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.

Quick Update – PH Clinic and fall

Well another day (and unfortunately a night) spent at the Mater Children's Hospital.  The Pulmonary Hypertension Clinic was on today, and I feel good about finally seeing his first Cardiologist back in the 'Charles in Charge' seat!  

Last night was spent in the ED after Julian slipped while hanging on to the top bunk at Mum & Dad's last night and banging his back and neck on the metal bed frame . . . arrrggghhhh!  Xrays came back with a shadow on his spine however the CT Scan was all clear.  It seems that his spine was jolted out of alignment for a while.  He'll be sore for the next few days, that's for sure!   

All we can say is, "Phew!"

Good people = Good results

As I mentioned in my previous post (Just when you start to relax), we got a call from Julian’s high school last week and were told by the Year 8 co-ordinator that his teachers’ had expressed their concern about how constantly tired he  is in class.  The outcome from that was the dropping of two of his subjects, which starts this week - half days on Tuesday afternoons and Wednesday mornings.  

Last Thursday came with another good result, with the Physiotherapist attached the Pulmonary Hypertension clinic, and the school/community physio attached to the hospital both coming up to Julian’s high school to do a review and help both us and the school work out a ‘program’ I guess, to safely get Julian out of his wheelchair at school more and more until – fingers crossed – it will only be at school for the inevitable so-so health day.  This meeting came about after my ‘Stand your ground’ post, which spoke about how Nigel and I were pushed to have Julian out of his wheelchair at school, despite no one from the clinic ever having actually attending his school to see how viable that was.  We had had no support, nor advice on what we should be doing or how this should happen, and the end result was that Julian’s health started to decline almost immediately, and we made an ‘executive decision’ along with support and advice from his GP to have him back in his wheelchair again until someone could assess the school.  

Well, both physios were in agreement when it came to the size of the school.  One actually used the word ‘sprawling’!  After a walk of one of Julian’s busiest school days and a very long meeting (thank you so much to the Year 8 co-ordinator for her patience during this time!), we established a plan to – slowly – get Julian out of wheelchair at school, and unlike the demands from the clinic, the physios have put together a plan to gradually ease him out of such a high need to use it.  We’ll be starting with one of his half days, where he has just one double-period, walking next to his wheelchair while one of his mates pushes it up to the eating areas and back again.  The physio will be presenting her suggestions at the next clinic (next week) to have it approved by his cardiologist before it’s implemented at the school.

Keep your fingers crossed they agree to this and don’t try to push it further!