Living with IPAH - Part Two

Warning- this input has one questionable word that no parent wants their 8 year old saying. Please proceed carefully (not that this blog series hasn’t had any language in it already, huh?)

 

From Julian’s point of view:

 

Wasup everybody. And welcome to MY point of view. The point of view that comes from the completely reliable 12 year old who never has anything dishonest to say…or at least not all the time.

 

Now, I have a rare heart/lung condition called Idiopathic Pulmonary Arterial Hypertension.

This entry is not of me saying (imagine these with a posh and British sort of accent) “Oh, look at me, I have a rare heart condition and the world should give everything I want… and if not I’ll throw a tantrum” NO. That’s just awful, don’t you think?

 

A question I get asked a lot is: “What’s it like to have your heart condition?” well, here’s the answer. IT SUCKS!!! Symptoms that I get are anything anyone else would not hope for. I get fatigue, hyperventilation, shortage of breath, and a whole lot more that I just cannot memorise. So, once again… MY CONDITION SUCKS.

 

The one thing that is the most irritating about my condition is the disabled car park. If you’ve read “You don’t look sick”, post #9 you’ll know. I just hate sitting or standing next to one of my parents argue over a stupid medical condition to some bozo who’s just slipped into a bloody car park just as our responsible father or mother has just been about to do the same.

 

AAAAAAARRRRRGGGGHHHHH!!!!!!!!

 

Anyway, now I’ve gotten that out of me...

 

I also have lots of food restrictions, like I can’t eat cranberries, grapefruit, or too many greens. And that fact just blows me away. Greens? Too many leafy greens? Come on!!!

One leafy green I can’t have too much of: Spinach. And that’s my favorite vegetable!!!

DOUBLE COME ON!!!!!!  

 

Anyway, I think that’s just about covered everything about being ME. From MY point of view. So, thanks for reading and I might be seeing you soon.

 

 

Blog Post by:

Julian Summers

Living with IPAH – Part One

From my point of view as a Mum:

 

One of the hardest things to accept about Idiopathic Pulmonary Arterial Hypertension was hearing that I was going to lose my son to it.  No one should have to hear that their child is being given a life sentence – especially from something that has no medical reason for happening.  And you know what?  It's not fair.  I'm sitting here at my laptop listening to Julian chatting with a mate he has over today, and it breaks my heart to think that one day, I won't be able to hear his voice anymore.  I won't see those silly faces he pulls, won't be able to wonder at how tall he's growing or feel the comforting squeeze around my waist as he gives me a hug.  One day, those bullet kisses he gives me on the cheek at night (the ones where he almost 'punches' his kiss on my cheek!) will stop.  I can't imagine a life without my first born son . . . I just know that I will lose a part of myself along with him.

 

My life seems to be full of disagreements with medical professionals, constant visits to the doctors, hospital stays for a ‘simple’ bout of gastro, trips to the chemist (I know almost all the staff by name now and definitely by face!), calls from the school . . . it goes on and on.  I’d love to be able to go a day without having to use the phrase “Jules have you had your tablets yet?” or, better yet, “Julian!  Why haven’t you had your tablets yet?!”  Right now, I’m constantly worried about the ‘flu, even though he’s had his ‘flu needle.

 

Sometimes I wonder what our lives would be like if Julian had never collapsed for that first time, if this awful condition had never become part of our everyday family life.  Would he be sporty and our afternoons be spent at sports practice and weekends at games?  Or would he still be my little bookworm that loves curling up with a great book?  I know we would be spending more time in the ‘great outdoors’ as a family, instead of Nigel taking the kids one at a time for a nice long bushwalk so our other children don’t miss out on the world.

 

Despite all this, I don’t think IPAH has to be the almost immediate life sentence (between 2 – 5 years) it once was.  I know that one day we will lose our son to IPAH but it obviously wasn’t as soon as we were told we would, and as his cardiologist – Dr. W – once told us, Julian’s life would have be about ‘quality not quantity’.  We really took that to heart (excuse the pun!) and I firmly believe that the ‘quality’ has given us the ‘quantity’ we were told we wouldn’t have.  We’ve made sure that Julian is as active as he can be, doing what he can and, being extremely sensible about it, has enabled him to lead as fulfilling a life as possible.  So many new medical treatments have become available since Julian was diagnosed, and I’m sure that there will be many more in his future.  Julian has told me that he’s just waiting for the research and technology to allow cloning of a person’s organs from their own DNA.  That way, he can have the heart/double lung transplant he may one day require and not have to worry about his body rejecting them!

 

Positive outlooks are so important, and allow our dreams to flourish where they might have otherwise perished.

 

      “Keep your face always toward the sunshine – and shadows will fall behind you.” – Walt

      Whitman

What is Idiopathic Pulmonary Arterial Hypertension?

Ha!  I just realised that after all this time, I still haven’t written about what Idiopathic Pulmonary Arterial Hypertension actually is!  *Face palm*

Pulmonary Arterial Hypertension is a rare disease where the blood pressure in the lungs is higher than normal.  PAH happens when the blood vessels tighten, and over time this causes fibrosis (scars) of the vessel and higher pulmonary blood pressure.  The Right Atrium and Right Ventricle chambers of the heart have difficulty pumping blood out to the pulmonary artery and through the lungs.  There is a large amount of strain on the heart to overcome this high pressure and the constriction causes the heart to become enlarged and weakened.  Eventually, the heart can no longer keep up with the demands placed on it by the body and can result in heart failure.

