Ramblings and thoughts

Do you ever stop and think about the start, stop, step backwards and altogether halt nature of chronic illness and healthcare? I was laying here trying to sleep musing over a few things provider related really thinking about upcoming appts, decisions, etc as I often do - driving and at night when I should be sleeping are often the times I think the most about this kind of things. I was just thinking about how things that happened years ago and have little relevance to current healthcare still have such a promiment part in overall thoughts while those things that to me would seem more important bc they are currently happening or changing seem to get little time. It is this odd phenomenon I find in my healthcare - some providers want to find an answer when either an answer no longer matters or when another healthcare provider has seemingly found a answer that equals a relevant solution and for which the answer made sense and gave a solution. Why is it we as a society are so stuck on what happened yesterday that we fail to realize what is going on today? I know I sound alot like I am rambling and really in my tired mind I probably am it just is something that really is an unanswered question to me. I feel like alot of appt time with some really good providers is spent with them trying to remember what has been and thus taking what little time there is away from what needs to be and I dont quite get this over and over and over again thinking I suppose. On the other hand there are providers who are cautious but inquisitive and who are willing to look at things from different perspectives and this is really the kind of care I think all patients especially all patients with chronic illness need. It certainly doesnt mean the other providers mean any less to the overall care just that less seems accomplished at those appts vs at the appts with the few who can move on while still not neccessarily forgetting what was if that "what was" should need to be dealt with at some point again. It's almost like a crapshoot to find a provider that blends with ones own personality, who meshes with the patients own thinking and who is receptive to what an capable patient knows about their own disorder I suppose. One who can rattle off the smallest and largest issues, who can tell you the obscure things about your disorder and who is willing to think outside the box and still be a decision maker even while not necessarily being an expert in the disorder with which their patient is diagnosed. Again as a patient myself with an extremely complicated disorder I dont expect every doctor to understand everything about me but I guess I expect at the least for those providers to atleast be willing to hear me out, listen to my experiences over time and be willing to accept what sometimes seems unacceptable in the medical world because in my case MPS is not 'normal' and thus defies normal standards. Partnership and equal listening really are key to moving on and functioning in healhcare...

I think it's a little like trying to change yourself to someone elses standards so they will like you - in other words become a cookie cutter of what our world views as acceptable. Ive always felt probably bc of how I was raised with 4 siblings and taught to be independent and independent thinking that I am the person I am and while I dont have to purposely try to alienate people I also should stay true to who I am and those beleifs I have been taught. While ive learned many lessons in keeping opinions to myself there also has to be a way for people to be able to share their own experiences and be a part even if their experiences havent been the 'mainstream' way... A friend of mine who also lives in the MPS world although with a child and I often talk about this, how with these disorders you would think there would be more all around openness and acceptance to what others think but when it's not within the realm of what people "know" people often refuse to "see". Ive never been the type to reach out and stay close with alot of people and in this world I think that and having an opinion are the 2 axes that grind a stone for many people. I really believe in our society we need more people willing to stand and not be bent to others wishes, we shouldnt change so someone else sees us as ok but instead stand for what you believe in, represent what you know to be true and judge not what others think because you in fact are the only person living in your body and the others judging know no better because they cant possibly see what your everyday life is like. We all are guilty whether we admit it or not of perceiving someone one way when in fact they are truly a very different person because we simply didnt take the time to listen, look beyond the cover or be open.


I will stop rambling non-sensically now and sign off for the night... will try to update after the Ortho appt at some point tomorrow or over the weekend.
Good night,

Erica

Few things Ortho, Easter weekend.

So I saw this new Ortho reccomended by my ortho yesterday (mon) and went fine though I was just to tired from the weekend to think of logical questions to ask to many and of course now have a few. It was a gorgeously awesome day outside and I had been at ERT all day already which really made it worse (the tiredness) as I just wasnt in the mood to be at infusion and sit around all day + w/so little sleep and then to go to the ortho appt. ZZzzz'zzzz's ZZzzzZZZzzzz's where in my brain! :) Anyways so he took some different apparently less often used type of xrays that hadnt been done yet (I did have other xrays and MRI ) and with those was able to calculate something to do with bone size and different measurements of abnormalities going on and with that told me while he feels surgery would help my symptoms that arthroscopic would not be appropriate. Because of the extreme high force they would have to use to dislocate the hip from the socket for this surgery and because 50% of my femoral head is covered in cyst or is cyst vs bone (not clear now if it is cyst covering bone or cyst replaced the bone) the risk of fracture when they dislocate the hip for arthro surgery and then put it back in place after is significantly high. With issues like this he said they only do the arthoscopic approach if there is maximum 30% involvement of the femoral head. He also said while artho is less invasive, open surgery for this issue can still be an option and here again I needed to ask what the difference is and clarify more. I can ask all these ques on fri to my ortho dr though so not particularly worried about my not having asked some questions. I know recovery time with arthro is shorter but not sure if Ortho will want to proceed with open - guess we will see.

