Monday, October 31, 2011

The Carrot, the Egg and the Coffee Beans...Adversity..

I liked this essay below it strikes a note I think because I sometimes dont quite know how to describe myself - Ive been described as resilient by alot of people but I also know inside I struggle sometimes (as do all people whether we admit it or not) to with all that is out of my control and finding a balance in the control I do have. ..

These past 6 years havent always been my finest moments, sometimes I am to argumentative and I realize and feel bad about this but yet other times feel no dismay at my actions because I also dont want to be a stone that others step on. I think ive grown and matured but I also know I certainly can still become even better both as a patient, a friend and as a sister, daughter, etc. I know my stubborness is an asset but I also know it can be a downfall and ive tried very much to reign in my opionated self but I know im far from perfect. I dont always care what people think of me and yet I care to much what certain people think. I want to be liked but yet I dont need permission to be someone other than who I am. I think I am fiercely loyal if you are kind, helpful or just listen and are there for me. I love what I get to do for others but have no illusions that I do things perfectly because I dont always. I want to be liked but I dont need to be liked.

I feel like over the past 4-5 months ive sort of put alot of things on the back burner including many friends and other than immediate things ive not been the best leader I can be for APF (my thoughts -ive never gotten anything but encouragement from managers/fellow leaders.) but I am always here for patients/others needing advice on pain and I will always work tirelessly (tiredly?) behind the scenes when not out in the open working on projects. I feel like other than my closest (distance wise) niece and nephews ive not been a great aunt but I also know the others are always in my thougts and as their aunt i'll always worry endlessly about how they are doing. Other than this new project for MPS I/Genzyme which hasnt been all that time consuming (a few hours a week generally)  ive not done much around MPS and feel so incredibly out of that loop but yet I dont think there is alot ive missed I suppose it's just a weird feeling since that is where I really started with advocacy.) Often these past 4-5 months ive looked back at the things ive done and it feels more like a cloudy memory than an actual memory I think because of so much going on H/A and this tiredness wise. I dont quite feel like there is a 'normal' routine to my life yet even 4-5 months later since that first surgery but I think that isnt really true (it just feels like it is because it's just different and a different normal right now than pre-May). This isnt to say that im not happy I had these shunt surgeries (and in general!!) - sure I wish the first one would have worked for longer than it did and the same wish with the subsequent revisions but there's no sense mulling over what I cant change about my body (finicky in-ability to regulate CSF with these shunts properly I guess). Im glad that I have the neurosurgeon I do whose willing to help and willing to stick with my care in the long run. I hope we get this figured out soon and I hope in the new year to figure out other aspects of my care that puzzle me or make me scratch me head in wonder. In all I guess this post is really just my mumblings and thoughts on what the past few months have taught me and what I think im trying to learn as time continues to march on with and despite MPS.

My conclusion at the end of this essay is that I think I was the carrot and am now the egg but hope to become the coffee bean and change those around me but never stop realizing that i'll never be able to change every situation and so instead a balance - a egg because it represents flexibility and adaptability and the coffee bean becomes it represents the ability to change the situations around you for the better. ...

A young woman went to her mother and told her about her life and how things were so hard for her. She did not know how she was going to make it and wanted to give up:
She was tired of fighting and struggling. It seemed as soon as one problem was solved, a new one arose.
Her mother took her to the kitchen. She filled three pots with water and placed each on a high fire. Soon the pots came to boil.
In the first she placed carrots, in the second she placed eggs, and in the last she placed ground coffee beans. She let them sit and boil; without saying a word. In about twenty minutes she turned off the burners. She fished the carrots out and placed them in a bowl.
She pulled the eggs out and placed them in a bowl. Then she ladled the coffee out and placed it in a bowl.
Turning to her daughter, she asked, 'Tell me what you see. ''Carrots, eggs, and coffee,' she replied. Her mother brought her closer and asked her to feel the carrots. She did and noted that they were soft.
The mother then asked the daughter to take an egg and break it. After pulling off the shell, she observed the hard boiled egg.
Finally, the mother asked the daughter to sip the coffee. The daughter smiled as she tasted its rich aroma. The daughter then asked,'What does it mean, mother?'
Her mother explained that each of these objects had faced the same adversity: boiling water. Each reacted differently.
The carrot wentin strong, hard, and unrelenting. However, after being subjected to theboiling water, it softened and became weak.
The egg had been fragile.Its thin outer shell had protected its liquid interior, but after sitting through the boiling water, its inside became hardened.
The ground coffee beans were unique, however. After they were in the boiling water, they had changed the water.
'Which are you?' she asked her daughter. 'When adversity knocks on your door, how do you respond?
Are you a carrot, an egg or a coffee bean?
I'd like to hope I was the carrot and became the egg and will become the coffee bean...

