Neurosurgeon call / Surgery plan change

I am still trying to figure my new netbook out so just going to post a quick update here.

Dr.Bragg called earlier today as she said she had been thinking a lot about tomorrows surgery and wanted to know what I thought of trying a different, less intensive approach first to the shunts and she explained her thoughts. Last year after we had removed the LP shunt (infection) and replaced the VP shunt we added a 2nd valve to the VP shunt to increase back pressures due to on-going occlusions and catheter issues to in-theory see if that would help keep the very small ventricles ok and catheter open. Later we added the LP shunt back and had 2 functioning shunts again but some issues.

Taking out this 2nd valve on the VP shunt is a simpler surgery and recovery then it would be to externalize an end of the shunt, drain outside the body and later have to put it back in also surgically. She said if this taking out of the 2nd valve doesn't work then in a week or two we will go back to 'Plan A' w externalizing a portion of the shunt and draining outside the body to see if under-drainage and CSF absorption issues where the cause.

I really hope this simpler plan will work. Praying!!  (I know God has a plan even if I don't understand it and lately I sure feel questioning about it all even though I truely do believe.)

My co-sunday school teacher and I where talking last wk ad I had let her know I was having surgery and she was asking ques about it all.(this thankfully falls on the first of by 2 off weeks (Joan and I do 2 on,  2 off so we each don't have to teach every Sunday which is nice). Anyways I got and email from one of my Pastors saying he and Joan where planning to drive to Madison to the hospital Weds to visit which I thought was nice especially since we've only known each other since the beginning of sunday school less than 2 mo ago. :)

I'll try to update after surgery tomorrow. Thanks for stopping by, Erica

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10/23 Pre-op Physical
Just a quick note, writing this from library and phone as my laptop harddrive is being fixed and I also have a new netbook ordered.
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Saw new PCP for pre-op today and she is talkin to my Hand surgeon to see if we cant use this pre-op physical/form from next weeks neurosurery shunt surgery also for the hand surgery at the end of the month. Usualy physicals have to be within 30days but we are within a week of being a month away from the hand surgery so she asked me if I wanted her to ask the surgeon; YES! She said maybe if nothing else she will be able to just add an addendum tpo this pre-op physical forms in a week or two for the upcoming hand surgery to cover bases. I hope!!

Risk for either surgery is moderate bc of the heart, airway and endocrine issues but she said as long as I dont have any complications w the shunt surgery she saw no reason to have an issue w the hand surgery. If I would have any un-forseen issues such as infection, heart issues/changes, pneumonia then I should just keep her updated and she will look for Dr.Bragg's clinic/surgery notes to and keep an eye out on whats going on vs having to come back in a few short weeks to see her. I am happy for that and glad she'll take the extra time to reach out to drs to try and make things easier given how complicated and tedious all this specialists and provider stuff gets on top of sunday school, GSF work and other things going on. :)  I like her!

Neurosurgery Appt: Externalize shunt, distal revision(s).

Not only will I be having the 3 part hand surgery on Nov 29th (carpal tunnel, tenosynovectomy (fancy word for cleaning the tendonds in the wrist and hand) and fat pad grafting to cover the released nerves in fat to form a protective barrier) but today I saw Dr.Bragg and she feels like we should go ahead w the shunt surgery to externalize 1 or both ends of the 2 shunts (likely just the VP shunt end) and see if having the shunt still open and internalized other than the portion draining outside my body can give us some answers. The biggest question being are the 2 shunts under-draining bc my body isnt absorbing the CSF properly? If this proves to be the case and I get good symptom relief as w the lumbar external drain (pre-2nd shunt placement) and EVD then we will look at where to revise the shunt(s) (again likely just one of the shunts) to w her commenting on several options such as chest, atrial drainage, etc. She said it is still possible we'd have problems w absorption but we are going to try and find the best answer(s) and she may end up doing several different changes this in-patient stay before we land on a final solution. The shunt drainage will likely be the same as w the external drains; draining in to a IV type bag and regulated to a certain amount of fluid per hour.

