Neurosurgery Fup, 8 yrs diagnosed...

Ugh, someday will I ever not be here as I am now writing about these stupid, blessed headaches and related symptoms? I should sure hope so but I guess I thought maybe the time would have already come and gone... and it hasnt... instead I am revisiting the same symptoms again and again as we try to figure out what to do. =/

Dr.Bragg talked to Dr.Dickson (of the IT studies) and which I kind of wondered if it wouldnt be this way as the current study is set up and bc of my VP shunt (drains) I wouldnt be eligible for the study. My shunt alone doesnt precluse me but the way they give the dose is by lumbar puncture and my low back is so scarred from all the previous LP shunts and related nerve root surgeries that doing lumbar punctures is pretty much not an option. Their study is also not set up for anything but looking at cognitive function outcomes and is not looking at could IT aldurazyme be given through a CSF reservoir to help clear the mucopolysaccharide (GAG) storage from the shunts and thus keep them working better.. We need/wanted to try IT to see if it could help clear the MPS (GAG) storage from the shunts and the meninges which is what Dr.Dickson told Dr.Bragg she feels is adding to our shunt issues as the meninges get thicker, stickier and clogged w the storage and then when this gets sucked in to the shunts it causes the shunts to gunk up in a sense. So while the shunts may flow it probably isnt enough and the storage is clear so it cant really be seen sticking to the shunts (If  I got this right) like other tissue can be w their scopes. Also bc it is more of a compliance issue (brain isnt able to expand properly I guess, nor are ventricles) and less of a high pressure issue when the shunts do drain at the lower settings I feel best at (I feel better draining alot of fluid as when we do the external drains) then the ventricles collapse  causing a suction effect which then causes the shunts to clog or intermittently clog.  A crappy cycle.  Goes back to my feeling anytime we've replaced the entire shunt(s) for one reason or another i've done really well albeit for short periods (weeks) while when we revise just a portion of either shunt say the valve we dont seem to get as good of results., maybe bc there is storage throughout the shunt?

Dr.Bragg is worried about the cardiac issues and asked when Dr.Earing thinks we will get the valves (severe stenosis) fixed as that would make her part easier as the Anesthesiologists now she said (and I believe) are unhappy w all the surgeries we've done as their job is alot harder between my airway issues + tacking on the additional monitoring needed bc of the stenosis. The severe valve issues require much closer monitoring w/  Arterial line, careful fluid balance, etc. Dr.Earing in Jan had wanted to wait if possible atleast 6 months to do valve replacements but at minimum 3 months to have me seen by Cardiac surgery (whoever we decide on) to give my immune system a chance to recoup from the shunt infections in Dec. A sobering fact unrelated to the shunts but related to the cardiac issues?  Most people will not live 3 years w severe stenosis; I think these cardiac issues are my "sticking my head in the sand" issue bc that was awesomely sobering but at the same time I am doing what I can and God has a plan +  I have  good drs. doing the best we can.

We talked about many things including she is still thinking about cranial expansion but doesnt want to put me through that unless she'd have a better idea it could work. Its a way last ditch thing for us I think but time will tell I guess.  The one thing we keep coming back to but dont know how to bi-pass is some kind of a internal mirror of the actual external ventricular drains as those and the pseudomeningocele's (CSF filled pocket) are what we've had fantastic results with while the shunts have been very short term "fixes" with a few weeks at most. She wondered if there was a way to create an internalized drainage point that would act the same as a EVD w the same continous flow while providing the needed back pressure to keep the venticles open and able to drain. I cant remember her explanation well but something about EVDs provide for a continous backup due to atmospheric or barometric pressure (??) so it's a constant drain whereas a shunt in my issues is only intermittently draining due to the compliance issues. I think that is something she may keep thinking about or asking around about as she and her partner do have several pts or atleast one patient anyways and she knows of others who instead of shunts have an external drain and drains in to an external pocket. We need this but a way to drain internally to avoid the infection risk due to all my other issues.

She is talking to the  Neurologist she works with on her complex shunt patients; they have spoken before about me but she wants to see if he will help her try to come up with a plan or does he have other suggestions she might try. In the past the week I was going to see him with her I ended up getting sick and admitted the day before the joint appt and so he saw me in the hospital but I was so out of it and they opted to try something else. I think that may be when we first switched from the LP shunt to the VP shunt after doing ICP monitor and the External drain.  She said she would call me monday which makes it 4 long days away and 4 long days to be nervous and though I dont often worry about care issues I do worry when it comes to these kinds of issues. I just truly hope Dr.Bragg doesnt give up and they are able to think of something together that might help/we could try. Its so frustrating (!!!) that we have such incredibly good results w the external drains and seems so simple (I know its not really) that we should be able to replicate. Wish it was so easy!

We'll revisit the IV Zofran when she calls on monday as my PCP Dr.Bragg commented must have said something to her (Dr.Bragg) about being concerned w the infection risk with our doing IV zofran through the continously accessed (but covered) Port. This has been a godsend so admittedly just one more thing to stress a little about. The zofran isnt perfect and I still have no appetite (Dr.Bragg herself as did my Pain Mngmt dr earlier in the day commented that I look like ive lost weight.) but atleast I am not throwing up here, there and everywhere in the mornings and at night when I am first up (first few hours are worst) and when trying to sleep at night is the other very worst.

Ironically today, 8 years ago my then GC called to tell me the blood results where back for the MPS testing (urine had come back a week or two earlier so we kind of knew, just not a type) and was positve. Good news then bc I had a name finally (though my Cardiol. had told me 2 or so months before he suspected it was a form of MPS). bad news bc who could have known it would be this crazy? Seriously? In that 8 years ive had atleast 2 surgeries every year I believe and years like the last 1 1/2 have had 20-some in just  a 1 1/2 year period. Again, CRAZY!

Im sure I am forgetting something but thats todays not un-expected but no less hard (disapointing, though not in anyone, just in general) news post-appt fup.

On a un-related note I teach sunday school for the first time since early Jan this sunday and our kids are practicing a new song but otherwise pretty un-prepated. Will be nice to see the kids but could use a boost of energy... =/

There's no manual how to do this disapointing news over and over and over again and is just as hard each time.
Thanks for stopping by, if you believe in it pray we figure something out,



Erica