Monday, February 27, 2012

Hydro info, update, news

Below are a couple responses from my neurosurgeon as well as from Emil Kakkis, MD regarding shunts and MPS - both are interesting and I think could be informative to MPS families. Ive taken out any names and inserted just initials plus the emails where edited to just contain the part I think would be informative so keep in mind there was more to both of these messages.

Received - 10/11
Sorry for the delay in response.  CSF pressures in general are not compartmentalized, but pressures can build in one area and cause a generalized increase in the brain pressure.  Pseudotumor is likely more of a problem with absorption of CSF, whereas hydrocephalus can be that or it can be an obstructive problem.  Problems with absorption of CSF are also seen in other syndromes, like glycogen storage diseases.  The problem isn't so much the definition, but defining what the problem is and working at solving it.  Sometimes addressing one or both still doesn't solve the problem because it doesn't necessarily deal with brain compliance (how stiff the brain is).  That is what Dr. H helps us understand as well. 

Received - 2/12
In MPS, the hydroscephalus is caused by obstruction or blockage to absorption of the CSF at the top of brain in special structures called arachnoid granulations.  This is the place CSF is absorbed back into the bloodstream.  But the blockage and slowness of the absorption leads to increased intracranial pressure, so the shunt is supposed to help over come the block by pushing CSF back into the body using a catheter.  The valve helps you control how fast it goes.  I think your shunt should be slow so it will be less likely to plug and so the slow release of pressure will help reduce pressure on your brain enough to feel better, but not too fast to cause the filter in the catheter to plug.  I hope it continues too.
Brain compliance has little to do with it.  The brain may be full of GAG and that may change the compliance but it does not matter how soft or hard the brain is, but more importantly how much fluid goes in and how much goes out.  Hopefully you have the balanced now.
I had a really good weekend headache wise - there was some headache but within an hour or so of turning down the shunt friday afternoon I was feeling pretty good and although I woke up with that same 'hung over' like feeling (that typically wears off a few hours after being up) I had a great day sat andon  sun waking up was improved (though i didnt sleep much then either as both Quinn and Zander spent the weekend and woke up REALLY early) + the day was also pretty good. Vision remains an issue but otherwise I was THRILLED to finally not have a nagging pressure sensation and nausea, etc! Today, hopefully maybe only because of the ERT fluid  load has been somewhat bad on and off - ive been getting these almost lightheaded like sensations although I dont have low pressure headache which is what id normaly associate light-headedness with! Instead its a pressure feeling and vision has been attrocious plus tired, really tired. I had a meeting after ERT over at FMLH (which is next door to CHW)  for the project I agreed to help with, with the new Adult Congenital Heart group; basically a meeting of the minds to bounce ideas off the 4 of us that met to discuss our website, fb page and overall ACHD group. Maybe because of not alot of sleep last night and then ERT all morning/early afternoon I felt like I was trying to focus from a million miles away which drives me insane!!

Tomorrow I have a sleep study (yuck) at FMLH for my Cardiologist and Pulmonologist and then see neurosugeon thurs at AFCH-UW. Im hoping and remain optimistic that today is jsut a fluke with the shunt!
I also got an email from the Genzyme person ive been working with on the Adult-MPS I project and we have a conference call tomorrow to go over the final survey and will go from there. I am excited we may be close to having this ready to send ou!

On a last note - Weds febr 29th is World Rare Disease Day - wear your 'genes' (jeans) and share your story!
Take care,


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