Trick or Treat

This weekend my sister, brother, sister-in-law and sister + brother-in-law took 2 of my nieces and my 2 of my nephews (ages 2 @ 5yrs, 1 at 9 yrs and 1 at 9mo) trick or treating - this was on sat and the kids where a witch, a ladybug and a bat - i'll include a few pics in this but it was alot of fun with reasonably warm weather - I forgot to charge my camera so this died not even 20mins in to the outing but my sister-in-law took over a hundred pics so I am really looking fwd to seeing those to! Bella is really to young to trick or treat of course but she seemed to like watching everything and Quinn, Zander and Jordan had a blast and we had a wagon along so my brother took turns pulling my niece and nephews and also Jenn and I. Ive to say my body wasnt any to pleased to walk around for close to 2 hours as my low back has been acting up again lately much like after May's laminectomy/shunt revision (this seems to be since the :LP shunt was tied off last month for whatever reason) and my hip was also trying to kill me on top of forgetting to wear better shoes (had flats that I tripped over just about any and every pot hole). Regardless of these aches and the cramps that came with it later in my leg it was to fun to walk around and take pictures and catch up with my brother and sister-in-law and just the best to watch the kids faces when they'd get candy they'd run back and jump around with excitement. Afterwards we went to my parents for supper (my Mom is a fantastic cook I always joke when I want a real meal I go to my parents to visit) :)

As far as headaches go ive continued to have these intermittent ones that are worse in the mornings or if I dont prop up enough at night and then occur on and off which my neurosurgeon thought was due to something with resistance and flow mechanics in the way this VP shunt is set up. We had decided to wait the weekend to see if this got better and so if it doesnt improve by tomorrow or weds i'll probably call her nurse. I am really ambivalent about this all and honestly even though I know she wants to help I just feel like a bad patient when these issues cont. Ugh, like ive said before I had being a patient not just with this but overall I hate it.

I finally called and made an appt with my hand surgeon for my L hand (writing hand) due to the thumb issues that have been worse since last spring and which get extremely painful (apparently my body is on a quest to hate me and thus get me to hate it!) :) This appt is later in Nov because of the travel to PA mid-Nov and other things going on next week. It has been a year since I got the cast and splint off from my R thumb tendon transfer and since I saw my Hand surgeon but ironically the secretary still remembered me - apparently I spent to much time there last spring and the previous year with that surgery to. In some ways it's nice when doctors office staff knows you but on the other hand it's almost like 'wow, do I really talk to these people that much that they recognize your name/voice/etc when you call? I guess it never ceases to amaze me really even though maybe it should aftter all these years. 

I also have a 6 mo echo (heart ultrasound essentially) and fup with my cardiologist in Nov and if good then it would mean the pulmonary venous hypertension, L Atrial enlargement and Aortic + mitral valve issues havent worsened. Given im not a big fan of the cardiac meds im already on I am hopeful it will be good! Tomorrow I also have a audiology test (yippee) and that to will hopefully go very well as I sit on the border of needing hearing aides for high frequency loss. I doubt id wear them anyways much like I dont wear my glasses

So there's alot going on this month - I will update when I know more headache wise and will update here and there on the other appts to.
Be well - stay warm!


Thursday, October 27, 2011

Neurosurgery fup appt, picture of ventricle

It has been a exhausting week - I know I should rest more but really I havent done anything that was soo strenous it's more that when I do lay down I cant sleep (I did take a nap 2 nights) and when I go to bed feeling worn out I take hours to fall asleep to. The past 2 nights have been especially bad with going to bed around 10:30-11:30 and not falling asleep till 2 or so in the morning and this morning I had to get back up at shortly after 5 to get over to my sisters as I was watching my youngest nephew till 12. Tomorrow in the same except i'll be watching him to 2 when I drop him off at his Dad's and will also have to drop off and pick him up at his kindergarten class.

                     Inside the VP shunt catheter at the ventricle/proxumal end of the catheter.

I saw my neurosurgeon today she took out the gauze and staples (there are sutures in) and we talked about how I am feeling. I always hesitate to honestly tell her when im not feeling a whole lot better not because I dont trust she wants to keep helping at this point but bc I dont want to sound whiny. Ugh sometimes I hate being a patient!!! She said it's possible the shunt could have clogged off again with debris because there isnt enough resistance and we talked more about putting in a second valve. We decided to give it through the weekend to see if these H/A's and nausea improve at all and I will call her early-to-mid next week to let them know how I am doing. She also said if it wasnt improved she would have me come back in, she would fit in a spot and she would repeat the scans. She explained how the thinking is that a 2nd valve could provide more resistance to the CSF flow and thus prevent the ventricles from collapsing under the extreme pressure/flow occuring due to CSF and my single shunt valve not being able to keep up and thus the ventricle collapses in on itself as CSF flows until so much pressure builds up they almost explode (my word) open again against the forced pressure. She thoguht this could very well explain my cyclical up and down headaches. (the ventricles collapsing due to not enough resistance and being forced open because of pressure).

