Wednesday, 7 August 2013

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!

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