First let me apologize for any sp and grammar errors in this post as I am unsure how to enlarge this screen (to make it more visible/less blurry) and well it is just that VERY blurry. =/
I called Dr.Bragg's ofc yesterday and talked to her partners nurse who in turn talked to Dr.Bragg + then Catie (nurse) called me back. She (Dr.Bragg) was just going to try calling this other dr she was wanting to talk to and told Catie to let me know she would call me back today (Weds). Ive not heard anything today so assume Dr.Bragg must be waiting to hear back from this other dr. which is understandable they all have busy schedules. I admittedly cant help but wonder though how come in cases like this our drs cant or dont have their nurses or secretary's let us know they are waiting to hear back, where they are in the process or that they will get back to us as soon as they can? I would sure feel alot better. =/
I get it, I really do and so I get this probably sounds harsh to which I dont mean for it to I guess I just feel so badly and so little energy and you sometimes just cant help but feel like your watching life pass by... By that I mean you dont really have the energy to do much, you dont feel great at all and your taking the max you can on the meds you have some of which are already being given in unconventional ways (IV Zofran, Ativan some nights oral)... and those arent always doing enough or anything though thankfully that isnt the case all of the time, just some of the time.
Instead you do those things you are committed to and reschedule the things you can, put off the specialists yet again that you can (Neuro-opthalm, Neurologist-for botox, Urologist for bladder symptoms, trip to Mn, etc) over and over and over again.. though some or many of the things are supposed to be done on a routine basis for maximum effect or in the case of Mn for study reasons. What can you do? To be perfectly honest you begin to doubt yourself, wonder if your depressed even though you know w absolute conviction your not but still it's still a nagging voice sometimes... Sometimes for me the hardest is how hard a simple thing like reading a book is, bc the words and pages blur together and so you spend more time getting frustrated trying to make out the words than you do reading the actual story line. Reading has always been a favorite past-time of mine, extremely relaxing and just nice I suppose bc you get lost in something less meaninful for awhile. I definitely would like normal vision so shunts please just work a little harder, please? Thank you!
Tomorrow's another day in which i'll pray I wake up feeling better, have a little more energy, feel a little more optimistic about all this shunt stuff and in general hope i'll feel a little more as if im letting people down a little less. With God all things are possible and so in him I must put my trust!
If you believe in it as I do please say a prayer I will hear from Dr.Bragg tomorrow. Honestly ive had a few moments tonight ive considered going to the ER but then I feel like what would that solve? We still wouldnt know what to do, id just end up (likely) back at UW and still without a plan so instead i'll stay here and hope for better days and answers and a call with some thoughts from Dr.Bragg.
Thanks for stopping by, my apologies if this seems melodramatic (or something), it's just how I feel,
Erica
Ps: Tomorrow Febr 28th is Rare disease Awareness Day, toast to a year of better answers, better treatments and more researchers studying these disorders - in my case especially a researcher(s) studying the CNS storage issues and headache issue in MPS would be wonderful.
Wednesday, February 27, 2013
Sunday, February 24, 2013
It's hard feeling this way, it's hard to wait..
Its now sunday night and a pretty quiet weekend (an ok thing) while really trying to not think to much about what might Dr.Bragg decide (w the Neurologist she planned to talk to tomorrow (monday) ) regarding my case and our on-going shunt issues. It's one thing to try and not think to much about something and an entirely different thing to actually keep it out of your conscious thought.. =?
I dont know that I will know anything tomorrow but when I get home from ERT (infusion) god willing if I have heard from Dr.Bragg and she has any news or updates or if she was able to talk to this Dr.Hsu (Neurologist) about me I will post here. I probably wont take my laptop with me but if I hear anything early in the day (very unlikely) will post to fb as well.
Please say a prayer she is able to talk to him and they can come up w a plan to potentially fix the shunts better.
I pray we can figure out a possible solution to try,
Erica
In between alot of time finishing prepping my sunday school lesson on Friday night and Sat (to in turn have just one kid show up!) I spent a fair bit of time trying to find articles talking about 'meningeal thickening', GAG storage in the brain, (Can I just say why isnt this being studied more???) Obstructive Hydrocephalus in MPS I, (again not much actual useful info) and other possible insightful brain related MPS I sources. =/ ARGGHHH!!
In all actuality I feel just as frustrated with just as much a lack of information and wish, just really wish some researchers would take an interest in the headache/storage issue in the CNS of MPS I pts. It's a very real and under-studied issues so whats the issue??
I dont know that I will know anything tomorrow but when I get home from ERT (infusion) god willing if I have heard from Dr.Bragg and she has any news or updates or if she was able to talk to this Dr.Hsu (Neurologist) about me I will post here. I probably wont take my laptop with me but if I hear anything early in the day (very unlikely) will post to fb as well.
Please say a prayer she is able to talk to him and they can come up w a plan to potentially fix the shunts better.
I pray we can figure out a possible solution to try,
Erica
Thursday, February 21, 2013
Neurosurgery Fup, 8 yrs diagnosed...
