Wednesday, December 21, 2011

Finished with the Zyvox!!

For a while I sincerely felt like this day would never come - I think this was definetly one of the worst medications but in a subtle way ive been on in a long, looonngg time. 5 weels ago I found out about the CNS infection and started the Zyvox - thankfully I am now finished with it as of last night-my last dose! This med has brought on such incredible fatigue in a way I cant even describe (not unlike that caused by beta blockers but different at the same time). When I had asked the Infectious disease docs about it the NP had said this was surprisingly a very common noted side effect in their patients on it although it isnt listed as a side effect in the literature. Atleast I knew I wasnt crazy!

Yesterday and today have been surprisingly tiring (in a different way I guess if thats at all possibl!) not only do I feel tired but I feel completely exhausted which is odd as the past 3 weeks (or is it 2 weeks only?) since surgery energy wise have been fairly decent other than the zyvox effect. I think the continuing annoying pinching/searing very present but intermitten feeling along the proximal portion of my shunt isnt helping I actually wonder if this isnt draining alot of energy as the past 2 days have definitetly been a little harder and a lot more often and occuring for longer periods of time. Ive also had a few symptoms re-occur but just beginning last night and today so maybe thats just a fluke and will again improve. - it sure would be damned nice if it did!!! Because of the vision issues ive taken to wearing my glasses for reading anything although the glasses annoy the h-e-double hockey sticks out of me because I despise the way they feel. I just cant make myself wear them all the time for this very reason.

Audiology testing yesterday was stable w/the mild hearing loss at sensorineural level (highest frequency equated with difficulty typicaly hearing in loud environments or differentiating conversation when more than 1 person is taliking in a crowded room. For me it just has been sensitivity to a lot of noises (air compressor, elevator doors sliding shut when they are slow closing, etc and being in crowded rooms. Not to big of a deal. The noise thing may even be more of a sensory thing but I guess that could go with hearing, who really knows, or atleast I dont off hand!

We're making slow - really slow progress on our collaborative effort (genzyme and myself) for the identifying un-met needs in adults with MPS I project which has been nice and although I think this is going to take us months still we have some really good ideas and a good group involved in this effort.
This is about all I know - not the most enlightening or entertaining update (are any of them really!?) I know.
Warm wishes for a Merry Christmas and Happy Holidays!


Thursday, December 15, 2011

Neurosurgery fup

I had fup appt this morning with neurosurgery and she removed all of the sutures, staples and dressings including the dissolvalble sutures (because she said she was impressed with how irritated my skin got even from these). The only thing left in was the dissolvable sutures in my head incision but the staples and dressing where removed there. In total 6 incisions are now free and I can wash my hair in total tomorrow which will be great as it has been incredibly itchy to both from the dressings and from bacitracin ointment they used over the sutures and under the staples/dressing. We talked about what could be causing the pinching at the R side of my reservoir/valve and she said it's possible it is either from drainage or from a disconnected piece from how they inserted the shunt (xrays where done the aftenoon after the surgery though) or that something ir irritated and/or possibly the ventricle collapsing when CSF is draining though that to would be odd (but not impossible) since we have the second valve in place. What I do know is ive not experienced this sensation before so it's a litte unusual.

She ordered a shunt series (xrays) and a add-in brain MRI which they got the radiology dept to do right away this afternoon to. I should know the results of xray series and the brain MRI in the next day or so. Otherwise if it doesnt show anything like a disconnected piece she to use her words 'doesnt want to torture me during the holidays' so we are hoping these symptoms improve or at the very least dont get worse as my headaches actually arent to bad - they defintiely arent perfect but they arent as bad as they have been sy before this 2nd valve was put in 2 surgeries ago (about 60-70% improved) so we're hoping the shunt just doesnt occlude and symptioms keep improving. I fup with her as long as nothing gets worse in a month.  On a good note the CSF culture from last weeks surgery also remains negative which means I should finish the linezolid (zyvox) next tues - I will be thrilled!! I am tired of being tired from it and taking a nap just about every day bc it makes me so tired!

Happy Holiday wishes,


Tuesday, December 13, 2011

1 week Post-Op

Incision at upper cest - shunt tracts down from front of forehead behind haieline to behind ear and then downward to where the 2nd valve sits under this dressing.

Reaction to the tegaderm used to cover eyes during surgery  "raccoon' look.

Lumbar incision - LP shunt removed.

It's no longer 'welcome to D6/4' but 'Welcome back' from the nurses.
 Lumbar incision a week later
 Stomach incisions from LP and VP shunt removal and replacement

Side incision from removal of LP shunt  reservoir and valve
 Shunt card for the 2ns valve. (paedi-gav)
Just  a quick note - I basically guessed and thankfully was right about what labs the Infectious Disease docs wanted since I hadnt received a lab order from the neurosurgery floor discharge team on fri. I called my PCP and asked them if they would be able to order the labs and they where fine with this. I'll probably see if I can do the other Lab at UW on thurs. Lisa, from my neurosurgeons office called today and she confirmed it was a CBC w/diff and also let me know that so far the CSF culture has come back negative (day 6) and that they would cont to watch the culture to make sure nothing grew back.  I see them thurs to get the stitches out of the various different incisions - in total I imagine there is around 20ish sutures and 2 of the incisions I think are dissolvable so it is the staples in my head and side + back incisions that will get removed + the small incision in my upper chest.

It was a completly exhausting and tiring weekend but we had alot of fun - sat we (sister, mom, sister-in-law and niece) went to Appleton and then on sun we baked pretty well all day. Needless once I did finally fall sleep sun night I slept well! Other than waking up pretty often having to go to the bathroom I did sleep and last night think I may have finally caught up some what. I will be thrilled when the linezolid (zyvox) is done - shoot I may throw a party, this med sure causes unending fatigue on top of all the other stuff!

Will update after tomorrow's Urology and thurs Neurosurgery appts.
Take care,


Friday, December 9, 2011

Post-op day 3 - Discharge, post 2

I just need to vent and this is the place I do it. So i got discharged as planned - I didnt hear from my neurosurgeon as she was in surgery so didnt get a chance to ask her what I wanted to (kind of makes it feel like there  are things un-finished when this happens but I get they are busy).  I guess when I have to ask the nurse every little question and for help on things that other nurses just automatically do (re-covering incisions, how to remove the Port access needle, meds, erc) and I am already overly tired from being unvcomfortable sleeping (pain is controlled just hard to find a good laying position) then all those otherwise little things really get to you. I didnt mind this nurse and she was nice it just felt like there was alot of things that werent done and we scrambled to do after I was already down in the Pharmacy-discharged. I guess as the patient I dont want to have to tell a nurse what needs to be done or ask for help on very little things. I already dislike asking people for help so topple that with my being exhausted and I just felt/feel unsettled.   I also know the more specialists you get invovled the more chaotic communication can get but in this case I felt like there was simply 0 communication between my surgery team and the Infectious disease team. Simple things like who was going to write the lab work order - each told me something different and then not providing their contact info even though im supposed to fax them lab results and/or if any issues with side effects of meds. We knew id need to be on the Zyvox (linezolid) for another 2 weeks so why would they wait until the day of discharge to try and get those prior auths??? If there is one thing that irritates the piss out of me this is surely it - I even asked on weds if this shouldnt be started on and then yesterday they told me it had been. (it wasnt). I dislike I think most of all how they have a NP on the floor who does the discharges - I just personlly think it would go sooooo, soooo much more smoothly if it where done by the patients own providers who've been in on all the communications and rounds and thus know what all is going on vs guessing in the end before discharge. The nthe NP never communicates and so you literally feel like even though you've dealt with this stuff before that she doesnt care and isnt going to listen albeit it would clearly make the process faster. I usually bring in 2 of my meds because of the brand they are -Dad had to run back up and get these thankfully he remembered as pharmacy and the nurses hadnt. It honestly was just one thing after another and to tired to think rationally. I especially hate that things are left in to the weekend and will have to be dealt with/figured out on mon - I always, always prefer to be discharged and things just run smoothly! COMMUNICATION!!!

I do still have the stapes and dressing in my head - my neurosurgeon had talked about removing this before I left (there are stitches underneath to) but I guess either the residents didnt think of it to come back or my dr had planned to do it but got busy. I know tomorrow i'll just deal with it and figure out how to make it coverable but it is much bigger this time than usual (she usually makes the residents cover it with a much smaller piece of teflex/staples) but said she had been closing another area when she realzied they had already put it in. This is life i guess. I just sincerely pray this is it for awhile - that the blurry vision improves and as does low back discomfort and headaches get back to a good place (they atrent so bad right now kind of up and down but hopefully improve more),  I get all the staples, dressings and stitches out next thues so will be happy about that and will ask my dr if there is anything I/we can do to manage the eye issues till they improve - I dont know that there is anything but was just what I had planned to ask her more about today. (we talked about it some the other day).

