May 4, 2012 Transplant +150 days

When a car is assembled, the parts are not made on location, but they come from various suppliers around the world.  The seats from one factory, the engine from another, the tires and wheels from another, etc.  These parts are delivered on a schedule so in a month if 1,000 cars will be assembled there is a delivery of 1,000 radios. 

The body’s immune system functions in a similar way.  Plasma cells in the bone marrow are the factories where proteins chains are made.  These chains are assembled outside the plasma cell and create the needed immune systems to fight infections.  On occasion, using the car factory analogy, some of the factories goes crazy and kicks out a large order of proteins that are not radios, but refrigerators.  This is a very simple explanation of what occurs with AL Amyloidosis.  Over eager plasma cells generate a large number of proteins that are ill-shaped and made wrong.  These proteins will float around in the body and not assembly with other proteins.  The overabundance the wrong or ill-shaped proteins will cause damage to certain organs in the body.  One protein called a Lambda Free Light Chain (λFLC) will cause damage to the heart and nervous and digestive systems.  This is what happened when I had heart failure and why I received a heart transplant back in September 2009.  If you look at the graph at the top of the page, there are two flat lines and a squiggly line. The area between the flat lines is an acceptable area of   production of the λFLC.  The squiggly line shows the production levels of my λFLC that have been assessed from blood draws since when I was first diagnosed with this disease back in February 2009.  When the doctors put me through chemotherapy or a stem cell transplant, the intention is to destroy the factories that are over producing the λFLC parts.  You can see each time I go on a heavy dose of chemo, the numbers of λFLC drops, but never reach the proper range. The squiggly line never gets down to where it should be.  Even after going through my last stem cell transplant with a high dose of chemo in December 2011.  In fact, the λFLC numbers have gone up slightly and have now plateaued off.  Going back to the analogy with the car factory; someone needs to bomb the factories that are making all those refrigerators!!!  The chemo should be doing this and this is the best weapon against this disease the doctors have.  The disease is very persistent.  In medicine, there are new weapons, better chemotherapy being developed all the time.  It is a forgone conclusion that I will need to be on some form of chemo for the rest of my life.  Right now, my young bone marrow environment is too tender to go through more abuse.  I still have a ways to go with recovery from the last transplant.