Ugh, someday will I ever not be here as I am now writing about these stupid, blessed headaches and related symptoms? I should sure hope so but I guess I thought maybe the time would have already come and gone... and it hasnt... instead I am revisiting the same symptoms again and again as we try to figure out what to do. =/
Dr.Bragg talked to Dr.Dickson (of the IT studies) and which I kind of wondered if it wouldnt be this way as the current study is set up and bc of my VP shunt (drains) I wouldnt be eligible for the study. My shunt alone doesnt precluse me but the way they give the dose is by lumbar puncture and my low back is so scarred from all the previous LP shunts and related nerve root surgeries that doing lumbar punctures is pretty much not an option. Their study is also not set up for anything but looking at cognitive function outcomes and is not looking at could IT aldurazyme be given through a CSF reservoir to help clear the mucopolysaccharide (GAG) storage from the shunts and thus keep them working better.. We need/wanted to try IT to see if it could help clear the MPS (GAG) storage from the shunts and the meninges which is what Dr.Dickson told Dr.Bragg she feels is adding to our shunt issues as the meninges get thicker, stickier and clogged w the storage and then when this gets sucked in to the shunts it causes the shunts to gunk up in a sense. So while the shunts may flow it probably isnt enough and the storage is clear so it cant really be seen sticking to the shunts (If I got this right) like other tissue can be w their scopes. Also bc it is more of a compliance issue (brain isnt able to expand properly I guess, nor are ventricles) and less of a high pressure issue when the shunts do drain at the lower settings I feel best at (I feel better draining alot of fluid as when we do the external drains) then the ventricles collapse causing a suction effect which then causes the shunts to clog or intermittently clog. A crappy cycle. Goes back to my feeling anytime we've replaced the entire shunt(s) for one reason or another i've done really well albeit for short periods (weeks) while when we revise just a portion of either shunt say the valve we dont seem to get as good of results., maybe bc there is storage throughout the shunt?
Dr.Bragg is worried about the cardiac issues and asked when Dr.Earing thinks we will get the valves (severe stenosis) fixed as that would make her part easier as the Anesthesiologists now she said (and I believe) are unhappy w all the surgeries we've done as their job is alot harder between my airway issues + tacking on the additional monitoring needed bc of the stenosis. The severe valve issues require much closer monitoring w/ Arterial line, careful fluid balance, etc. Dr.Earing in Jan had wanted to wait if possible atleast 6 months to do valve replacements but at minimum 3 months to have me seen by Cardiac surgery (whoever we decide on) to give my immune system a chance to recoup from the shunt infections in Dec. A sobering fact unrelated to the shunts but related to the cardiac issues? Most people will not live 3 years w severe stenosis; I think these cardiac issues are my "sticking my head in the sand" issue bc that was awesomely sobering but at the same time I am doing what I can and God has a plan + I have good drs. doing the best we can.
We talked about many things including she is still thinking about cranial expansion but doesnt want to put me through that unless she'd have a better idea it could work. Its a way last ditch thing for us I think but time will tell I guess. The one thing we keep coming back to but dont know how to bi-pass is some kind of a internal mirror of the actual external ventricular drains as those and the pseudomeningocele's (CSF filled pocket) are what we've had fantastic results with while the shunts have been very short term "fixes" with a few weeks at most. She wondered if there was a way to create an internalized drainage point that would act the same as a EVD w the same continous flow while providing the needed back pressure to keep the venticles open and able to drain. I cant remember her explanation well but something about EVDs provide for a continous backup due to atmospheric or barometric pressure (??) so it's a constant drain whereas a shunt in my issues is only intermittently draining due to the compliance issues. I think that is something she may keep thinking about or asking around about as she and her partner do have several pts or atleast one patient anyways and she knows of others who instead of shunts have an external drain and drains in to an external pocket. We need this but a way to drain internally to avoid the infection risk due to all my other issues.
She is talking to the Neurologist she works with on her complex shunt patients; they have spoken before about me but she wants to see if he will help her try to come up with a plan or does he have other suggestions she might try. In the past the week I was going to see him with her I ended up getting sick and admitted the day before the joint appt and so he saw me in the hospital but I was so out of it and they opted to try something else. I think that may be when we first switched from the LP shunt to the VP shunt after doing ICP monitor and the External drain. She said she would call me monday which makes it 4 long days away and 4 long days to be nervous and though I dont often worry about care issues I do worry when it comes to these kinds of issues. I just truly hope Dr.Bragg doesnt give up and they are able to think of something together that might help/we could try. Its so frustrating (!!!) that we have such incredibly good results w the external drains and seems so simple (I know its not really) that we should be able to replicate. Wish it was so easy!
We'll revisit the IV Zofran when she calls on monday as my PCP Dr.Bragg commented must have said something to her (Dr.Bragg) about being concerned w the infection risk with our doing IV zofran through the continously accessed (but covered) Port. This has been a godsend so admittedly just one more thing to stress a little about. The zofran isnt perfect and I still have no appetite (Dr.Bragg herself as did my Pain Mngmt dr earlier in the day commented that I look like ive lost weight.) but atleast I am not throwing up here, there and everywhere in the mornings and at night when I am first up (first few hours are worst) and when trying to sleep at night is the other very worst.
Ironically today, 8 years ago my then GC called to tell me the blood results where back for the MPS testing (urine had come back a week or two earlier so we kind of knew, just not a type) and was positve. Good news then bc I had a name finally (though my Cardiol. had told me 2 or so months before he suspected it was a form of MPS). bad news bc who could have known it would be this crazy? Seriously? In that 8 years ive had atleast 2 surgeries every year I believe and years like the last 1 1/2 have had 20-some in just a 1 1/2 year period. Again, CRAZY!
Im sure I am forgetting something but thats todays not un-expected but no less hard (disapointing, though not in anyone, just in general) news post-appt fup.