I k now I sound whiny and grumpy and I apologize - tomorrow will be better and I will be better for it.
What a week!


Revision 13 (VP shunt 4th) Post-op day 3

Should be getting released today, my goal was by sat so I could go with my Mom, sister and sister-in-law to Appleton.  Some oif the things that have to be done before I am released today include the PA (prior auth) for the Linezolid (zyvox), logistics of the lab work, and Infectious disease docs figuring out concrete plan of action which right now stands at Linezolid for 2 more weeks, Lab work 2-3 times a week here and they'll call if the CSF culture comes back positive which they expect if it's going to it wont until day 4 or 5 as the last 2 cultures havent shown up till days 3 and day 5 - a slow growing bug. Given I have the Port I wouldnt need to have a PICC line inserted but we're still hoping the labs come back negative.

This time has been so weird - every post-surgery it honestly feels like healing has went really different - this time I suppose because not only did we take out 2 shunts in 2 completely different locations (8 incisions) but then we put in a new VP shunt to boot. My lower back is pretty swollen yet although the incision is about 3/4 the size of my previous lumbar laminectomy/shunt revision in May using the same incision.

I think honestly I just feel tired and exhausted and worn out by all this - it's not that  I dont think it will get better or even that im worried what if this shunt doesnt work given the VP shunt removal showed us there was yet another occlusion in the process. It's more that im just tired from all the other things that go along with surgery and cardiac stuff has been a little nutty (increased-racing heart beat and skipped beats) and the usual re-adjusting to anesthesia getting out of my body and lack of sleep and head adjusting yet again (not low pressure just pressures in general always go through a 'process' period of ups and downs the first week especially. Honestly I think athough I like the nurses here on the floor and find them almost all very sweet (several have told me they specifically requested to have me on their case load when they find out I am coming in/or here which is always a near thing to know even if it's sucky having spent so much time on a hospital floor that the staff likes you and apparently not a pain as a patient.  I think im just tired of feeling good, not feeling good, surgery, healing, surgery, healing, feeling good, not feeling good and then feeling good again. I will be extremely excstatic (and I bet my neurosurgeon and her nurse will be to) when we have the shunt figured out and in a good place for a long while! That all said I am still very grateful to have my neurosurgeon and her team who want to help and are funny and just plain care despite all of our ups and downs over the past few months - the fact that they continually have some other ideas amazes me and I am grateful to them for that!

I will update again soon but atleast I am headed home today - very excited about this and more excited about the weekend. I know it's going to be hard the next few weeks trying to cover all these incisions for showering and then re-cover them afterwards when some are in some hard to reach areas but im thinking maybe I can go every couple days changing the dressings as long as I can keep them dry in the shower and just have my infusion nurse change them on mon and if im here for labs can always ask one of hte neurosurgery nurses to help or famiy - will figure it out - in the mean time my ever thinking mind is always try to figure out the best details just because.

Take care and thank you for following the ups and downs over the past few months,


Tuesday, December 6, 2011

Shunt surgery: VP shunt revision 3, Revision 13

12/7/11 - 12:10
 Infectious Disease docs stopped by again a bit ago; they'll watch the CSF culture for about 5-7 days and while we all expect/hope it comes back negative (infection under control) if not or if i begin to have negative side effects like bone marrow suppresion they will switch to a IV antibiotic and the antibiotic would be given for longer than the current planned 2 weeks (if negatve). Suprisingly they said that tiredness is a common reported side effect in their pts of zyvox but not reported in literature.

Surgery wenr pretty well with a few surprises but started about 7:30 and finished a little past 10:30. Dr.Bragg started with the LP shunt removal whch she said when she got in to the lumbar portion of this she fiound that the 2 shunt pieces which should have been 1 where disconnected completely in to 2 pices (should have been connected as it was tied off but this area hadnt been worked on since the 2nd shunt revision in august and was initially replaced during the laminoplasty in May. The august revision  was just re-anchoring the catheter as it had come lose in that area. This she said was likely why there was still a small amount of CSF build up but Dr. Bragg does not think this will have any impact on the lower back pain as that she feels is from not enough CSF draining through the shunt. She was happy that the spine tissue was scarred off though so she did not have to mess around in the area of the nerve roots or with the nerve roots as happened during May's surgery. There are 3 incisions from this shunt removal. The VP shunt apparently right before I went to sleep from anesthesia I asked Dr.Bragg if she could in fact move the 2nd valve on the VP shunt although I fell asleep then before she could ask questions or we could talk about it - normally I ask not to have the 'sleepy' IV med right before they take you in to surgery but this anesthesiologist gave it anyways which kind of ticked me off and after I explicilty but nciely explained why I didnt want it.  Anyways so she when they got to that point did move the valve up to my upper chsst and commented how in the other location upper stomach/lower chest the valve clearly moved around quite a bit and she could definitely see why that would have been annoying as itwould rub on the bra line and clothing to.. When she got to the proximal catheter (in ventricle) and 1st valve/reservoir she found that these where once again partially occluded and said that this is probably why my headaches had improved so much but then basically plateaud. If this happens again she is planning to move the proximal catheter to a ventricle with more fluid space that is farther from the choroid plexus (near the front of the head and a coral like material that often gets occluded in catheters placed in the front ventricle. Placing the catheter tip att the back of my head/brain she said would give more space and she could place the catheter further in to the ventricle. In total I have 6 incisions and actually feel far worse pain wise tonight than I did this morning or afternoon after surgery. Sleep has been elusive which is a bummer as I am incredibly tired!!! - update I did sleep on and off for about an hour here and there.

The infectious disease doc stopped by 2x's today and decided that they will cont the zyvox (linezolid) for 2 more weeks with 3 times weekly lab tests as once a person is on this med for more than 2 weeks it can begin to suppress the bone marrow and I have been on it now for 3 weeks already. My labs where low currenly but the docs said it wasnt worrisome at this point and just needed to be monitored closely. If the CSF culture from todays surgery would come back positive they said they'd re-eval the treatment approach but we are somewhat limited in meds as I am allergic to the other 2 oral meds that cross the BBB and help CNS infections. Our hop is though that since ive been on this med for 3 weeks now the infection will be well under control and removing the 2 shunts will prevent a recurrence. (this type of infections act much like scum on plumbing when it clogs - very difficult to get rrid of and would  re-occur if the shunts hadnt been removed which is why shunts are often removed and eplaced with CNS infections.

Im sure im forgetting something so will update soon - thanks for the prayers. Sorry for the typos this is all  really blurry so having a hard time re-checking the spelling. =/
Hoping for sleep,


Monday, December 5, 2011

5:30/7:30 arrival/surgery (shunts)

Surgery at 7:30 - arrival 5:30. (Dr.Bragg does this when I am her only adult pt that day so that she doesnt get caught up in Peds neurosurgery cases and so I dont  have to wait around for her with no definite time). This means leaving my house 4:15 my parents house 4:30.  My Mom is attending my Great Aunt's funeral so will just be my Dad and I.

Pre-op called today and then the Pharmacist called (severaltimes) - as well as an email from my neurosurgerns nurse confirming the antibiotics to be cont'd. I will still take the cipro (UTI) and zyvox (linezolid-CNS/shunt infection) tomorrow morning along with all other meds other than the lasix which is usually given later in the day in these cases. Im not entirely sure how long i'll be in-patient but hoping to be out by the weekend as my sister in law and niece are coming and spending the weekend + they, myself, my sister and my Mom are going shhopping sat and baking sun. I know i'll be beating up on myself after this big of surgeries but will do what I can handle and by that point as long as out of the hospital will be craving some normalcy regardless of how I feel!

My infusion nurse left my Port accessed after infusion today so that is in place for surgery tomorrow and the only thing will be if anesthesia needs to do an arterial line and/or place a 2nd bigger peripheral vein which they often do in addition to the Port access. Non the less one down is nice!

I'll update when I can either tues night or Weds.
Saying my own prayers for this to all go well and the CSF cultures to come back negative for any residual infection!


Friday, December 2, 2011

Communication: the ever elusive issue?

I should probably start if you'd like auto updates sign up as a follower (or for email updates) to the right of this post.

