Okay - Like I always say - when I go, I go big.
So I thought I would corner the market on Rare Diseases and Disorders.... Right!
As everyone knows by now, The Big S (Scleroderma) is pretty rare. But, my body decided to invite a few other friends to the "rarity" party - and among those invited was MGUS
Sounds pretty gross right. kind of like your clearing your throat? Or what comes out of your nose when you blow it.
MGUS is a bunch of letters that stand for Monoclonal Gammopathy of Undetermined Significance.
What the heck does that mean? Hell - my life is full of things with undetermined significance - lol
MGUS is basically a precancerous condition which has a 4 in 10 chance of turning into Multiple Myeloma eventually, and the longer you have the condition the more chance you have of it turning into myeloma. As I was young at diagnosis, I am at a higher risk because I have more years life expectancy in which to develop the myeloma. Multiple Myeloma is cancer of the plasma cells and therefore the bone marrow, as this is where plasma cells are made within the body.
MGUS is basically a term used to describe the finding of a monoclonal protein (Immunoglobulin) in the blood. A monoclonal protein is an immunoglobulin which keeps replicating itself and producing identical clones of itself. An immunoglobulins normal job in the body is to attack and destroy infections, they fight off the bad germs for us. We have several different types of these good immunoglobulins and they each have a letter for instance IGg, IGa, IGm. In MGUS one of these immunoglobulins goes crazy and starts multiplying clones of itself, leaving less room in the blood for the other immunoglobulins. We all need a good balance of immunoglobulins to maintain an effective immune system, when one immunoglobulin gets out of control and crowds out the others, the balance is severely upset causing the immune system to fail. The overproduced immunoglobulin is useless at fighting infection because of its crazy mutated state.
MGUS can be happily lived with as long as it is monitored and as long as the amount of protein stays stable. If levels of protein keep rising then the MGUS is not stable and it may mean that the condition is progressing towards myeloma, so more testing needs to be carried out.
I have been diagnosed with IgG Kappa type with a current M-spike of .5. It has risen since last year from .1 which is why I am now invited into the "labs every 3 months" category. My particular protein also has an Alpha-2 elevation. What significance that has or means is beyond me, that's just what the labs tell me.
Scleroderma has been found to increase the risk of cancer, as it is an immune system disorder - however- the blood cancer risk with The Big S is historically Non-Hodgkin's Lymphoma not Multiple Myeloma. They are both blood cancers - and both ARE caused from abnormal proteins in the blood, which I find ironic and leads me to the question of; Are the Big S and Monoclonal Gammopathy related or coincidence?
There is some literature and studies that feel Monoclonal Gammopathies may be autoimmune related, in that those with an autoimmune disease are at a greater risk of getting an abnormal protein ab in the blood.
Again, I find the particular cancer risk for Sclero being Non Hodgkin's and the monoclonal gammopathy needed to have MGUS very interesting??? Monoclonal gammopathies DO produce auto antibodies, but it is rare - but then, so is Scleroderma. Hmmmmm.....
I also find it interesting that a monoclonal gammopathy is a B-cell disorder and I have Centromere B antibodies. The centromere causes primary constriction of the chromosome - (region of specialized chromatin found w/in each constricted chromosome that provides the foundation/assembly/attachment ) Errors to centromere are catastrophic for cell and lead to aberrant division and chromosomal instability which ARE OBSERVED IN CANCER CELLS.................Again, Hmmmmmmmmm
But who am I - no doctor. Just a girl who wants answers and maybe a ribbon or something for going big with rare disorders.
Just to warn you - The party doesn't only have The Big S and MGUS - they also decided to invite POTS - but I will leave that for my next blog
At this point my MGUS is pretty low and I am stable... with the exception of the rapid increase this past year - albeit - the increase itself was large, the numbers are not - which is great and all is good.
I also did a skeletal bone survey which shows no lesions, also good. I am to do a 24hr urine collection to see if there is a certain marker that is only found in urine is present, if so - that may change the game a little - if not, then its "to the Lab" every 3 months with a smile and a lot of hope!
IF - and that's a big "if" there is another increase, then I will do a bone marrow biopsy. So when that number rises - that's whats in store. Good times, eh?
I think that is all on the MGUS front - I do labs again here pretty soon, so I will keep you all posted.
Fun Times with Blood Work - LOL!
Ahhhhhh Life is Good
Peace and Love to all