On a un-related note I teach sunday school for the first time since early Jan this sunday and our kids are practicing a new song but otherwise pretty un-prepated. Will be nice to see the kids but could use a boost of energy... =/
There's no manual how to do this disapointing news over and over and over again and is just as hard each time.
Thanks for stopping by, if you believe in it pray we figure something out,
Erica
Dr.Bragg talked to Dr.Dickson (of the IT studies) and which I kind of wondered if it wouldnt be this way as the current study is set up and bc of my VP shunt (drains) I wouldnt be eligible for the study. My shunt alone doesnt precluse me but the way they give the dose is by lumbar puncture and my low back is so scarred from all the previous LP shunts and related nerve root surgeries that doing lumbar punctures is pretty much not an option. Their study is also not set up for anything but looking at cognitive function outcomes and is not looking at could IT aldurazyme be given through a CSF reservoir to help clear the mucopolysaccharide (GAG) storage from the shunts and thus keep them working better.. We need/wanted to try IT to see if it could help clear the MPS (GAG) storage from the shunts and the meninges which is what Dr.Dickson told Dr.Bragg she feels is adding to our shunt issues as the meninges get thicker, stickier and clogged w the storage and then when this gets sucked in to the shunts it causes the shunts to gunk up in a sense. So while the shunts may flow it probably isnt enough and the storage is clear so it cant really be seen sticking to the shunts (If I got this right) like other tissue can be w their scopes. Also bc it is more of a compliance issue (brain isnt able to expand properly I guess, nor are ventricles) and less of a high pressure issue when the shunts do drain at the lower settings I feel best at (I feel better draining alot of fluid as when we do the external drains) then the ventricles collapse causing a suction effect which then causes the shunts to clog or intermittently clog. A crappy cycle. Goes back to my feeling anytime we've replaced the entire shunt(s) for one reason or another i've done really well albeit for short periods (weeks) while when we revise just a portion of either shunt say the valve we dont seem to get as good of results., maybe bc there is storage throughout the shunt?
Dr.Bragg is worried about the cardiac issues and asked when Dr.Earing thinks we will get the valves (severe stenosis) fixed as that would make her part easier as the Anesthesiologists now she said (and I believe) are unhappy w all the surgeries we've done as their job is alot harder between my airway issues + tacking on the additional monitoring needed bc of the stenosis. The severe valve issues require much closer monitoring w/ Arterial line, careful fluid balance, etc. Dr.Earing in Jan had wanted to wait if possible atleast 6 months to do valve replacements but at minimum 3 months to have me seen by Cardiac surgery (whoever we decide on) to give my immune system a chance to recoup from the shunt infections in Dec. A sobering fact unrelated to the shunts but related to the cardiac issues? Most people will not live 3 years w severe stenosis; I think these cardiac issues are my "sticking my head in the sand" issue bc that was awesomely sobering but at the same time I am doing what I can and God has a plan + I have good drs. doing the best we can.
We talked about many things including she is still thinking about cranial expansion but doesnt want to put me through that unless she'd have a better idea it could work. Its a way last ditch thing for us I think but time will tell I guess. The one thing we keep coming back to but dont know how to bi-pass is some kind of a internal mirror of the actual external ventricular drains as those and the pseudomeningocele's (CSF filled pocket) are what we've had fantastic results with while the shunts have been very short term "fixes" with a few weeks at most. She wondered if there was a way to create an internalized drainage point that would act the same as a EVD w the same continous flow while providing the needed back pressure to keep the venticles open and able to drain. I cant remember her explanation well but something about EVDs provide for a continous backup due to atmospheric or barometric pressure (??) so it's a constant drain whereas a shunt in my issues is only intermittently draining due to the compliance issues. I think that is something she may keep thinking about or asking around about as she and her partner do have several pts or atleast one patient anyways and she knows of others who instead of shunts have an external drain and drains in to an external pocket. We need this but a way to drain internally to avoid the infection risk due to all my other issues.
She is talking to the Neurologist she works with on her complex shunt patients; they have spoken before about me but she wants to see if he will help her try to come up with a plan or does he have other suggestions she might try. In the past the week I was going to see him with her I ended up getting sick and admitted the day before the joint appt and so he saw me in the hospital but I was so out of it and they opted to try something else. I think that may be when we first switched from the LP shunt to the VP shunt after doing ICP monitor and the External drain. She said she would call me monday which makes it 4 long days away and 4 long days to be nervous and though I dont often worry about care issues I do worry when it comes to these kinds of issues. I just truly hope Dr.Bragg doesnt give up and they are able to think of something together that might help/we could try. Its so frustrating (!!!) that we have such incredibly good results w the external drains and seems so simple (I know its not really) that we should be able to replicate. Wish it was so easy!
We'll revisit the IV Zofran when she calls on monday as my PCP Dr.Bragg commented must have said something to her (Dr.Bragg) about being concerned w the infection risk with our doing IV zofran through the continously accessed (but covered) Port. This has been a godsend so admittedly just one more thing to stress a little about. The zofran isnt perfect and I still have no appetite (Dr.Bragg herself as did my Pain Mngmt dr earlier in the day commented that I look like ive lost weight.) but atleast I am not throwing up here, there and everywhere in the mornings and at night when I am first up (first few hours are worst) and when trying to sleep at night is the other very worst.
