Friday, November 18, 2011

Surgery date, Philly - CHOP MPS I study

I picked up the Zyvox on tuesday morning at UW (cancelled and rescheuled other appts) and have been takig it 2 x's a day ever since. Thankfully no side effects minus a minor all over body itching that occurs a few hours afrer I take it and lasts 1-2 hrs. Since it started the day I started the Zyvox I assume it's relatedf. I'll take this antibiotic for the next 3 weeks and then am not sure if I will be on an antibiotic for a while after the new shunt is placed or not. Surgery to take out the LP and VP shunts and replace with the new VP shuntwill be Tuesday, Dec 6th. I am very grateful that Dr.Bragg and her nurse, Lisa worked it out so I did not have to go in to the hospital this week for IV antibiotics and surgery! Im not sure how will they know if the infection is truly gone (since I didnt show signs of it) and also have to check to make sure the hardware in my neck is fine but otherwise am just looking at this like atleast I am getting a reprieve for the hoslidays and atleast I am still feeling pretty good. My vision and low back/leg issues continue to occur unabated but those may still heal. On my questions to ask Dr.Bragg about if not improved by surgery - I do wonder if taking out the LP shunt wont help the low back pain since it seems to be along the same lines and areas as the type and area of pain I experienced after the LP shunt revision and laminoplasty in lumbar spine last May. We will see I guess and hope!

Weds since I had Dr.Bragg's permission I flew out to PA for the MPS I-non-sense mutation study at CHOP (on Penn  State grounds) which weds night I had supper with my good friends Jenn and Anisa who are from Toronto, Ontario but whom I met several years back at a conference in Vancouver. Anisa is 6 with MPS I and completely reminds me energy and talkativ wise of my 5 year old niece - she remembered me to which I was really surprised by! They where also participating in this study and we by lucky chance ended up on the same dates. My other friends Jenn and Austin live in NJ and drove down for supper with Jenn and myself and the kids - was really lovely. I'll try to uload some pics in to this post. Thurs Jenn, Anisa and myself took a shuttle over to CHOP with another MPS I family - the Moore's from Mn whom ive also met and we all had breakfast with the study Genetics Counselor and then went up to meet the study doctor, Paige Kaplan. Skin biopsy atleast in my opinion was very easy and lots and lots of very cute pictures! We should know in a few weeks how successful the study is as it takes a few weeks for the biopsy to grow in to a sheet of skin which the ataluran (study drug) is then tested on. If these studies are successful Dr.Kaplan and Sara expect this to go to clinical studies in spring - a relatively short time frame because the medication is already in FDA hands being reviewed for approval for another disorder called Duchenne Muscular Dystropy clearing the way for MPS I studies in humans sooner. I asked if the medication would be a replacement for ERT as many families appear to be under the assumption or would it be a adjuvant therapy and Dr.Kaplan said they will test it in patients on ERT infusions and believe it will be an adjuvant oral treatment to the once weekly infusions that we take (and BMT pts do not receive but will also qualify for the study) and they believe even if approved by the FDA down the road that it would not be a replacement therapy. The hope and expectation is that ataluran will get in to the blood, brain barrier and in to the bones which ERT does not do effectively. Very promising - stay tuned.

I will update other things soon - will kee updated on how this shunt stuff goes and if anything changes with the surgery, etc. For now pre-op is scheduled for tues and I also see my Cardiologist then for a Echo and 6 month appt. (so ironic that at last 6 month Echo an appt we where looking at the first shunt revision back in May.)
Take care - God Bless,


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