Easter weekend was hectic - totally 100% hectic but fun. Both of my nephews 8 1/2 and 4 1/2 where here and sat went with my sister to FDL and took Zan for pics (which I did update new pics but will post easter ones soon to). That was quite funny and interesting - Zan is 100% a ham but was not having much to do with getting his pics done. My sister while we where there going through the 5-7 good pics Zan did take after took a cute pic of Zander, Quinn and I together - Q is draped over my shoulder, Zan in my lap and we look like the Zoo crew ie silly! :0) Easter was also well - the kids and I went to church which is much easier when just 1 but with both was an experience no doubt and during the instruments (trumpets) for I know that my redeemer lives and Christ is Risen Zander had his hands clamped tightly to his ears. Was very pretty but quite loud! My sister, mom and I combined easter baskets this year so the older 2 got wind up wooden airplanes with their baskets (big mistake not buying Zander one to due to the spinning propellar factor!), Alissa my oldest neice got a assortment of small webkinz with her basket (she wasnt able to come nor was my brother so have this) and two youngest got animal/butterfly carriers/butterfly nets and small webkinz as well as baskets which we hid the baskets and then also did a easter egg hunt hiding 60 eggs around the yard at my sisters. Lots of cute pics!

Appts

Orthopedic Appt with Dr.Birmingham my current Orthopedic drs colleague is next monday afternoon at FMLH after my infusion at CHW which these two are connected.

Neurosurgery Appt with Dr.Bragg at UW for second opinion per my Nsg's reccomendation is next thurs at 2.

May the peace and grace of our Lord and Savior's death and resurrection be with you this holy week and during the special events we celebrate friday (good friday for those who celebrate this our Christ death for our sins) and Easter sunday for the risen savior!
I am looking forward to being with family and for those MPS Families who are away from home I pray for peace and continued steps fwd.

Erica

Abnormal Hips; arthroscopic surgery

Not to often there will be this many posts in a week! This morning I saw Interventional Radiology for fup from the port placement and all is well there and we should have the right equipment at infusion this week to access w/as the nurse this week is planning to bring what we need. The steri strips on the main incision have yet to fall off so they left these alone and we expect they will fall off in a few days. I also saw Orthopedics in follow up from the hip injection and physical therapy and my orthopod had done a little further research + took my xray and MRI films to another of his colleagues and talked to him about me. (the guy I see now handles the "complex" cases but I was sent to him by another orthopedic dr in that same clinic who does more chronic illness cases that affect orthopedic issues and whom my Pain dr has worked w/+ reccommended and sent me to. Anyways this 3rd colleague is more a hip "expert" and deals almost solely with hip issues and told my current Orthopedic dr he thought going in arthroscopically and looking around would be of benefit and give us a better idea. He wants to see me and go over options but feel arthoscopic surgery to clean out the gag storage, re-mold the edges of the hip socket/re-form it more properly and clean off the edges of the hip socket as they are jagged and malformed. (I think I got this all right). This would be only 1 side at a time and I think different than what is done in alot of MPS cases but I am not certain about that either and have to research it some more and will learn more when I talk to this new orthopedic dr. (also still in the same clinic which I jokingly said we where going to run out of partners at FMLH to see though since it is an academic center there are alot of ortho drs.) :) The reasoning is that the hip socket is to wide and deep and when you look at imaging you can clearly see what appear to be deposits and the boney edges of the hip/hip socket are abnormal though thankfully cartilage looks good. I will update soon, Erica