Image of PH Hearts from Nationwide Children's Hospital

Idiopathic (or Primary) Pulmonary Arterial Hypertension  is when pulmonary hypertension occurs without a known cause and is not the result of another medical condition.  IPAH is extremely rare, occurring in approximately 2 – 10 people per million per year and although it affects men, women and children it is most common in women between the ages of 20 – 45 years of age.  When Julian was diagnosed it was not believed to be a family or hereditary link, however this belief has changed over the years, and it is possible that there may be this link.

IPAH symptoms are rather common, and can be mistaken for Asthma, or in cases like Julian and a friend of his, Epilepsy.  Symptoms include:

• Breathlessness, especially on exertion
• Tiredness/Dizziness during physical exertion
• Swollen ankle and legs
• Fainting
• Chest pain during physical activity
• Blue tint to the skin (cyanosis)
• Recurrent nausea
• Exercise intolerance
• Poor growth in children
• Recurrent respiratory infections

It can be diagnosed through a variety of tests (although not all)  – Echocardiography, Six Minute Walk Test, Blood tests, Sleep Studies, Lung scans & function studies, and Right Heart Catheterisation (this is a definitive test to prove the diagnosis and confirm pressures).

IPAH has a poor prognosis and must be investigated.  The outcomes from treatments are largely dependent on the cause of the disease and how quickly the diagnosis is made.   When Julian was diagnosed, we were told that the mortality rate for children was between 2 – 5 years (9 years ago now!).   

Whilst prognosis is still poor for anyone diagnosed with IPAH (adults and children alike), living with IPAH has become easier over the years thanks to improvements in treatments and procedures.

10 Years Ago To The Day

Julian with his favourite author, John Flanagan

10 years ago to the day, Julian collapsed for the first time at my Nanna’s 80^th Birthday party.  Imagine if you will, the heart-wrenching sound of your first born (and only at the time) wailing at the top of his lungs and as you and your husband race towards the sound you know came from your boy, you see him collapsing in a heap before you can get to him.

“Oh my God, what’s happened?!”  After it’s all settled down, you’re never really sure if you actually yelled those words aloud or in your head.  Hands are fluttering uselessly as your husband scoops him up on the floor and together you bump your way through a gathering of your very large family and head towards the office where your Mum has worked for the last 9 years of your life and see her holding the door open with the phone in her hand, waiting to call the ambulance.  Questions, so many questions are asked by the operator, and after you hear “Call us back immediately if the situation worsens” you’re not even sure you were very coherent when you answered them.

Then, when you hang up you see two concerned women hovering near the door.  If you weren’t so worried and almost in tears it would be a face palm moment.  In your hurry to get to a phone, you’ve raced past two of your Aunties who are registered nurses!  They are quick to come in and check over your boy, and you can slowly see the awareness come back into his eyes.  Your nephew (who was playing next to your son when he started wailing) is in tears waiting to see if he’s okay, so you put your own worries aside to give him a cuddle, reassure him and ask if he knows what happened.  He doesn’t.  No one does.  Julian was simply on a chair at a table when he stumbled down and started crying for us.  

The ambulance comes, and the Ambulance Officers joke around with all of us while they check our 2 ½ year old over.  He looks so tiny on his Daddy’s lap with them kneeling down, putting a stethoscope to his chest and doing their best to coax a smile out of him.  His collapse has been put down to winding himself.  He’s an active boy, perhaps he hit his chest getting up to the table?  It’s possible, and heads are nodding, including ours.  After all, the kids have all been pretty rambunctious, enjoying their own little party with the baby-sitter in the other room.  The ambulance leaves without us, and we go back to enjoying the company of family and waiting until it’s time to cut the birthday cake.

Looking back, I can’t help but wonder what would have happened if we had decided to go to hospital anyway.  Would Julian have been diagnosed earlier?  I don’t believe so.  We had a long journey and a lot of – let’s say ‘disagreements’ – with medical professionals over the next 8 ½ months and it wouldn’t have made any difference if we had gone to hospital then. 

10 years ago to the day, Julian collapsed for the first time and set us on a journey we will never forget.

"That's been going around a lot lately"

It’s so hard when your kids get sick (and boy oh boy would I love just one week without someone calling out “Muuuummmmm!”), and even more so when there’s something “going around”.  You know, that ‘thing’ that’s always going around when you call the school to let them know you have a sick child at home.  “Oh yeah, that’s been going around a lot lately.”  Crap.  I hate it when they say that.  It means that all my kids will drop like flies from whatever ‘that’ is within days of each other!

It’s Julian this time.  I got the dreaded phone call from the high school yesterday.  “Hi Coleen!”  Why do they always have to sound so darn chipper when they call???  “Julian’s in the sick bay feeling ‘vomity’ in the stomach.”  Blech.  Vomity?  Or struck down by the dreaded ‘Maths Class’ that’s been going around lately???  Unfortunately, it’s not ‘Maths Cl ass’ or even ‘Exam on today’, it’s Gastro and a particularly nasty version of it too.

This might sound a little strange, but it’s always so much easier when one of his younger siblings has a stomach bug.  It’s off to the shops I go for Hydrolyte ice-blocks, Coke and Salt-and-Vinegar chips (a brilliant combination by the way, one told to me by an Ambulance Officer when Ayan had to go to hospital with Rotavirus last year), and then it’s just sit until the call of “Muuuummmm!” until it’s run its course.  With Julian though, it’s much different.  Nigel and I are on egg shells, counting how many times he’s been sick or has had to go to the toilet.  Too many and it’s off to the doctors to have an injection to stop the vomiting, or if it’s bad enough, a trip to the hospital a dose of Ondansetron and get put on a drip.

He seems to be coming good now though, so no trip to the doctor or hospital this time around, and he’ll be home recovering tomorrow.  Keep your fingers crossed that I don’t hear “Muuuuummmmm!” anytime soon . . .