Our Journey to Tubie Town

One of the topics for Tube Feeding Awareness Week is how we got here.

If you have read my blog at all in the last couple of months you probably know a bit about our journey thus far.  If you are new to this blog try THIS and THIS post or the CdLS page for a description of Kaylee's condition and how we got where we are today.  But I will start at the start.

When Kaylee was born I thought "Wow she is tiny.  Those are some funky little hairy patches on her thighs I wonder what that means.  She is beautiful.  How am I going to get this tiny baby to latch and feed?  How am I going to keep her warm?"

I am a long term breast feeder I guess, having fed 5 kids previous to Kaylee's birth.  In fact, for the first half of my pregnancy with her I was still feeding Kaylee's older brother.  Feeding my babies has always been one of the most pleasurable aspects of mothering a tiny baby for me.  I treasured those moments when I would be forced to slow down and savour their tiny perfection as they guzzled greedily at my breast.  For a couple of them, it required work and patience and time to get things working properly, but we always managed it and while I knew it would be a challenge with the tiny infant I had in my arms it didn't occur to me that we wouldn't have a "normal" feeding relationship - I just figured it would take some work.  After some rather horrid afterpains I was curled up in bed with a tiny bundle of baby carefully expressing clostrum into her mouth.  She lapped at it and opened her mouth but showed no signs of really wanting to attach.  After a while we trundled accross to the hospital where I thought they would tell me what kind of syndrome she had and I would take her home to figure out the feeding thing....little did I know.

The next weeks - perhaps months - was a complete blur of hospitals, transports, tubes, wires and a million other interventions and experiences which I do not really care to relive.  I know her feeding tube was placed sometime in there - perhaps during her first PICU stay the day after we flew in to Melbourne?  That would seem to make sense.  I expressed with dogged determination.  I remember the first time I managed to express a decent amount of milk.  It was the middle of the night and I was reading a message from my oldest daughter which read "Compared to my love for you the sky is like a dot".  I carefully screwed the lid on my milk and RAN to Kaylee and cried out to her "Look baby, I made you milk!!"  and he nurse very carefully started it going down her feeding tube and into her little tummy.  And thus began my love-hate relationship with feeding tubes. As I said a few days ago to another tubie mama - feeding tubes, love that they keep my kid alive, hate every other dang thing about them.

Kaylee was a few days old when we made it up to the cardio ward.  It had been a rough few days for both of us but she was finally stable.  With the help of Sue, one of the lactation consultants, I put Kaylee to the breast but I have to admit my expectations were very low.  I was completely blown away when she actually tried to feed!

It was a balancing act to put her to the breast to try and feed without exhausting her.  A combination of a cleft palate, heart condition, poor suck and swallow coordination and poor muscle tone and control all due to her syndrome made feeding very difficult.  Because she had the feeding tube, she was able to stay hydrated and well fed which gave her the best chance of learning how to feed for herself.  Without the feeding tube, Kaylee would not have survived her first week.  We did try her with a special bottle designed for children with special needs but she only got a few mls more than she did at the breast (8ml as opposed to 3 ml, and the bottle tended to try and drown her a bit which wasn't good) so I opted to keep trying at the breast instead because of all the other benefits of direct breast feeding and I had the option to do that because all her milk was getting into her anyway through her tube.

