The valve I have now is set at a high pressure; high resistance setting and so while saturday morning and afternoon where very good (I suspect because CSF would drain off to some extent during the surgery so my body would have that to make up for the difference in the high pressure setting - the fixed pressure valve was medium pressure before). My neurosurgeons partner was seeing her patients today and so I asked him about lowering the valve setting some as last night I was miserably bad feeling with a high pressure headache and nausea, etc + very drowsy/sleepy till the pressure wore off some. He thought we should wait and watch for a few days so I am still in-patient on the neurosurg floor until atleast tomorrow and will see what my own neurosurgeon says. I know sat she said she was hoping we would be able to tolerate the shunt at the high pressure-high resistance setting (less likely for ventricles to collapse and then less likely for intermittent and eventual permanent occlusion as has been occuring almsot every month to date.) She did say if my headaches got bad though she was fine with lowering the shunt and we would see what I could handle as we knew doing this I may even need to drain more CSF than I was with the previous medium pressure valve. Hopefully having the second high resistance-high pressure valve in place at my collar bone will provide back up pressure if we do have to lower it to lowest settings in order to help prevent future occlusions.
Dr.Bragg and I talked about seeing an Opthalmologist yesterday (I have one at UofMn but would like to have one locally and at Dr.Bragg's hospital since to some extent the 2 will go hand in hand.) I also need a Opthalm to follow the corneal clouding which so far is just at the back and top of my cornea. Dr.Bragg was going to talk to her nurse Lisa who has worked here longer than Dr.Bragg and they where going to set me up with whomever of the docs they work with and think is best.
Right now pressure is building up at the valve and is alot like a piercing feeling down in to my eye but not outright painful just makes you take notice and somewhat uncomfortable. This will eventually lead to headache and then draining.
I think I am finding amazingly it easier to talk about all that I have going on face to face with people - there are plenty of times I dont feel like talking about any of this MPS stuff and so will change the subject or turn around the conversation but I do think I am getting better at explaining whats going on and not feeling so self-conscious talking about myself. I have no problem talking to large groups of people as that can be so informal and I can basically let myself get lost in the crowd while talking but 1 on 1 it is different and has always been really uncomfortable for me im not sure why. Especially providers I dont know well or people I dont know well and even sometimes family bc often I just need a break from thinkng about all this MPS stuff and when by family just want to think about other things. There are a few people ive gotten to know really well including a couple providers and I have no issue for the most part talking to them but in their cases some of them have been so open about their own lives and what they or their family are dealing with and this almost makes a mutual connection. I dont think im ever going to be one of those people who has as easy of a time talking person to person about my life as it is easy for me to say write here or write about what im dealing with on fb or the MPS Forum. A work in progress I guess.
The nurse and I tried to see if we could get the aldurazyme infusion done here at UW while I am in-patient on the floor but UW does not carry Aldurazyme (as far as I know there is only 1 other little boy in this area who has MPS I and he is post-BMT so no longer on ERT thus UW would have no reason to carry the drug). I am going to call my Insurance Case Manager tomorrow see if she has any ideas otherwise will try to get the infusion done later this week at CHW if one of my infusion nurses is able to come and do it. Hopefully.
I have fwd'd all the IT communications from Drs. Dickson, Kakkis and Elsa Shapiro to Dr.Bragg and will talk to her about her thoughts. In the mean time Dr.Shapiro and her colleague Kate who does the actual testing and scheduling for UofMn's part of the cognitive study is looking in to my having the fMRI done at UCSF and then UofMn will do the neuro-cognitive testing. From there I will have to make a decision if I am interested in doing the IT and doing the pre-study testing to see if i'd qualify. I havent asked Dr.Dickson but assume this is the study she would want me in but then get IRB approval to use my shunt valve/reservoir for the actual injection to see if it helped slow down or prevent the occlusions and clear the MPS-GAG storage from choroid plexus and brain matter. I plan to talk to Dr.Bragg this week and get her thoughts on this all as I dont think I would try for the study if she didnt think it would be of benefit or if she where against it for some reason. We will see - stay tuned.
Please keep my good friend Lea in your thoughts, she has been one of my closest friends in the MPS community and yet we are comfortable enough we can go what seems like eons without talking and still know whar the other has going on/is dealing with. Her son Gabe is having his funeral today and tomorrow and Gabe had MPS II another form of my disorder. Gabe was very young and is incredibly sad. I cannot wait to hug my nieces and nephews extra hard.
God bless,
Erica
My life's but a weaving between my Lord & me,
I cannot choose the colours...
Oft times He weaveth sorrow
And I in foolish pride forget
He sees the upper but I the under side.
Not till the loom is silent & the shuttles cease to fly
Shall God unroll the canvas & explain the reason why
The dark threads are as needed in the Weaver's skillful hand
As threads of gold & silver in the pattern life has planned. -B M Franklin
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