When she went out to get the staple remover kit she must have run down to her OR locker as this was where she had told me last weekend she had a picture for me from the surgery. This is a 8x10 shot from the scope camera she uses to thread the catheter in to my ventricle and that she used to see what was going on with my shunt last fri. The white spots scattered around the opening are body matter and she said although the picture doesnt show it these where scattered all throughout the upper (proximal) portion of the catheter and definitely the cause of the shunt failure. The picture shows the tip of the ventricle and then there is black space further in the hole and this is the tunnel created by the catheter (to use her description much like when an animal burrows underground and into the dirt but they leave behind a tunnel that doesnt fill up) - apparently this will always be the way my ventricle and brain look regardless if the shunt was takenout (which it cant be), Drfinitely kind of fascinating! Apparently also the majority of neurosurgeons do not use scopes to place shunts and she herself said she rarely used them before coming to UW/AFCH but the other neurosurgeon there convinced her to learn and it helps her better be able to thread catheters in to very small ventricles like mine. Interesting stuff to learn! (to use another of her analogies I said something about how I thoought being able to take the pictures would be fun but wouldnt want to do the rest (not because of blood and stuff just wouldnt be good at it!) and she jokingly said 6 year olds could probably do it well these days because of video games - having to operate the remote while looking at a screen to get the visual of the problem areas. I will forever laugh and think of neurosurgery in a funny way when my older nephew is plying video games here. :)

I'll update other things soon -
Take care,


Sunday, October 23, 2011

Day 6 - Neuro Release!

Day 6

Dr.Bragg came in this morning as did her Resident previous to her and the resident asked how I was doing and what I thought about going home - I just said while I think there has only been some improvement since fri (some times are sooo good and some are just plain not great) that I still wanted to go home as I thought I could do the same there as I am doing here. This way I wont miss ERT (infusion) tomorrow and when Dr. Bragg came in she said I could fup with her later this week plus we would eval. She also said she had the picture(s) from surgery but the reason she kept forgetting to bring them was she had it in her O.R locker and stowed in the back of this so as not to get lost but that she would bring it to clinic if this was ok. She just said that the picture was for me and my name was on it so I could take it when she did bring it if I wanted. I will post it here. She said they took the pic when she was looking through the scope in to the shunt catheter and could see granules or something like that scattered throughout the shunt catheter. She seemed to think it was really cool so I bet it will be interesting to see and I will post here.

I follow up with her thurs so im sure she'll eval the valve/r/eservoir for refill time as this was how she initially figured out what was going on with my shunt last week and a way for her to eval patency. Otherwise she said she could take off the gauze and staples from my head and I think any sutures would be dissolvable but im not sure if the incision itself is any longer than the previous, initial VP insertion so if not it would likely only be 2 or 3 sutures if that. Several of the nurses ive had over the past 6 days have stopped by to say bye and wish me luck staying out of the hospital - no one wants to guess how long it will be this time so we all are just wishing for a long period of no shunt issues. I pray this just improves further and further!

Today I plan to just go to the grocery store so I can get the basics like milk and bread and cereal so I can figure out something good to take for infusion tomorrw - it always makes it a little better when I have something good to eat even if I dont always eat what I bring due to appetite. Nothing beats good food even if just to have. :)
Will update soon - if nothing enlightening becomes before thurs I will try to update how the neurosurgery appt goes.

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Saturday, October 22, 2011

In-patient day 5, Post-op 1

I think i'll stop thinking I know how long something is going to be or how something will turn out because clearly I stink at figuring out my own body! In this case I just mean when it comes to the shunt and this admission as when we went to the ER we thought it would be just that day and then we thought it would just be over-night admission while Dr.Bragg figured out what was going on/if it was the shunt nad then it became surgry on fri - so while I was sick I still felt like an slight imposter on the neurosurgery floor as I could take care of myself and other then increased meds for the headache and pain and the IV I essentially did not really require nursing care. My neurosurgeons depressing the shunt valve/reservoir and then the shunt tap which was weds and showed the CSF flow was slow/obstructed where really the only tests she did and so I was here and sick but also at times did feel pretty fune - like aflippd switch, fine one moment and headache the next. The time still went fairly fast but I didnt have mhy laptop here and didnt have my planner so I felt a bit like a big fish out of water and also each day when I would learn I was going to be here I had to scrammble to have my cousin or family go tgake carfe of my carts. I thnk because im the type of person wo likes well planned out occurences ie if goibng to be in the hospital I like knowing ahead of trime and if going to have surgery I find it easier to know ahead of time to. Yesterday we thought I would get out today as my neurosurgeon said if I felt good she had no problem letting me go home. Last night I had alot of gtrouble falling asleep due to the shunt pain, muscle spasms in my low back and L leg and hip pain because I essentially could only lay on my L side which happens to be my worsg side for low back and hip pain and the side my Port is on (ipper chest) and this was being miserable due to irritation and the needle sticking out some. This morning when I woke up after finally falling asleep I ws groggy and exhausted and headached/bufned. I think when my neusorugeon came in she could tell I felt pretty crappy bc while I would still have went home she just said she would keep me today and let me go tomorrow if feeling better. She wanred to try to depress the shunt to see if the re-fill was normal which would tell her how well it was working again but she said she didnt want to cause further pain so she'd wait and we'd see. how it goes. Today I am still sooo tired this afternoon but after being up a few hours this morning I did start to feel better H/A wise and get a litttle more energy. Late this afternoon I got tired, tired, tired again so after a walk in the halls tried to take a nap but that was a no go. Hopefully I sleep gvood tongiht.

