Welcome to NZ Amyloidosis Patients Association (NZAPA). We are Aubrey and Jaime Christmas. In October 2013, Aubrey was diagnosed with Familial Amyloid Polyneuropathy (FAP ATTR val47). We had prayed that this disease which claimed the lives of his grandmother, father, brother, aunties, uncles and cousins would somehow skip him but that was not to be.
His condition got progressively bad with the protein depositing in his gastrointestinal tract, nerve (familial amyloid polyneuropathy/ FAP), central nervous system and unfortunately his heart. He has been diagnosed with Cardiac amyloidosis (“stiff heart syndrome”) due to the amyloid fibrils depositing in between the cardiac muscle cells. So the heart thickens and stiffens as the amyloid deposits prevent the muscle fibres from relaxing properly. A restrictive cardiomyopathy, the deposition of amyloid fibrils in between cells preventing or restricting the muscles from doing their job ie. allowing the heart from filling up with blood.
On 9th Feb 2016, he underwent NZ’s first domino liver transplant at Auckland hospital and since then, his donor liver is doing excellent in his body despite the fact that the other organs compromised especially his heart and nerves continue in their unrelenting deterioration.
From the point of diagnosis to even now, the one thing we have discovered is that proper support and care for those afflicted with Amyloidosis, the ATTR gene mutation particularly, are severely lacking in NZ. Perhaps this stems from the lack of resources or time on the medical practitioners side but clearly something must be done to cater to the needs of the patients and their carers.
Fortunately for us , we are personally connected with the National Amyloidosis Centre (NAC) in the UK (Prof Hawkins & Dr Julian Gilmore) as well as the UK Amyloidosis Advisory group (UKAAG) and thus have first hand access to medical information and development in this area.
What we hope to achieve is to be a bridge between the expert entities overseas as well as in New Zealand, and all patients with the disease here in Aotearoa. We also want to connect and identify those ‘in the same boat’ as us to offer support, not only to the afflicted but also their carers. If we do not band together and create one voice, we are unlikely to be heard and this lessens the chance of accessibility to treatment drugs that are currently available, as well as receiving funding from our local Pharmac.
At present, all efforts by us to access any form of drug currently in the outside market have been at a slow alarming rate. We have taken it upon ourselves to make our voice heard by working together with other similar patient advocacy organization such as Rare Disorder New Zealand, Australia Amyloidosis Network, Amyloidosis Alliance Network in Europe and Patient Advocacy group in South Korea. This website is set up to promote awareness as well as be a platform to show the larger community that we are here and we need backing. We are confident that together, we can make a difference in each other’s lives. Together we can eventually eradicate the effects of this cruel and disparaging disease.