Just Breathe

I'm not sure what words will describe how I'm feeling right now, but I did feel the need to get all this out, even if it is extremely personal.  Actually, I do know what words will work.  I just can't repeat them in polite company.

I have found it harder and harder to hold on to my emotions these past few weeks.  I'm sure Jules can see the worry in my eyes whenever he gets out of breath, despite my best intentions to hide it from him.  I'm trying desperately to stay calm when he collapses to the floor, resorting to pathetic jokes about how out of shape I am while I put my arms around him from behind and help him to stand up.

When I think about what's coming up, my heart races.  I feel sick.  My hands tremble.  So does my chin.  Hell, my whole body does.  My throat gets tight and starts to ache.  But I grit my teeth and swallow it all back down.

Because Will and I only cry when Julian's gone to bed.  He's not supposed to be our rock, and he's not supposed to see our tears.  We can't put that responsibility on him.

Dammit.  It's not fair.

A few months ago, while Julian & Will were in New Zealand - thanks to the generosity of a number of people - we found out that there were a few locals getting quite nasty about us because we had started a GoFundMe page for Julian to travel to New Zealand.  To fulfill his big dream before starting 'Veletri'.  One of the things that was said was that we "always had our hands out, just because we had a 'sick' child".  There's a long story behind that.  I won't bore you with the details though.

If only.  If only he was just 'sick'.  I would do anything for just 'sick', instead of terminal 'terminal' and 'incurable'.

You know, on a day to day basis I can usually ignore the fact that my son is dying, bit by bit.  One heartbeat at a time.  Most days, it's easy.  Julian looks 'fine'.  He's got great colour.  He growing so tall, and his feet are bigger than his Dad's!  He's funny, he's a ratbag, he's so much fun to be around.

And then, out of nowhere, he goes pale.  He struggles to breath.  His heart pounds so hard that it vibrates the sleeve of my own shirt when I put my hand on his chest to check his heart rate.  His heart races so fast I can't keep count of the beats.  He collapses to the floor and can't get up without help.

He wakes up out of breath.

I'm just happy he wakes up.

Jules has gone purple!

 

Why?  Because it's Pulmonary Hypertension Awareness Month!

11.5 years ago, my husband and I were told this strapping young man (who is turning 15 next week) only had between 2 - 5 years to live when he was diagnosed with IPAH at just 3.5 years old.  He continues to fight with everything he has, so what else can we do as his parents but fight with and for him too?  Julian is an awesome - although somewhat irritating at times - big brother to his 2 little brothers and his little sister. 

He is a ratbag, he is hilarious, and he - like every other teenager his age - is trying to figure out where he stands in this world. 

He is my hero.




Next Thursday, on the 26th November, he will be going in to the Lady Cilento Children's Hospital to have a PICC (Peripherally Inserted Central Catheter) line put in.  He'll be coming home that day so he can spend his birthday - 27th of November - at home.  On the 30th November, he will be admitted to the LCCH for 2 - 2.5 weeks to go on a medication called 'Veletri', which is administered through the PICC line via a 24hour pump.  My husband and I have to learn how to mix his medication, change the medication cassettes, change lines, change dressings, not to mention learn and understand all about this new medication.  Can you believe we have to buy a cupboard to put all his medication, lines, dressings, etc in????  We will be away from our other 3 children for this time, and my MIL will be staying with them so they can finish off their school year without too much disruption.  Thankfully, they go to an awesome primary school with awesome staff and teachers!

So, can I ask you all to 'Like' & 'Share' my Facebook page - Saving Jules​ - to help us to raise awareness of this terrible condition.  Follow Julian's journey with IPAH and have a bit of a laugh at his antics.  You'll be amazed at how he faces each day.

Straw Challenge

I read about this great idea to help people understand how Julian feels each day, so I would like to present a challenge to you all!

I would like you to take a straw and breathe through it for a few minutes. You might think, oh that's easy! But it's not. Keep going. Little bit more. Little bit more. Are you starting to struggle? Are you breathless, do you feel an ache starting in your chest, even your stomach?

This is how Julian, and other PH, PAH & IPAH sufferers feel. Every. Single. Day. No break.

Now, take the straw out of your mouth. Feel a bit of relief? Unfortunately, Julian never gets to feel that, never gets a break from breathlessness. It's just a matter of how breathless he is, not if he is or isn't.

Let me know how you go!

Brave Warrior

12 months ago, the world lost a star that shone so bright that in the time we all took to shelter our eyes from his brilliance, he was called home to the Angels.

On the 19th September 2014, at just 4 years of age, little Talon lost his battle with Idiopathic Pulmonary Arterial Hypertension.

I am sitting here in my lounge room, trying desperate to think of the right words to honour his memory, and it's hard.  How can I explain to you all the connection I saw between Julian and Talon?  How proud I felt watching my own 14 year old son finally find his own personal hero in a little boy 10 years younger than him?