To continue on the theme of yesterday - communication. So I think I mentioned that my neurosurgeons nurse e-prescribed a continuation of the antibiotic my PCP prescribed as neurosurgery wanted it extended up till the morning of surgery but that my PCP felt it only needed to be for 3 days. (that is the recomendation for an uncomplicated UTI which this would fall under since although I do take a bladder med for retention issues I have had no issues of a UTI symptoms). So anyways I get a message back from my PCP today se still feels only 3 days is required but I should do what I feel is right... Ummm, ok????? I do really like this provider and I also really like my neurosurgeon and her nurse very much but since when is a patient supposed to be in the middle??? I know my PCP left a message for the nurse w/her antibiotic recomendation but it wasnt with my Nrsgns nurse and I dont think she ever actually talked to anyone on the phone beyond that VM.. Ive done all I can (and more) as a patient to smooth edges and presumably feelings in this matter but feel like no matter which way I take this I am stepping on toes - I dont think I have a choice but to take the extra 5 daysof antibiotic till tues mornings surgery given we need to know for certain there is no infection given we are already taking out the 2 shunts because of a different unrelated infection and im not sure if they will have to cath or not during surgery (foley) usually depends on length of surgery and if my neurosurgeon will have me lying flat for 24-28 hrs due to the spine portion of the LP shunt removal. I can understand neurosugeries concern given my history of UTIs over the past 5-6 month (due to the laminectomy and LP shunt revision-catheter placement in May and resulting nerve + bladder issues.) and desire to be extra cautious with this in preventing a infection post surgery to but also am more than a bit confused on why this has even turned in to the saga it has between these 2 providers. Who knows!?!

So readers, im curious how do you deal with providers disagreeing (in the MPS world is so frequent atleast in my experience) and how do you decide when providers give differing opinions? Im definitely struck time and time again by how often patients are (probably unintentionaly) put in the middle in not only diferring opinions but also making sure providers share info, communicate (or dont), and in trying to decide what to do.  

More soon,


Thursday, December 1, 2011


This week has been busy and yet times ive had a little more time to get things done or work on projects (genzyme) and spend time with my nephew and today he and my niece and sister this morning.

To highlight exactly how frustrating my care can be at times (im not sure if it's anyone's fault or if I am just to charitable) but monday I receied a messaged via the hosptial  electronic system from my NP that there was she suspected a bacterial infection brewing in my urine and so she was calling in a antibiotic to be taken on top of the zyvox (linaxolid) ive been taking for the past 2 1/2 weeks for the CNS/shunt infection. Tuesday morning my Nsg's NP called to make sure a antibiotic was started as they had gotten the pre-op form/info and also to ask me to let her know if the antibiotic wasnt prescribed up till surgery. I picked up the cipro tues afternoon and it was written for 3 days 2x's a day so I called back the nurse (Lisa) and let her know as I didnt have the NPs # directly on my cell. She talked to the NP and they asked if I could find out if my PCP-NP would re-prescribe the Cipro up till the following tues (6th) so I sent  a message back to my PCP-NP and she responded saying she had left  a message for neurosurgery NP and it was ok the way it was. Today I talked to Lisa again (I swea I talk to her more in the past 4 months than I have my own family) and she wanted it up till surgery - in any case she e-prescribed it for another 5 days 2x's a day. Wouldnt it be easier if providers just talked to each other??? Dont get me wrong I do really like both of these offices but good golly!! 'the gift of patience' is not always gift enough! ;0)

Symptoms such as the low back pain and vision continue without improvement which is really weird so i'll have to talk to Dr.Bragg about this before or after surgery but I do wonder if  maybe the low back pain might by some small chance improve once we take out the LP shunt (due to the shunt infections) 0 I guess we'll see..

Otherwise we are continuing to move along albeit slowly on the genzyme - adult project and this week I spent several hours identifying and contacting adults ranging from late 20's to late 40's and have heard back from one lady already who would like to participate. Myself and the mrketing + patient advocacy reps at genzyme are aiming for Jan to do our first part of this project w/about 5-6-7 other adults ive indetified and using pre-determined questions/information we've come up with. It has been interesting and I think will be useful not just to MPS I but across the spectrum of MPS's to other adults.

I'll update next week likely but in the mean time take care and be well,


Thursday, November 24, 2011

I am thankful for....

Today im thankul for...

My Family including 2 parents who I love and who love me dearly. Who are there to help me when I need it, who take all the time off from work they need (sick days, personal days/FML) to be there for my many surgeries and procedures. Who visit me every night at the hospital when I have been in-patient which this year has been to many days and nights to count despite the hour drive one way generaly after they've each worked a full 8 hour day and in my Dad's case often when he has had to go in to work early. I am thankful for the many times this year my Dad has been able to leave work suddenly to take me to the ER and to his employers for letting both my parents take this time with little to no questions asked. Most of all im just thankful to have these 2, my Mom and Dad as MY parents - I dont think I could ask for better parents! They dont meddle, they dont try to rule my life (despite what I know is often alot of worry) and they treat me no different than my other healthy siblings of which I most appreciate. I am thankful they raised me to be so idependent and for instilling the ability to deal with the chaos that sometimes is.

I am thanikful for my sister who although like any siblings we have our disagreements I know is there and I can talk to her and I know she cares. Im thankiful for the beautiful, adorable nephew and niece she has given me and thankjful most of all she wants me to be a huge part of their lives!

I am thankful for my sister-in-law because I can truly talk to her about almost anything and although I rarely talk to people about how I feel with all this MPS stuff I know with Jenn I can vent and while she may not always understand it she listens and doesnt judge and I can talk to her and when with her we just have such a good time. Im thankful to have her and my sister because I dont think a person could ask for better sisters!

I am thankful for my brothers who although sometimes they frustrate me (my oldest and twin) I love them and I know they care in their own ways and I through them I have also been given a wonderfully beautiful couple nieces and another nephew all of whom I adore. I am thankful for being an Aunt several times over!

While this may sound cliche I am thankful for my apt and to know I always have a place to call home if I need it (my parents house). There's somethin to be said for a place of our own and I am incredibly thankful to be able to live on my own but there is also something to be said of always having the ability to rely on our childhood home if we need it!

This may sound a little odd but im thankful to have the cats outside my apt to take care of - Im thankful I have a Dad and brother who can build me shelters for the cats and that I also have a live trap compliments also of my Dad for when I can catch these cats and for the local humane society. On the fli;p side of this I am NOT thankful for people who abandon their cats like yesterdays garbage - no one person or animal deserves this cruelty. I am glad I can help these cats a little. There's something to be said of helpfing something otherwise abandoned!

I am thankful for Genzyme's willingness to take on this 'identifying un-met needs of Adult with MPS I' project as it is something ive been intersted in doing for a long time and I am thankful they've committed the people/resources they have for our population.

I am thankful for a promising new treatment for MPS I which although it would not replace ERT would benefit some of the areas of which I am most affected by MPS (CNS (brain/spine), Heart and joints. I am also thankful for the researchers who are invested in this disorder and genuinely interested in us their patietns lives and helping.

I am thankful for my faith and for my church and being able to be renewed in a sense each week at church. Also my Pastors who have visited each surgery and who care about how things are going.

Lastly I am very grateful for those providrs old and new on my team who go out of their way to help me and to help solve the complicated health issues I/we deal with. I am (not to exclude any providres but these deserve special thanks) especialy grateful to my Pain Mngmt dr who is invested not only in my pain care and treating the various aspects of my pain (neuropathic, joint/muscle) but who also is interested in my life in general and a cheerleader for what I do both with APF, with MPS I and with my life in general. I of course am thanikful to have found my neurosurgeon who while Pediatric trained is also interested in helping adults with complex shunt issues and for her interest in sharing what she knows, always making a way to make it seem understandable and for her ability to pick up on when I just dont seem right even at times when I dont quite realize it yet.
Thanki you to all these people and for all these things!

Tuesday, November 22, 2011

Pre-Op Appt and Cardiology/Echo changes..

Busy day so i'll just jump in and start from the last appt which was Cardiology on down. Rcho was done first per the usual and saw my Cardiologist right after (adult congenital) who said there continues to be progression of both the aortic and mitral valves as there has been for the past 2 years now. We do 6 month fup appts and Echo's and it seems there has been some progression in atleast 1 area in every appt since around fall 2009. I asked some questions but of course didnt think of one that I wonder about now  - we are on diltiazem (a calcium channel blocker) and lasix and have been for about 1 1/2 years but if there continues to be this progression I wonder should we re-visit the dreaded beta blockers. Ive tried these in the past, a couple different ones and felt terrible with each med we tried (even after several weeks when ones body is supposed to adjust and the fatigue should improve) so thus we went on the diltiazem which my Cardiologist uses although he doesnt love it for my particular issues (but has less side effects, it just increases something with afterload pressures on valves/heart). I wonder now would something like the beta blocker be less aweful since so many other non-cardiac symptoms are under control. I dont know. My Cardiac issues currently stand at: Moderate Aortic stenosis and mod. insufficency/regurgitation, Mitral stenosis-moderate and mild insufficency. Moderate Atrial enlargement and Pulmonary Venous Hypertension. I fup in 6months but of course can always call or email them if I so desire with my question. For now I think it's fine. Concerns are if breathing issues worsen (Atrial enlargement/PVH) and if sporadic abnormal heart beats would increase to much longer occurences that uncontrollable-longer lasting (changed) shortness of breath occurs with this.

