard. 

Photo of her having her newborn hearing test:

Rosie's follow up appointment with audiology was when she was 5.5 weeks old. They carried out a number of more in depth tests with her. One required her to be completely asleep and still as any movements would interfere with the results... Rosie is a very active sleeper and likes to wriggle a lot so this appointment took a very long time to finish! We had to go for another appointment to follow up as they needed further tests - this happened 2 weeks later. 

It was discovered that Rosie had good responses in her right ear but congestion in her left ear. It was felt that the congestion did not affect her hearing however. 

 We will need to have a follow up around the time of her cleft repair.

Unrelated to her hearing appointment, I did my first public NG tube feed on the day of her first audiology appointment. With help from my mum being an extra pair of hands for me - I completed a feed while in the audiologist office! Another win to be proud of!

 

Feeding Roslyn

Due to Rosie's cleft she was unable to create suction in her mouth which meant typical bottles were not suitable for her. This meant all the prep I did and the bottles we had already bought for her were useless. Seeing items I had got for her not be able to be used due to her condition was sad to go through as it made it really settle in how much we would need to adapt to meet her basic need of being fed:

Rosie had an NG tube put in from birth and has been feeding via that ever since. My husband and I had to complete competency training on how to feed her via the NG prior to her discharge. She does have specialty bottles but most of her feed goes down her NG. It's been difficult managing all the supplies - the different syringes sizes, the ph strips and not to mention the aching arm from gravity feeds. It's also difficult in that we realized it would be hard to have time for ourselves or use a babysitter at all within the first year as it's not common for them to be NG trained. We spoke with our community nurse who gave use the go ahead to train family member to feed her via NG and have been slowly doing so - this will allow us to have childcare once in a while to have time for ourselves for a date night or just a little break now and then:

One of the things about Rosie's condition which has caused me a lot of upset is being unable to experience breastfeeding with her. It was something I was looking forward to doing and I had heard many stories about the special bond that it can create. I had lots of things at home ready like comfy breastfeeding pillows, nipple cream and shields and was so prepared to give it a go. I was excited to have that bond with my baby. But due to Rosie's cleft and being unable to create a suction she was not able to breastfeed.

I wanted to try and give her the best start I could so I made the decision to try and feed her exclusively via pumped breastmilk. As my supply was getting started she was supplemented via formula but I was quickly producing enough for nearly full feeds. It felt good to feel like I was doing something for her while I was at home and away from her in the hospital but it was grueling to getup and pump every 2-3 hours constantly to establish a milk supply. I am proud that I did this for her but I made the choice to pout the pump down at one month in when she was due to come home as it was too much to mange on top of lengthy NG gravity feeds and even in the hospital I felt I couldn't just enjoy holding her as I was clock watching when the next pump would be. 

I was very upset considering switching fully to formula but fed is best and it was ultimately the best choice for me and for us as a family. I was surprised to be told by a nurse at GOSH that I had already beaten the national average of feeding breastmilk as that is just under a month. 

As mentioned earlier, Rosie does have specialty dr brown's bottles with the valve as this allows a flow of milk to come through the bottle without suction. 

It is not usual for cleft babies to have to have NG feed, and many can feed 100% orally with specialty bottles. However, in Rosie's situation her cleft in combination with her compromised airway managing the swallowing and breathing routine is very difficult and tiring for her. 

She started on ultra preemie teat with the DR brown bottles and enjoyed using them from the get go. She would only ever take about 5-10ml from the bottles before tiring out. It's more about giving her a continued oral feeding experience to make weaning off the NG easier later on. We also use dummy dips in milk and allow her to have that during feed so she associates the taste with her tummy being full.

See below for demonstration of the specialty bottle and Rosie have her first few attempt at taking it:

Once she was fully discharged home we had the opportunity to visit Broomfield cleft team feeding clinic at 7.5 weeks old. We met with a dietician, a SALT and specialty cleft nurse. They watched her have the bottle and were all very happy with how she managed it. They gave us the go ahead to move form ultra preemie teat to the preemie teat and advised us that we could decide when she was ready to try the level 1. 