Should I tell you about the first time I attempted to chat with this shy little human, and how I had to resort to bribery in the form of chocolate brownies?  Should I tell you how he looked at his Mum and Dad as if to say "Who on earth is this woman?  And can I have a brownie?"

Should I tell you about the too short amount of time Jules and Talon visited in the hospital, when they were both admitted on the same ward?  And how Talon finally came out of his shell when Julian found a way to connect with him?  Card tricks.  Clearly my brownies stood no chance again the magic of my own son!

Should I tell you how Julian's attention transformed from a shy little boy to a chatterbox?  How I felt a lump in my throat when he started to show me his fire truck?  How proud I was as a mother when I watched Julian teach him how to grit his teeth and growl "Just do it!" when the nurses had to do a blood test?  The connection I felt with his own Mum, while we watched our soldiers, both big and small, grinning at each other with the same glee only boys can grin with?  You know that cheeky grin I'm talking about.

Should I tell you how Talon's favourite nurse is Julian's favourite nurse?

Or should I tell you how my chest went cold and my heart stopped for a while, only to start pounding so hard I could hear it's echo in my ears when I read the devastating news that Talon had lost his battle, that his too small body had let go of this life?

I'm finding it so hard to find the right words to tell you all how it made me feel to watch him, in that short amount of time, fight with all his heart.  He was such a brave boy and an enormous inspiration to Julian, who said to me when we left the hospital "Mum, he's my hero.  He's so brave.  I've never met anyone like him, I can't wait to watch him grow up.  He reminds me of me at that age."

Talon, thank you.  Thank you for opening your heart to Julian.  Thank you for your friendship with him.  Thank you for being fascinated by Julian's card tricks.  Thank you for helping Julian to remember why he fights IPAH every day.

Thank you for the smile that reached your eyes, lit up the room and brought an answering smile to everyone's lips.

Thank you for showing us what it means to be brave and fight on even when we're too tired to join the battle over and over again.

A Message From Jules

My younger siblings and I

I am feeling shocked. Well, to be honest, I am feeling like any teenager right now, with all these emotions raging inside me. But most of all I was feeling shocked. Here, let me tell you why.

Last Thursday (25^th June) I was at my eight-weekly clinic appointment (or check-up, whatever you want to call it) and I was bored out of my mind. It was literally one of the most boring days of my life. When I was talking to friends, or seeing one of the doctors, that, was different. I had something to do. Even though it is tiring and tedious talking to cardiologists and physios and psychiatrists and cardiac fellows, etc. It gives me something to do. But, when we’re just sitting there, doing nothing but waiting…. Ugh. Talk about not having anything to do. And then, when I go to make a cup of tea for mum, I get told off, getting told that “us children aren’t allowed in there”. Being one to respect older people (in most cases) I walked out and left it alone. That didn’t mean I wasn’t just a little ticked off though. 

Anyway, while I was waiting for my final appointment, I sat in a beanbag (well, more like lay in my beanbag) wondering about what I was going to do when I got home, when I fell asleep for about two hours. Although we thought that my final appointment – the one with all my doctors – would be at either 1:00 or 2:00, I ended up waking up at about 4:00 for the appointment. Go figure.

I walked into the consultation room, still dozy and groggy from sleep, expecting the normal brief about how my INR has been going, keep doing my regular exercise, blah blah blah. What I did most definitely not expect was for my cardiologist – Dr. W – to tell Mum and I that I was going to have to be put on a new medication called Prostacyclin (Veletri). If you aren’t familiar with this medication, let me tell you about it. Basically, this medication will hopefully make me the healthiest I have ever been, along with the added bonus of making me feel a whole lot better. But, like any good thing, it comes with a price. Basically, this medication being given to me is

“Like a doctor telling a paraplegic that his legs are cured but he still can’t walk for the rest of his life” – Will (Dad) Forester, a couple of days ago.

So, basically, I won’t be able to do most of the stuff I can do right now. Things such as:

Rollercoasters

Water Slides

Swimming

Kicking a soccer ball around

And more.

Which is honestly going to drive me absolutely crazy over the next couple of years, or until the oral medication comes out. But that’s OK! This will give me way more time to practice my hobby. Shhhh, my hobby is a secret. No-one shall know.

The way I felt when I was told that I’d have to start Prostacyclin was a very strange feeling. Because I had been told in the past that it was only a medication to be used as a last resort, I was furious. So very, very angry. But, then, there was the feeling of excitement. The feeling that said to me that maybe – just maybe – things would go upwards for a change.

Right now, at this moment, I still feel the same way, sort of. I still feel angry that I have to go on a “last resort” medication, but it’s more suppressed. Also it’s suppressed because Mum told me that Dr. W has never led us astray before. But things are definitely going to get better, based on past results. So I will be looking forward to that.

