Amyloidosis

My husband George was diagnosed with Primary Cardiac Amyloidosis in late 2005. Since the initial diagnosis, I have kept a journal of his early treatment – through stem cell and chemo – as well as his clinical trial participation.

Let’s start with a definition of Amyloidosis.

The clinical definition – thanks to the Amyloidosis Foundation – here’s their site: http://www.amyloidosis.org/facts/al/

“The bone marrow in the center of bones produces cells in the blood system, including “plasma cells.”  These plasma cells are the part of the immune system that makes antibodies for fighting infections.  The term “immunoglobulin” refers to the class of proteins that function as antibodies. Immunoglobulins are composed of four protein chains: two light chains, either kappa or lambda light chains, and two heavy chains, of which there are several types. 

These proteins are produced by the plasma cells in the bone marrow.  In AL patients, these plasma cells produce an abnormal antibody (immunoglobulin) protein. For AL amyloidosis, it is the “light chains” that become misfolded, and the abnormal, misfolded result is the forming of amyloid.  With AL amyloidosis, the “A” is for amyloid and the “L” is for light chain.

These misfolded amyloid proteins are deposited in and around tissues, nerves and organs. As the amyloid builds up in an organ, nerve or tissue, it gradually causes damage and affects their function. Each amyloidosis patient has a different pattern of amyloid deposition in their body. It often affects more than one organ.  AL amyloidosis does not affect the brain.”

The personal definition – life is crazy, right? Family, home, work, bills, friends – all the everyday stresses that make me pine for a glass of wine.

Now add the dimension of potential death to your beloved spouse. The everyday issues fade to the background as you start to contemplate the immediate and future steps on this journey. Your goal isn’t to get rid of the disease because that is impossible – your goal is to create and manage a treatment plan that will help you to live longer with the best quality of life possible.

Over the next few days, I’ll be sharing my journal from the initial treatment phase – hope you can stay and listen! Feel free to leave comments below!