Diamox short term trial; Lumbar drain info

Dr.Bragg and her NP both stopped by separately; Sue is writing the discharge orders. Dr.Bragg thought trying the acetazolamide (diamox) would be a good idea and even if we cant do it long term (checking with cardiologist and will fwd any response to her) it may give us another idea how well a 2nd drain would work. She mentioned that she would be looking at my scans again and talking to Interventional Radiology to make sure placing a lumbar external drain could be done in xray (I believe she would do it there awake) so that she could have a clear picture/image when she is placing the drain and be safely above the laminectomy from last May and know we are away from the areas that are abnormal. She thought a few weeks rest. I think 2 weeks on the diamox would give us more than enough info if it would help and how it would help my body/CSF; I am grateful she will let me try this in the mean time (and Cardiologist already responded back that a short term trial was ok) and maybe will do even more to bring down the last CSF pressure that is driving my vision batty and making sleep impossible. Literally waking up in the morning is like trying to pull myself from what feels like a dazed coma. Here at the hosptial I slept upright; literally almost leaning forward and last night finally slept for about 4 or 5 hours. Today the headache is there but not as bad as before this revision and for that matter it never is really like a headache it's the pressure or as if your wearing a head band or something compressing the skull from the inside out. Not nice!

We (I) know pretty well that this latest revision did the job in restoring my CSF pressure back to where it was when we placed the programmable medtronic-strata valve in Febr. (low setting) and a few weeks will do good for my body (I think we will likely do a 2 week trial on the diamox before I go back and see her but uncertain). In the mean time it allows other things to get done and also can gather info which everyone knows is what makes me tick. :)

I will continue updating throughout this all and about the other upcoming tests, appts, etc.
Thanks,

Erica

External Lumbar drain, LP shunt addition and Cardiac Perfusion testing

After a miserable night of no sleep (maybe an hour and sitting up the whole time) and the nurse asking multiple times if I was ok because of the way I was falling asleep/did sleep Dr.Bragg came in this morning and I think the first thing she said was "your still feeling pretty crappy yet, huh?" Meaning she wondered yesterday if this shunt catheter move would help as much as we needed as she thought more CSF needed to be drained. The catheter being moved is a good thing as it still will be less likely to occlude but she must have thought that we would likely end up doing some form of additional drainage to as she came in and said she had been talking to her partner about why he did the LP shunt in one of his pts that also had a VP shunt and that patients symptoms she said  had very similar mechanics and issues to mine including small the small non-expanding ventricles. That patient has done really well with the 2 shunts and her partner apparently told her he would recommend trying the 2nd drain in me. Her partner as ive said previously is well respected in the hydro community and has alot of experience with shunts so although I am apprehensive about him I respect that he is willing to help my neurosurgeon plan the best course for me. Both feel our best bet is likely the 2nd drain so lumbar and brain pressures are equalized/level and if there is any issue with communication between the spine and the brain this will help mitigate this.  She wants to give it a couple wks for my body to re-coup from this surgery and initially she thought we would place a lumbar ELD via radiology using Xray guidance so as to go higher than the previous laminoplasty and also ensure proper placement. I think if im not mistaken she said we would be able to do this awake and then would monitor for a few days. If this showed improvement then we would place the lumbar peritoneal shunt in general surgery under anesthesia and at the higher lumbar level. Im unsure what valve setting she would use or if she would do a programmable with this to but she said she still has tricks up her sleeve and we have options. If needed and miserable she said we can also condider moving this ELDS (external lumbar drainage system) to sooner rather than wait the couple weeks. I think we will likely decide final when I see her in normal clinic fup which is typically 1 week after discharge. I could have gotten out of here tonight but have some questions for Dr.Bragg and although i do look fine I just wanted to ask her specifically about short term diamox in the interim. I would rather be home but know they have things here to help that I dont have and i'll get out tomorrow.




The CHW-ACHD (heart center) clinic coordinator called this afternoon and scheduled the Nuclear Med Perfusion Stress test - she  scheduled this test which will take 3 over hours over at FMLH for April 10th. I'll then follow up with my Cardiologist a month after this. This test will tell us if the heart muscle is getting enough adequate oxygenated blood and if so we can postpone valve replacements fairly safely as well chest pain not as likely to be serious (it also identified coronary artery disease which is known to happen in MPS although not well documented in data). If it came back abnormal with areas of the heart not getting enough oxygenated blood then we would know we have to consider surgery sooner. Please pray it is negative and we can continue to manage symptoms with medications for a while! I will start the new beta blocker (bisoprolol) likely on mon to give my body essentially a week post-op recovery.

Wish me better sleep tonight and the next few nights - it hasnt helped that the much higher  post-surgery(every 12 hrs) stress cortisol replacement dose is given at around 7:30 in the morning and 7:30 at night. I am down right exhausted tonight and vision getting blurrier and fuzzier despite wearing glasses so I am hoping to be able to sleep even if it is upright.

Will update soon,

Erica