She is planning to do the distal end externalization under general anesthetic  as she said she didnt want to torture me w/knowing how toughened the skin gets and how much scar tissue builds up very quickly around my shunts in the past. She thought id be able to go to the regular Neurosurg floor vs having to go to the Neuro-ICU bc I still will have the shunt valve implanted as well as the 2nd shunt will still be internalized. Some of the possible revision locations she talked about where pleura, (lining around lungs), chest, and atrial although she is aware of the cardiac issues and my Cardiologist is really against us putting any of the shunt in the heart as I already have the central line there and would increase infection risk alot to Port, shunt and heart (endocarditis) especially given the cardiac issues I already have w multiple valves and severe enlargement of L sided atrial issues. I think she'll be planning this right up till we have to go back in and revise the actual shunt to a location.


Sat's WALK was amazing; I just volunteered in the kids area - there where 3 of us running the kids soccer event (for any kid that participated we gave away soccer balls and had 75 of the balls and by the end still had kids asking if they could have one after we where out. Amazing turn-out! My neurosurgeon was actually the invited guest speaker which I loved that in her short speech talking about shunts and research and causes of Hydro she even mentioned the genetic component of hydrocephalus (hydro as a secondary issue such as my case from the MPS I dx). The room/hall we where in at the Zoo was literaly huge so it was impossible almost to see everything and she wouldnt have known I was there and I had forgotten she was there so I didnt go looking for her to say 'hi'. I mentioned to her today that I was in the kids area and she said to me she wished she would have saw me/known I was there and we talked about the event as a whole for abit. The fact that she would take her time, on her day off, drive that hour+ to the Walk and give a short presentation is amazing and apparently she also attended last year as well! She's pretty amazing. :)

All for now; I see my Pain Mngmt dr on Thurs., back to regular Infusion monday, Pre-op on Tuesday next week and PT every Weds for an hour. It is also back to my 2 weeks on for sunday school.
Thanks for stopping by,

Erica

Hand Surgeon; Surgery, Neurosurgery

Tuesday I saw the new Hand surgeon and while at first he didnt actually realize I had an underlying diagnosis he told me he has had a few other patients who've had a 3rd carpal tunnel release surgery and so while we both know it isnt going to restore lost muscle and tissue it can help with symptoms; that is good enough for me. I asked him if it bothered him that it would likely re-occur again and he just seemed to look at it like we'll deal with it as it comes which is what I also think, no sense worrying about that now.
He recommended a 3rd release and a tenosynovectomy (cleaning of the tendons in the hand, my fingers are not affected) and additionally he would like to do a fat pad graft in which he will take fat/tissue from the side of my hand (in this case R hand first) and use whatever he can get (as he put it there isnt alot of fat to begin with there as I have so much muscle loss and thin to begin with) and then use this to "pad" the nerve which has now been cleaned and released 2x's already and a 3rd time will leave very little protection/cushion especially given my amount of previous damage (prior to 1st CT releases which where pre- MPS dx.  That is then splinted for 5-6 days and then bandages changed and I think re-wrapped plus stitches removed at 10-14 days + follow up in 2-3 weeks after that. I will wait till that is completely healed to do the other hand. Other than the tendon transfer in my ring finger to thumb (a pulley like system now that goes from wrist to thumb now) which was 2 yrs ago this surgery will be bigger than a typical CT release but he said still only take about an hour for the actual surgery.

I see Dr.Bragg tomorrow and hoping either the MRI gives us a clearer answer or she has thought over the things I mentioned here last week as these symptoms seem to be happening more and more frequently and progressively worse/longer lasting. It is almost like the pressure headaches and then to top it off the c-spine/upper neck into skull headache. =/ YUCK!

Sat I am volunteering at a Walk for Hydrocephalus at the Zoo in Milw in registration and the kids area and then going to my brother and sister in laws who are moving that day to an place about 30mins from the Milw Zoo. Should be fun if it doesnt rain and really is in the 60's as predicted - I sincerely hope it is a good day!!!! (especially symptom wise it is sooo un-predictable.)

Below was written monday  monday after getitng home froma long day in Milw and at ERT (infusion).
Thanks for stopping by,

Erica


Monday Oct. 9, 2012
This morning I was at ERT and up front getting paperwork from the secretary after coming back from across the parking lot/walkway of our clinic building (Pharmacy Children's Hospital, infusion is done in Genetics clinic not an infusion center) and after stopping back up-front before heading in back my former GC (genetic counselor) happened to also be standing at the desk talking to several people and she commented to me: "You look really great, Erica!" - this was the first morning ive felt semi-energetic (bouncing off the walls a bit actually) in probably a week (feeling particularly mind-numbingly bad on/off this past week) and so it was kind of nice actually to have someone think I looked really good when in fact I still hadnt combed my hair this morning (the hidden blessing I guess of naturally wavy, shorter hair)  and no makeup, no nothing special on! I love when even when you do feel like garbage run over and really havent done anything to look good people still think you look good probably bc of your mood and they dont see the fact that you dont feel good! I actually do not much like talking about how I feel (a product of too many drs maybe) and so days like today when I was feeling "better" but still hadnt done anything in particular are (perhaps selfishly!?) mini mood boosters, reminders that what I  have  or am feeling doesnt have to get the best of me! =)

Neurosurgery, Appt, C-spine MRI, Further discussion on options

And so it continues....