I talked to Dr. Bragg about the muscle spasms I hav been getting for atleast a 1 1/2 mo period now and she - feels this could be from the shunt catheter fix last time and nerve root re-irritation + this  may heal and the spasms jsut go away now that the shunt is fixed. Otherwise Dr.Bragg said she had other treatment options she could use if this didnt heal and she was also going to look at my medication list to see if there was anything in there that could give her hints.

To switch gears temporarily monday I will be on a conference call with 4 Genzyme reps; several from the marketing dept regarding moving forward on our Adult-MPS I project and identifying our next step. From this call we will make the neccessary arrangements for the conference call with the 5 or 6 Adults also living with MPS I and how we will do this ie phone call or using something like skype combined with the phone. I am looking fwd to this project and found it pretty easy to identify adults who also want more information regarding our age group and for healthcare providers to be better aware of the needs of individuals in this subset. Many times simple things like having a single provider willing to act as the 'Case Manager' or 'Gate keeper' ive found makes a huge difference. Someone usually a provider who wants to learn about MPS I and is active in getting articles, sharing  what they learn with their patient and keeping core providers communicating with each other and invoving their MPS I patient in care decisions. Many folks with MPS I I spoke to where more than willing to participate simply out of interest which is awesome!

I Cancelled a Neuro-Endocrine appt which was fri and my dr is booked out but looking through her schedule to see when she can get me in. This provider tracks cortisol, thyroid and other hormone levels and especially so when the hydro is problematic as it has been in the last 4 1/2 mo with these 6 revisions so close together. She is the dr that has considered writing a paper on the effects of long term hydrocephalus on endocrine levels and how my hormone levels improve when the hydro is well controlled and how they decrease/go down when the hydro is not well controlled. She was the biggest backer of my having problems with the hydro due to her ability to look at my endocrine levels during the times I said I was having pressure headaches but very few believed me.

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I will update soon,


Friday, October 21, 2011

VP shunt revision 1, Revision #6 in 4 1/2 mo, 9th overall. aka Craziness!!

Certificate of appreciation for APFAN work

Shunt card - VP shunt (fixed pressure PS Medical valve)
Well I never did write about New York at the APFAN training meetng thurs-sat but the flight out of Madison went well, lay-over in Detroit was just the right amount to stretch and walk around a little and the plane from there to Albany was much bigger and more room to shift which was great! Our meetings started right away thurs around dinner time and ended about 9 at night, Friday started at breakfast 7-8 and then we had meetings/training until 8 or 9 at night with a few short breaks and  1, 2 hour break from 4-6 w/a reception rom 5-6 jointly with Albany Medical Center if we wanted to go.. Sat began again early and emded around 2:15 and by then I was due to head to the airport which I rode with another Leader from Iowa and her husband - she was a Pediatric Nurse and I was getting tips from her for our Pediatric Pain meeting at UW. Susie and I and 2 other leaders where on the same plane to Detroit and so also spent time at the airport talking firther. I got in to Madison about 8:30 sat night and was home shortly before 10 after getting my bag. Sadly I found out monday that Susie my fellow leader from IO passed away monday evening due to a unexpected brain aneurysm - this was shocking and sad due not only to Susie's wonderful addition to APF and one of the leaders I felt closer to as well as having just saw her 2 days prior made it all the harder. Her fineral service is today and I pray for Stephen her husband and her family who where blessed with such a wonderful wife and mother.