Ironically today, 8 years ago my then GC called to tell me the blood results where back for the MPS testing (urine had come back a week or two earlier so we kind of knew, just not a type) and was positve. Good news then bc I had a name finally (though my Cardiol. had told me 2 or so months before he suspected it was a form of MPS). bad news bc who could have known it would be this crazy? Seriously? In that 8 years ive had atleast 2 surgeries every year I believe and years like the last 1 1/2 have had 20-some in just a 1 1/2 year period. Again, CRAZY!
Im sure I am forgetting something but thats todays not un-expected but no less hard (disapointing, though not in anyone, just in general) news post-appt fup.
On a un-related note I teach sunday school for the first time since early Jan this sunday and our kids are practicing a new song but otherwise pretty un-prepated. Will be nice to see the kids but could use a boost of energy... =/
There's no manual how to do this disapointing news over and over and over again and is just as hard each time.
Thanks for stopping by, if you believe in it pray we figure something out,
Erica
Sunday, February 17, 2013
Home (Again).... Wishing to feel better..
I am home and while happy to be so I wish I felt better. I hate this and hate these headaches + other symptoms. Sure I can function in the afternoons and if I take the IV Zofran in the a.m am able to eat better but I dont have a great appetite although by supper it tends to get a little better. Appetite in general isnt anywhere near as good as when we have had the shunts working splendidly well. I dont think the shunts are not working I just dont think it is enough fluid that is being taken off with the shunts set where they are but when I do drain at the lowest setting which should be alot of CSF (fluid) I initially feel much better but then my ventricles collapse and thus the catheters collapse causing intermittent occlusions and the eventual problems with the shunts leading to all the revision surgeries.
I am supposed to follow up with Dr.Bragg on Thurs and am praying she has been able to talk to Dr.Dickson by then and that she will have some thought on trying this CSF reservoir for taking off extra CSF (in addition to the shunts) as I am tired, just really tired of these headaches. It is apparently supposed to snow on Thurs and I am praying that the snow will hold off or the weather people will be completely wrong this time. I have an appt in Milw and then the appt w Dr.Bragg in Madison and really need to go to both Appts. The second appt is for my Pain Mngmt dr and for med refill + I havent seen this dr since early Nov. due to the long Dec and Jan stays + short interval in-between those 2 in-patient stays. She and Dr.Bragg talked several times during this last in-patient stay and Dr.Bragg's team wrote for the long acting pain patch during these past two stays in Jan/Febr and Dec but I need to get the patch and immediate release med refilled + see about other ideas she might have to help.
This is short but please say a prayer for Thurs's appt and that I am able to figure something out with Dr.Bragg + the snow holds off. Also pray the rest of the weekend and busy week ahead (ERT monday, Dentist in Milw Tues (if up to it), and Weds an Endocrine Appt., followed by the 2 appts on Thurs in Milw and in Madison. Oh vey.
One other note, Dr.Bragg's NP asked that I call her monday to let her know how I was doing and check in so I pray maybe she will have some update from Dr.Bragg; that would be ideal. .. Ive said it before and am sure this wont be the only time I say it again but I strongly dislike waiting for answers or waiting to hear from providers what they are thinking as far as a "next step". If anything stresses me out it is definitely this about my disorder and care.
Anyways enough of my venting, does it feel like groundhog day alot when you (my reader(s) come by and read this as it seems always to be about the shunts, often to be about my frustration at the shunts and my wishing we could figure this out sooner rather than later. =/
Thanks again,
Erica
I am supposed to follow up with Dr.Bragg on Thurs and am praying she has been able to talk to Dr.Dickson by then and that she will have some thought on trying this CSF reservoir for taking off extra CSF (in addition to the shunts) as I am tired, just really tired of these headaches. It is apparently supposed to snow on Thurs and I am praying that the snow will hold off or the weather people will be completely wrong this time. I have an appt in Milw and then the appt w Dr.Bragg in Madison and really need to go to both Appts. The second appt is for my Pain Mngmt dr and for med refill + I havent seen this dr since early Nov. due to the long Dec and Jan stays + short interval in-between those 2 in-patient stays. She and Dr.Bragg talked several times during this last in-patient stay and Dr.Bragg's team wrote for the long acting pain patch during these past two stays in Jan/Febr and Dec but I need to get the patch and immediate release med refilled + see about other ideas she might have to help.
This is short but please say a prayer for Thurs's appt and that I am able to figure something out with Dr.Bragg + the snow holds off. Also pray the rest of the weekend and busy week ahead (ERT monday, Dentist in Milw Tues (if up to it), and Weds an Endocrine Appt., followed by the 2 appts on Thurs in Milw and in Madison. Oh vey.
One other note, Dr.Bragg's NP asked that I call her monday to let her know how I was doing and check in so I pray maybe she will have some update from Dr.Bragg; that would be ideal. .. Ive said it before and am sure this wont be the only time I say it again but I strongly dislike waiting for answers or waiting to hear from providers what they are thinking as far as a "next step". If anything stresses me out it is definitely this about my disorder and care.
Anyways enough of my venting, does it feel like groundhog day alot when you (my reader(s) come by and read this as it seems always to be about the shunts, often to be about my frustration at the shunts and my wishing we could figure this out sooner rather than later. =/
Thanks again,
Erica
Thursday, February 14, 2013
2 1/2 weeks... Going Home...
