Friday, October 12, 2012

Where are we now...

I have been considering doing a post like this since Kaylee's birthday six weeks or so ago but have only now had time.  Well, not so much had time as made time.  And today is another anniversary.  A year ago today Kaylee almost died.  She was revived by the Medical Emergency Team.  She was navy blue.  I was too dazed to be scared.  The support we got through this blog when Kaylee was small was amazing and I can never thank my readers enough for the way they supported us.  In the end, maintaining the blog was overwhelming and when I started to have people write to me privately to tell me I shouldn't think or write certain things, when people told me I had an "obligation" to my readers to blog regularly and when people in our real life began to be offended by what I wrote, I knew it was time to step away from the blog.  It was a small minority, but for me this was enough for me to say "enough".  I will not be returning to blogging publicly about our lives however I do feel that I need to wrap things up a little better.  This Blog still gets visited in dribs and drabs - mostly by people googling Cornelia De Lange - and I know that some of our regular readers are curious.



She is downright ADORABLE.  Seriously, this kid is cute as a button.  Healthwise and development wise, as is Kaylee's style, she is complicated but today I would say "doing well".

Heart:  When her heart was repaired in March the surgery went very well.  Unfortunately, the valve on the pulmonary artery (the artery going from her heart to her lungs) had to be sacrificed so at the moment about 50% of the blood that gets pumped out of her heart toward her lungs actually refluxes back into her heart, needing to be double pumped.  Because of this, her heart is bigger than other children her size and/or age and as she grows it is likely that her heart will start to struggle to cope with this extra load.  At some point, and nobody can say if it is 2, 5, 10 or 15 years away, Kaylee will need to have her heart valve replaced which means another open heart surgery.  Unless there are some pretty big advances in technology in this time, Kaylee's valve will then need to be replaced every ten to fifteen years for the rest of her life.  Right now, however, it is doing great.  In fact, during a nasty chest infection earlier this year Kaylee's O2 levels were so good she didn't even need extra oxygen!

Stomach/feeding:  Kaylee's Fundoplication and PEG insertion was largely a success.  Kaylee no longer has reflux as severely and tolerates having food in her stomach.  I feed Kaylee mostly via her "special tummy button" which I connect a tube to and either syringe feed her or connect a feed pump.  Most days she has a go at eating orally - by which I mean I offer her some tastes of yoghurt or something on a spoon and/or some formula in a special sippy thingy.  Sometimes she takes a bit, sometimes she takes a lot (as in, 3 teaspoons or 20 mls), sometimes she is not interested, sometimes she puts it in her mouth, plays with it and then spits it out!  Some people ask me if she will ever eat "normally" or be able to get rid of the button.  The answer truly is maybe - but maybe not.  A side effect of the surgery is she cannot burp so she currently needs to be "burped" with me connecting a tube to her tum then using a 30ml syringe to release bubbles.  In fact, as I type now she is sitting on my knee and I am holding the syringe in my teeth.  If I do not do this when she needs it she will be in a whole lot of pain and also vomit - yes, she can't burp but she can vomit past the fundo!  Talented girl this one.  It is very likely that her stomach surgery will need to be repeated in the future which we knew before hand.  I don't like this as it is a very painful procedure (I would say more painful than her heart surgeries) but it is better than living with the daily pain of reflux and the risk that would pose to her lungs.  I am praying it will be a while off before we need to worry about this anyway.

Cleft:  Kaylee's soft palate cleft is not really a hassle for us right now.  We had a chat with the plastic surgeon and we all agreed that Kaylee has been through enough for now.  We are all tired and we are taking a break for a while from surgeries.  We will re-evaluate where we stand with this in six months.

Things to Watch:  Kaylee has something I like to call "funky monkey feet".  Her toes are curled and her instep curves her foot around suggesting that perhaps some tendons are a bit tighter than they should be.  We are still waiting for assessments to come in on this but our plan for now is to massage often, watch and wait.  When she starts to stand or walk we may look at something to strap on her little feet.    Kaylee's eyelids droop to the point that she sometimes has to tilt her head back to see things in front of her at eye level, so we will need this assessed too, to evaluate the effect on her vision and decide if an eye-lid lift is on the cards.  I kind of hope not.  Kaylee's kidneys have always been a little kooky (small and with cysts) but they are functioning normally.  We probably wouldn't even know about them if she hadn't had so many ultrasounds etc with her heart.  None the less, they are on the "things to watch list".