Also had Pre-op appt this morning w/labs which was fine, since this is only the 3rd or 4th pre-op appt in the last 5-6 months for the shunt nothing unexpexted. My PCP was just going to verify with Endocrine dr that cortisol dose should stay the same for this next shunt surgery and talk to Cardiology to verify all was blessed there. We talked about providers and how it seems to be working a bit better with communication and having Dr.Bragg manage the whole neurologic issues (vs having my neurologist) is probably going to work quite well since she is so thorough and also has the Neurologist she works with when it is an issue she isnt sure of. PCP asked how I cope with all this as she said she has concerns when especially younger pts have to deal with a chronic illness and in my case she just said  mine is so overwhelming seeming because so many different specialists and issues and concerns to manage. I never really have a good response for that question because what choice do we have? I dont really believe in the 'God doesnt give you more than you can handle' chain of thought but I do think you either sink or swim. As long as I have providers who incorporate me in decision making (and this has gotten 10x better with addition of new providers, although not perfect)  and welcome my opinion or concerns and I can have say/control in when things occur (appts/tests/surgeries when possible) that makes a big difference so I can still have a semi-normal (to me) life.  I am surrounded by family including my nephews who remind me to be resilient - Zander especially at 5 years old dpesnt stop just bc of the craziness autism brings to his life and his chaotic, unending schedule (insane for a 5yr old) and so I joke that when I need a reminder there is plenty of good around to I just go visit Z or other nieces and nephews. I also have a community of MPS friends who although I rarely talk about how I feel with this all (to anyone) I know some of these friends get it and I can get advice there on the medical stuff. And really it's not about me, it's about making the best of what I do have. Doesnt mean im perfect just that we have to make do with what we do have and can change.

I talked to Lisa at Dr.Bragg's office as I had asked how would we know the infection was gone since I dont have symptoms and she said the particular strain of bacteria (P.Acnes) is susceptible to the antibiotic  I am on which crosses in to the CNS very well and wouldnt be able to survive it. If we where to only do the antibiotic and no shunt removal then the bacteria which will maintain to the side of the shunts would grow back but removing the LP and VP shunts eliminates those variables. She also said they would do maintenace dose of the Zyvox (linezolid) after surgery I guess although im not sure how long. I also asked to double make sure if the hardware in my neck was fine and she said because this sits outside the CSF that it will not pose a problem which is good news!

My thought of the day is I wish I was one of those people who could instantly think of questions the minute information is presented to me especially from medical poviders - but im just not. Doesnt matter if I write questions down because frequently questions change based on new or changing information from providers. I usually am so busy processing and I suppose figuring out what if anything new information means that I just dont think of the questions I have. Thus I envy people who can instantly think of questions!
More soon,
Happy Thanksgiving!


Friday, November 18, 2011

Surgery date, Philly - CHOP MPS I study

I picked up the Zyvox on tuesday morning at UW (cancelled and rescheuled other appts) and have been takig it 2 x's a day ever since. Thankfully no side effects minus a minor all over body itching that occurs a few hours afrer I take it and lasts 1-2 hrs. Since it started the day I started the Zyvox I assume it's relatedf. I'll take this antibiotic for the next 3 weeks and then am not sure if I will be on an antibiotic for a while after the new shunt is placed or not. Surgery to take out the LP and VP shunts and replace with the new VP shuntwill be Tuesday, Dec 6th. I am very grateful that Dr.Bragg and her nurse, Lisa worked it out so I did not have to go in to the hospital this week for IV antibiotics and surgery! Im not sure how will they know if the infection is truly gone (since I didnt show signs of it) and also have to check to make sure the hardware in my neck is fine but otherwise am just looking at this like atleast I am getting a reprieve for the hoslidays and atleast I am still feeling pretty good. My vision and low back/leg issues continue to occur unabated but those may still heal. On my questions to ask Dr.Bragg about if not improved by surgery - I do wonder if taking out the LP shunt wont help the low back pain since it seems to be along the same lines and areas as the type and area of pain I experienced after the LP shunt revision and laminoplasty in lumbar spine last May. We will see I guess and hope!

Weds since I had Dr.Bragg's permission I flew out to PA for the MPS I-non-sense mutation study at CHOP (on Penn  State grounds) which weds night I had supper with my good friends Jenn and Anisa who are from Toronto, Ontario but whom I met several years back at a conference in Vancouver. Anisa is 6 with MPS I and completely reminds me energy and talkativ wise of my 5 year old niece - she remembered me to which I was really surprised by! They where also participating in this study and we by lucky chance ended up on the same dates. My other friends Jenn and Austin live in NJ and drove down for supper with Jenn and myself and the kids - was really lovely. I'll try to uload some pics in to this post. Thurs Jenn, Anisa and myself took a shuttle over to CHOP with another MPS I family - the Moore's from Mn whom ive also met and we all had breakfast with the study Genetics Counselor and then went up to meet the study doctor, Paige Kaplan. Skin biopsy atleast in my opinion was very easy and lots and lots of very cute pictures! We should know in a few weeks how successful the study is as it takes a few weeks for the biopsy to grow in to a sheet of skin which the ataluran (study drug) is then tested on. If these studies are successful Dr.Kaplan and Sara expect this to go to clinical studies in spring - a relatively short time frame because the medication is already in FDA hands being reviewed for approval for another disorder called Duchenne Muscular Dystropy clearing the way for MPS I studies in humans sooner. I asked if the medication would be a replacement for ERT as many families appear to be under the assumption or would it be a adjuvant therapy and Dr.Kaplan said they will test it in patients on ERT infusions and believe it will be an adjuvant oral treatment to the once weekly infusions that we take (and BMT pts do not receive but will also qualify for the study) and they believe even if approved by the FDA down the road that it would not be a replacement therapy. The hope and expectation is that ataluran will get in to the blood, brain barrier and in to the bones which ERT does not do effectively. Very promising - stay tuned.

I will update other things soon - will kee updated on how this shunt stuff goes and if anything changes with the surgery, etc. For now pre-op is scheduled for tues and I also see my Cardiologist then for a Echo and 6 month appt. (so ironic that at last 6 month Echo an appt we where looking at the first shunt revision back in May.)
Take care - God Bless,


Monday, November 14, 2011

Shunt good, infection bad..

The theme of the day appears to be 'keep your friends close and your doctors closer"