It was so exciting and reassuring the know that she was doing well. 

She was also weighed and measured when here and she is tracking very well on the 75th centile for weight and the 98th centile for height. She has maintained this for the past 3 months of her life so she is putting on and maintain her weight very well. 

Photo of us at Broomfield hospital waiting to go in for a feeding clinic:

 

Diagnosis

Below shows screenshots of how I was feeling at the time of Rosie's birth and how I tried to take in the information. It shows initial panic at the idea of a surprise medical issue and at the extra care needs she would have. It also shows me being able to come to terms with it and try to have some hope for the future. 

I found it very hard suddenly having my baby taken from me without me being able to hold her. It felt like I didn't have a baby - there was a disconnect. I was pregnant, then suddenly I was not - but where was my baby? 

After the first day I was only bale to hold her for 20 minutes and then I was left in my bed unable to move after my CS. I couldn't sleep because of the pain and I was panicking. I didn't have my baby with me and I didn't know what was wrong. I did the only thing most people would do and this is go to dr google. 

I ended up finding out about PRS this way before she was officially diagnosed. Of course I found worst case scenarios and had all thoughts about how I could cope and manage in this unexpected situation I found myself in. I felt so alone. Again, I am so thankful to my family, my partner and the delivery team at Colchester for heling me get through this difficult time. 

I spent this first night panicking but quickly after I started planning and having hope about my little girls' future:

First sleep study and NPA

 At 5 days old when it became clear that waiting for a bed at GOSH would take a long time Colchester decided to do a sleep study of their own to see if they could help speed her GOSH referral up with some more information. The sleep study indicated severe airway obstruction when on her back so they inserted a nasal airway (this is called an NPA). They advised their nasal airway was only a temporary one and that GOSH would be able to put in a permanent one and provided us with training to mange it ourselves. Colchester stated they were not able to give the training and neither was anyone at Broomfield cleft team so we HAD to wait for the bed at gosh. 

Colchester stated that as their sleep study indicated her airway obstruction was severe she would be treated as a priority for GOSH to admit her. We were told that a call could come in any day about a bed being available and that we would need to move fast, but it wasn't until another 24 days that one became available.

Photo on the left is of her getting hooked up for the sleep study and on the right is her with the temporary NPA the day it was inserted. The temporary one has plasters all over her face to keep it secure. It was sad not being able to properly see her beautiful face and stroke her cheeks and head with plasters in the way:

Colchester NICU

We spent 25 days in Colchester NICU. She was stable and purely waiting for a bed at Great Ormand Street for the majority of her stay. She needed to be at GOSH for them to do a more detailed sleep study than Colchester could perform and for them to look at a more permanent nasal airway PLUS give us the training to manage said airway. 

There were 2 occasions in those 25 days where a bed was suppose to be available but one time another baby needed it more than Rosie so she was back at waiting and the other time the baby who was due to be discharged relapsed and couldn't be moved anymore so Rosie continued to wait.

These 25 days were the longest in my life. It was awful not knowing how many more days or weeks we would be waiting. I was desperate to have her home and it was exhausting going to the nicu everyday for hours while I was still recovering my CS. The seats aren't comfy, the rooms are sweltering and there is buzzing and beeps going off all around you as babies have drops in their stats. It's not a nice place to be and my heart goes out to the parents who have this be their life for months. 

Most of the babies in the NICU were preemies - it seems quite uncommon to have a full term baby in there. This made it extra hard as she was having period of becoming more and more alert but she was stuck in her crib - unable to be taken out for tummy time and be played with much due to wires and needing to be hooked up to monitors. Other babies who were premature mostly slept - so I started getting very upset about her missing out on social interaction. I was so desperate for her to come home. 