As I researched more and more about Cornelia de Lange Syndrome I learned that reflux  was a major life long issue for most people with the syndrome and I became very proactive in asking for her reflux to be assessed and managed with this in mind.  Because of the excellent care she received we had little trouble with reflux until after Kaylee's first cardio surgery.  Unfortunately after her shunt was placed Kaylee needed to go on a medication which interacted with her reflux medication.  She was still on a different medication but it was not as effective and Kaylee's reflux started messing with her heart stuff.  Just as we had started going to 3 hourly feeds rather than hourly or continuous - which was a big step toward "normal" - we had to place an NJ tube instead of her NG tube.  This is a tube which goes through her stomach and sits in her small intestine.  I love the NJ tube because without it Kaylee's heart condition would still be requiring her to be in hospital.  I love the NJ tube because without it Kaylee (whose reflux has gotten more and more severe) would probably vomit a large portion of what she was fed and would not be growing or putting on weight at the rate that she needs to so she can get ready for her surgery.  I love the NJ tube because it keeps my baby alive!  It gets my milk into her which has helped her stay reasonably healthy and grow delightful fat rolls.  It means I can give her medicine without worrying about her vomiting it up or spitting it out.  I can keep her well hydrated - a very important thing as dehydration could make her shut stop working.  But I hate it for every other reason.
 
The NJ tube needs to be placed by a radiologist so they can take pictures of where it is which means if it gets clogged or kinked we need to go to hospital - and if that happens after hours (which, for some reason, it almost always does) we need to stay overnight until they come in the next day.

The NJ tube means that Kaylee must be fed small amounts continually.  This means she does not get hungry - which SOUNDS good.  But learning what hunger means and that one needs to eat to stop feeling hungry is something that most babies do without us really thinking about it.  Right now Kaylee is not learning that lesson.  The cycle of hunger and feeding also means that a baby wakes up and interacts with either Mum or Dad and uses mouth muscles (the same muscles used for speaking, chewing, swallowing saliva etc. - all things that Kaylee was going to find challenging anyway with her other issues) several times a day - and in some cases several times a night!  The nerves that feel the sensations of hunger, being touched and held for a feed, milk being drunk, tummies getting full all send messages to the brain not only about food and eating but about where the baby's body is in space (sitting, lying, being touched etc) and the brain creates pathways with this information which we use every day for our whole lives without even thinking about it.  To help Kaylee develop these pathways I massage her gums, give her skin-to-skin contact, put expressed milk on her dummy so she can experience taste (she used to suck her dummy but since her reflux has worsened Kaylee has largely lost her sucking abilities, we will have to re-teach her to suck).  There are fabulous therapists who will help us with all of these things as we come to them.  So as with most big medical interventions, there is the good AND the bad.

After Kaylee's heart surgery we will start doing more with her feeding tube.  Trying out an NG which is a tube through her nose to her stomach (which I can learn to place at home, eliminating the need for quite so many hospital visits).  We may have a G tube placed which goes through her the wall of her tummy into her stomach and sometimes (in the case of a GJ) into her intestines which would eliminate the irritation of a tube in her nose and throat.  Perhaps we will be able to move to bolus feeds where we give feeds every few hours rather than continuously which would be wonderful.  There is no reason why Kaylee can't start to learn to eat "normally" as we get her other health issues sorted out - but it will take her a while.  In the mean time I will be able get all the breastmilk and medicines into her which she needs.

We have learned just how common the tubie experience is since we were introduced to this world.  It isn't just people who are as unwell as Miss K.  There are some people who look "normal" who lift their shirts and hook up a pump to their belly for a feed.  For most who have them a tube is a life improving - if not life giving - measure and the best chance they've got at getting well.  We have been blessed by the knowledge, support and information provided by other tubie families and support groups and it helps us make informed choices for Miss Kaylee, our little tulip.

So that's how we got here and this is where we are going for now.  If you ever find yourself in a position where you are having to manage a feeding tube there are some links to resources at the bottom of our Cornelia De Lange page

Of laughter, books and tubes

When Christopher laughs he has this belly laugh that is full of mischief and mirth.

When Andrew laughs it is the uninhbited laugh of a toddler and anyone in earshot is forced to smile, if not laugh too.

When Anna laughs it is like a tinkling bell.

When Erin laughs it sounds so grown up now days.

When Billy laughs his whole face breaks into a grin so big it's a wonder he doesn't fall apart.

When Kaylee laughs it cracks me up every single time - with mirth and delight.....but I have to explain to onlookers that she is, indeed, laughing.