Well Lisa from my neurosurgeons office (she is Dr.Bragg's nurse) called this morning - it's never hard to figure out when it isnt going to be great news but she just said we have some somewhat bad news for you. Ironically she was like I barely got done sending you an email about how happy I am your doing well with this shunt revision and Dr.Bragg walks in looking like she's about to cry. Apparently my shunt tap from last week which was testing CSF fluid grew back a bug which means the VP and the LP shunts are infected. This is never a good thing. Initially they though i'd have to come in tomorrow  for 4-5 days of IV antibiotics and then remove the 2 shunts on fri and replace the VP shunt. ( The LP shunt is tied off so not necessary we just had left it in in case we ever went back to that type of shunt gviven how difficult it was to place in my lumbar spine). We talked for a little longer and Lisa was also talking to either Dr.Bragg or one of the NPs in the background and they thought maybe we could do the IV antiobiotics at home since I have a Port but we'd need to get a home health company to do the set-up and could have likely left my Port accessed from todays infusion. Then because im supposed to go to Philadelphia this week she called me back and said they thought maybe we could do a strong oral antibiotic and Dr.Bragg paged and spoke with the infectious disease doc. They recommended a med that is used in antibiotic resistant infections and pretty powerful but thought it was fine I did it at home as long as the antibiotic was continued until surgery was done to take out and replace the shunt(s).  Because this med (zyvox) is used in such specialized populations it requires a prior auth and infectious disease referral so the rest of the afternoon was spent figuring out the PA and Dr.Bragg's nurse working out the issues. We also had to figure out when the LP and VP shunt surgery would happen which we could have done next tue but Dr.Bragg was concerned I might not bounce back for thanksgiving especially given so many other recent surgeries and this will be more than 1 shunt - many incisions.) I didnt really want to risk it knowing how I usually take a few days to bounce back and so we decided on the 1st week in Dec (Dr.Bragg will be gone the week after thanksgiving) and i'll take the oral antibiotic (zyvox) till then. Because the medication is so costly my primary insurance pharmacy benefits management company did not want to approve the med but Dr.Bragg's NP is married to one of the UW Pharmacists and he + Lisa kept on them and because they had the infectious disease medication referral they needed for the drug pre-auth - they told navitus if they didnt approve the oral med (About $5000) they where going to admit me in-patient for IV antibiotics which of course would cost far more than this. Apparently almost immediately Navitus reversed their decision and approved the med.  I liteally think I talked to Lisa no less than 6-8 times today.  Because  I have to pick the med up from UW tomorrow  also had to call and cancel/reschedule two appts I had in milw tomorrow as well as schedule a pre-op physical and call the study coord at CHOP to make sure things where fine for later this week. I also called my insurance company and secondary pharmacy benefit as well as insurance case manager a couple times. All this took place during and after ERT infusion. A little nutty! Lisa said to me on one of the later conversations something about my being so calm through all this and she thought it amazing that I just have 'the gift of calmness' when everything seems to be crazy in my life - what with all these on-going shunt issues and also all the other medcal issues I deal with and have going on on top of trying to balance a 'normal' life. I often dont feel to calm when in my head im furiously going through who I need to call for what and making sure I dont forget to either be somewhere im supposed to or in cases like this reschedule those appts I need to because of things that come up. It's nice that apparently I appear calm to other providers though and nice that some appreciate how complicated it is and how stressful it really is even if I keep a cool front. Like I said to Lisa it's not hers or Dr.Bragg's fault really that the shunt is infected - it happens and though I dont like it and hate that it means more surgery and means rescheduling everything tomorrow I certainly am not going to take it out on them. I truly think Lisa felt as bad about this if not worse and I can appreciate how hard it has to be on them to have a patient they struggle to help and get answers to and then when you just get done exchanging happy emails about how good things are finally going the crap hits the fan again so to speak. I just told her I couldnt hardly get upset at her or Dr.Bragg since they have done so much to help me and have been so sweet but that I would admit I probably wouldnt be that calm headed/collected if it was a particular provider of mine whom I dont get along with well and whom I do clash with. It's much nicer to be nice to people who want to help you and are dedicated vs people who are just in it for personal gain or the money or whatever their motive is beyond helping patients!

So that is the scoop on the latest go round with these shunts - flippin ironic that it has to be an infection in the one that finally seems to help atleast a great deal better and seemed like we may have finally hit a good answer. What are yah going to do though, right?
If I think of anything ive forgotten i'll go back and re-edit this.
God Bless - Take care,


Saturday, November 12, 2011

Good News, Good thoguhts

It hasnt actually been to busy of a week - not real low key but not real busy either and for once I actually managed to sleep in several days - this is a good sign as usually I wake up exhausted but in no way, shape or form can get myself to fall back asleep. I feel more like a human being - a human being with some achy body parts but human no less! The 2 mornings I visited my nephew and niece I think I actually had more energy to play with them and making conversation wasnt like talking through glue which also is nice. (talking through glue would be when your brain is so foggy and tired and so you can make conversation in your head but to much effort to get it out). :)  The leg spasms and low back pain + vision continue to persist and nightime has gotten a little better but is often made worse by the cardiac breathing issues but these to I think will iron out and in time will heal - hopefully not alot of time though! :)

I had sent a quick email to my neurosurgeon last night that things where going good as she and her nurses always ask to be kept up to date and honestly email is just the easiest way sometimes - this way no one has to answer a phone or listen to a voice mail and is at everyones convenience. I havent heard anything  minus on monday several hours after the shunt tap about the culture (which was negative then-see previous entry) and so asssumed it was negative which I was extremel;y happy about - if there is one thing i'd like to avoid it is the hospital (in-patient) for a good long while. I like my dr and her staff and the nurses but as we joked monday would rather not see them! :) She just emailed back this morning letting me know about a different question I had last week and she would respond soon + to keep her/them updated.   .. I often wonder how do other people/families keep their multitude of providers up to date or on the same page medical wise regarding whats going on? I think it's an important piece of a patients puzzle but since it is no easy feat I am curious!? For myself my core providers and I do a group email when there is a surgery or some unusual test result going on - this usually doesnt amount to often w/sending an email (and any one of them will respond if they have a particular piece of input) except lately because of the surgeries and so much going on often time unexpectedly. And in this case I just havent updated as often when there is surgeries because it just seems like over-kill and the same basic info just different reason. So then when I do see a provider they will ask why ive not updated and want to know whats going on. I like keeping em on their toes. ;) (just kidding)  One of the hospital systems I am at where a majority of my core providers are at has a electronic system called 'mychart' which can be used to contact providers but doesnt allow for sending a message to more than 1 provider and since a couple of my providers are outside this system ive always said i'll either send one email to the group or otherwise wouldnt do any updates and they all would be on very different pages (because it is next to impossible to get commincation to happen w/this group and getting records sent from one doctor to 4 or 5 or 6 of them is also not likely to happen and I dont plan to be a record ferrier.)  So anyways with this reminder we usually end up back at these emails and it works for the most part and equals less wasted time at appts + all have agreed they'd like the updates so it is purely their choice what they do with them. 

Tomorrow we are headed to Circus World in Baraboo - should be fun and interesting - several of us in my family have read many of the old autobiographies about the first circus's as well as how things have changed safety wise then vs now and so should be a good time.  I'll upload some pictures some time in the week ahead.

God Bless - Take care,


Monday, November 7, 2011

CSF Lab growth and re-test for potential shunt infection

I got released from UW Hospital Saturday and my Mom, sister and nephew came to pick me up once my sister got off work. We went out for supper and stopped at a few places.

Last night was pretty rough with headaches anytime I even attempted to semi-prop up either to read or when going to sleep I got  a splitting headache but then it would improve again once I was upright. I ended up falling asleep around 9 and woke again at 11 but fell back asleep sometime after midnight. This morning I was still feeling really tired, almost literally worn out or exhausted so I slept in and planned to miss ERT + go to my other appt in milw which was later in the afternoon. Not long after 9 my neurosurgeons nurse called and she said that a lab culture from weds's. shunt tap in the OR grew out a infection but that it was a possble contaminant. (The shunt tap was to see if the proximal end-the end of the catheter in the brain was working).She asked that I come in either this afternoon or tomorrow (tues). Since I skipped ERT today  I cancelled my other appt due to some other things I really need to try and get done tomorrow from last week. The shunt tap was done by the NP's at UW and they asked that I stick around Madison till they had initial lab results. I got those late this afternoon and so came back home which was good - I didnt expect not to come hom but still I didnt really want to go shopping so basically just went to one of the grocery stores I like in Madison as well as to a trendy shop in the mall looking for christmas gifts. My big whole buy was a cute teddy bear kntted type hat with a pink flower on it for part of my older nieces present. Not to long after that I called back to the nurses and the initial lab result is clear with glucose and other potential infection markers where negative so far. The neurosurgery team will cont to watch the CSF results as they come back for the next 3 days and make sure nothing grows in that time since it took til day 3 on this first lab for the potential infection to grow. Otherwise as long as things continue going well/improving and no problems i'll fup with them in a month  Cross your fingers and say a prayer!!!!

Other appts coming up (this month will be crazy due to some 6 month appt fups and also appts ive had to reschedule from last month. These include Echo and Cardiology appt at Children's in Milw, Audiology at FMLH (also milw), Urology later this week for the bladder stuff - just a fup back at UW. Pain Mngmt dr normal (ha! funny, how did any of this ever become 'normal'??)  1 month fup and probably something else im forgetting. Next week I also go to Philadelphia for the MPS I-investigational study though that will take just a couple hours and i'll see several friends who are coming in from Vancouver and who live in that area. The study is initially just a 1 time thing but because it is promising for those of us with atleast1 of our mutations being a  non-sense type and the drug is oral + may get in to the BBB (blood, brain, barrier) and bones I dont think it can hurt to do it - right now it is simply at the skin biopsy testing so will be awhile before it goes to human studies but non-the-less promising. The 2 families I will see ive met 1 and been friends with the other for several years and so we plan to have supper the night myself and Jenn's family get in and breakfast the next morning with the researchers. I never enjoy flying mostly because of the small space (some airlines/planes wayyy better than others though!) and having to sit still for so long on top of the pressure sensation in the air on head but maybe it will be better head wise this time - can hope! It always makes me incrediby tired.