Various Appts

Hello, busy week here so a few updates with all that is going on although mostly appt updates as the rest is status quo and just working on various projects for the MPS Society and for APFAN. I saw Pulonary this morning which went quite well and pulmonary tests actually turned out really well - there is still the mild restrictive lung disease but otherwise no change so as far as the breathing issues I experience my pulmonary dr does also believe they are cardio-pulmonary related and that the choices my cardiologist has made are good ones. Yay good news! :) Otherwise my Pulmonary dr is looking into seeing if I can do the sleep study at home as she and I to feel this might give us more accurate results and we dont neccessarily need the actual leg and sleep level portions of the test just the parts that look at breathing and oxygen and hours of sleep. I also followed up w/Pain Management which is usually always a good appt and always informative - not only for my own care but bc my PM dr really is awesome about sharing her knowledge and what she learns/hears going on in the background in the pain world. In this case it was alot of what changes are coming down the pipeline w/the govt and with some more pharmaceutical change overs (this would make the 3rd or 4th one that I can think of this year alone in this case Cephalon in a hostile take over and some of the others have been King being taken over by Pfizer and of course Genzyme by Sanofi. Must be something in the Pharma blood lately. =/ In some of these cases these are really good pharma companies that are really commited to pain management and improvements and in the case of cephalon it is expected to be broken up and sold off by various divisions which is a shame. We just talked alot about some of what needs to be done, what we each are working on and what we would like to see change/happen. As far as medical stuff we talked about GI affects of opioids and are going to do a trial run of a medication called remeron which is typically known for it's use in depression but because it affects both norephineprine and serotonin (a tetracyclic) it also falls into a class of drugs called neuromodulators which can help cervicogenic and cerntral nervous system headaches. In this case it is a disolvable medication taken at night and we will try it for a few weeks pending side effects. (im a big believer in not taking a med if it has significant negative side effects except in certain instances where the medication truly has a big impact on my quality of life in which case I will weight the side effects vs the good effects. She was a little concerned w/the Port bc of my thin body type and the very apparent nature of the port ie meanin the skin is very stretched but we all will just watch it to make sure the skin does not erode over time and it really should be fine. I do also wish the port was less visible as ive not seen anyone whose port is quite this apparent - it is just a very apparent bump but still not all that appealing! Lastly I see Orthopedics tomorrow for follow up to the hip injection several weeks ago. Next week I am meeting with the Insur Plan provider - err the middle company manager to go over the different plan policy changes that the school district/union are looking at and considering - my Dad being union president has told me some but this will give me a chance to get more indepth information. I also was able to find out from talking to my insur case manager that as long as we stay w/Dean which we are expecting to do she will stay as my CM which takes away some stress as she has actually been very good and very helpful at getting information and getting providers to communicate with each other! Another piece of good news! Really looking fwd to the weekend, to seeing my nephew whom ive not seen now for almost 2 weeks which just about kills me I miss him to pieces (he has been by his Dad) and also will see my youngest neice and brother + sister in law - fun!! God Bless and take care, Erica

Neurosurgery news

I saw the new Nsg who was surprisingly nice although maybe this shouldnt be surprisingly since any dr who will take on a pt without knowing much about them and takes on the patient via email bc of an email I sent to the Pain Coord I work with via APFAN and which she then sent to fellow co-workers of hers. Anyways I am appreciative of this APF colleague passing my email on, of her colleagues passing my email on and then of this Nsg accepting me as a patient! Hopefully I dont look back and regret saying this but he seemed to get that MPS is not clear cut and seemed to get that i'm not textbook and nothing about my history has followed any of the "normal" MPS cases but then why be classic right? I did appreciate that he got and acknowledged that lack of papilledema and lack of enlarged ventricles doesnt mean much in MPS I and I told him ive had drs for years noow who still dont really beleive me about that 2 issues and so it was nice that he readily agreed that those issues cant really be used to rule out having hydrocephalus.. He had a couple opinions about what we could do as he isnt 100% certain abour what is going on and we both agree it may just not even be the shunt. 1 was that he is going to talk to his Peds Nsg colleague who is relatively new (as is he but ooh gosh extremely easy going and nice and seemed to know more than some of my drs combined about MPS, not that I have anything against my other drs just that it was nice he seemed to either have seen MPS before or had studied it before I came) but one of his colleagues specialties is Hydro and he wants to see if she concurs with his thoughts. 2 if she agrees he would like to do a shunt study to test if the shunt is flowing to slowly ie is fluid moving through but not adequately or is it partially clogged as some of my drs have thought in the past and which ive always wondered (bc my 2nd revision was due to inadequate shunt intracranial flow) which could explain why my headaches and nausea and occassional (couple times a week or sometimes just once a week) vomiting are occuring (or it could show that the shunt is working fine) and 3 another ICP study over a couple days depending on the shunt study. 4 if we where able to demonstrate it was shunt related he would scrap the LP shunt altogether and put in a VP shunt as he commented how less effective LP shunts are, how much more problematic/failure rate they have and that they are much harder to test than VP shunts. Time will tell.

