AWARENESS
Last weeks Appt with the Pediatric (Peds) Neurosurgeon at
UofMn seemed to go ok. He’d clearly done some research through my med records,
looked at imaging they had requested and remembered the various conversations we’d
had over the past 8 or so yrs. As a complex Pt these are always nice to hear from new drs and saves a fair bit of trying to explain!
He made many of the same observations Dr.Bragg has over the
years about my skull bone being overly thickened (he commented how noticeable
it was from the imaging he had looked at), how he also feels that likely has played in to our not
being able to keep the shunts working well for long periods and how my brain wouldn’t
have the capacity to adjust for even mild pressure changes be it weather,
elevators, anything that people with normal skull compliance their bodies can
automatically adapt to.
He brought up and commented about cranial vault expansion (but its not technically that procedure, in my case he seems to think like Dr.Bragg has that IF it would work (he said he wasn’t sure it would be the best approach for my case and is a pretty big surgery) this could create more room in my head for the spinal fluid (CSF) to circulate and thus help the shunts work better by creating more room.
He did make the comment that my ventricles are not slit like
(not slit ventricle syndrome) though I am not 100% sure what imaging he was
looking at (older imaging I assume) and would be curious actually what my
ventricles look like now. They do not routintely get very big but when in shunt
failure my vents do enlarge some these days.He brought up and commented about cranial vault expansion (but its not technically that procedure, in my case he seems to think like Dr.Bragg has that IF it would work (he said he wasn’t sure it would be the best approach for my case and is a pretty big surgery) this could create more room in my head for the spinal fluid (CSF) to circulate and thus help the shunts work better by creating more room.
I had known this before the Appt but he is going to be out of the
ofc for about 1.5wks but said he wants to set up either a 3 way call or Video
Conference with Drs.Bragg and Iskandar to discuss my case and hopefully they
can come up with a treatment plan. I mean this in the sincerest way, but I pray
to God, sincerely they are able. I have had about enough of the headaches
(pressure) but as much as that even more sick of the low-to-mid back pain, it
literally feels like a searing pain. I can only pray.
It did seem from his comments that he is interested in staying a part of my Team or if needed potentially even doing any surgery but I am not completely sure on this. He seemed very passionate about trying to help figure out a longer term plan.
As my Mom said when I’d talked to her the other day after I was back from Mn, in as many words ‘It would be nice if someobody would do something to give you some relief while they figure out a longer term plan’. I couldn’t have agreed with her more!
It did seem from his comments that he is interested in staying a part of my Team or if needed potentially even doing any surgery but I am not completely sure on this. He seemed very passionate about trying to help figure out a longer term plan.
As my Mom said when I’d talked to her the other day after I was back from Mn, in as many words ‘It would be nice if someobody would do something to give you some relief while they figure out a longer term plan’. I couldn’t have agreed with her more!
Re the Lumbar-to-Thoracic (LP) Shunt he made the observation
that he also like Dr.Bragg and like my 1st Neurosurgeon with Lumbar
Shunts places the valve and reservoir (valve controls how much CSF gets
through, Reservoir is used much like my Port-a-Cath for my weekly infusion to
draw off CSF (Spinal fluid) when needed and/or a direct way to test how the
shunt is functioning). Anyways he commented he never places these on low back
as mine sits now and positions them along the shunt but to sit on a Pts side. – Yet another
Nrsgn who does not do as mine currently sits. He seemed to feel the current
placement could have some to do with my significant low back pain.
Now if only someone would do something about it!?!?!? I can only hope and am certainly praying..
I actually get that I likely look very good to most people and most can't/don't know me well enough to see the subtle ways when I don't feel good that I am different but those who know me best know and can see it. Oh how I just wish to feel better! For the shunts to work better again!!! I reallllyyyy miss Dr.Bragg, atleast she was always thinking and trying to figure something out to help. I pray Dr.Guillaume will be able to set up the 3 way call with Dr.Bragg and Dr.Iskandar and something comes of it and SOON!!!!!
Peds Neurosurgeon (Dr.Guillaume)
Friends and Fun - National MPS Society Conf.
NATIONAL MPS SOCIETY CONFERENCE
Re the Conf. I think I can say this was the closest Conf I've been to yet that's had at least a little focus on Adult issues though mostly/mainly young adult issues/transitioning. STILL it's slow progress!
The Adult Committee with the MPS Society has been looking for new members and many Pharma/BoiTech and Families asked me at the Conf why I am not on it/why I wasn't talking on the Adult panel so I guess i'll try to get involved. I definitely have enough other things going on but I do think I can help and that I have a good voice (experience) as an somewhat older adult with these disorders so I did send in an email about possibly joining this and adding another older adult voice (kinda funny b/c really I'm not actually that old but in our MPS/ML world 34 is kinda old-older). =)
I think to me the best part of the Conf though some people don't apparently like that they are there is talking to and meeting some of the new/newer Pharma/Biotech folks and getting to learn about the various new research coming down the pipeline. There are several studies either going on or starting (Armagen, with Aldurazyme re-formulated to cross the BBB, Sangamo just recently started a study looking at a gene editing (sort of like gene therapy) study. RegeneX Bio is another newer company I talked to a bit and who wants to do a Conf call next wk (they and I meant to meet up to talk more indepth at the Conf but ran out of time) to get my opinion or thoughts I guess on their study and to share some about what they are working on.
The Sangamo and Armagen folks also talked to me and shared info about their studies. Dr.Whitley with Sangamo had a copy of the Sangamo gene editing consent form he had given to me with his # on (he's been trying to recruit me to this for awhile, I had given him quite a bit of personal data to use when he presented before the FDA for study approval).