I should be getting out tomorrow (fri) after another marathon 3 1/2 weeks in-patient stay. This roller coaster of emotions while waiting on responses related to other drs Dr.Bragg has reached out to is really hard and by far the hardest part of this disorder. ... So far we've heard frrom Patti Dickson regarding the IT and she gave me her cell # to pass along to Dr.Bragg to have Dr.Bragg call her. I passed that info along to Dr.Bragg and she was going to try to call Patti in between her clinic this afternoon or otherwise this evening and said earlier today if she did connect with Dr.Dickson she would try to stop over and see me tonight and let me know what they talked about. I assume because I didnt see Dr.Bragg that she didnt get a chance to call Dr.Dickson or left a message and they didnt connect. I am really, really to see Dr.Bragg in the morning and that she will have had a chance to talk to Dr.D and have some thoughts on out next steps.
On a slightly different note when I asked Dr.Bragg about if placing a CSF reservoir to remove extra CSF to relieve the "left over" headaches she said it was a possibility we could conside and possibly do in the next couple of weeks. I hope so as these headaches while especially wicked in the morning and at night sure act up during the day some days to (liike today) and are a complete bear to deal with. Not fun at all and so wish we could find a way to deal with them sooner than later. The reservoir would like be implanted in-line with the VP shunt and we could likely use it if we did do IT through the shunt but for now we would use it to drain off extra CSF as needed. I hope, I truly, really hope.
The only other bit of news is that I am going home w IV Zofran which I will self administer up to 4 times a day as needed through my Port which will stay accessed for now and be changed at ERT + if needed I can have it changed by my infusion nurse at her access team dept at CHW. This will help alot as the oral zofran no longer does much.
Stay tuned and pray Dr.Bragg is able to talk to Dr.Dickson + also that we can do this external CSF Port soon as I truly think it would make a difference.
Erica
On a slightly different note when I asked Dr.Bragg about if placing a CSF reservoir to remove extra CSF to relieve the "left over" headaches she said it was a possibility we could conside and possibly do in the next couple of weeks. I hope so as these headaches while especially wicked in the morning and at night sure act up during the day some days to (liike today) and are a complete bear to deal with. Not fun at all and so wish we could find a way to deal with them sooner than later. The reservoir would like be implanted in-line with the VP shunt and we could likely use it if we did do IT through the shunt but for now we would use it to drain off extra CSF as needed. I hope, I truly, really hope.
The only other bit of news is that I am going home w IV Zofran which I will self administer up to 4 times a day as needed through my Port which will stay accessed for now and be changed at ERT + if needed I can have it changed by my infusion nurse at her access team dept at CHW. This will help alot as the oral zofran no longer does much.
Stay tuned and pray Dr.Bragg is able to talk to Dr.Dickson + also that we can do this external CSF Port soon as I truly think it would make a difference.
Erica
Monday, February 11, 2013
Dr.Bragg's potential thoughts, other doctors ideas.
Just an interesting tidbit which ive written a little about previously is the airways issues I have (secndary to the MPS dx) and which Dr.Bragg was telling me the Anesthesiologists frequently comment to her + she is able to clearly see on the endocscope viewing monitor as they are intubating me w the various instruments is how my airway is more closely resembling that of an overly obese person (despite being 5'5 and 112 pds) probably due to the MPS storage. She also talked about how I have such a Floppy epiglottis: A floppy epiglottis is frequently seen with a condition called `laryngomalacia`.
This is a condition seen in newborns and in some conditions like MPS where the structures of the larynx above
the vocal cords, called the supraglottic structures, are softer and floppier
than usual. As well as there is Limited opening also secondary to the storage material causing stiffness and narrowing.
Dr.Bragg stopped by as usual today and some of her ideas include she is waiting to hear back from the IT drs/researchers in CA about their suggestion we try the IT injection using Aldurazyme through the shunt. She is interested in their ideas and talked about placing a different valve such as a Codman which can essentially be turned so high temporarily that the shunt is in an 'off' setting to let the Aldurazyme sit in the catheter for a few hours and then turn the shunt back on after those few hours + the aldurazyme would flush through w the CSF. (the idea being to try and thin the brain tissue that these researchers believe is adding to the intermittent shunt occlusions as the MPS (gag) storage is a clear, sticky material that causes the brain tissue to become thickened much like the rest of the tissues in the body.)
Other ideas she had include she has reached out to a dr she trained under I beleive in Utah during her Fellowship who she talked about has a patient with a permanent external drain in-place of the patients shunt and which CSF drains to. She doesnt feel that this external drain would be optimal but she does wonder if something similar with less infection risk would be an option. She talked about placing a reservoir that would act much like a Port-aCath that I currently have but this reservoir would feed in to the CSF and we would be able to draw off fluid several times a week and I imagine id learn to do it on my own too. She is getting info about this option and I am unsure if it would be in addition to the shunts or replace one or both of them. I asked her what would happen with this if say I went on vacation and couldnt get to her to draw off of it the CSF but dont remember what she said as she had an idea. We also would just change the needle a couple times a week so likely on mondays when I get ERT and I could have it re-accessed likely by my one infusion nurse who is on the access team at CHW and has accessed my Port for surgeries multiple times.
The other idea was to place a different valve such as one of the Codman valves which have several more options for settings than do my VP shunt (non-programmable) valve and their is a choice of either an 8 setting shunt or a 18 setting shunt. I believe both of these have to be changed using Xray and a magnetic re-programmer in which Dr.Bragg has said its more complicated but not overly so as she would just go with me to Xray, re-program the valve to whatever setting she wanted and then take the image + check to make sure it re-set properly. I am hoping we do one of these options mentioned above and here as I cant imagine living many weeks with mornings and nights like this.