While I dont see other drs at UW Hospital (all drs are in Milw and a couple in Mn) It turned out to be good since I also met with the Pain Coords (adult and Peds Coords) I work with at this particular hospital and we are going to start working on a state wide meeting for Pediatric pain outreach and awareness meeting for families and for professsionals with the plan to have 1/2 the day for professionals and 1/2 the day for families but w/professionals in attendance. The goal would be for this to occur in about a year or so and just trying to reach out to schools (school nurses in particular) and hospital staff so that people start looking at this issue more (bc kids as you know cant verbalize their pain and parents do not always know what to look for and providers do not always ask).

I had the Pulmonary testing yesterday which took a little over an hour and a 1/2 due to some issues with a few of the tests which we had to repeat quite a few times and 1 test we scrapped after the 2nd time (5 minute test of pure oxygen and a flush to rinse out nitrogen from the lungs while breathing through a tube each time) and tried a different test altogether inside of a sort of sealed box. I see my pulmonary dr (or one of them this particular one is sleep medicine pulmonary) on thurs so will get those results and her opinion of the cardio-pulmonary issues and what she thinks and then will do the sleep study next week. Ironically when finally leaving the pulmonary tests monday after ERT I ran into my other pulmonary dr who is the dr that originally referred me to this dr I will see thurs. I also see Pain mngmt for normal fup and Orthopedics this week. All for now will update soon. Take care Erica

Port-a-Cath placement via Interventional Radiology today

Hello All, Last week I had the hip injection through my Pain Mngmt dr ordered by my Orthopedic dr which was done under twilight sedation simply bc this dr is an anesthesiologist and well aware of my airway and lung issues. That went well other than the delay in getting to the procedure (1 hour wait in getting solu-cortef injection and overall delay) and afterwards while I dont remember much other than the initial injections (dye and numbing) it all went fine and no complications though minus the soreness (just different than the discomfort and "clicking"/tightening I get already in the hip joint and from trocanteric bursitis ) other than really low BP but that isnt altogether unsurprising either as my BP tends to run pretty low day to day. Today I had to be at Froedtert in Interventional Radiology for the Port a Cath placement which was placed in my old cardiac-reveal monitor (a continous EKG monitor which is read much like a continous feed device such as pacemakers, etc but purely monitor) scar site at the left upper shoulder and jugular vein region (2 scars - 1 new, 1 old). This was pretty much on time other than once again the order was written ahead of time by my endocrine dr for the solu-cortef (stress dose of cortisol replacement) so that was run an hour ahead of time and the clindamycin IV antibiotics as well. Ports are usually put in under sedation but my Interventionalist gave me the option of doing it under a nerve block while awake (I could have done twilight sedation or even some kind of nerve block and versed combo) to avoid us having to deal with the lung issues and having to worry about the airway issues. So while the normal precautins where taken like monitoring oxygen, heartrate, etc., I was able to talk to the interventional people the entire time which was definitely interesting! The only minor complication (and not really a complication) was that my provider had to place and remove the Port a couple times while he dealt with what he called a "quite a bit" of old scar tissue from the old cardiac monitor in order to get the cathether in properly and without it being kinked. Altogether the actual procedure still only took about a 1/2 hr from top to bottom and was totally worth the mild discomfort of having to lay flat for that period with my head turned in order to avoid dealing with airway!! I am home now and it is a 'baby' port secured and covered by surgical glue and steri strips which will fall off on their own and i'll follow up with Intrvnt'l Radiology clinic sometime next week - the glue and strips will fall off in 7-10 days. The area is a little sore (but still numb) and turning quite black and blue + the port itself is much more visible than I actually thought it would be but I think that is because of my thin build overall. All for now - Dont forget to vote if your in WI! Erica