That’s all from me this time. I’m not sure if I’ll be writing more blogs for Saving Jules, but if I’m asked to I will definitely do it. It was a pleasure writing down my feelings and getting them off my chest.

Until next time, have a good night and good day.

     – Jules

Hit by a Bombshell

At Julian's last appointment of the day last at the Pulmonary Hypertension Clinic last week (Thursday 25^th June 2015), we had a bit of a bombshell dropped on us.  It was one of those appointments when I wished Will had been with me, but we have 3 other children in primary school and someone needed to be home for them when they came off the bus.  I’m just thankful my Mum had gone in with us this time to visit my teenage cousin who was in hospital for a ‘tune up’ for Cystic Fibrosis, because I was absolutely shell shocked when we walked out of our final appointment.

Exhausted after a day of testing

Long story short, sometime between September and the end of the year, Julian will be started on a medication called 'Prostacyclin', which 'inhibits platelet activation and is also an effective 'vasodilator'. It will be given through a PICC line or a Hickman line.  As I understand it, this used to be a 'last resort' medication, but Julian’s cardiologist has been in contact with other specialists in the field, and it is now thought it is a far more effective preventative, rather than a last resort, as there is no guarantee how effective it would be when people got that sick that they had no choice but to be put on it.

At first, Julian was on see-saw of emotions, ranging from “At least I will feel better” to “You would all be so much better off without me, look at all the problems I cause."  Now, he seems to be very accepting about it. 

This is going to be such a huge lifestyle change for all of us and there are so many things I want Julian to experience before he has to start on Prostacyclin - which will be very restrictive to what he can and can't do. I want him to go to town on all the things he won't be able to do once he starts. New Zealand. Water parks. New Zealand. Roller coasters. New Zealand. Swim with dolphins. Did I mention New Zealand???   As if Julian doesn't miss out on enough of life, now he will be even more restricted.

This is completely unexpected for us, and not something you can ever plan for. We have done our best to make sure we can do everything together as a family. Some of you may not know, or may not realise that Julian's condition - Idiopathic Pulmonary Arterial Hypertension - is terminal. That means it will kill him. Not might. WILL. It's not a matter of if, but when. This is why we want to get as much done for him as possible.

Our family is so appreciative of all your support.  Julian especially loves the messages of encouragement he receives from you all.  Keep an eye out for a blog post from him, to all of you, letting you know how he felt, how he feels now, what he will miss and what he’s looking forward to.

Recording Life

On the way home from the new Lady Cilento Children's Hospital late Monday afternoon, Julian was being his usual self in the car – and by that, I mean a little bit nutty.

So, I told him he should video himself more often – record his life – and this is what he came up with!

 

A trip to the new hospital!

We were at our neighbours son’s 7^th birthday party last Sunday morning when my 11 year old came up to me and told me “Mum, Julian’s not feeling well.” 

I looked over to the swings – where all good teenagers and pre-teens go to play at a playground! – to see him slumped over one of the swings on his stomach with his forehead resting on the ground.

When I got to him and helped him to stand he said that he was having chest pains, so I walked him over to the picnic tables and helped him to sit down.  He flat out begged me not to call an ambulance – he didn’t want to frighten the little kids at the party – so I kept a close eye on him for a while. 

Thank you Fairy Raine!

He rested for a bit, then we did a slow treasure hunt and he had his face painted by a lovely lady called “Fairy Raine” who went along brilliantly with my dare to have his face painted like a Pirate Princess (we were at a Pirate Party to, after all) – he got $10 out of it too!  By the way, I can’t recommend her enough.  She was absolutely brilliant, related fantastically to all the kids, and my own 4 thought she was fantastic. 

So, long story even longer.  He tried to get comfy on the picnic bench, slipped, bent his finger back and promptly sat on it.  Arrrggghhhh!  It swelled up like a bloody balloon, and we toddled off to the local Ambulance station to see if they thought it was broken or not.  No one was there so Will and I made the decision that I would take him to Robina Hospital ‘just in case’.  He is on Warfarin, so our worry wasn’t just that it was broken, but may be bleeding in the joint.

Waiting for results . . .

When we got to Robina Hospital, they took us straight through once they heard how he had injured his finger.  He was hooked up to an ECG, bloods taken, xrays done and they were in constant contact with the Cardiac Fellow at the Lady Cilento Children’s Hospital.  

 

HUGE tellies in the ED at Lady Cilento

  

A few hours later, he was transferred to the new Lady Cilento Children’s Hospital where he spent the night, and then spent Monday having tests done.  The new hospital is great, I just wish we hadn't had to see it so soon!

 

HUGE rooms too!

Nothing was found, and his results were all almost identical to the results from the Pulmonary Hypertension clinic in November.  The doctors are of the opinion that he just did too much.

So, now to figure out how to keep a teenager quiet for the next few days . . .