I also heard from the new Metabolic Neurologist I emailed at UofMn as well as the Neurosurgeon there who formerly worked at OHSU and who has experience w lysosomal disorders. He wasnt sure of ideas but was going to also think about it some and was headed to a meeting where he said he would talk to some other Pediatric Neurosurgeons to see if they had any thoughts on things we could try. I am hoping he will get back to me. :)
She is talking about discharge later in the week, maybe mid-week? She talked about sending me home on the increased med doses + the pain med (different than my normal one) + I also found out from my insurance that IV zofran is apparently covered at no tier charge so I am going to ask Dr.Bragg if this might be an option I could use at home for nausea and vomiting (through my Port) as it works better than oral zofran.
Will update again if anything new or if Dr.Bragg has any new ideas or hears from any of the drs she reached out to.
Thanks for stopping by,
Erica
Dr.Bragg stopped by as usual today and some of her ideas include she is waiting to hear back from the IT drs/researchers in CA about their suggestion we try the IT injection using Aldurazyme through the shunt. She is interested in their ideas and talked about placing a different valve such as a Codman which can essentially be turned so high temporarily that the shunt is in an 'off' setting to let the Aldurazyme sit in the catheter for a few hours and then turn the shunt back on after those few hours + the aldurazyme would flush through w the CSF. (the idea being to try and thin the brain tissue that these researchers believe is adding to the intermittent shunt occlusions as the MPS (gag) storage is a clear, sticky material that causes the brain tissue to become thickened much like the rest of the tissues in the body.)
Other ideas she had include she has reached out to a dr she trained under I beleive in Utah during her Fellowship who she talked about has a patient with a permanent external drain in-place of the patients shunt and which CSF drains to. She doesnt feel that this external drain would be optimal but she does wonder if something similar with less infection risk would be an option. She talked about placing a reservoir that would act much like a Port-aCath that I currently have but this reservoir would feed in to the CSF and we would be able to draw off fluid several times a week and I imagine id learn to do it on my own too. She is getting info about this option and I am unsure if it would be in addition to the shunts or replace one or both of them. I asked her what would happen with this if say I went on vacation and couldnt get to her to draw off of it the CSF but dont remember what she said as she had an idea. We also would just change the needle a couple times a week so likely on mondays when I get ERT and I could have it re-accessed likely by my one infusion nurse who is on the access team at CHW and has accessed my Port for surgeries multiple times.
The other idea was to place a different valve such as one of the Codman valves which have several more options for settings than do my VP shunt (non-programmable) valve and their is a choice of either an 8 setting shunt or a 18 setting shunt. I believe both of these have to be changed using Xray and a magnetic re-programmer in which Dr.Bragg has said its more complicated but not overly so as she would just go with me to Xray, re-program the valve to whatever setting she wanted and then take the image + check to make sure it re-set properly. I am hoping we do one of these options mentioned above and here as I cant imagine living many weeks with mornings and nights like this.
I also heard from the new Metabolic Neurologist I emailed at UofMn as well as the Neurosurgeon there who formerly worked at OHSU and who has experience w lysosomal disorders. He wasnt sure of ideas but was going to also think about it some and was headed to a meeting where he said he would talk to some other Pediatric Neurosurgeons to see if they had any thoughts on things we could try. I am hoping he will get back to me. :)
She is talking about discharge later in the week, maybe mid-week? She talked about sending me home on the increased med doses + the pain med (different than my normal one) + I also found out from my insurance that IV zofran is apparently covered at no tier charge so I am going to ask Dr.Bragg if this might be an option I could use at home for nausea and vomiting (through my Port) as it works better than oral zofran.
Will update again if anything new or if Dr.Bragg has any new ideas or hears from any of the drs she reached out to.
Thanks for stopping by,
Erica
Saturday, February 9, 2013
Too sum it up... A Frustrating week
What a week?! I am sorry for not updating I just havent been feeling very in the mood and hasnt really been alot to say other than this week has been trying emotionally. Ive "fought" in a sense with my parents bc ive been crabby about people asking how I am doing or whats going on which isnt fair to family especially and I feel badly afterwards after ive gotten upset. I hate being upset, I hate being tired and thus so much shorter tempered about stupid questions and in many cases even reasonable questions. It is hard being here in the hospital, sleeping ok but not super great. Anytime anyone wants to come in they can be it the aides, the hospital pastor, or whoever doesnt really matter if very tired or in the middle of something. I appreciate everyone who stops by but w the hosptial pastor or friends it is definitely easier to be given a heads up!
