Friday, May 27, 2011

Related to surgery

A quick note - surgery is scheduled for tuesday morning arrival at 5:30 (which means leaving my apt 4:00 am and my parents at 4:30 (AAGGHHH!!!!) and surgery is scheduled for 7:30. As of this morning the pre-op nurse didnt have orders written for the solu-cortef pre and post cortisol replacement infusion (cortisol is a stress hormone and for those such as myself who are deficient in this any procedure or surgery can be life threatening and risky if cortisol replacement isnt given ahead of time - in this case my neuro-endocrine dr wrote the orders she faxed for IV Q8hr (every 8 hours the 1st day and the 2nd day will go back to oral meds at high dose tailoring down for 5 days).. So anyways apparently my Nsg has to re-write the orders my endocrine dr sent and so the pre-op nurse was going to make sure this got written before tues. She also said anesthesia would come in the morning  of surgery and meet (as will my Nsg of course to go over last minute questions). Nsg's secretary called late this afternoon they hadnt gotten the cardiology notes/release yet and needed my cardiologisrs info - (see this is why I like control - it is hard when you have to rely on various drs offices to send their various info - vs just getting it done yourself! Needless I have worried more than a little this week which is a little unusual for me and last night laid awake after waking up tossing and turning trying to not think about this. I really could use lessons in giving up control better! :)

On a different note I can tell I am getting sick - I am already taking antibiotics for an ear infection caused I think secondary to wisdom tooth issues (being removed the end of the month) so I pray these antibiotics which I had just started kick this all in the bud - say  a prayer if you will!

Earlier this week I spoke w/the new Director of US Marketing for Aldurazyme and he seems very motivated as well to try and get things done for MPS I. My big fovus with him was the need to focus on adults with MPS be it getting proper diagnosis, getting doctors to teach adult specialists about these disorders and getting these adult specialists to really understand the differences in our disorder and your average joe with cardiac or neurologic or even spine issues, carpal tunnel, etc. And also another thing we talked about was just getting materials tailored to adults and at meetings getting talks focused to adults or having adult speakers. We are each working on this on our end and conferencing again in a couple weeks.

Will update more later,


Wednesday, May 25, 2011

Odds and Ends

Surgery check-list:
Pre-op physical (x)
Endocrine related pre/post-op meds arranged (x)
Pain Mngmt dr calling Nsg w/her recommendations (x) - have to fup on this though
Anesthesia and other records - MRIs sent (x) - just have to send Visual Fields
Cardiology (x)

While this is actually the 2nd surgery and 3rd overall procedure ive had this year involving surgery/some form of anesthesia this has been much more involved than any of the previous recent surgeries or procedures probably due to multiple factors. 1. New hospital, new drs and thus new anesthesia team and 2.. Can affect cardiac issues due to being an implantable (though far less likely as the shunt is not in the bloodstream but in the CSF pathway) and length of surgery is an overall stressor on already stressed heart. From previous shunt surgeries my guess is this one will take between 2- 2 1/2 hrs but really dependent as much on anesthesia as anything. 3rd factor is because of my neck surgery/plates/screws and inability to flex neck backwards the intubation also is affecred in this fashion in addition to actual airway size. I plan to ask the anesthesia drs to write down their actual airway grade and assessment for me again. My Cardiologists words where regarding my PCP-NP wanting him to 'clear' me for surgery where "I dont "clear" pts but i'll give the ok and while your heart is an issue my biggest concern and a  very big concern at that is the actual anesthesia/intubation." (something to that effect)  Really with all this prep you would think this was a much bigger surgery but atleast they are thorough.

I thoroughly feel unprepared in a way im really not used to for this surgery - since I only spoke to the nurse of my Nsg after my dr spoke with my Adult Nsg (her colleague whom I 1st saw) and they decided what they wanted to do and not directly to my Nsg the only thing I really know is that surgery is tues and they are planning to replace the LP shunt with another LP shunt. I dont know why they chose doing another of this type vs VP (what her colleague the adult Nsg originally said he would likely recommend before I saw this Peds nsg and before the 2 drs spoke to each other to hash out a plan for trying to get on top of the headaches) . I dontt know if she is adding something different to the possible new LP shunt, I dont quite know if she is going to try a programmable (I have a fixed medium pressure shunt but they wondered if the H/A's werent bc of positional CSF (cerebrospinal fluid) backing up due to my other spine issues and also not working right w/laying down vs being upright had a difference) . I dont know why they chose this eoute - I guess I just feel a lack of control and while I do well trust these drs (anesthesia will have to be seen how they rank) anyone who knows me knows I like information, control and going along with the former being informed of everything!!