She squinches her eyes shut and her mouth opens and the corners of her beautiful lips move sideways toward her ears.  Her cheeks bunch up, dotted with dimples.  And this noise comes from her that sounds a little like an adolescent goose or the large blue bird in this clip

Onlookers could be forgiven for thinking that she is crying or in pain but when you know her like I do, you know it is a honk of pure, unadulterated joy.  She did it this morning when I was kissing her hands and neck and it was like she was saying to me "Mummy, you are SO FUNNY!!!!!  Seriously Ma, have you thought about taking this act on stage?  You are HILARIOUS!!"

People are like books.

Some are best sellers with glossy, inviting covers and clear, accessable language.  A page turner that you can enjoy in a sitting then go back and read through slowly enjoying things you missed the first time around.

Others are like my collected works of Shakespeare.  The outside a bit banged up, the print a little too small, the language a little strange in places and requiring quite a few readings to even skim the surface of the stories within - but so, so worth the time and effort.

Kaylee, and so many people in the "special needs" category, are a whole genre of their own.  Imagine picking up a book and looking at the title only to find it is written in Russian.  Flipping through to find a conglomeration of Japanese Haiku, Ancient Greek chorus, Hebrew prose, Egyptian Hieroglyphics and beautiful Arabic calligraphy.  Intimidating.  Inaccessible.  Intriguing.  No one expert can explain it all.  She doesn't even share an alphabet with me.  I have to build my own Rosetta Stone so I can learn to read my girl.

Yesterday I was kneeling on the floor next to the wheelchair of a beautiful young girl with CdLS and I honestly did not know where to start "reading" her.  She is almost 12.  She has four words and a few signs.  But I dove in.  I asked her parents questions, I looked her in the eye and smiled when I wasn't sure what to say, I made an effort.  And each time we meet I will learn a little more, understand a little more.

I am learning that of anyone parents and carers understand kids with special needs best and usually are more than happy to help other people understand them too.  It is not the young child that barrels up and shouts "Hey, what's that thing in your baby's nose?" that offends - it is actually more hurtful when the embarrassed parent hushes the child and rushes them away.  While admittedly it gets tiring to answer the same questions over and over again - and there are moments when people let ignorance and stupidity out of their mouths without thinking - someone wanting to put the effort in to understand my complicated and intriguing little girl - even a little bit - makes me happy.

Parents, siblings, carers and friends act as bridges to help Kaylee and kids like Kaylee understand the world and be understood.

For this reason, I will be writing a series this week in honour of Feeding Tube Awareness Week to help others understand this surprisingly common aspect of the special needs world.

So they won't be frightened by the seeming complexity and and strangeness of a tube taped to someone's face or hooked up to someone's belly.

So they won't glance at the front cover and move quickly on to an easier read.

So others will learn to know when she's laughing - and laugh along too.

Still here at the RCH

In Kaylee's first two months we did three hospitals, five wards, three air transfers, one road transfer, three general anaesthetics, one scary-as-all-get-out open heart surgery (scary for me, the surgeons were magnificent and didn't even twitch) and by the time we got home I was SO READY TO BE HOME.

I do not love being in hospital.


I do, however, love the Royal Children's Hospital.

They moved to a brand spanking new hospital building last year on the 30th of November (kindly discharging Kaylee before the crazy of moving buildings) and it is the prettiest hospital I ever saw.

By far the best part - they have eliminated the walk-away wherever possible.

In the first two months of Kaylee's life I was walking away from her the majority of the day.  To go and get food, to express, to shower, to sleep.  Right now I am stretched out on the couch/bed in our room with the bathroom door just accross the room.  My belly is full of the meal they delivered to our room.  I do not have to leave this room at all. 

Along side this is the other brilliant thing.  I can go if I need to and know that she will be OK.  The monitor read out is not only in our room but also at the nurses desk so they can always see what she is up to.  Every single nurse that I have worked with is top quality.  I know that they will not only look after her body, but cluck and coo over her little person if required.  And I love them for it.

