Hello everyone, here's an update of our ongoing PRS journey. I haven't logged on and written a story in a while, thats because truthfully not many medical interventions have taken place, see Summer's main medicine was growth. Her airway needs to grow enough to support itself without needing airway support & tracheal tubes.

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Summer is 1 of 50 babies born in the UK a year diagnosed with PRS. As soon as she was born she needed breathing and tube feeding support immediately.

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We were told during a visit to our local hospital that Summer actually has a grade 4 airway in comparison to other babies who are born with a grade 1 (normal size) airway.

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Summer is now fast approaching 11 months old, and up until recently still had both of her tubes, nasopharyngeal airway & a nasal gastric tube.

In a sleep study in early December at Great Ormond Street Hospital the consultants confirmed that Summer can breathe on her own during the day almost perfectly, however night time held more challenges as her tongue is still slipping back over her airway and closing it, causing her to obstruct.

A little disappointed in the sleep studies results we still had a fantastic Christmas and New Year, as a family stronger than ever before determined and ready for what 2018 had brewing for us. 

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At almost 11 months old Summer is still Nil By Mouth. She is fed through her nasal gastric tube 5 times a day at around 220ml a feed. In early January Summer started projectile vommiting during and after most of her feeds, concerned she'd lose weight I contacted our dietician who suggested we introduce a feeding pump.

After long consideration and a battle of acceptance towards the pump we agreed to have it in our lives.

A feeding nurse was sent out to us to teach us how it works and how to set it up and what to do if certain alarms go off on it etc. Finally we quickly reaped the rewards of having the pump in our lives.

We set the amount of the feed and set the time we want it to run over, run the milk through the tube making sure theres no air bubbles then press start. The longer the feed goes on the slower it goes through the tube, through Summer's feeding tube and into her belly, the slower the better as theres less chance she'll be sick.

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More recently I noticed something with Summer's breathing tube. As she was being fed she was nudging her tube (that sits at the end of her tongue) forward through her nose. It was so weird. Assuming it was a one off I didn't think much of it, until it happened again the next feed. I phoned the cleft nurse at GOSH and told her, she assured me that it was Summer exploring her tubes and pushing her boundaries to explore her limits with pushing the tube out, we hung up the phone. Later that day I was telling Paul, we were then worried the tube was not long enough and not doing its job properly. I also truly believe in listening to your babies as if they're trying to tell you something. The next day I phoned GOSH again, this time talking to another cleft nurse. She suggested Summer could have potentially outgrown the tube 'i'll contact the respiratory consultant immediately and call you straight back' she said. I anxiously waited as my phone buzzed.. 'The respiratory Consultant says if you feel confident enough, remove her breathing tube but monitor her closely, keep her on a sats machine overnight - if her oxygen and heart rate dips refit another tube and come up to the ward for a resize immediately' *WOW* Obviously we were over the moon at this point, its a milestone we've been longing to reach but shit have I just put my baby at high risk? Am I pushing her too much?  What if she's not ready? Am I just asking for an emergency to happen? All these thoughts went through my head. Maybe I should have just kept quiet until our appointment a few weeks after this call was made.

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Saturday 3rd March 2018. There was heavy snow on the ground and it was freezing outside, Paul and I decided to removed Summer's npa - breathing tube. We attached the sats monitor to her big toe on her right foot, her sats were fantastic! She was happy immediately. PHEW! 

We enjoyed the entire day one tube down, Summer was so happy and so were we, she kept rubbing her cheek and smiling. 8pm arrived and it was time for bed, we'd taken lots of photos, we'd bathed her, we done lots of things incase the tube had to go back in during the night. It was a long night for us as parents. It was indeed the first night Summer slept 12 hours without any breathing support. Tears of joy hit me hard Sunday morning. Summer has had the npa longer than any other PRS baby I've met.

By her nudging it I believe she was telling us she was ready. Ready to breathe all by herself.

Paul & I trusted our instincts and made the choice together to see if she was ready like she was trying to tell us she was.

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At almost 11 months we are one tube down. Summer has another sleep study in a week, this will determine if the tube can stay out and if she's ok during the night, we measure her sats but GOSH do more in-depth tests.

 

Our girl, finally we can kiss your cheek and properly see your nose. 

Since having her breathing tube out Summer has learnt how to belly laugh. She is such a happy baby. We say 'Summer wheres your tube gone?' She laughs her head off pointing at her nose.

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Once we get the go ahead from GOSH about her airway being strong enough to support itself and that she has retained a safe swallow, we can then restart oral feeding alongside our Speech and Language therapist. I can't wait to wean and try different recipes. Summer will have her feeding tube for a little longer but theres no real rush, again we will trust when she's ready she will simply let us know.

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Our next challenge is to learn how to fit the NG feeding tube as now she is at an age where she pulls at it, it will save time travelling up to a hospital. 

Especially as our last change was traumatic. We took Summer swimming which she loved, she accidentally caught her tube which was fine these things happen, I phoned our community nurses but all were busy so was asked to bring her up to Barnet. We were met with a very nervous nurse which immediately made me nervous.

The nurse seemed to struggle fitting the tube, it seemed a little forced when usually it should just slide down. Summer does resist with her body but I usually hold her still and keep her calm while the nurse fits it. Once the tube was in the nurse tested it to make sure it was in her tummy, when she aspirated the tube she pulled back pure blood, tears filled my eyes. What the hell have you done to my baby? Up to two hours after the tube was fitted summer was bringing up blood in her vomit.

 

We pledge to learn how to pass a tube from our daughters nose to her tummy by the end of April.. wish us luck!

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Most recent lesson learnt, theres no manual to parenting. Simply trust your instincts.. your babies will let you know!

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Thanks for reading Trust Your Instincts...

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Jessica  x