Bittersweet

Manny got a broviac (also called a central line or line, a type of semi-permanent IV) on February 7, 2012.  It has been through a lot.  (Understatement).  And I even explain more details here in the Blog entry called, “Bye Bye Broviac”.  http://destinyshousechurch.blogspot.com/2014/03/bye-bye-broviac.html

April 1, 2014 they will remove this broviac due to its instability. 

Plan A:  They will attempt to put a new one back in the same vein.  This might be tricky because it’s been in for 25 months.  Scar tissue and other things might complicate it.  (UPDATE:  Due to the ultrasound results, this is no longer a viable option.  The surgeon said the angle of the old one is too sharp of a turn to try that or it might rupture the vein.) 

Plan B: To put a new broviac in another vein in his chest.  Today they did an ultrasound to check patency and it appears there is still a viable alternative in his right chest.

Plan C: To put a new broviac in his leg.  This would be a last resort and quite frankly, a pain in the butt. 

In addition, we are having a nerve biopsy done.  A muscle biopsy is quite common but a nerve biopsy is relatively rare.  This is being done as a diagnostic test.  He has Merosin Deficient Congenital Muscular Dystrophy (LAMA2).  That account for his muscle weakness, why he can’t walk, etc.  But it does NOT account for his intestinal failure.  And it seems progressive (meaning getting worse with time).

An EMG (nerve conduction study) was done and had two results.  For LAMA2, the nerve relay signal is delayed.  The signal still arrives at the destination but slower.  (Think a car going 30 instead of the expected 60 mph.)  And his did this. 

What was unexpected was that there were reduced axions in the nerve bundle.  Every nerve is actually made up of lots of axions.  (Think about how there are lots of wires inside a cable for example.)  Say there should be 250 in a nerve bundle.  His had like 50.  This gives us a clue. 

When the doctor performed this test I was like, “Cool, it’s a hint.  What does it mean? And what causes this?” He told me he didn’t know.  I asked who would know and he said a neurologist.  I said, “Uh, aren’t YOU a neurologist?”  Yes.  But in true Manny fashion, this is RARE. 

The only way to know what type of neuropathy this is … a nerve biopsy.  Now if this was only to give us a name, I wouldn’t do it.  But it’s possible that with the name MIGHT be a treatment.  Or a way around it. 

And if this was only affecting his arms and legs, we wouldn’t pursue this answer.  But we think this is affecting his Autonomic Nerves as well.  That means the heart, lungs, kidneys, bladder, brain … important stuff.  And this biopsy could help “Stage” how far this has progressed. 

It will be several weeks before we get results.  Hoping we get answers though and that I’m not putting him through this for nothing. 

So around 11am EST, think of Manny.  Send up prayers for him, us, the whole surgery team.  For wisdom on what to do and how to do it.  For no complications.  For no issues with anesthesia.  For no complications in recovery.  For pain control.  For a quick discharge.  For an easy transition back to home.

The whole thing is bittersweet.  The central line lasted 25 months.  That's HUGE.  And I hate to see it go.  But it's time.  While it's technically still working, there are things we cannot do with it.  For example, we are supposed to do a "power flush" to help clear the line of "gunk" like clots that build up inside the catheter.  But that now breaks the line instantly.  So there's no way to clean it.  And this could cause MAJOR problems (like the line to rupture, the vein to rupture, infection, clots, etc). So it's time. 

Today is bittersweet because he has to do another surgery.  I hate putting him through it.  He's scared.  He freaks.  He's in pain.  He throws up.  He's miserable after.  It's just so pitiful. But it's necessary. 

Same with the nerve biopsy... pain inflicted.  But hopefully it's for a good cause.  But still, it's pain. 

But if we don't do the broviac replacement, he can't get his TPN and he can't go home.  So forward we push. 

But it's bittersweet.