On a side note my Insur. Case Manager called today and she just wanted to let me know the surgery was authorized and also that Dean has approved my weekly ERT infusions for another year when the BDSSA contract expires June 30th and a new plan is begun July 1st. As of yet we are pretty certain of what the new plan is - same guidelines and a POS still but I thought it was really great that the insur approved my infusions and everything more than a month before the current plan year is even up and before our new plan is even signed!

The Cardiology appt went fine yesterday - I never quite feel like my Cardiologist is telling me everything or that he tells me one things and then dictates something completely different in his reports - he somewhat glossed over everything and it didnt help that his fellow who has been there since last summer has been less than helpful - she comes in before him (I HATE when drs do this!!!!!!!!!!!!! A complete waste of TIME!!) and you can tell she really isnt writing down what you tell her and she is more interested in your life not related to the appt which fine I dont mind sharing what I do because im proud of my accomplishments but if im paying (or rather my insur is paying) to see my dr id really like for him to just come in with her together and actually trust me!! Ive thought more and more about seeing the Cardiol. again in Mn even just 1x to get her opinion but havent decided. We'll see. It's not that I dont trust him it's that I just dont quite know if he quite knows 'enough' about MPS I - sure he knows some since he was the one to dx me but maybe I also need a cardiol. who knows 'alot'?

I will update more soon or atleast after I hear what time surgery is scheduled for fri and after I talk with anesthesia.
God Bless,


Thursday, May 19, 2011


Not sure if it should be a happy day or what I should feel - have been misplacing things like crazy lately but alays found everything and so I thought that was the case with my camera to. I searched my apt high and low and throughout my car thinking maybe I had left it out there. I noticed when looking in my car glove compartment that my GPS was also missing. Apparently some thieving jerk went in to my car at some point in the last week or so when I left it unlocked and went through my stuff stealing my camer (with like 4 discs of photos feom past 4-5 years and stealing my GPS as well. The camera I had saved up for and bought and the GPS I had received as a gift from a very kind MPS family several years ago. Im just incredibly sad, frustrated nsd upset. I dont understand how people can just have no quelms about going in to other peoples personal space in this case my car and stealing stuff. I dont think it was here at my apt bulding although with the crazies that live here yah never know - bc my spare key is still in my place i kep it. I dont know what to do really. Anyone who knows me knows I love taking pics just bc years later it is nice to be able to share those pics with family and with younger generations -ironically just last night I was going through pics of my grandparents for a party.

On a side note I was planting flowers and vegetables on my patio earlier this afternoon in between some things and went to move the wooden cat house I have on my patio in the corner so I could clean off  dirt and noticed one of the mama cats sitting in the house. Now if you know anything about feral cats its that the minute they see people they run so I immediately suspected she had, had kittens in there. Sure enough I gloved up and put on a heavy coat (it is warm out!) and peered in (by this point the mama ran out and was watching me from the base of a big tree about 5 feet away. I looked in and there where these beautiful kittens  - 5 of them all orange. Mama has now been moving the kittens but I know i'll be able to catch them when she comes to feed and they get old enough to feed cat food. Ive always kept straw in this house so  I imagine it would seem a safe, comfy place to have kittens since they sleep in there in the winter and is sealed off from rain. The pic shows 2 of the kittens and the other 2 are under the ones visible.  I will post more soon.

Tuesday, May 17, 2011

Pre-op Arrangements

What a crazy, busy day!  Lots of phone calls between the Nsg's nurse regarding fax #'s, surgery arrangements and other of my  endocrine drs and Nsg nurses requests regarding pre and post surgery care. My Endocrine dr sent an email this morning in response to the group email I sent to my core group of specialists (PCP, Pain Mngmt, Neurology, Endocrine, Cardiology, GC and Insur CM)  which is something I normally do for bigger, more pertinent health issues. Anyways my Endo dr wrote in her email to remind me to have my Nsg order the  stress dose of  solu-cortef for pre and post-op given by IV and then at an additional doses back on the regular hydrocortisone by mouth I take daily. So I arranged this with the Nsg office and they have the order + all worked out for that. I also had to get anesthesia records from FMLH and took care of that given the concerns with difficult airway/difficult intubation. I also had the pre-op physical with my PCP's NP (who is as familiar with my care and also very involved with my specialists) who ok'd the surgery other than a little concern with the long term use of hydrocortisone which we really have no choice on as that replaces the deficient cortisol production caused by pituitary insufficency. Her other bigger concern was that my Cardiologist release/ok me for the surgery which as I mentioned in yesterdays post I see him anyways next week for normal fup (Echo and appt) and she also was going to call him just to make sure to. I like that these drs are so thorough and yet not overwhelming. (by that I mean they would rather let me make decisions regarding my care and keep them updated and are not afraid to listen to what I tell them and accept that im not making something up + work well with my other specialists in trying to keep everyone on the same page). There where a few little minor things she pointed out that I hadnt heard before but nothing really concerning or that she seemed to concerned about.

