Julian's treatment for IPAH

Julian is on 3 specific medications specifically for the treatment of his IPAH:

·         Sildenafil (Revatio) – Sildenafil relaxes the arterial wall, which leads to decreased pulmonary arterial resistance and pressure.  This then reduces the workload of the right ventricle of the heart and improves symptoms of right-sided heart failure. 

·         Bosentan (Tracleer) – This is a medication called an ‘Endothelin receptor antagonist (ERA)’.  It helps PAH patients by blocking the effects of the extra ‘Endothelin’ their bodies produce.  Endothelin is a naturally occurring chemical in your body and is involved in blood flow.  Don’t worry, I don’t really understand how this works either!

·         Warfarin – This is an anticoagulant that lowers the risk of blood clots forming by increasing the time it takes for blood to clot.  There are two brands of Warfarin available in Australia – Marevan and Coumadin.  These brands are NOT the same, and come in different tablet strengths and colours, so if you’re taking one, you have to use the same brand unless your doctor specifically tells you to change.  Although Warfarin is very effective, many commonly used medications and foods can interact with it, and can change a patient’s ‘INR’ (International Normalised Ratio – basically how long the blood takes to clot).  INR has to monitored by blood test or a ‘Coagu-check’ machine (similar to a blood sugar machine).  An INR that is too high increases the risk of bleeding, and an INR below the nominated target increases the risk of the dosage being too low to be efficient.

Julian is also on 2 other medications to treat issues either from side effects of his medications or variables of his condition.  One is for migraines he suffers from which are both hereditary and a side effect of the Sildenafil, and the other is to help with the difficulty he has sleeping (both getting to and staying asleep), which for him is a ‘variable’ of his condition.  I guess he’s just lucky as none of the other 3 kids with IPAH in Queensland have either of these!

More and more treatments are being developed to treat IPAH.  Atrial Septostomy (making a hole in the two upper chambers of the heart – the atria- to relieve the pressure in the heart due to the IPAH) and Prostacyclin (a drug given via a portacath – in layman’s terms, a portacath is basically a ‘permanent catheter’, and in the case of Prostacyclin, is put in the chest) can be offered to sufferers who are failing their other medical therapies, and when all else has failed, lung transplantation and heart/lung transplantation is also an option for some –although not all.  It seems that different countries follow a different regimen when it comes to using the different medications available to patients with IPAH.

Julian’s health started to deteriorate a few years ago, and the decision was made to perform an ‘Atrial Septostomy’ in 2011.  This was supposed to happen in June, however due to a bad chest cold, it was postponed to August 2011.  In his case, doctors had to first create a hole between the two atrial chambers, before placing a dumbbell shaped stent in to keep the hold from closing up.  While his health never really got back to what it was before it started to deteriorate, we were very lucky to have had this procedure performed when it was.  According to Julian’s cardiologist, he was borderline with the pressure in his heart to perform it safely.

Nigel and I are continually looking for anything that helps Julian remain as healthy and as active as he can, and we think we’re doing a bloody good job!

I look forward to the silly things he’ll do

I know this sounds a little silly, and a lot of parents dread the phone calls from the school letting them know their child has run into a pole, jumped off the slippery slide, or fell out of a tree they weren’t supposed to be climbing, but I'm really looking forward to silly things Julian is likely to do as a pre-teen!

 

Over the past couple of weeks, we’ve had 2 phone calls from the school that went a little something like this, “Hi Nigel/Coleen!  Don’t worry, Julian’s fine, but.. .”  They’ve been greeted with that parental groan we all know so well, and along with the mild annoyance I’ve felt at his silliness, I’ve also been pleased at the same time.  When you’re told that you will outlive your own son, you start to regret the fact that he’ll likely never fall off the monkey bars and break his arm – not that that’s the good part! – and then feel the puffed up self-importance that all kids feel when there’s a rush by friends to sign the cast.  There will be no war stories told over the doctors head while he stitches a leg or hand, no scars to share and brag about with his mates.  These are the injuries that no parents really want for their kids – who wants to see their kids in that much pain? – but it’s something that I always had a mild, somewhat guilty, hope for him to experience.

 

His first misdemeanour was the week they all went back to school, and involved putting his head together with a mate and deciding that having said friend push him down the hill on the oval in his wheelchair would be a break from the boredom of high school!  Let’s just say a clean-up of the wheelchair was in order after that one . . .

 

 

 

Then, just a week ago we got a call to let us know about a magic trick gone awry!  Long story short, it involved glue, a leather neck band and what looked like carpet burn on his neck . . . I’ll leave your imaginations to fill in the rest!

 

We may have been annoyed by his silliness, but boy oh boy, were we pleased he got to have those experiences too!

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.