So the only real change this week is Dr.Bragg talked to Dr.Bratanow my Pain Mngmt dr and they are not working together. Dr.Bratanow texted me this morning to see how things where going and then after Dr.Bragg stopped by and said Dr.B had just called her + given her a few med suggestions. We are going to play around w the lyrica dose and then the topamax dosing. Yesterday Dr.Bragg had asked me to touch base w anyone I knew who might have suggestions for her on the shunt issues and so ive reached out to a few drs. I also was given the name of a Metabolic (Genetics) Neurologist formerly from Baltimore area now at UofMn who I in turn emailed and heard back from soon after yesterday. He admitted he did not have alot of experience w MPS I but recommended I look in to the IT Aldurazyme studies (taking the enzyme replacement and injecting it intrathecaly or via the spine) and also I think was going to look for info. Several other drs have also recommended I consider the Intrathecal study for my shunt issues and have the aldurazyme injected via reservoir in to the shunt + then re-adjust the valve to a higher almost "off" like setting for a few hours + then after those few hours the valve setting would be re-adjusted again back to the original setting. Dr.Bragg when she came in today said it was worth looking in to and she could replace the valves I have in my current shunts w ones that would be more appropriate for this type of treatment. Obviously we dont know if I would be eligible but I am already in the cognitive function Neuropsych testing study at UofMn and in the past have demonstrated on those tests memory issues if not other issues that might qualify me. Another portion of the study is taking a dye and injecting it in to the spine to see if it travels to the brain and down the spine in a certain amount of time which would demonstrate if the Intrathecal injections would even work. That said if we could get approved for the study and passed the tests we would try to get IRB approval to do the IT injection via my shunt reservoir. When I was in the original IT-compression study we had gotten a special IRB approval to do the injections via a cisterna magna tap (at the base of the brain, more risky) and so I would think this could get an approval to. So stay tuned and we will see how this all turns out.
In the mean time I am still in-patient and truly hope to get out soon.I am unsure what Dr.Bragg's plan is but cant believe she isnt feeling frustration at these shunts too, I sure am. =/ I truly hope she doesnt give up - I tend to think she wont but yah never know.
Thurs we turned the TPL shunt down to 1.0 and then fri late afternoon we turned it down again to .5 with mornings comtinuing to be the hardest of all of this. Its a shame I cant figure out how to just avoid mornings. Symptoms ive been experiencing include the headaches (way worse in the morning), nausea, some vomiting, very odd (but not new to this time) double vision and fuzzy. Turning down the shunts helped a little with the 'throughout the day' symptoms but not the morning headaches. I have been reading alot trying to learn more and if nothing else I do think ive learned some.
About all for now, thanks for stopping by... See below for a short paragraph from earlier in the week..
This was written earlier in the week of Febr 2nd -
Whew, it is 2 days shy of being a week out from the last shunt revision (changing out the TPL shunt valve) and it has been a hard week; the weekend was better but the past 3 days have been harder and pretty hard. Headaches, nausea, some throwing up (IV zofran), and other symptoms have made it a plain hard week topped by struggles feeling like I am disappointing my parents bc I know they want me to feel better and come home. =/ I want to come home to but above all I just want these shunts to work better.
Erica
So the only real change this week is Dr.Bragg talked to Dr.Bratanow my Pain Mngmt dr and they are not working together. Dr.Bratanow texted me this morning to see how things where going and then after Dr.Bragg stopped by and said Dr.B had just called her + given her a few med suggestions. We are going to play around w the lyrica dose and then the topamax dosing. Yesterday Dr.Bragg had asked me to touch base w anyone I knew who might have suggestions for her on the shunt issues and so ive reached out to a few drs. I also was given the name of a Metabolic (Genetics) Neurologist formerly from Baltimore area now at UofMn who I in turn emailed and heard back from soon after yesterday. He admitted he did not have alot of experience w MPS I but recommended I look in to the IT Aldurazyme studies (taking the enzyme replacement and injecting it intrathecaly or via the spine) and also I think was going to look for info. Several other drs have also recommended I consider the Intrathecal study for my shunt issues and have the aldurazyme injected via reservoir in to the shunt + then re-adjust the valve to a higher almost "off" like setting for a few hours + then after those few hours the valve setting would be re-adjusted again back to the original setting. Dr.Bragg when she came in today said it was worth looking in to and she could replace the valves I have in my current shunts w ones that would be more appropriate for this type of treatment. Obviously we dont know if I would be eligible but I am already in the cognitive function Neuropsych testing study at UofMn and in the past have demonstrated on those tests memory issues if not other issues that might qualify me. Another portion of the study is taking a dye and injecting it in to the spine to see if it travels to the brain and down the spine in a certain amount of time which would demonstrate if the Intrathecal injections would even work. That said if we could get approved for the study and passed the tests we would try to get IRB approval to do the IT injection via my shunt reservoir. When I was in the original IT-compression study we had gotten a special IRB approval to do the injections via a cisterna magna tap (at the base of the brain, more risky) and so I would think this could get an approval to. So stay tuned and we will see how this all turns out.
In the mean time I am still in-patient and truly hope to get out soon.I am unsure what Dr.Bragg's plan is but cant believe she isnt feeling frustration at these shunts too, I sure am. =/ I truly hope she doesnt give up - I tend to think she wont but yah never know.
Thurs we turned the TPL shunt down to 1.0 and then fri late afternoon we turned it down again to .5 with mornings comtinuing to be the hardest of all of this. Its a shame I cant figure out how to just avoid mornings. Symptoms ive been experiencing include the headaches (way worse in the morning), nausea, some vomiting, very odd (but not new to this time) double vision and fuzzy. Turning down the shunts helped a little with the 'throughout the day' symptoms but not the morning headaches. I have been reading alot trying to learn more and if nothing else I do think ive learned some.
About all for now, thanks for stopping by... See below for a short paragraph from earlier in the week..