And Miss Kaylee loves it so much she is playing with us.

This morning we switched her O2 off for about the third time and she was doing brilliantly for hours.  So we took out her drip seeing as she no longer needs IV antibiotics and would not be transported with oxygen and a little while later we took off her prongs (which had not been giving her oxygen for several hours) and she started to de-saturate all over the place.

She is back on very low-flow oxygen and from here we just keep trying and seeing how she goes.  How long this takes is totally up to her.  We miss Jon and the kids, but I am taking this opportunity to rest and recharge.

And I am so very thankful for this place.

Boiling frogs and warm fires

It's 4:30 am and I am sitting up using my breast pump and watching Kaylee sleep. 


She is doing super well.

We have only had to suction her twice this shift and then only a little bit.  I am going to ask for her oxygen to be turned off to see how she copes with it - I think she will do fine without it.  Over all she is doing brilliantly.

But I am not sorry we came.

Truth be told I was getting very tired and she was dangerously sick.

At home I was giving 6 hourly meds, 8 hourly meds, daily meds, managing her feed pump, using the breast pump and suctioning her nose every few hours day and night.  I didn't realise just how insane it was trying to manage all that plus do the other things I was doing until I stopped doing it all.  Just like I didn't really realise just how awful Kaylee was starting to look until after she was on oxygen and started to look better!

There are two reasons things got as crazy as they did.

The first is, it happened slowly.  If you put a frog into hot water it will jump out but if you put it in cold water then slowly add heat, the frog will boil alive - not realising that the water is getting hotter and danger is imminent.  I think I was a bit of a boiling frog by the time I brought Kaylee in to hospital.  I was looking at photos I took a day or two before we came in and  I was startled at just how pale she was.

The second reason is, Kaylee is my sixth baby but I am still finding where the panic buttons are with her and working out when I need to call in help.  A bit of a snotty nose that you just keep an eye on with another baby, really needs to go to the doctor with Kaylee.  A bit of a cold you'd just take to the doctor with another baby is a trip to the hospital for us.  A hospital trip for another baby - a plane trip to the mainland for us.  I still find it totally crazy that a slight cold that would have been a mild inconvenience for my other kids would have killed Kaylee if we hadn't come in for oxygen and treatment.

And it is further evidence that we are indeed in a whole new world with Kaylee.

Sometimes well meaning people try and reassure me that after Kaylee's heart surgery she will by "right as rain"  - but the truth is she won't be.  Her heart surgery is a huge hurdle and getting over that will free us up to address some of Kaylee's other issues - but on a day to day basis the biggest change initially will be two less medications to give.  And that's it.  Kaylee's feeding stuff, reflux stuff, cleft stuff, developmental stuff and all her other stuff will still be there and still need dealing with.  This isn't just a long term thing - for Kaylee, and for us, this is a life long thing.

Usually when your kids get sick you know they will get better.  They get chicken pox and as they whine and itch through the rash you know that in two or three weeks time they will have a few scars and childhood memories of watching TV on the couch.  They get appendicitis and usually, after the initial trauma of hospital and surgery, there is an expected recovery time and a life afterwards with only a scar for a souvenir.  With Kaylee she will not get better.  It is like a fair ground game where we use mallets to hit the gophers that pop out of the holes. 

I honestly do not know how people walk this road without a faith.

Because I believe, truly and deeply, that my daughter will be miraculously healed.  There will come a day that she runs and jump like other children.  There will come a time when she will have a voice to speak her mind.  There will come a time when she will bite into the fruit of the tree of life and use the leaves that are for the healing of nations.

But right now God is using this time and all the evil and horribleness that goes along with this world, to sand back the rough edges of my character.  He is blessing us and giving us what we need even while Very Bad Things happen.  And the good things don't make the bad things better, the bad things are still very bad.  But the bad things only make the good things sweeter.

And HOPE for the future is like a fire by which I warm myself on a cold night.