Monday I had teh usual ERT and that evening I began havng headaches again and woke up tuesday with estreme nausea and vomiting - after this went on for several hours I called my neurosurgeons nurse hesitantly because I just didnt know if I should call them or not. I have such a hard, very hard time deciding when to cal a doctors office regarding symptoms./ The nurse (Lisa ) advised me to go to the ER at UW and she would alert my dr (who was in surgeries otherwise they would have had me come in to the clinic)  and have the resident meet us in the ER. Within 2 hours (I live about an hour from the hospital) I was in the ER and my Dad had gotten off of work. At the ER they started  an IV (in Port) and fluids + dilaudid and zofran. An brain MRI was done and after a few hours and the Neurosurgery NP, the resident and later on shortly before being admitted up to the neuro floor my surgeon stopped by to share her thoughts to. Initiallly they thought maybe I would just need to be admitted over night and could get out weds. My surgeon depressed the shunt valve initially and said this was very sluggish to refill which suggested a problem to her. They then did a shunt tap withdrawing CSF fluid and also had problems with this. The resident couldnt easily get CSF to drain out and said it was very slow. He pressed on various sides of the valve to try and get it to flow with little success. Both he and my surgeon said this would suggest that my ventruckes due to being small to begin with where collapsing on the catheter but then like a straw when you suck out a shake tthey initially get clogged and collapse and then pressure builds up so forcing the ventricle open and CSF out to drain through the shunt relieving the headache but then the cycle would start over again and again. The decision was made weds afternoon to do surgery so that was scheduled for today (fri) and Dr.Bragg suspected ahe would end up replacing the proximal catheter in the ventricle to the valve.

Surgery was today at 10:30 so I went down to pre-op from my room quarter to 10 and the anesthesiologist + CRNA and anesthesia resident came by. Showing just how often ive been here in the past 4 1/2 months the CRNA worked with me at my last surgery and her + the anesthesiologist said they had poured over my prior surgeries both these last 5 here at UW and then those previously done at FMLH.  I think my neurosurgeon makes it a point to make sure the anesthesiologists know to use the fiber optic scope and about my airway history. As with other surgeries they let me skip the pre-op sedative and I was fully awake while monitors where hooked up and the initiation steps/time out where taken. I was then put asleep by the mask which usually takes a bit but is such a weird feeling! Apparently my neursourgeon had a conversation with me in post-op (which I dont remember and she said as much) but then she talked to my parents and also came up to my room after she was finished with her next surgery.  She said she replaced the prxximal cathether that goes in to my ventricle as this was coming out of the ventricle and initially had not been placed as  far in to the ventricle. She re-threaded it further in the ventricle and said that the catheter was clearly clogged.  I think she said right up to the valve and this nas why the valve wasnt working well  She said that when she opened this area up CSF came shooting out as there was so much pressure built up. She thinks this will help and provide headache relief but if it would not or the headaches came back she would plan to place the second valve which she would-place further down in the shunt catheter near my upper chest/shoulder and this would if I am remembering right aid in the resistance and thus allow fluid to drain more easily no matter the pressure  (how fast the CSF came through/drained) - I am not 100% sure this is how it would work but I know it had something to do with flow of CSF and helping prevent clogging and  fluid back-up thus headaches.
I am in a single room (all UW rooms are which is very nice! Since ive been here so often ( =// ) the nurses remember me and everytime id go for a walk several stop and say 'hi' or come by my room to see how im doing. Much like with the anesthesiologist its nice to have familiar faces but at the same time it means ive been here to much! Atleast the majority of nurses are very nice! My neurosurgeon took a picture of the clogged catheter and ventricle and showed my parents but bc she was coming by after another surgery when she came to my room she said she'd bring the picture by tomorrow. I am curious to see it and going to ask if I can take a picture of the picture! How interesting and fascinating since it is of my brain!
She also said that another sign of  differing response to the hydrocephalus (build up of CSF causing the abnormal high pressure) was that most patients when their ventricles collapse would feel better lying down but I am the opposite and laying down during this makes my headaches worse vs relieving it. She said to my parents she could somewhat understand how the other neurosurgeons including my former one who initially placed the LP shunt did not believe I was having pressure related headaches bc I present with such different problems of the hyro/shunt failure  but she did not agree with their essentially blowing me off. She  felt that more should have been done + she could identify clear signs pointing to pressure secondary to the MPS and she really felt she could continue to help.
My Mom brought up to Dr.Bragg about my hesitation when to call or go to the ER when having these headaches or nausea/vomiting and my worry about going if there wasnt really  an issue. Dr.Bragg  said that if symptoms recur and I have no other symptoms of cold/flu I should not hesistate to go to the ER as I have not been wrong so far about the shunt and when there was an issue. She just said she knows I know my symptoms. (how wonderfully awesome to hear a provider say this - I love her for this alone and her trust and sapport of me!!!)
If I do well tonight and feel okay Dr.Bragg will release me tomorrow which would be nice since ive been here on the neuro floor since tues morning! (ER  till mid-afternoon then up to the floor).
Will update when I know something,


Tuesday, October 11, 2011

Neurosurgery, Neurology...Many drs., one patient.. ooh so many specialties..