During our wait for a bed at GOSH there was a time were Colchester NICU were thinking of discharging us with their temporary nasal airway and allow us to wait for GOSH as an outpatients, but suddenly that changed. It got all very confusing as so many professionals had different opinions and they were trying to communicate between Broomfield cleft team, Colchester hospital and GOSH. 

We made the best of it and tried to take photo's and videos each day to send to family. Her grandparents especially found it hard not being able to meet her and spent the first few weeks of her life getting to know her via photos. Unfortunately, due to covid only parents were allowed in the NICU - so no one other than us got to meet her. 

First few weeks in the nicu:

First time in an open crib as oppose to an incubator at 2 days old as she was regulating her own temperature very well. Unfortunately, it didn't last long as she caught a cronobacter infection a few days after and was put back in an incubator to prevent spread as a cautionary measure. But I still remember feeling so proud seeing her in a "big girl crib"

NICU stay continued:

Rosie's first post from her cousins. They were excited to give her a birthday card for her BIRTH day!

 

Rosie passing her car seat stress test at 19 days old!

Christmas in the NICU obviously wasn't ideal and not the way we had thought we would spend the day as a new family of three.

Waking up it didn't feel like Christmas at all. But going in to the NICU that morning. we were greeted by gift bags on the incubator... Santa had been. What a lovely surprise! It made things feel slightly more Christmassy and it was beautiful to open presents with Rosie on her first Christmas. There were lovely gifts like rattles, children's books and gift cards in there and we had a great time going through everything. It felt like we were children ourselves as we opened things with Rosie. The gifts where donated by amazing charities such as wish upon a fairy (a charity that raises money for Colchester NICU)

The staff all dressed up and there were pretty decorations around us. It was the best that they could have made the day an I actually found myself really enjoying Rosie's first Christmas. Even Santa came to see us!

I'm so thankful to the charities and NICU staff for doing all they could to make the day special.  

Birth story

I started losing my mucus plug at about 36 weeks bit by bit whenever I wiped after peeing. I didn't think too much of it as I know it can regenerate but I was happy my body seemed to be preparing for baby somehow.

I had a bloody show at 37w1d. I called my midwife team and they said that is was fine I didn't need to come in. I didn't need anti d or to come in be checked, she just advised me to keep an eye out on contractions or waters breaking. They were all suggestive it likely meant nothing and could be weeks until any labour happened.

Well... The next evening my waters broke. I had only just had my ELCS date given to me to be on 39w4s. I was worried that the scheduled date would be too late, as I was hearing people having it closer to bang on 39w to avoid the risk of going into labour. But everyone said I was silly for worrying about that as most FTM go closer to 41w before labour starts... Which was not the case for me.

I went to labour and delivery having constant gushes of water. Contractions started about 2 hours after my waters initially broke. I had steroid injections just in case baby girl needed help with her lungs at 37 weeks.

They were umming and erring about when to take me for the CS as doing it at night meant less staff and they also wanted to balance giving the steroid injection time to work. I wasn't making fast progress, so in the end it was a further 14 hours until I went in for my CS. It was quite traumatizing waiting that long not knowing when things would happen. I also hadn't expected to go through labour in any stage due to planning a CS so I did not have a plan to deal with the pain I was feeling.

Overall the CS surgery part was fine. The spinal tap part was the most painful part and it really felt like they were taking heir time to get it right. It felt like a very deep muscle pain and it was hard to not flinch via reflex. It took them a very long time to get it how they wanted it and they have you sit in an awkward hunched over position. I think mine took about 30 minutes in total until they were happy with it. The don't allow support people in with you whilst they put in the spinal tap - they can only come in after it is done - so I only had my midwife with me for support and she was fantastic.

