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 . . .  

Every rock needs a solid foundation

Recently I saw an image on Facebook where someone had created a number of circles – like a target – showing how comfort should be given to people in a crisis.  The basis was that those in the larger circles give comfort to those in the smaller circles, all the way into the ‘bulls-eye’.  Those in the smaller circles can ask for comfort from those in the larger circles, however not vice versa.  The closer you are to the crisis, the more comfort you’re supposed to receive, not give.

When I looked at this image, I wondered who the rock is for ‘the rock’ of the family this crisis is affecting.  In a lot of families, the Mum is the rock for everyone else, and it’s the same way in mine.  Although I know that I can turn to Nigel whenever I need to, I also know that I ‘cope’ better with Julian's condition than he does.  Maybe it’s because I grew up with an older brother with a life-threatening condition.  I’m also the perfect person to help his younger brothers and sisters through their worries, because I’ve been in their position as well.  What I do know is that I have learned to ‘shrink’ all the worries down and hold it all in one tight ball.  If I didn’t, I’d explode!   

Within our family, we have a lot of these ‘targets’ all overlapping each other.  Obviously Julian has one bulls-eye, and the outer circles are Nigel and I, his siblings, his grandparents, uncles, aunts, cousins, friends, teachers, etc.  BUT!  Each of his siblings also has a ‘bulls-eye’, just like Nigel and I do, and Julian is learning to be one of our outer circles as well.  Just as we are still learning to help him cope with what is happening in his life, he is also learning to help us cope with having a son and brother with a life-threatening condition.  It is as hard on us as it is on him, just in a different way.  While we can never really understand what it’s like to have this threat hanging over his head every day, neither can he truly understand what it’s like for us.  Nigel and I live every day with the knowledge that we may outlive our own child - something no parent should ever have to live with – and his younger siblings live with the knowledge that they may end up growing up without their much loved big brother.  I know how this feels from both sides, as a younger sister and as a Mother, and it is hard.

So, in a family like ours, each ‘rock’ has to have a firm foundation, and that foundation is formed by the outer circles.  Nigel and I are each other's rocks, but I also know that if either of us fail to cope, then our foundation is wide enough that there are other ‘rocks’ for us to turn to.  I know that I lean on my own Mum a lot, and her rock is my Dad, just as she is his.  I also know that they can turn to my brother, and he can turn to his wife.  Our foundation is large and it is strong.  It’s not just made up of one rock, but many.  If it wasn’t, we would all crumble.