So in MPS (and im sure in many chronic conditions) there is this semi-struggle to figure out 'do I really need this provider?' 'How many providers is to many providers?' And even if you needed a provider for a period of time re-evaluating at times and figuring out if that provider is still needed on the overall team... I know your thinking (a healthy person anyways those who've experienced MPS for isntance know better!) that how hard could it be to figure out if you really need a specialist as part of your group of providers or not? Believe me sometimes it should be easy but atleast with a disorder like MPS it's just not typically. Why? Well with MPS what is an issue now may not be an issue 3 months from now and whats not an issue in 3 months may become an issue again in 6 months. Add to this that specialists (atleast many of mine) tend to be so uber-specialized (Ortho (Plastic surgery)-hand, Ortho-spine, Ortho-hip for instance) and you tend to end up with lot of specialists who all begin with the same speciality but branch off in to secondary specialties or areas within their field. So it seems like you should have only 1 Ortho for many issues but in reality with MPS to get a dr who has experience you end up with 3 drs. Atleast with this field ive found for my own care that I can see these drs on an as needed basis and when surgeries are needed follow up with them in that post-surgery period. Sounds easy enough, sure but then you add in 10 or more other specialists, a PCP and you end with some confusion and in it all have to get these providers to communicate with each other. Some are amazing at communicating with other providers and others could use a few lessons.  At the question well which drs do I need and which dont I need? Sometimes you dont know if you should see a specialist (we'll stick with Ortho) and because your care is so managed by specialists you tend not to see the PCP very often and so they tend not to be as involved and cant always guide you when it comes to symptoms. I keep coming back to the lower spine issues back in may and the un-expected lumbar laminotomy (lamina removal) because of the nerve roots being compressed. Generally with MPS imaging is done every 1-2 years to make sure there is no compression going on but this hadnt been done and I cant exactly blame any dr alone because as the patient it is semi-my responsibility to be on top of my own care to. But if we had caught the compression on imaging would we have been able to prevent some of the side effects of the surgery? (believe me im not blaming anyone and im not blaming my neurosurgeon) I just wonder if it would have been better to catch it sooner but I dont know the answer and I dont know that we would have done anything different. I guess thiese are just things that a person wonders and part of the 'how do you know' puzzle when it comes to care, providers, and communication. It sure would be nice if someone wrote a 'how to navigate alot of providers and lack of communication' manual! :)

So my whole reason for the wonderings above actually have more to do with a question I asked my neurosurgeon and which she graciously answered. Relating to the above ive wondered about my Neurologist and given it is quite fragmented wondering with having a Neurosurgeon managing my shunt now did I need a Neurol to manage the autonomic stuff and spine stuff or what did other (non-MPS) pts of my neurosurgeons do when they have non-neurosurgery related neurologic stuff? My neurosurgeon responded that she felt pretty certain she could help manage these areas for me and if she did need any help figuring something out she could talk to the Neurologist she works with on her complex shunt pts. (and who I met this last hospital in-pt stay prior to the VP shunt)  to get his in-put. This is great news as typically neurosurgery and neurology is another area where the neurosurgeon manages surgery and then the neurologist manages all the other neurologic issues. This effectively means i'll be able to have just one neurologic dr vs having my neurosurgeon and having my neurologist. My dr will help manage the MRIs and as well any other issues we have come up and need their help for. I am happy she will do this and to have one less provider and to top it off this neurosurgeon is so good at communicating with/working with others that I feel like it will be ok and be a good thing. :)

I'll udate from NYor soon,


Monday, October 10, 2011

Spare some energy please..

I dont know what it is the past 2 or so weeks but I feel a little like I cant get myself motivated - I know I shouldnt be hard on myself because sometimes ones body just needs a break but I am really, seriously not liking this no energy, not feeling that well. Having a hard time convincing myself to do the normal things I normally do and that I typically enjoy (little things, my nephew it doesnt matter I always have time for him). I know this makes me sound quite depressed but I dont think it's that I think it's just that I want so badly to have a reprieve from issues cropping up and from waking up with headaches (tend to still improve as the day goes on it just is sooo maddening that my body cant get it together and feel better for a better part of the day.) This spending 1/2-sometimes more of the day with a headache and thus really tired is pissing me off I guess. Not pissed off at anyone just pissed off at my body I guess and I wish I knew how to make it better. I feel like it's this one step forward, 2 steps back dance and dang it I just want to plain dance forward! The past few days I have been waking up 3-5 times a night again and that isnt helping anything. My L leg is being all weird with charley horse type cramps that had been already occuring for several months now in my calf and foot 2-3 times a week but now occuring more often and for long periods of time (5-20mins) - these usually occur at night i'll wake up or in the morning but I think because the spasming has been going on for a while now and often for these long semi-intermittent periods that my calf is very tight and painful to touch or with walking. I sent a message via mychart to my PCP and the nurse who intercepted the message wrote back that she thought I needed to make an appt (ooh joy whats one more thing) to check this out bc it hasnt gone away and seeming to increase. Ive not made an appt yet but will do so at one of the days I am already in milw for either infusion or something else since it doesnt seem emergent more just bothersome and frustrating to me and concerning to them I guess. The other thing has been hips/low back - it really has been a little crazy - me against my body, lol. GI system other than last night which I do think sometimes is more jsut my body trying to absorb CSF drainage has been better than early a week ago and these symptoms are so similar to what I experience when I have to pee that it is what makes me think maybe my body just is reacting to so much CSF draining and working to compensate. I obviously dont know it's just a guess really.

