Hi all. I would only like to add that this forum has been at times a therapeutic tool in itself. I am a tech professional and like professionals in other areas some of my colleagues don’t favor sharing technical instructions because they feel that laymen might get confused or misunderstand concepts and procedures. While that may be true, I much prefer this forum because we not only share our own experiences but also we pray for each other and uplift each other. I know some mistakes, misunderstandings get into our conversations but still I think most of us value the strength and love far more than recommendations.

Just my 2 cents

All true Sheldon. After almost 5 years on Tafamidis, my recent echo and ecg shows significant loss of heart function. Pace maker and defibrillator are in the works. But, how many people live to 84 years with or without Attr-wt? I think the key is to enjoy what we have.

The disassociated (broken-up) Transthyretin protein clumps and accumulate as amyloid fibrils in-between the layers of heart muscles. The TTR protein is a c loverleaf shaped tetramer but it end up as monomers as it misfolds.

In both ventricles, the muscle fascicles of the myocardium are arranged in 3 main directions, forming 3 muscle layers within the ventricular wall. The amyloid fibrils fill-in spaces between the layers of heart muscles in the ventricles as well as being deposited in the atria.

The Fibrils which are the fallen-apart, deposited TTR are proteotoxic so they not only are the cause of heart wall thickening, they disrupt the ability of the heart muscle tissue to conduct electrical signals and trigger arythmias.

I just went through, in the Pavia Amiloidosys Center in Italy, the review after one year of Tafamidis treatment. Blood and urine test show the same values of one year ago, In particular S-NT-proBNP is 213 (before Tafamidis it was 456). The echocardiogram show that the hearth tickness is still the same of 18 months ago (SIV=18mm;PP=17mm).
It seems that Tafamidis works well enough. I will have another echocardiogram and Holtercontrol in 6 months.

I had my heart thickness tested after being on Tafamidis for 6 months after my initial diagnosis . Thankfully the thickening of my heart had stopped. My doctor said that with that news, unless i have other symptoms that show up, that he didn't think i needed to go back to him for a year... Thank God.!!!! it gave me a boost of positive feelings about living with this monster and now i just want to keep qualifying for the assistance program. Looking ahead i have a lot of life i want to live and I'm not planning on sitting around to wait on it just because i have amyloidosis.
Happy Thanksgiving to all!!!