I'll update when there's something to write on - take care,


Friday, November 4, 2011

Madison Neurosurgeon Dr.Josh Medow creates possible life saving device for children with Hydrocephalus

Ten years ago, working on a night shift as a resident in a Madison emergency room, Josh Medow found himself treating a child with hydrocephalus, a disease in which fluid accumulates in the brain. The child had a headache and the anxious parents feared the worst — that a shunt designed to drain the fluid had failed and potentially lethal pressure was building up in the boy's brain.
Medow realized there was no way to check whether pressure was indeed increasing, short of intrusive and painful procedures. The child ended up in the operating room.
Today, Medow, 38, and an attending neurosurgeon at UW Hospital, is on the verge of patenting a device he invented that allows doctors and even parents to easily keep track of cranial pressure in a child with hydrocephalus.
The long journey from that night in the emergency room to the invention of the tiny silicon implant that now sits on his desk is partly a tale of how medical devices come to be. But it is also a story of invention, full of twists and turns, moments of insight (that light bulb going on over the head), night-long sessions in Medow's basement where he initially cobbled together a prototype, and a trip or two to Radio Shack.
A tinkerer from age 10
Medow has long had an interest in how things work. Before getting his medical degree at UW-Madison, he earned a degree in applied life studies and biomechanics at the University of Illinois. When he was a boy, he liked to build radios. When he was 10, he bought himself a Commodore 64, a very early computer, and taught himself computer programming.
"I used to tinker," Medow said. "I worked at a Radio Shack when I was in high school."
Though he is now a doctor, Medow still views the world through the eyes of that tinkering boy. He sees problems that could be solved by devices and he thinks about how to build them.
About 700,000 people have hydrocephalus, a disease in which the body is missing the ability to re-absorb the cerebral spinal fluid that bathes the brain. That fluid is normally made and drained three times a day, Medow said.
But in those with the disease, it builds up and creates dangerous pressure that can lead to brain damage, stroke and blindness. Normally, a shunt keeps the fluid drained, but studies show half of all shunts fail within two years. Sometimes, for example, they get clogged; fluid builds up and pressure increases.
Medow couldn't stop thinking about the problem. There had to be a better way, he thought, to know whether a shunt has failed than doing surgery on the shunt itself, an operation that can cost as much as $15,000 and cause considerable pain.
That night, when he got home, he made the initial drawings for a device that could eventually be made small enough to be implanted to monitor pressure and allow parents and doctors to know whether a shunt had failed without doing invasive surgery.
Simple idea, modest tools
The initial idea was fairly simple and drew on a basic principle of magnetism and electricity arrived at by English scientist Michael Faraday in 1831: electromagnetic induction. Faraday found that when he manipulated the magnetic field around an electromagnet by closing and opening an electrical circuit he had built around the magnet, an electrical current could be detected in a separate conductor nearby.
Perhaps, Medow thought, he could build a device using electromagnets that would collect electrical signals from the brain and send them to a separate device that would translate the information into something useful — such as a pressure reading. He could use a device called a transducer to translate pressure into electrical current. The higher the frequency, the higher the pressure in the brain.
Medow went to Radio Shack and bought $75 worth of electronics gear. Working at a coffee table in his basement he cobbled together a prototype on a piece of plywood.
It worked.
Now a tiny implant
But, for a long time the prototype remained a prototype, a bunch of electrical equipment mounted on a piece of plywood that Medow had painted red. He was deep into his residency, working 100 hours a week at the hospital. Sometimes, he'd take friends down to his basement and show them what he'd made.
"They'd say, 'That's really nerdy, now let's go out for a beer,'" Medow recalled.
Eventually, however, Medow found numerous supporters on the UW-Madison campus who helped him move the work forward. They included John Webster, a professor emeritus of biomedical engineering, who became a champion of Medow's after he realized the value of the invention. Other supporters included Dr. Robert Dempsey, chair of neurosurgery at UW Hospital, and Dr. Berman Iskandar, professor of neurosurgery.
Last April, Medow was awarded a $300,000 grant from the Hartwell Foundation that would help the Department of Biomedical Engineering downsize the prototype into a tiny implant made from pliable silicon and shaped to fit neatly beneath a rounded skull.
Signals from the implant — called a transcutaneous inductive pressure monitor — will be read on a handheld device about the size of a television remote.
The final product of Medow's imagination now sits on his desk. He hopes to see it tested in human trials over the next three years.
"It's neat being a doctor and knowing there is a problem to solve," Medow said."And it's neat knowing how to build a device that seems to have the potential to work. I just knew there had to be a better way."

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Day 4, Post-op 2

Dr.Bragg came in this morning and we decided on either this afternoon or tomorrow for discharge. Well since it is tonight I am still here - I felt incredibly sick this afternoon around noon and after zofran fell asleep till about 5 after which I woke up with the headache still and very blurry vision but that dissipated after being up a while. I do think the headaches might decrease quicker with this new valve when I get up form laying down - a few moe days will show yes or no but this would be awesome as mornings and naps are definitely the hardest for the headaches and symptoms occuring! A good sign is the reservoir built-in to my 1st valve at top end of shunt (proximal end) now automatically refills and isnt collapsing thanks to the 2nd paedigav valve in my upper stomach area of shunt. This should help prevent the cyclical headaches and pressure spikes as body acclimates to new valve as my body gets used to new, better pressure and the nerve signals calm down from being so compressed for so long. My Neurosurgeon just said she thinks we havent had the right pressure setting in months and so the up and down increase/decrease in H/A's will keep improving as the nerves in brain calm down. I might still have some (lesser) H/A's at the moment but glad for good signs pointing fwd and will take this - I can take that it might take a few weeks to see if all symptoms will go away, atleast we are working towards something vs not knowing when it might or even if it will improve! She also said she thinks the blurry vision will improve as the nerve signals calm down and pressure evens out. This goes along with the fuzzy/numb feeling in my legs/feet and muscles spasms in my calves and feet as she feels those to are from the shunt not working for so long and pressure building up in the spine and already having had irritated nerves from previous lumbar spine surgeries throughout these revisions and from the LP shunt problems. I am hopeful all these things will even out and completely believe in my neurosurgeon - I truly like how committed she is to my care and to wanting to help me.  I feel like im getting more to the point especially with this admission that I can talk to her more easily - not that she ever personally made me feel like I couldnt ask her things but I just have a hard time bringing up concerns to people/asking for help and  I just never wanted to inundate her with my concerns/or questions. I think the talk her and I had yesterday was the best thing that could have been and I am incredibly thankful that she is intuitive and could figure out something was the matter on my end emotion wise and I had things on my mind regarding overall neurologic issues.

Dr.Bragg explained to me yesterday how different causes of hydro (congenital, trauma, or hydro such as from MPS) present different and react differently to shunts and thus require neurosurgeons to treat them differently - to think outside the box. She explained how often neurosurgeon treat all hydro cases regardless of the underlying cause the same and this can cause problems with how a person adapts to a shunt/how a shunt works. Anyways I liked the way she explained it and was something I hadnt really thought of before other than how hydro presents a bit diferently in MPS than in 'plain' hydro cases. So I think I am going to ask Dr.Bragg if she might be able to write down her explanation of different shunt issues in different hydro ptients and differing support needs whenever she has a moment so I could share that with my other providers as I really think it could help them understand why it hasnt been just that simple to get the right shunt fix and why we have gotten good results but then the shunts will occlude or headaches re-occur. I will share it here if she is able to write this information down.

I emailed my neurologist last night about Dr.Bragg being willing to manage my shunt and overall neurologic care (spine, autonomic, etc) and that Dr.Bragg had said if she had any questions she can contact the neurologist she works with on complex shunt patients. So Dr. Peltier said she was fine with this and would just like to be kept in the loop and I should keep her in the loop if we/Dr.Bragg ever had questions regarding past neurologic history or in general. Dr.Peltier said she felt there wasnt anything she could do that wouldnt require her consulting surgical input anyways so I think Dr.Bragg managing all issues may work well since we get along very well I think. I think Dr.Bragg has gotten an incredible idea of what my life is like overall with MPS even just  a small idea and this has really helped my overall care and our overall working together to. In some ways it feels like she understands how MPS complicates almost everything more than some providers ive had for years understand the overall situation! I see my cardiologist in a few weeks and am going to have him send his reports as well as other pertinent drs send then reports to Dr.Bragg as I think she of anyone cares to get them and be involved.  Sometimes I feel like when it comes to providers I should pinch myself, how did I get lucky enough to find providers like her and a couple others I have?????