Milestones:  I have a love/hate relationship with milestones.  I love it when we get there, I love supporting Kaylee to get there - I hate it when people ask if she is doing things that are so not even on the horizon yet!  I suppose developmentally Kaylee is at about the stage of a four month old however she does not support any of her own weight on her legs when you hold her up, she kind of hangs there as if to say "yeah right!  Not today!".  She rolls from side to side from her back and somehow (nobody can work out how) manages to migrate around the floor by doing this but as far as we can work out Kaylee has no idea where she is going, she is just rocking and rollin'!  She can sit for short periods in a bumbo chair.  She can reach out and grasp things and usually shoves them in her mouth (especially if it is her feeding tube which she loves to chew, much to the annoyance of her mother!).  She has a brilliant smile and a unique laugh which is a bit of a gravelly honk.  She kind of grunts and honks and "sings" sometimes too.  She is all gums still which isn't unusual for kids with Cornelia De Lange.

Goals:   Right now we are working on helping Kaylee learn to sit, encouraging her roll, working on her upper body strength (she can't hold her self up with her hands if placed on her tummy leaving her in an uncomfortable face plant position!) and helping her to learn to eat something orally.  

Will she ever... When these words leave people's mouths about Kaylee, I never know what to answer.  The future is a big bundle of unknown.  Things could go super well.  She could need minimal surgeries in the future, her development could all of a sudden speed up and, if not catch up with her peers at least keep her somewhere in the ball park, she could suddenly start talking or walking or eating and doing a million other things.  Or she could start having seizures, aspirate, her lungs or heart could become compromised - Very Bad Things could happen.  Likely, the future will be somewhere in the middle of that.  With Kaylee, it is a matter of climb on for the ride.

The Other Kids:  Are brilliant.  They are amazing.  I am prouder of them every day.     

Me:  So how am I?  Honestly, most days I am trying to work that out still.  I look back at this blog sometimes to see what we were doing this time last year and it is almost surreal.  To be honest, I think I sugar coated a lot of things when I blogged without meaning to.  Frankly, it was hard to admit to myself how bad it was.  It really was harder than it looked, there was less support than I let on and it was scarier and more exhausting than I could ever convey.  I am happy and content now for the most part and in many ways I have a greater acceptance of Kaylee's diagnosis than many of the people who love Kaylee but don't live with her, because I understand those intangible bits of wonderful that she brings so much better than they do - I see the wonderful every day.

But I have been angry a lot of the past year too - and not always justifiably.  I have been angry at the loss of autonomy we have endured, being forced to make impossible choices and let go of dreams and plans that we had.  I have been angry at people whose own internal dramas and pain have caused them to make our lives - already impossibly difficult - harder.  I have been angry at people who have been all too willing to spout words on the internet but when it was possible to visit us or help us in a practical way, they were all of a sudden too busy.  I have been angry at people who tried to help with the best of intentions but because they did not first listen or ask, actually made our lives more difficult or placed us in an awkward position.  I have been angry at people who tried to tell us what to do or how to manage our lives.  I have been angry at people who simply stopped showing an interest in our lives once the "drama" stopped being blogged for their convenience on the internet.  I was angry at my own limitations.  Sometimes, I was just angry because being angry is easier than being scared, sad and hurting.  Sometimes there has been a big, complex ball of emotion inside me and I have not been able to separate anger from joy, happiness from sadness, frustration from relief - or anything else.  At times my anger and frustration has spilled out toward good people with good intentions who made innocent mistakes or whose good intentions did not work out.  I have done damage to relationships and have a lot of rebuilding to do.

My patience and reserves have been low.  I have found it hard at times to relate to my "ordinary" friends.  More than once I hung up from talking to someone about their breastfeeding trials and had a cry, remembering the hope I had that first time Kaylee tried to go on the breast, the ache I had as I cried for fifteen minutes before expressing  when she was six months old because I just didn't want to do it any more and the gut wrenching grief I experienced as I made the (right) decision to stop expressing when Kaylee was eight and a half months old and I was beyond exhausted....and knowing that this may well have been the conclusion to my journey of feeding my babies.  I do find it harder to be generous with myself to friends and family these days.  While Kaylee is healthy right now, her care is still demanding and exhausting and constant.  Several friends I have an agreement with that if I am not in a place to hear their tales of ordinary trials and triumphs, I just need to say and we will connect again later.  I am thankful for friends like this.

I get awfully frustrated with people who call me 'inspirational' and tell me they could never do what I do.  Firstly, I don't need those kind of expectations to live up to.  I don't find it encouraging, even though it is well meant.  Secondly, I find that people often say these things as a way to distance themselves.  Someone who is "inspirational" doesn't need help or encouragement or friendship or a listening ear.  Thirdly, some people seem to use this as an excuse to under achieve in their own life.  I have pushed myself to the limits of my endurance in the past year or so and let me tell you, they are further than you think.  It isn't because I was strong or special, it was because I simply got off my backside and did it.  I believe most people are stronger and more capable than they realise if only they get up and work at it.  Most of the time I take it as intended and realise people are trying to be encouraging but there is more than one person who I have had to simply ask to stop saying it.  One or two haven't listened, but for the most part people are indulgent if not understanding.