The irony of this surgery is that since it will be at UW I will be on the receiving end of a project I am working on there for the 'peri-operative pain mngmt' committee -will give a interesting perspective no doubt as while I am the patient representative to this committe ive never actually been a patient in any of UW's pain care programs as up to this point I didnt see any drs at UW and my own Pain Mngmt dr is in Milw (UW is in Madison) near all of my other specialists. The Pain care coord. I work with at UW on this committee and other outreach awareness for APFAN (including a meeting we are going to begin planning in June for Peds Pain awareness w/both providers and families) has already asked if she could stop by post-op which is very nice of her.

I was reading through prior anesthesia and shunt surgery reports looking for the ones pertinent to keep for my records and which where neccessary for Nsg nurse for this surgery and something struck me (as often things do in these reports).  There was a comment about my lack of response related to some bad news I cant remember what it was but it made me think about other appts and the change in how I used to respond to providers telling me something unnerving or bad vs now. The first few years I was diagnosed I would have shed a few tears most often and openly showed my emotions - not because I wanted to but bc I was so upset. Somewhere along the way I got to where I almost shut down emotionally - while I still respond to whatever it is bein said I almost shut down emotional responses and clam up. I wonder why that is? I mean I realize when I am doing it but I dont always care bc I just dont want to reveal to alot of people what they are saying is bothering me. I wonder how many people are like this?

As far as the above meeting I mentioned I am meeting with this Pain Coord, her colleague a Peds Pain coord and my regional manager for APF in June (the week after my surgery so I pray I am back in shape bc I am looking fwd to this new project!

Monday, May 16, 2011

Neurosurgery surgery date

I talked to the Peds Neurosurgeons nurse this afternoon and she relayed that this Nsg and my adult Nsg both felt they could handle my care and outlined what both agreed was probably a good plan for moving fwd. So the plan will be that I will see the Peds Nsg as my Primary neurosurgeon and then the adult Nsg will be there for back up if something should change or should the Peds Nsg not be available and I need care in the future.

The plan is to do surgery on May 31st to directly explore the shunt I have now throughout direct visualization and test it's flow characteristic, etc. She is planning on testing the shunt and probably revising with an additional potential ICP monitoring afterwards. I am to plan on being in-patient for 3-5 days and one of the things we had talked about when I first saw this dr was that part of the problem might be that when I stand up due to the type of shunt I have it is able to flow properly and CSF (cerebrospinal fluid) is able to build up enough pressure and get down to the shunt which is located at L3-L4 in my lumbar spine. But then when I am laying down bc LP shunts have such a smaller cathether to carry the built up fluid and possibly due to spine issues I have or just in general due to gravity fluid may not be able to be building up enough force/pressure to flow adequately through the shunt and thus I get the headaches at night worse. The potential fizes for this are 1. a VP shunt or 2. add an additional device to the LP shunt that regulates horizontal/vertical positioning. This would regulate fluid regardless of being upright or laying down. We all agree that the shunt is working to some extent just that we think there is a problem with the dynamics of flow either due to the positioning/gravity issue or due to just plain some form of blockage in the shunt (which has happened in different ways in my other 2 shunt revisions).

I have a physical scheduled tomorrow (the nurse just called today but asked if I could get the physical done as soon as possible) so that is at 3:00 on tues with my Internal med-primary drs NP who is very familiar with my care. I also will be faxing anesthesia records to this drs office (Nsg) as ive not actually had any surgeries done at UWHC and they want to get an idea of what is needed. Anesthesia is often the most difficult part of any surgery I have and especially when general anesthetic is required as I have very narrowed airway (grade 4 of possible 4 direct visualtization by a former Anesthesiologist I had who wrote down the details for me) which requires special instruments to get the breathing tube in. I dont have the surgery time yet but will keep everyone updated on all these details.

