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
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