Wednesday, April 5, 2017

The Fatigue!

More on the immune system...

Since the immune system regulates so much in the body, many things can go wrong with you when you have immunodeficiency.  When the immune system crashes, AUTO immune diseases visit you more frequently.  The problem is the immune system largely dictates what your blood work looks like.  When the Dr looks at your blood work, sometimes it is a guessing game what is going on. Example: My lupus blood work is all over the board.  One blood test the ANA is off the charts, the next time it is below normal.  The same with other parts of my blood tests.  Does it mean I have lupus?  Possibly.  They often start going with symptoms...

My immunologist explained that it is because my body is clueless what is wrong.  I have a dis-regulated immune system, so my body can't 'read' the natural signals because they are not there.  

So, I have been diagnosed with SLE Lupus, Sjogren's Syndrome, Fibromyalgia, and Sarcoidosis of the lungs - in addition to ICL.  If you look up all these syndromes/diseases you will see each one in itself causes fatigue - and a landslide of other problems.  Did you read the symptoms on all of those?  

Since my immune system crashed, the Drs have said it is like a domino effect; auto immune diseases will continue popping up.  I hope they are wrong, but the immunodeficiency support board I am on says otherwise.  2,044 members - and the majority have landslides of auto immune problems in addition to their initial immune problem.  And I am the ONLY ICL patient.  

Also, immunocompromised patients (esp with ICL) are susceptible to opportunistic infections and cancers - especially lymphoma.  The t-cells (specifically CD4) recognize those nasties in your bodies, but, (here we go again) remember I am at 39.  The nasties would have to slap the 39 to get noticed, but are more likely to scoot right on past, into the body and wreak havoc.  And that is what we watch for.

Oh.  My.  Gosh.  The fatigue.  It is unexplainable!  It is deeper than bone marrow.  If I ran a marathon, I believe I would still have more energy after it than I do after walking 200 ft - I have worked extremely hard occasionally in my life, and it doesn't even begin to feel like I do on a daily basis.  Every day, I am fatigued.  Extremely fatigued.

I have finally given in to the fact that I can no longer be superwoman.  I do what I can and forgive myself for what I cannot do.  I have always felt guilty for not pulling my own weight, and I still do, but I have also conceded that if I go overboard, we suffer for days;  Steve and I both do. So we shut it down when I start getting wobbly. He shuts me down before I collapse - I am still learning my limitations.

If you look up Idiopathic CD4+ Lymphocytopenia (ICL), you will find very little information about it because there are so few of us that have it.  I am HIV negative which is a huge factor.  I have basically the same problems HIV patients have except I don't have HIV.  However, my CD4 count is 39 - very critical. They are extremely concerned when your CD4 gets below 200.  

Mine is 39.

There have been studies on ICL since 1992 - the same year my dad was diagnosed with (we think) the same thing.  Of course they didn't know that then, but we assume that is what he had, because he died of Micro-bacterium Avium Intercellular - an opportunistic infection that kills people with ICL.

Because of that - they assume my ICL is genetic.  

The Drs at the National Institute of Health (NIH) are trying to find out what is happening to my t-cells.  They have found that my antibodies are sticking to my t-cells and smothering them, but they have no idea why.  So they fly me back to Bethesda Md to study me and my blood.  Yes, I am a guinea pig.  And yes, I pray for an answer. But if they offer me a treatment, I would seriously have to weigh the options of taking it.  They have already tried stem cell (killed the patients), bone marrow (killed the patients) and transfusing the t-cells into the patients, but they died also.  Since our t-cells are the supposed to be the ones that jump start the immune system, we have no way to fight infections that can come with those choices.  How bad do I want to be a guinea pig?

Right now there is no help or cure for ICL.

Maybe someday there will be. And maybe it will be a safe cure.  That part scares me.  I go to NIH in case any of my ancestors have/get ICL.  I want to be part of the cure.  And if I can save someone else from this awful disease, I would gladly do it.

Until then, oh my goodness, the fatigue! 

Enough for today.  Make it a good one!

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