This was written earlier in the week of Febr 2nd -
Whew, it is 2 days shy of being a week out from the last shunt revision (changing out the TPL shunt valve) and it has been a hard week; the weekend was better but the past 3 days have been harder and pretty hard. Headaches, nausea, some throwing up (IV zofran), and other symptoms have made it a plain hard week topped by struggles feeling like I am disappointing my parents bc I know they want me to feel better and come home. =/ I want to come home to but above all I just want these shunts to work better.
Erica
Saturday, February 2, 2013
Revision #25 - TPL shunt surgery
We opted to tao the TPL shunt Friday morning and was not flowing so Dr.Bragg suspected it was either an intermittent occlusin issue or the catheter was just not working due to being blocked. We kept the OR time and te 2 Anesthesiologists I had opted to place a arterial line this time for which I stayed awake through most of and then they gave mild sedating med after which I dont remember the rest. As far as intubation they used the same equipment as previous surgeries w the lighted scope and device to help visualize better. Dr.Bragg when she stopped by this morning talked about how she can now tell the Anesthesiologists exactly what equipment will help as she isnt usually familiar w this + the mechanics of whats going on. She talked about the Anesthesiologists told her I have the aitway of a severely obese person due to so much extra tissue (tonsils/adenoids where taken out as a kid) probably caused by the mucopoilysaccharide storage. The limited opening they think is probably also likely from the MPS storage.
As far as the actual Thoracic Pleural shunt surgery revision Dr.Bragg told me she opened up the area and initially thought she would have to dissect down to the catheter (I am unsure if she ulimately ended up having to) to free it and replace this as it wasnt flowing at all. I think she said she manipulated part of the shunt some that was closer to the surface (?) and after a bit this would flow then stop, flow then stop confirming her suspicion of intermittently occluding due partially to not enough back pressure. (the pressure in the lung is alot lower than that of say the normal drainage point, the stomach so the catheter is more likely to occlude and thus ultimately (I guess) fail. She must have moved the catheter some and un-coiled it from the round-about way it was initially placed, shortened it (?) and secured it with a metal type clip. I do have to ask if the clip is titanium or is it actual metal for future scans. I imagine it is titanium. She commented she would like me to have help putting on a bra (fastening it behind my back to which I said I cant reach behind me and do already turn it around to fasten it then re-position to behind my back.) Showering i'll have to be careful w washing my hair over my head as well as reaching for objects she said; I am unsure why but will try to remember to ask her tomorrow and think it may be she is concerned that anchor will come loose and the shunt move? Right now any reaching doesnt sound appealing so will find other ways to get things.
She also wanted to leave the setting on the programmable valve blinded so she can adjust it and if symptoms say improve w the setting at a higher setting (5 choices to set it at overall) it would help her know that say the valve at high resistanc, lower drainage helps keep the catheter open better or lower setting, higher flow helps headaches but intermittently ( think I got that right!? on-the-less we will see. We also talked about the low pressure hydrocephalus article I sent her and which she had a chance to read + she said the reservoirs work well for people who have enlarged ventricles but bc mine are so small the risk of bringing brain tissue/debris in to the shunt catheter is high (similar to how we had reservoirs last year w the VP shunt valve nad reservoirs for testing the shunt.) and thus occlusion/failure is high. I think she wished it was an option; I sure do!
Will update if or when there is anything new,
Thanks for stopping by - please keep a family friend Sue in your thoughts as she lost her husband earlier this week and I cant imagine the grief she feels.
God Bless,
Erica
As far as the actual Thoracic Pleural shunt surgery revision Dr.Bragg told me she opened up the area and initially thought she would have to dissect down to the catheter (I am unsure if she ulimately ended up having to) to free it and replace this as it wasnt flowing at all. I think she said she manipulated part of the shunt some that was closer to the surface (?) and after a bit this would flow then stop, flow then stop confirming her suspicion of intermittently occluding due partially to not enough back pressure. (the pressure in the lung is alot lower than that of say the normal drainage point, the stomach so the catheter is more likely to occlude and thus ultimately (I guess) fail. She must have moved the catheter some and un-coiled it from the round-about way it was initially placed, shortened it (?) and secured it with a metal type clip. I do have to ask if the clip is titanium or is it actual metal for future scans. I imagine it is titanium. She commented she would like me to have help putting on a bra (fastening it behind my back to which I said I cant reach behind me and do already turn it around to fasten it then re-position to behind my back.) Showering i'll have to be careful w washing my hair over my head as well as reaching for objects she said; I am unsure why but will try to remember to ask her tomorrow and think it may be she is concerned that anchor will come loose and the shunt move? Right now any reaching doesnt sound appealing so will find other ways to get things.
She also wanted to leave the setting on the programmable valve blinded so she can adjust it and if symptoms say improve w the setting at a higher setting (5 choices to set it at overall) it would help her know that say the valve at high resistanc, lower drainage helps keep the catheter open better or lower setting, higher flow helps headaches but intermittently ( think I got that right!? on-the-less we will see. We also talked about the low pressure hydrocephalus article I sent her and which she had a chance to read + she said the reservoirs work well for people who have enlarged ventricles but bc mine are so small the risk of bringing brain tissue/debris in to the shunt catheter is high (similar to how we had reservoirs last year w the VP shunt valve nad reservoirs for testing the shunt.) and thus occlusion/failure is high. I think she wished it was an option; I sure do!
Will update if or when there is anything new,
Thanks for stopping by - please keep a family friend Sue in your thoughts as she lost her husband earlier this week and I cant imagine the grief she feels.
God Bless,
Erica
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