Ive said it before but will say it again I am eternally grateful to Dr.Bragg (neurosurgeon) for continuin to want to help me, for believing me and for caring when she easily like many others could just refuse to to anymore in trying to help me..

Today's appt she opted not to tap either of the shunts which was originally the reason for the appt but instead reviewed the MRIs with me and her NP (whom I also really like) as I was telling her about how the headaches cont to be worse anytime I bend my neck even if for very short periods (such as fastening pant buttons, straightening shirt, washing hair, looking at the computer (ive found better ways around this one) and all the little times we un-knowingly bend our necks to do some activity for a short period of another. Originally c-spine MRIs where ordered w the last MRIs done 2 weeks ago but for whichever reason they appear to not have been done and she when thinking about this H/A w/neck position issue wanted to make sure there isnt a phenomena of induced chiari (where the tissue of the brain is pulled down and essentially plugs the brain stem area - I think I have that right?) and/or to check the area around the previous laminoplasty (screws/plates) at C2-4 for position and is there narrowing in these areas or above/below these previous decompression/partial fusion as compression is a common issue in MPS pts spines.  It looks like that is scheduled for next thurs) - her NP was trying to see about getting it done today but since this 1. isnt Dr.Bragg's normal clinic day and 2. she has to reset the 2 shunts after any MRI imaging we opted to schedule for one of her clinic days.

She again talked about some options she has been thinking further about including is there an absorption issue going on w the 2 shunts being in my peritoneum (a pocket of the stomach essentially)  and would it be useful to externalize 1 or both of the 2 shunts either at the peritoneum area or for the VP shunt at the chest level and see if these while being 'open' and 'functioning' but draining outside the body bring better symptom relief and more efficacy similar to the external drains but this time using my own shunt)s) as the external drain and then when done w the external drain she would just have to revise those areas and replace that specific parts vs having to monkey with the 'minefield' that is my lower back. She wondered from a few articles she has read and 1 I sent her if in MPS we have absorption issues secondary to storage (I think thats how she put it)  could the shunts conceivbly work better in a diferent way, she doesnt know as we have restriction in almost all organs and even our skin is very tight and thickened but she has experience w other patients (non-MPS) who the distal catheter has been moved to the chest and because of the increased back pressure this area provides (vs the peritoneum) that that might help keep 1 or both shunts functioning better. She thought possibly moving the distal end of 1 or both shunts to the chest area after a trial might be a good idea to see if draining here would be more efficacious. -Clearly we dont know but feel like we have to explore various ideas as right now it isnt working as well as it should given the on-going symptoms I have and yet how good of symptom relief I had with the lumbar external drain trial (prior to our placing the 2nd shunt) and the same w the Ventricular external drain last fall and the pseudomeningocele (fluid collection pocket under the skin) we dealt with but which I had no symptoms during.

Another possible option is to move the trajectory of the VP shunt from the inner ventricle where  the  ventricle catheter now sits in to the back of the head where she could get a better angle of catheter placement in to the ventricle and  where there is more area (again I think thats what it was) to work with in placing the catheter given the very tiny ventricles.

I am sure I am forgetting some things as we literally talked about many ideas and possible options between Dr.Bragg, Sue (NP) and myself.

Nothing is ruled in or out yet as far as what we will do to manage this shunt(s) issues but we are getting closer to narrowing down our options and what we might try. Too quote Dr.Bragg "she still has a bag of tricks we can try." - for that I am grateful as this shunt stuff does wear you down. I sometimes actually laugh to myself bc I know even when I am in pain the vast majority of the time I dont look like it and can still be engaged and asking questions and sometimes I think that can actullay w many providers be a downfall as they cant look past "how good you look" and so I am grateful she and her staff try to help seeming w/these endless issues and can look past how I "appear" to know me well enough that I am not simply making up what I say.

I am grateful,

Erica