I unfortunately/fortunately (however you want to look at it) am already signed and consented for Dr.Polgreen's Humira (anti-inflammatory) study.
Perhaps at some point i'll share a overview of what each of the studies are about. Matthias, the CEO from Armagen (whom I've met before, VERY nice guy!) also stopped for a bit as he was leaving the Conf to give me his card and chat a bit. He emailed me again last night and I have to say seems like a genuinely caring type of person.
Honestly I feel like the HUGE take away from this Conf. is that SOOOO MANY Biotech, Pharma and Researchers are working on my MPS I and though it seems as though everything takes forever and a day there is more HOPE!!!! Potential treatments that may help some of my brain (especially) and heart and spine issues for which Aldurazyme is currently not. Atleast I have a treatment but it's kind of exciting stuff!
Almost above all I pray some of the other disorders that do not yet have treatment will be able to get treatments sooner than later. I am grateful I atleast I have something even if it is imperfect.
I feel like there is so much to say about this Conf and yet I've written a reealllyyy long post already. So instead i'll try to update again when I know anything re the Neurosrugery stuff. If you believe in prayer please pray we can sort something out!
Dr.Polgreen, Humira Study
Lastly I just heard from Dr.Polgreen's study coord. this morning, they are needing to do 2 separate initial visits to CA for the study (we where trying to avoid this) which kind of does bite but this will take place Aug. 31 and Sept 13th if I remember the dates right, right off hand. That is kinda going to stink (I don't typically mind flying just that with the headaches/pressure it's harder and all around flying is exhausting!). Is what it is I guess though to try and get something to potentially help some of the areas.
Will try yo update soon. Thanks for stopping by,
Erica
Now if only someone would do something about it!?!?!? I can only hope and am certainly praying..
I actually get that I likely look very good to most people and most can't/don't know me well enough to see the subtle ways when I don't feel good that I am different but those who know me best know and can see it. Oh how I just wish to feel better! For the shunts to work better again!!! I reallllyyyy miss Dr.Bragg, atleast she was always thinking and trying to figure something out to help. I pray Dr.Guillaume will be able to set up the 3 way call with Dr.Bragg and Dr.Iskandar and something comes of it and SOON!!!!!
Peds Neurosurgeon (Dr.Guillaume)
Friends and Fun - National MPS Society Conf.
NATIONAL MPS SOCIETY CONFERENCE
Re the Conf. I think I can say this was the closest Conf I've been to yet that's had at least a little focus on Adult issues though mostly/mainly young adult issues/transitioning. STILL it's slow progress!
The Adult Committee with the MPS Society has been looking for new members and many Pharma/BoiTech and Families asked me at the Conf why I am not on it/why I wasn't talking on the Adult panel so I guess i'll try to get involved. I definitely have enough other things going on but I do think I can help and that I have a good voice (experience) as an somewhat older adult with these disorders so I did send in an email about possibly joining this and adding another older adult voice (kinda funny b/c really I'm not actually that old but in our MPS/ML world 34 is kinda old-older). =)
I think to me the best part of the Conf though some people don't apparently like that they are there is talking to and meeting some of the new/newer Pharma/Biotech folks and getting to learn about the various new research coming down the pipeline. There are several studies either going on or starting (Armagen, with Aldurazyme re-formulated to cross the BBB, Sangamo just recently started a study looking at a gene editing (sort of like gene therapy) study. RegeneX Bio is another newer company I talked to a bit and who wants to do a Conf call next wk (they and I meant to meet up to talk more indepth at the Conf but ran out of time) to get my opinion or thoughts I guess on their study and to share some about what they are working on.
The Sangamo and Armagen folks also talked to me and shared info about their studies. Dr.Whitley with Sangamo had a copy of the Sangamo gene editing consent form he had given to me with his # on (he's been trying to recruit me to this for awhile, I had given him quite a bit of personal data to use when he presented before the FDA for study approval).
I unfortunately/fortunately (however you want to look at it) am already signed and consented for Dr.Polgreen's Humira (anti-inflammatory) study.
Perhaps at some point i'll share a overview of what each of the studies are about. Matthias, the CEO from Armagen (whom I've met before, VERY nice guy!) also stopped for a bit as he was leaving the Conf to give me his card and chat a bit. He emailed me again last night and I have to say seems like a genuinely caring type of person.
Honestly I feel like the HUGE take away from this Conf. is that SOOOO MANY Biotech, Pharma and Researchers are working on my MPS I and though it seems as though everything takes forever and a day there is more HOPE!!!! Potential treatments that may help some of my brain (especially) and heart and spine issues for which Aldurazyme is currently not. Atleast I have a treatment but it's kind of exciting stuff!
Almost above all I pray some of the other disorders that do not yet have treatment will be able to get treatments sooner than later. I am grateful I atleast I have something even if it is imperfect.
I feel like there is so much to say about this Conf and yet I've written a reealllyyy long post already. So instead i'll try to update again when I know anything re the Neurosrugery stuff. If you believe in prayer please pray we can sort something out!
Dr.Polgreen, Humira Study
Lastly I just heard from Dr.Polgreen's study coord. this morning, they are needing to do 2 separate initial visits to CA for the study (we where trying to avoid this) which kind of does bite but this will take place Aug. 31 and Sept 13th if I remember the dates right, right off hand. That is kinda going to stink (I don't typically mind flying just that with the headaches/pressure it's harder and all around flying is exhausting!). Is what it is I guess though to try and get something to potentially help some of the areas.
Will try yo update soon. Thanks for stopping by,
Erica
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