Kaylee's Fund

Spending time in hospital has given me a bit of thinking time.

Last December I blogged about how people have been wanting to be generous but I had conflicting feelings about accepting the generosity.  See, I would like to take advantage of everyone but I don't want to take advantage of anyone...if you get my drift!

You see, there is something very wrong feeling about someone walking past the new scanner/printer we bought to give us a box of groceries or money that had been donated for needy families.  It just feels dishonest somehow.

However, while we have no specific needs right now, down the track Kaylee's needs will probably get expensive with feed pumps and other speciality items we may need to get for her.  We would like to keep our family together as much as possible which means finding money to transport and accommodate Jon and the kids when we need to go to hospital in Melbourne and Hobart.  And we will need to finish our renovations and move house sooner than we originally planned so we may need to employ people either to help out around the house so Jon can get into the DIY or to do some more of the renovating than we were originally planning to get other people to do.

And we have discovered over the past five months that people are generally brilliant, generous and extremely lovable and they DO want to help us out in any way they can.

So here's my plan.

On our "Prayer and support requests" page I put a donation button like this:

and I have an account put aside for Kaylee related expenses so people can donate to us via paypal.  If you know us personally and would prefer to give us money in person or via post that's fine too.  This way I know that we are accepting help from people who understand our situation, not people who are trying to help out destitute families!  We will keep this money aside and use it as needs crop up.

I do thank all those who have helped us out over the last five months.  I am awed and humbled to have such wonderful people in my life.  Kaylee has been treasured by so many people and we feel privileged to be able to help her touch the lives of so many in such positive ways.

The Verdict

We are pretty sure Kaylee aspirated - inhaled some vomit and/or snot - which caused this lovely little infection.  It is possible that she did pick up a low grade upper respiratory infection (that is COLD to those of you who haven't had your minds warped by too much time in hospitals) before or after this aspiration, we can't really tell for sure.  The tests from her nose came back negative in spite of the copious quantities of thick, creamy SNOT we are suctioning out of there several times a day (hope you aren't eating dinner as you read this....or vanilla slice) but it is possible there was a virus in there still, just not one of the ones they tested for.

So, as usual, we know a bit - not everything - but enough to keep going.

The plan now is to get her lungs in tip top condition before her heart repair. 

To do this she needs to spend a little bit more time on oxygen and with IV drugs but we may be transferred back to Launceston kids ward for this.  I do not get as much rest there because I won't let the nurses touch her feed pump without direct supervision - the first night I was there I went to sleep and woke up to find 300ml of my breastmilk hanging in the feed bag when she was being fed at a rate of 21 ml per hour.  It is only meant to hang for a limited amount of time and nobody could tell me how or why or WHEN this happened so I had to discard 300 ml of breastmilk.  Not happy Jan.  I also had to discard another 500ml when I got there because it took me over an hour and a half to get someone to put it in the fridge - by which time it had been out of the fridge for over five hours and become useless.
*sigh*  I also have to chase almost every single medicine because they forget or get busy.  Last time we were there I had to record all of Kaylee's de-saturations by hand as the monitors there do not record and there was not enough staff for someone to sit and write down the numbers.  I'm afraid I was very spoilt with nurses trained for ICU or high dependency work and it takes some getting used to working with a ward that is as understaffed as that one and does not have a good understanding of Kaylee's condition or how to handle EBM and continuous feeds.

We will see how we go.

Kaylee had not had reflux since we settled in here and I decided to test a theory.  I sat her up this afternoon.  She loved it for the first five minutes and I got SMILES and COOS and LAUGHS!!!  And then bang, the reflux hit.  I don't know if it was simply being moved that brought it on or sitting up making her tube press the valve on her stomach and letting acid out.  Either one is a decent theory.  I don't know what to do with that one.  I can't just not move her for a few weeks! 

The other kids are all having fun with Nanny and Poppy and Dad.  They miss me but this is not as traumatic as the first time I came across unexpectedly.  Kaylee is not as sick and they are in Smithton .