I have learned a lot about listening.  The most helpful to me have been the friends who simply asked then listened.  I have been blessed beyond measure by these people.  There are so many who just keep talking and talking to fill up any silence, so many who surged ahead to push assistance on me regardless of my feelings toward that assistance or how helpful it would actually be.  The golden few who truly served as balm to my soul simply listened.  At times prattled about their own lives and shared their beautiful ordinary and frustratingly ordinary with me - but when I needed it, just listened.  I hope I can listen like that to my friends when they need me.

Managing our medical and therapy staff has left me wishing I had about six different degrees and x-ray vision.  Turning up to a hospital means I will have to educate people about Kaylee's syndrome and condition which I don't mind terribly (I love med students and usually get at least half a dozen through if we are admitted - last time I had four in Emergency alone!) but there are days when it is hard.  Because it is not just the students that don't know about Kaylee's condition, it is the nurses and the interns and the registrars and the consultants.   The truth is, I have no access to any expert who knows everything there is to know about Kaylee's condition.  I can access heart specialists, plastics specialists, physio specialists, CdLS specialists and a million other specialists - however all of them are specialists in one aspect of Kaylee, not all of Kaylee - and all of them are very busy people.  I am constantly on duty if she is unwell and I have found myself having to second guess and question some very clever people simply because I know Kaylee better than they do.  Fighting red tape is a full time job.  Everywhere is under funded.  Because it wins more votes to launch a new program rather than improve funding for an old one, there are about a million different programs that could contribute to Kaylee's care and all of them are under funded and many have waiting lists that are hugely long.  However I have met some brilliant people with amazing knowledge and generosity of spirit and I thank God every day for so many of the nurses, doctors and specialists we work with.

Support groups are great, but the flip side is we always know of someone who is sick or fighting for their life and I have written more letters and messages of condolence in the last year than I ever have before - for beautiful, bright young people who have held a place in my heart.  But there are some things that nobody except parents of kids with special needs will understand.  They are in many ways a part of our family now.

We are slowly finding our new normal.  We have dreams of camping this summer.  We have some dear friends who drop us off a meal or two every now and then - which was a life saver recently when we were all taken out by an awful bug, Jon and I hit worst of all (Kaylee managed to avoid it by being on hefty doses of antibiotics already due to her chest infection!).  We have also hired  a friend to help keep us up to date with the laundry and cleaning once a fortnight which is so helpful.  Being that most of our family and a great many of our friends live far away there is little in the way of practical help for us so the ministry of this family is invaluable to us.

There are days when things hit me.  Earlier in the year Kaylee had her heart check up.  It was largely a tale of "nothing new, we will see you in 6 months" however it all of a sudden occurred to me that Kaylee will likely need heart surgery when she is an adult, and I am old.  A million "what ifs" could hatch out of that.  What if she doesn't understand?  Will she be as frightened as a little girl but in a woman's body?  If I am too old, who will advocate for her?  Take care of her?  There are a million what ifs and I could drive myself crazy if I try and travel each road before we take that turn.

Most days I soak myself in dimples.  In crazy hair and impossibly long eye lashes.  I watch her siblings play with her and dote on her.  And I realise that my blessings are really without number.  I live for each moment.

Yesterday was what it was, tomorrow will be what it will be but today is good.


Bek said...

No comment. ;)

Liz P said...

Thanks honey for this lovely update. Kaylee is gorgeous! I've wanted to ask so many questions but I don't want to burden you with having to explain yet again about Kaylee. Wish I could help but being almost 200kms away and with a newborn there's not much I can do. I think about you often and pray for you sometimes (not so good at that, sorry). Loved hearing about all your kids and miss it now you're not blogging, but respect your right to privacy and your right to not blog!

Momma Bug said...

My heart was hungry for that picture Jess! Tulip she is, and she is beautiful! Of course I knew that without seeing her, but it makes me feel a little closer.

Loving you,


Seaweed and Raine said...

Smooshy, yummy bundle of adorableness is what that photo conveys to me. :) (not all that you see, I would guess, but it just makes me want to pick her up and cuddle her and give her a big smooch on the cheek!)
This post I am guessing took a lot of heart moments. And I love you even more for conveying them and sharing them. I don't often have the words. You are right... listening might be the better option ;) (Not an easy thing for me! LOL).
Hugging you from afar.
S x0x

Vanessa - SA said...

thanks so much for the update Jess. I was wondering what was going on with your 'lot'.

LOVE the pic of Kaylee, she is a gorgeous baby.

Ness xx

Emma said...

As a far away blog reader who doesn't know you in real life, thank you for posting this update. I really appreciate you sharing your thoughts and feelings, and am glad to hear more about you and your family. Best, Emma

Leisa said...

Thanks for your update Jess. I don't have the words to say, but your post was so raw and honest. Thank you for letting us into your world. Kaylee is so so sweet.

Hoping for good days.