During them tying to get the right placement I felt pins and needles in my leg and went to adjust it my legs position as I usual do when I get pins and needles. I got told off "DONT MOVE" and cue panic in me... Am I going to accidentally paralyze myself now? I start crying and having a panic attack, which obviously is hard to stay still during. My midwife was amazing and held my hands and let me squeeze her. She explained that pins and needles were good as the spinal tap was starting to work... (why couldn't I have been warned before i move and almost paralyze myself!)

Soon my lower body goes dead and its the most alarming feeling. I kept trying to test it to be sure that I would feel anything when they cut. So my brain would send signals to move my foot and it wouldn't. This made me panic its a feeling like I've never experienced. It made me feel so not in control. It was scary. But of course it was GOOD that I couldn't move as that way I wouldn't feel pain of the operation. Yet logic wasn't really getting in my mind at that point.

Next they put a cold spray down my side and I had to say whether I felt the cold or not. This is because the spinal tap numbs feeling of pain (which is similar to the feeling of cold) but not all sensations. So you can feel pressure etc but no pain. It's a very bizarre feeling. Once they were happy I was numbed from the waist down they began the operation.

Once she was delivered I knew something was wrong. They were supposed to drop the curtain and let me see her the first time as she was lifted out and my husband was supposed to be able to cut her cord.

That didn't happen. Instead alarm bells sounded which had me panicking. I asked my husband what was happening and he tried to white lie to calm me down saying it was just an ambulance we could overhear in the parking lot but I knew this wasn't the case. I could hear a team of people at the back mentioning the term "neonatal emergency" and calling for help. A bunch of people rushed in. I later learnt from her notes she was blue and floppy at birth and required rescue breathes. 

Eventually we heard a little cry. They were able to bring baby girl over to us to see but only for a moment before they whisked her away to the nicu. Before they took her away they said they saw a small hole in the roof of her mouth and that may be related to the cause. They also noted she has a little chin. 

I couldn't hold my baby despite carrying her for 9 months. It was awful. I didn't feel like I had a baby. It felt like she wasn't mine. It was so hard to be alone without my baby. 

Eventually the next day we found out that the "small hole" in her palate was actually very large of the soft and hard palate and her small chin was actually micrognathia. I started googling and found PRS online and thought that looked and sounded exactly like what our girl had... Obviously dr google shows worse case stories and horror stories so I panicked. It wasn't until the next day that our cleft team saw us and confirmed PRS. Because I had googled it the day before and saw all the worse case scenarios I just broke down at the confirmation she had that. It took a dew days to process and come to terms with everything. 

There was no indication of PRS on her scans. It was an extra shock as we had to have a second anatomy scan at 22 weeks purely for her lip and palate as she hid her face at the first one at 20 weeks and that looked clear! 

She was on CPAP for just over a day but was able to manage her own oxygen very quickly. She was out of the nicu incubator within a few days and in a standard cot as she could regulate her own temperature. So my little fighter did great. 

Overall, it's been such an adjustment and shock coming to terms with being nicu parents/having a child that will have a lot of extra medical care needs at least for the first year which we didn't expect. Imp also coming to terms with the trauma of her birth and the grief over not being able to experience breastfeeding with her. But despite that all I'm feeling blessed my little girl is here safely. My husband and I are so in love with her and so proud of all the progress she is making.

The nurses were able to bring her around to be delivery suite for me to hold her for 20 minutes. They had to bring a portable oxygen tank as she was still on CPAP at that point and she couldn't stay away from the NICU for too long. Holding her the first time was the most amazing feeling and it was so hard to let her go.

I was discharged 2 night post CS. I delivered my girl during COVID so technically partners were not supposed to be around after birth over night. However, the delivery staff at Colchester were amazing and accommodated us very well we stayed down in the delivery suites instead of the postnatal rooms as I didn't feel comfortable being around other parents who have their baby while I didn't have mine. This meant we had our own private room cubicle and they could wheel in an extra bed for my partner. I am so grateful to the delivery team at Colchester, I don't think I could have got through it without them and my partner.