It just so happens I see my Cardiologist next week for a 6 month fup so I guess that is good timing - please pray that the atrial enlargement I have had which has gotten progressively worse over the last year will not have worsened and that the valve issues which had also worsened a fair deal will also not have worsened. I am currently on a calcium channel blocker and lasix daily and really, REALLY do not want to have to go on to a beta blocker which I know is what my cardiologist will do/push for if things havent improved/stayed the same. It was all I could do to get him to agree to let me try a CCB for 6 months instead of trying another beta blocker (I had been on toprol xl) to see if we could get improvements in breathing (due to atrial enlargement and possible pulmonary venous hypertension) and to see if it would keep the valve issues from worsening. BB's and myself are not friends! (Ive always felt taking a medication that has many side effects such as unending fatigue to mitigate side effects from a disease process is pretty ludicrous but also understand that sometimes it just requires playing around of a class of meds to find a good match - hopefully it wont come to that as I know 110% my Cardiol will not hesitate to stop the CCB - diltiazem CR if there is further issue as he was very against doing this class of med or the Ace inhibitor class of meds to begin with.

This past sat my uncle passed away from metastasized cancer and complications from a stroke suffered last summer after a TBI and fall. The funeral will be this fri nad burial on sat - this would be my Dad's sister husband and while we didnt grow up close to this particular relative due to distance and really just differences in family style I guess I ask for prayers for my uncles family as his wife and kids are still good people who are mourning a huge loss.

All for now - God Bless,


Sunday, May 15, 2011

MPS Awareneness Day - May 15th

Today, May 15th is MPS Awareness Day. The MPS's are a group of rare disorders caused by a missing or deficient enzyme and in all but 1 type of autosomal recessive (inherited by both parents who must b e carriers) With MPS type I the form which I live with the incidence rate is approx 1:160 with a 1:4 chance of being affected and 67% chance of being a carrier.

The word MPS (mucopolysaccaridoses) essentially means 'many thickened sugars' caused by GAG deposits throughout the cells, organs, joints and CNS.

Symptoms include coarsened facial features, hearing loss, corneal clouding, hydrocephalus, joint contractures and stiffness, spinal compression, kyphosis, scoliosis, cardiac abnormalities, lung issues, enlarged liver and spleen and many other symptoms and in MPS I the treatment consists of enzyme replacement infusions weekly for my form and transplant for the severe younger kids.

The gene is found on chromosome 4p16 and enzyme Alplha L Iduronidase

The inheritance rate of MPS I atteniated is estimated at 1:500,00 to 1:2million

Saturday, May 7, 2011

Neurosurgeons and Orthopedists

Just a quick note - sorry for the delay.

I saw the Pediatric Nsg on thurs that the adult neurosurgoen I will now be working with reccomended I see (a colleague of his) just to get her opinion and see if it gelled with what he thought. She was well informed of my history (very nice!) and said she had spoken at length with my other Nsg and pretty well concurred with everything he thought. She said I could either chose to work with her or with him and/or they could tag team and work together on my case which seems like a good enough idea - 2 minds that seem very open and seem to want to look outside the box is good in my opinion! She ordered a xray shunt series (in my case with my particular shunt just 2 images) and said she would talk to my other Nsg and then call me on monday and we would have a plan of what tests next to see how the shunt is flowing. She differed only in that she also spoke about how LP shunts are much more prone to breaking/malfunctioning in various ways due to the smaller cathether but said if testing did show a problem either in scar tissue, flow or pressure they could either re-do the LP shunt or as the other Nsg said switch out to an VP. We will see if we get to that point though.

I also have been emailing back and forth with the ortho at UMn and he is willing to see me and his nurse is going to call me to set up an appt either at the end of his clinic day on mondays so we have more time or on one of the other days they will fit in an extra appt on a day he wouldnt normally see pts.

All for now - busy weekend here!


Sunday, May 1, 2011

Apparently I totally forgot to post how the appt on fri went so here goes - I saw my Orthopedic dr and he told me that he just doesnt have the experience to do hip reconstruction and doesnt feel he could guarantee a good outcome with this surgery for me. (how awesome is a surgeon who is honest!) - He did comment how he loves doing surgery but this surgery is just very complex and most cases of reconstruction are done under arthroscopic approach but since that isnt an option for my case he feels out of his league so to speak with this. Instead he said because there are underlying complicating factors he would like to help me find an orthopedic dr who does reconstructive hip surgery - open approach he talked about in chicago but I told him I had been talking with the dr at UofMn who has a lot of experience with this atleast with MPS with MPS I kids. So I he asked for the UofMn ortho's name and I am in the process of figuring out if I will go see the dr in Mn or see one of his adult colleagues or see another reconstruction dr elsewhere. The recovery is alot longer and more indepth with the open approach and I dont know that other drs would reccomend it but I will be talking to drs at the very least who have experience.

Had a very good weekend - have a good night - will update after the Nsg appt if not sooner on thurs.