It has been nice to catch up with some of my lovely Melbourne nurses.  I keep showing them photos of one of our old room mates who I have kept in touch with and they get very excited.  It is rare to be in a place where SO many people are brilliant at their job and genuinely seem to be dedicated to their work. I consider myself truly blessed.

The new hospital itself is amazing and I can't wait to bring the kids over here.  I am sleeping in with Kaylee COMFORTABLY!  How often can a parent say they are sleeping on a hospital ward comfortably?!  The whole place is designed beautifully.  I am comfortable and cool (until I go outside where it is humid and hot!) and the room is flooded with natural light in the day.  All over the hospital there are little places to sit and watch the kids play.  It is a truly wonderful place for sick kids and their parents.  If I could change one thing I would make the parents retreat room larger because there are times when your kid in ICU when you need to wait in there and if there are some large families in there, it would get crowded and uncomfortable.  But other than that, I really do love this place even if I do not love the reasons we are here.

Anyway, tonight I will be good.  I am going to bed EARLY.

I will update as I get information.

Thanks all for your prayers and encouragement!

Where things lie..

So it is presently looking like our biggest issue is, wait for it, SNOT.

When we got in last night a clot in the shunt (the most concerning scenario) was ruled out by various tests.  I have a feeling that if I had been listened to when I told everyone that it was OK if her sats went up to 100 and Kaylee's suctioning needs had been attended to a little bit more diligently when I asked in Launceston and perhaps we had tried a few snot busting tricks that we are going to try now THERE, we would still be in Tassie.  But unfortunately we could not get intensive nursing over in Launceston due to low staffing levels *cough cough* and people not always doing what I tell them when I tell them *cough cough*.

But to be honest, I was ready to be here where the more intensive nursing is available and I can rest and I have been very concerned about Kaylee's general health for a while.  It is very hard to distinguish between reflux symptoms and cardiac symptoms and I'm-holding-my-breath-because-I-hate-what-just-happened symptoms and add in a slight cold - I am happy to have monitors to give me a little more insight.  She may well be outgrowing her shunt and her full repair was imminent anyway.

So where do we go from here?  Possibly home to Tassie to get well then come back for surgery.  Possibly stay here and get well for surgery.  Right now, I really don't know.

Hopefully tomorrow after rounds I will know more.

Coming to you live from RCH

Kaylee decided that she wanted to further check out this fabulous place of 2 story aquariums and fabulous, new shiny rooms.

On Wednesday she had a big chuck and stopped breathing for long enough to cause me to say "Um, let's go to the hospital."

She had several "de-sats" (episodes of the oxygen levels in her blood going down) some of which were low enough and long enough for the magnificent Rob (our cardio dude) to want to see us in person again.  So we flew out tonight, Kaylee's air ambulance taking off the same time as my commercial flight in wonderful and quirky answer to my prayer that we be able to fly together.

I am waiting in the spunky new parents room while they settle Kaylee on "Rosella Ward" and looking out the window to an amazing playground.

I feel good that we are here.  It was time.  She has been steadily going down hill for the last few weeks and it is too long since I saw my baby smile.  We need intensive nursing right now as I haven't slept for a few days doing the 24 hour shift.

Jon and the kids are with my lovely in-laws.  From here we could end up on monitoring for a few days then home again, surgery or monitoring until surgery.

I am not totally sure what to pray for right now.  Wisdom, discernment and compassion in our fantastic medical staff.  Love and protection for myself and our little family.  Protection from infection for Kaylee and peace during invasive and/or painful procedures.

I didn't get to pack like I would have chosen to, but I do have most of what I need this time.  I am not sure what I want in terms of accommodation yet until I know what is happening with Kaylee.  My phone died and I got a new one - but didn't get a chance to transfer numbers.  So if you have my mobile number, SMS me your number so  I can contact you!

Right now things are a bit up in the air so we won't be taking any visitors for a bit.