I leave for NY on thurs and so far bc ive been so tired and feel so crappy in the mornings ive just not even felt excited for the trip   - if anything  I wish this meeting had been several months from now or I didnt have to go! Sad, I know. =/ I will probably never be happier then when I am back on WI soil sat night! Ive definitely come to the concluion I would much rather do day trips that dont require flying and being able to come home to my bed at night then I would to take 3-4 day long trips and have to deal with the airport and sleeping in a hotel bed! Our conference is at the Hilton-Garden Inn by Albany so should be ok id imagine. I just sincerely have no ambition to travel right now!

On a separate unrelated note trick or treating this year falls on sat by my sister so i'll get both my nephews to take and my nieces will also be along for the day with all of us. Sun is trick or treating by my sister but am not sure if we would take Z and Bella a 2nd day. Should be fun! Next weekend sun we are going to the pumpkin patch about 45mins from my sister - myself, my sister and her family, my Mom and my sister-in-law and her family.
Will try to post again soon,

ps for those reading this forgive my whininess in this post - didnt mean for it to come across as so negative but I think it kind of did!

Thursday, October 6, 2011

Why i'm blessed...

Why i'm thankful....

Sometimes you dont realize the hidden blessing in a seeming problem until many years later.. These days one thing I realize is how in a somewhat twisted way I am blessed to not have been diagnosed with MPS until I was older. Sure I wish perhaps I had been diagnosed a wee bit sooner and wish I had not had to defend myself and my symptoms to so many people but on the other hand I am thankful that MPS does not define me. Because I was not diagnosed until a very older age (in MPS terms)  I never had to find out if I would still be the same stubborn, independent person today that I am if I had in fact been diagnosed as a kid. I never had to grow up trying to figure out who I was as a person separate from this diagnosis and I never had to worry my parents would treat me differently than they treated my 4 siblings. Do I think my parents would have treated me differently? Probably they would not have because in our family independence, stubborness and being strong willed where instilled at early ages. My parents taught us and showed us that we could be our own selves and could have our own beliefs - that we need not follow everything others did but we could also do anything we wanted whether that was going to college or whether it was getting a job and working right away. My parents made us work throughout high school to buy cars, and pay car insurance and to buy the clothes or other things we wanted but they also encouraged us to join sports or do things outside of just sitting at home. Sure as a kid I wondered about little things I couldnt do as well as other kids (jumping jacks sticks in my mind) but I still joined cross country in grade school and basketball and in high school played a year of soccer because I thought that was interesting. In high school I waitressed part time after school my sophmore, junior and senior years and took classes I was interested in when we had more choices our junior and senior year. I took the short course to become a Certified Nursing Assistant right after I graduated and went to college to become something in the health profession, at the time I wanted to be a nurse. Health issues cont'd to worsen and my junior + senior year especially I saw dozens of doctors until a year after graduating I moved to a school I transfered to and began seeing new specialists and eventually landed in the office of a newly graduated (from fellowship) adult congenital heart dr and within 3 months I had the diagnoses. Did MPS become a much bigger part of my life and change my life drastically? Definitely, id be lying if I said it didnt. It took me several years to figure out who I was now going to be - I was very involved in this community and within several months had given my first talk on being an adult with this disorder as well as began being involved with the Pharma co that markets  the weekly enzyme replacement drug I was receiving. Regardless of all these adventures I still didnt really know who I was with this disorder and didnt know what I would do next - I needed something that wasnt necessarily MPS related to be a part of. That came about 3 years ago with my becoming a leader for WI with the American Pain Foundation Action Network. With this I have personal experience with pain and can help others but it also is not about me and my issues instead it is about what can I do to make others lives better, to bring about change and to help others realize that there is still life beyond living with chronic pain or chronic disorders. But I go back again to why im grateful to have been diagnosed later - while I think my parents would have still raised me similarly to my siblings would I have had all the great experiences I did? Would I have been so independent with my health care and with making decisions regarding my own care even as a teenager? (by the time I had a car I was going to specialist fup appts on my own and going to other appts on my own and recognized when issues where important). Would I have relied on my parents to make those decisions still today if I had been diagnosed as a kid? While many people say it's better to take someone with you to appts I am a whole hearted supporter of people with chronic diseases taking on atleast some of their care and when possible all of their care - the insurance stuff, the appts, the doctor decision making and even the ultimate decisions whether to keep or drop a provider.  (doeant mean you dont ask others for their insight or advice or with bigger things like surgeries/procesures have someone go with you) These things all help foster better personal care in my opinion and help the patient to have a better stake in their own care and also be more involved with the small and larger communications that take place around and with providers regarding care. I know it's not possible for every MPS pt to take on their own care and im not advocating that im simpling talking about my own care and my own experiences and how it all shaped  me. I know lots of people have no desire to do their own care but it really is a great feeling when you, the patient are engaged and are the person that providers call, email or talk to when decisions are being made. In these respects this is why I am grateful to have been diagnosed later - sure of course I wish some of the issues could have been treated sooner or diagnosed sooner but overall im glad to be the person I am today, fairly optimistic, not defined by MPS, not afraid to go after something if I want it and ok with having a life that is different from most peoples. Embrace who we are, right?