More soon - hopefully getting out for real tomorrow - my sister, mom and nephew are coming to pick me up and go out for supper on the way home! So excited!!


Thursday, November 3, 2011

Surgery (revision) #10, VP revision # 2

Surgery went well yesterday see below for more info but affter testing Dr.Bragg didnt need to revise the portion of my shunt in my head again so she just ended up disconnecting and placing the second valve in my upper stomach area. Total surgery time was apparently 1 1/2 hrs from anesthesia to Dr.Bragg finishing with Dr.Bragg's portion taking only about 25mins. I didnt sleep very well last night either and got about 3-4 broken up hours of sleep.

Dr.Bragg when she comes by especially after surgeries always has funny sayings she comes up with to compare what she has done to something in real life in order to make it more understandable and in my case she wanted to make me laugh and said when she was trying to get the valve in to the tissue all she could think of was how my skin and tissue is just like rubber.  She said the 2nd valve placement (a paedigav valve) should have taken 5 mins but took 25 mins to place  due to shunt tubing being adhesed in to tissue already and catheter moving around quite a bit when it should have stayed in it's placement. She said it's very unusual for scarring and buildup to occur in such a short amount of time as my scarring did (about a month 1/2). I mentioned how with IV's my skin is the same way and especially at ERT infusion the nurses always commented how tough my skin was and this is really common in most MPS pts. Interestingly she said the skin in my head is no where near this tough to navigate catheter placement through. Apparently catheter in the chest and stomach should slide right in like a necklace against skin and much like kids linking blocks should be easy to connect pieces together.

I have been so tired the past 2-3 days due to so little sleep and having an incredibly hard time falling and staying asleep that I have been emotional today and was in tears of frustration with symptoms (low back, muscle spasms, vision and just wishing it and the headaches would go away). She first said how again she wasnt going to abandon me and I shouldnt worry about telling her things - she wants to help and the worst she can say is "zi dont know" but that she will always try to find answers for me be it the headaches or other things going on and will be honest with me and wasnt going to leave me care. She just mentioned how it helps her for me to tell her whats going on so she can get a small snippet of what my life is like and helps her in making decisions for us/my care  I asid to her how I appreciate this and just need to learn that she is different and isnt going to suddenly change and say "I dont know what to do with you anymore" or something like that. I also said how even though I have a hard time telling her when different things are occuring health wise and asking for her help she is so different from many providers ive had and I didnt know if she realized it but she really is a good dr and good at listening to/helping her patients. She said the low back pain and muscle spasms could be from when shunt isnt working and pressure building up. She is going to try a muscle relaxer as needed to help and thinks it may improve although may make me tired so we'll just try it today while im here and see if it does anything for the spasms.   They are looking at relistor to see if it would help with GI affects (constipation) of pain medication per GI drs thoughts. Did Xrays this morning on shunt which is generally her standard protocol after surgeries to make sure it's all together and right.  She also talked about how when she came up to the floor to find my parents she was talking to the nurses explaining to them why my shunt and situation is so different from alot of her patients and explaining why we've had to do so many revisions. She said the nurses told her how much they like me (so sweet of them) and she said it was good for her to be able to explain to them why my case is a little different and how MPS factors in + complicates the situation and her and I have to find the right fit. Why cant all healthcare providers be this down to earth and caring? Granted I have some very good providers but non quite like Dr.Bragg - I think the biggest thing I told her is with some of my providers I do feel like I am banging my head against a wall trying to explain this shunt stuff and surgeries and she said that she really tries to think outside the box and no 2 patients of hers are alike + she thinks with hydro and shunts thats why some providers have such a hard time getting when there are may issues.

Below is a entry from yesterday -

10:42am - Nov 2nd.
No certain time for surgery due to it being an 'e-case' but neurosurgeon stopped by and she is planning to re-explore shunt and will place the second valve (usually in upper chest but due to my thin frame she plans to place it in my stomach area of the shunt) which the idea is this will slow down/provide added resistance to the CSF which the 1st valve isnt keeping up with and thus prevent ventricles from collapsing and occlusion. Thinks it wont be a long surgery.

Be thankful for those around you who want to help, take time to listen and care! Be it family, friends or providers even!


Wednesday, November 2, 2011

Back in UW Hospital admitted through ER, surgery Weds.

Just a really quick update - i'll post more details likely tomorrow - but I called my neurosurgeons nurse today and filled her in on this weekend/the past few days since thurs when I saw my neurosurgeon and how things havent improved. She asked a few questions and specifically about the throwing up ive experienced again in the past few days since I got the staples out and as these headaches seemed to progress again. Anyways Lisa called me back after talking to Dr.Bragg and initialy they where going to find a spot on the surgery schedule for the next week but because of the vomiting they asked that I go to the ER because they wanted me to be admitted and to put me on for an 'elective' surgery tomorrow but the only way to do this since it isnt one of my neurosurgeons normal surgery days was to admit me through the ER. Initially my Dad and I had a bit of a wait as the ER was backed up but then once we where in a room things moved swiftly as Lisa and Dr.Bragg had put in orders that surgery was going to be tomorrow and I was to be admitted to D6/4 (neurosurgery floor) from ER. A short brain MRI was done and many labs as well as the neuro assessment by the ER doc and Neuro-Resident. From ER room to being on D6-4 the time was less than 2 hours which is actually quite impressive. Once on the floor quite a few more labs where drawn after the IV Access Team accessed my Port and a EKG was done as well as 2 X-rays. Ive had zofran (throwing up) and dilaudid by IV but otherwise just chilling in my room here with the headaches occuring intermittently. I'll see in the morning what time the surgery will be and if it will in fact occur tomorrow (always the possibility it could get pushed back because of a full schedule). From what I understand the plan is to explore the shunt again and then likely place a second valve on the shunt which would slow down CSF and thus prevent the hope is the ventricle from collapsing and causing headaches and shunt failure. I'll post more in my next update on what the surgery will be or correct info about the thinking behind this second valve if I am posting anything wrong here.

All for now -  I hope and pray this is the last surgery for a while, a long while with this shunt!
Thanks for prayers,

Monday, October 31, 2011

The Carrot, the Egg and the Coffee Beans...Adversity..

I liked this essay below it strikes a note I think because I sometimes dont quite know how to describe myself - Ive been described as resilient by alot of people but I also know inside I struggle sometimes (as do all people whether we admit it or not) to with all that is out of my control and finding a balance in the control I do have. ..

These past 6 years havent always been my finest moments, sometimes I am to argumentative and I realize and feel bad about this but yet other times feel no dismay at my actions because I also dont want to be a stone that others step on. I think ive grown and matured but I also know I certainly can still become even better both as a patient, a friend and as a sister, daughter, etc. I know my stubborness is an asset but I also know it can be a downfall and ive tried very much to reign in my opionated self but I know im far from perfect. I dont always care what people think of me and yet I care to much what certain people think. I want to be liked but yet I dont need permission to be someone other than who I am. I think I am fiercely loyal if you are kind, helpful or just listen and are there for me. I love what I get to do for others but have no illusions that I do things perfectly because I dont always. I want to be liked but I dont need to be liked.

I feel like over the past 4-5 months ive sort of put alot of things on the back burner including many friends and other than immediate things ive not been the best leader I can be for APF (my thoughts -ive never gotten anything but encouragement from managers/fellow leaders.) but I am always here for patients/others needing advice on pain and I will always work tirelessly (tiredly?) behind the scenes when not out in the open working on projects. I feel like other than my closest (distance wise) niece and nephews ive not been a great aunt but I also know the others are always in my thougts and as their aunt i'll always worry endlessly about how they are doing. Other than this new project for MPS I/Genzyme which hasnt been all that time consuming (a few hours a week generally)  ive not done much around MPS and feel so incredibly out of that loop but yet I dont think there is alot ive missed I suppose it's just a weird feeling since that is where I really started with advocacy.) Often these past 4-5 months ive looked back at the things ive done and it feels more like a cloudy memory than an actual memory I think because of so much going on H/A and this tiredness wise. I dont quite feel like there is a 'normal' routine to my life yet even 4-5 months later since that first surgery but I think that isnt really true (it just feels like it is because it's just different and a different normal right now than pre-May). This isnt to say that im not happy I had these shunt surgeries (and in general!!) - sure I wish the first one would have worked for longer than it did and the same wish with the subsequent revisions but there's no sense mulling over what I cant change about my body (finicky in-ability to regulate CSF with these shunts properly I guess). Im glad that I have the neurosurgeon I do whose willing to help and willing to stick with my care in the long run. I hope we get this figured out soon and I hope in the new year to figure out other aspects of my care that puzzle me or make me scratch me head in wonder. In all I guess this post is really just my mumblings and thoughts on what the past few months have taught me and what I think im trying to learn as time continues to march on with and despite MPS.

