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!
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