Next week at our yearly APFAN training (in Albany, NY this year) I was asked to give a joint talk on 'balancing advocacy and self care' - do I have any profound ideas on this yet? Not really, other than one needs to take care of themselves first and do their work second. We are no good to others if first we do no favors to ourselves after all. I will either write more about this before I leave for NY or will write more on the talk after. It is set up as a talk and discussion so should be pretty interesing. The overall meeting and trainings are in conjuction with Albany Medical Center and so it promises to be a interesting few days full of new inormation, new topics and im very certain very tiring to! :) It looks like I will get the chance to see a couple of friends 1 of whom ive been friends with since I was dx'd 6 years ago and 1 whom ive not seen in almost 2 yrs now so that to should be nice!
Take care - God Bless,


Tuesday, October 4, 2011

And so it is..not to much (neurosurgery appt and vacation)

Headed to vacation - Zander and Jordan riding together
Jami (my brother) and I doing a puzzle - Jordan's
Playing cards

Back from vacation which my family (parents, sister and her family (including niece and nephew) and brother and his family (including niece) went up to the Mn/WI border near Winona, Mn for the past 4 days which was a tad stressful the first day but then pretty nice and we did a lot of driving around to parts of WI area and up to Rochester area of Mn as well 2 days. Just nice to get away but nice to be back to! I'll try to post new pics soon as I have a bunch and my sister in law also took over 400 or so which i'll only pick a few to put up in the photo slideshow.

Headaches where actually pretty decent this weekend  - I get these very odd episodes where I think the fluid is draining as I can feel a very unusual sensation throughout the valve to ear portion of the shunt in my head but mornings minus minor symptoms where not to bad which was really great. I do wonder if there can be issues with CSF taking a while to absorb from the peritoneum in to the body but non the less I was just happy to feel pretty good almost all the trip! Now if only I could get an appetite back that would be really nice - mostly id just eat a meal once a day and then just snack on little things which has been the habit for some time now and the cause of quite a bit of weight loss (7pds on a small/thin body already). Hips also while painful several days where relatively mild the other days so 50/50 is duable pain wise to me! :)

I'll try to update again soon but below is the entry regarding seeing my neurosurgeon last thurs that I started thurs night but never finished.


Later on in the drive (about 4 hours) I am with Zander and Jordan in my brothers car - tight fit but fun!
Sept 30th 2011
Today was the day ive been waiting for, for the past almost 2 weeks - I got the staples and gauze taken out of my head and my neurosurgeon also took out part of the sutures in my abdomen/stomach which she said those along with the sutures in my head will dissolve. I think they where as curious to know how I was doing ass I was happy to get the staples out. I commented that mornings are still rough but that it seemed like every few days I still have noticed improvements in symptoms and so maybe we just need more time for my body to heal and soon enough my mornings would catch up with how my afternoons and evenings are ie much less headache and more energy. My neurosurgeon recommended trying to sleep upright (I sleep semi-propped up but maybe need to revise this as perhaps the way I have been sleeping could be partially occluding the shunt at night or maybe the way I am sleeping just isnt elevated enough for CSF to get through.) In any case my neurosurgeon said she thought maybe bc we have done so much manipulating to shunts and my body in the last month or so (4 surgeries) that with healing this would improve - we both agree that we think we should give it more time which I am fine with! I am sooooo happy to have the staples out and although the area that is shaved/clipped is going to be interesting to match up with the other hair atleast I have thick enough hair I can part it differently and will cover it! I think that it be will be pure awesomeness to wash it fully tomorrow!! I am not at all worried if for some reason the shunt wouldnt acclimate and I wouldnt start to feel better in the morning (although believe me im hoping and praying and optimistic and so to is my neurosurgeon) bc I at this point know my neurosurgeon would do what she could to improve any residual symptoms as she is a rare kind that truly believes me when I say something isnt right. I totally love this! Being the absentminded person I am I forgot to write down a question I had for her about a neurology/care issue but she also asked me to keep them up to date on how things are going and if I do get to the point before I see them where I am hopping out of bed in the morning with energy (I had said to her I couldnt wait to feel so good h/a wise that id wake up in the morning and be jumping out of bed bc I had so much energy.) ;) and so I know I can always call her nurse or even email this dr. the question I forgot. Atleast I remembered a few other things.