My conclusion at the end of this essay is that I think I was the carrot and am now the egg but hope to become the coffee bean and change those around me but never stop realizing that i'll never be able to change every situation and so instead a balance - a egg because it represents flexibility and adaptability and the coffee bean becomes it represents the ability to change the situations around you for the better. ...

A young woman went to her mother and told her about her life and how things were so hard for her. She did not know how she was going to make it and wanted to give up:
She was tired of fighting and struggling. It seemed as soon as one problem was solved, a new one arose.
Her mother took her to the kitchen. She filled three pots with water and placed each on a high fire. Soon the pots came to boil.
In the first she placed carrots, in the second she placed eggs, and in the last she placed ground coffee beans. She let them sit and boil; without saying a word. In about twenty minutes she turned off the burners. She fished the carrots out and placed them in a bowl.
She pulled the eggs out and placed them in a bowl. Then she ladled the coffee out and placed it in a bowl.
Turning to her daughter, she asked, 'Tell me what you see. ''Carrots, eggs, and coffee,' she replied. Her mother brought her closer and asked her to feel the carrots. She did and noted that they were soft.
The mother then asked the daughter to take an egg and break it. After pulling off the shell, she observed the hard boiled egg.
Finally, the mother asked the daughter to sip the coffee. The daughter smiled as she tasted its rich aroma. The daughter then asked,'What does it mean, mother?'
Her mother explained that each of these objects had faced the same adversity: boiling water. Each reacted differently.
The carrot wentin strong, hard, and unrelenting. However, after being subjected to theboiling water, it softened and became weak.
The egg had been fragile.Its thin outer shell had protected its liquid interior, but after sitting through the boiling water, its inside became hardened.
The ground coffee beans were unique, however. After they were in the boiling water, they had changed the water.
'Which are you?' she asked her daughter. 'When adversity knocks on your door, how do you respond?
Are you a carrot, an egg or a coffee bean?
I'd like to hope I was the carrot and became the egg and will become the coffee bean...

Trick or Treat

This weekend my sister, brother, sister-in-law and sister + brother-in-law took 2 of my nieces and my 2 of my nephews (ages 2 @ 5yrs, 1 at 9 yrs and 1 at 9mo) trick or treating - this was on sat and the kids where a witch, a ladybug and a bat - i'll include a few pics in this but it was alot of fun with reasonably warm weather - I forgot to charge my camera so this died not even 20mins in to the outing but my sister-in-law took over a hundred pics so I am really looking fwd to seeing those to! Bella is really to young to trick or treat of course but she seemed to like watching everything and Quinn, Zander and Jordan had a blast and we had a wagon along so my brother took turns pulling my niece and nephews and also Jenn and I. Ive to say my body wasnt any to pleased to walk around for close to 2 hours as my low back has been acting up again lately much like after May's laminectomy/shunt revision (this seems to be since the :LP shunt was tied off last month for whatever reason) and my hip was also trying to kill me on top of forgetting to wear better shoes (had flats that I tripped over just about any and every pot hole). Regardless of these aches and the cramps that came with it later in my leg it was to fun to walk around and take pictures and catch up with my brother and sister-in-law and just the best to watch the kids faces when they'd get candy they'd run back and jump around with excitement. Afterwards we went to my parents for supper (my Mom is a fantastic cook I always joke when I want a real meal I go to my parents to visit) :)

As far as headaches go ive continued to have these intermittent ones that are worse in the mornings or if I dont prop up enough at night and then occur on and off which my neurosurgeon thought was due to something with resistance and flow mechanics in the way this VP shunt is set up. We had decided to wait the weekend to see if this got better and so if it doesnt improve by tomorrow or weds i'll probably call her nurse. I am really ambivalent about this all and honestly even though I know she wants to help I just feel like a bad patient when these issues cont. Ugh, like ive said before I had being a patient not just with this but overall I hate it.

I finally called and made an appt with my hand surgeon for my L hand (writing hand) due to the thumb issues that have been worse since last spring and which get extremely painful (apparently my body is on a quest to hate me and thus get me to hate it!) :) This appt is later in Nov because of the travel to PA mid-Nov and other things going on next week. It has been a year since I got the cast and splint off from my R thumb tendon transfer and since I saw my Hand surgeon but ironically the secretary still remembered me - apparently I spent to much time there last spring and the previous year with that surgery to. In some ways it's nice when doctors office staff knows you but on the other hand it's almost like 'wow, do I really talk to these people that much that they recognize your name/voice/etc when you call? I guess it never ceases to amaze me really even though maybe it should aftter all these years. 

I also have a 6 mo echo (heart ultrasound essentially) and fup with my cardiologist in Nov and if good then it would mean the pulmonary venous hypertension, L Atrial enlargement and Aortic + mitral valve issues havent worsened. Given im not a big fan of the cardiac meds im already on I am hopeful it will be good! Tomorrow I also have a audiology test (yippee) and that to will hopefully go very well as I sit on the border of needing hearing aides for high frequency loss. I doubt id wear them anyways much like I dont wear my glasses

So there's alot going on this month - I will update when I know more headache wise and will update here and there on the other appts to.
Be well - stay warm!


Thursday, October 27, 2011

Neurosurgery fup appt, picture of ventricle

It has been a exhausting week - I know I should rest more but really I havent done anything that was soo strenous it's more that when I do lay down I cant sleep (I did take a nap 2 nights) and when I go to bed feeling worn out I take hours to fall asleep to. The past 2 nights have been especially bad with going to bed around 10:30-11:30 and not falling asleep till 2 or so in the morning and this morning I had to get back up at shortly after 5 to get over to my sisters as I was watching my youngest nephew till 12. Tomorrow in the same except i'll be watching him to 2 when I drop him off at his Dad's and will also have to drop off and pick him up at his kindergarten class.

                     Inside the VP shunt catheter at the ventricle/proxumal end of the catheter.

I saw my neurosurgeon today she took out the gauze and staples (there are sutures in) and we talked about how I am feeling. I always hesitate to honestly tell her when im not feeling a whole lot better not because I dont trust she wants to keep helping at this point but bc I dont want to sound whiny. Ugh sometimes I hate being a patient!!! She said it's possible the shunt could have clogged off again with debris because there isnt enough resistance and we talked more about putting in a second valve. We decided to give it through the weekend to see if these H/A's and nausea improve at all and I will call her early-to-mid next week to let them know how I am doing. She also said if it wasnt improved she would have me come back in, she would fit in a spot and she would repeat the scans. She explained how the thinking is that a 2nd valve could provide more resistance to the CSF flow and thus prevent the ventricles from collapsing under the extreme pressure/flow occuring due to CSF and my single shunt valve not being able to keep up and thus the ventricle collapses in on itself as CSF flows until so much pressure builds up they almost explode (my word) open again against the forced pressure. She thoguht this could very well explain my cyclical up and down headaches. (the ventricles collapsing due to not enough resistance and being forced open because of pressure).

When she went out to get the staple remover kit she must have run down to her OR locker as this was where she had told me last weekend she had a picture for me from the surgery. This is a 8x10 shot from the scope camera she uses to thread the catheter in to my ventricle and that she used to see what was going on with my shunt last fri. The white spots scattered around the opening are body matter and she said although the picture doesnt show it these where scattered all throughout the upper (proximal) portion of the catheter and definitely the cause of the shunt failure. The picture shows the tip of the ventricle and then there is black space further in the hole and this is the tunnel created by the catheter (to use her description much like when an animal burrows underground and into the dirt but they leave behind a tunnel that doesnt fill up) - apparently this will always be the way my ventricle and brain look regardless if the shunt was takenout (which it cant be), Drfinitely kind of fascinating! Apparently also the majority of neurosurgeons do not use scopes to place shunts and she herself said she rarely used them before coming to UW/AFCH but the other neurosurgeon there convinced her to learn and it helps her better be able to thread catheters in to very small ventricles like mine. Interesting stuff to learn! (to use another of her analogies I said something about how I thoought being able to take the pictures would be fun but wouldnt want to do the rest (not because of blood and stuff just wouldnt be good at it!) and she jokingly said 6 year olds could probably do it well these days because of video games - having to operate the remote while looking at a screen to get the visual of the problem areas. I will forever laugh and think of neurosurgery in a funny way when my older nephew is plying video games here. :)

I'll update other things soon -
Take care,