"The truth is rarely pure and never simple" (Oscar Wilde).
This blog reflects my personal life with but not defined by MPS I- reflecting the peaks and valleys of this dx., and my life despite a rare disease while rarely falling within the standard definition of this disorder.
See below for an article on the Heart procedure next wk - it is not specific to what will be ablated (killed/burned basically) in my case but the area being located and ablated/killed next wk is the Sinus node or the heart natural pacemaker.
The risks with this or the biggest risk really I guess is the procedure causing to slow of heart rate and thus a need to place a pacemaker (basically a device that controls/mainrains the heart heart rate/function )
Hopefully we won't end up on this route as it would for sure make things like MRIs (which are needed for the MPS bone/spine issues that occur and MRIs help us with things like the Hydrocephalus mngmt when it comes to surgery planning BUT as Dr.Bragg said a few mo's ago if it did come to this then we'd just have to go to CT Scans and deal w the excess radiation that goes along with these.
Anesthesia wise we are using the same Anesthesia (airway) dr i've had for all 3 of my Heart (OHS) surgeries and the 2 prior heart procedures in the past (now 3) yrs but actual airway wise I am not entirely sure what the plan is.
I know they have the Endocrine (stress dose steroid) requirements from my Neuro Endocrine dr so I guess given it's the same anesthesia (airway) dr as prior and same Heart Team as prior im not to worried and am sure she'll (Dr.Taylor, et all) will have a good plan.
At this point the plan is to leave my Port accessed after Tues's (ERT) Infusion and use that for initial anesthesia then once under Dr.Taylor or her Team will insert an peripheral IV as she said the Port isn't ideal for use for this procedure.
I'll stay on Coumadin for the procedure, so I am not entirely sure how that affects recovery BUT hope they'll let me atleast lay with my head propped up some in recoveryrecovery after the procedures if I keep my lower body flat (to prevent bleeding, especially due to INR will likely be high ( thin blood). - We've had issues keeping INR stable due to the pressure issues but have figured out a few tricks that atleast help some so we'll see where it is when I re-test Tues.
As long as no complications it's expected to be just an overnight stay at CHW.
Below is a mix of copied from an article and my own writing so if it seems weird the would be why! =)
Article on Heart Rhythm (EP) Ablation Study
Related terms: cardiac ablation, radiofrequency ablation .
Electrical impulses from the heart muscle cause your heart to beat (contract). This electrical signal begins in the sinoatrial (SA) node, located at the top of the heart's upper-right chamber (the right atrium). The SA node is sometimes called the heart's "natural pacemaker."
For most people with arrhythmias, medicines work quite well to control the abnormal heart rhythm. But when medicines don't work, doctors may suggest catheter ablation.
The procedure is performed in the Cardiac Cath lab) or the Electrophysiology Lab.
Once you are in the cath lab, television monitors, heart monitors, and blood pressure machines. You will lie on an examination table, which is usually near an x-ray camera. Small electrodes (EKG) will be placed on the chest. These electrodes hook up to an electrocardiogram machine which monitors your heart rhythm during the procedure.
After gaining entrance into a blood vessel in the groin, arm, or neck, doctors insert several long, thin tubes with wires, called electrode catheters, through a sheath and feed these tubes into the heart. They use a video monitor (like a TV screen) to see the process.
To locate the abnormal tissue causing arrhythmia, doctors send a small electrical impulse through the electrode catheter to activate your arrhythmia. Other catheters are used to record the heart's electrical signals and locate the abnormal tissue.
Doctors will then place a catheter at the exact site of the abnormal cells in your heart. The radiofrequency energy is sent through this catheter to cauterize the tissue causing your arrhythmia.
A Heart Cath will be done initially to measure pressures in the various areas of the heart and lungs before the Heart Rhythm study is started.
From the last Appt w my Heart Team they had planned to do some fluroscopic guided views of the lung space or maybe it was the area around the lungs to better view this. I admittedly am not 100% sure what that was but using X-ray guidance to view the abnormally elevated chest wall if I remember right and perhaps the abnormal fluid spaces.
Given the VPL Shunt drains to the 1 Lung space I'm not to sure off hand how that works though at the time it was explained it did make sense.
After the catheter is removed, firm pressure is applied to the site the groin to stop any bleeding. To avoid bleeding at the catheter insertion site, you will need to lie very still for several hours, either in the recovery area or in your hospital room.
Catheter ablation usually takes 2 to 4 hours. If you have several areas of abnormal tissue, then the procedure may take longer.
Otherwise nothing new in the shunt saga which if its not already been obvious has been aggravating! I don't see the other Peds Nrsgn (Dr.Bragg's former Partner) till Aug. 11 which given 1 of the shunts isn't working right and we almost virtually know it given both the scans and the CT Scan is nothing short of frustration there!
I asked Dr.Brahh her opinion on this if she tight her former Partner would atleast let the NPs do Shunt
Intermittent shunt taps till I can see hmm and she was not thrilled this has still not been resolved.. I had an eye Appt last wk to have glasses script increased yet again (2nd time in a yr) as the shunt/pressure issues really affect that.
Dr.Bragg in turn messaged me back and was going to send another msg to this former Partner, see if he'll see me sooner. She strongly seems to feel this needs to get taken care of sooner than later which its been going on for mo's and several different scans show there's an issue not to mention vision changes amongst other things so I don't disagree with her!
I really don't want it to come to that (as much as I like Dr.Bragh and hated her moving to AZ, not being here) but numerous if my Providers really feel at minimum of should contact at least some care w her once she has "Adult privileges" at her new hospital system. It's. A mess right now we the shunts and my care though certainly not for lack of Dr.Bragg trying to help, she's been amazing and persistent when there's no reason she really has to be!
Dr.Bragg and I have talked about if really needed once she gets privileges at the Adult hospital affiliated with her new Hospital I can see her in Phx which I am glad that option is there and I really like Dr.Bragg of course but what a pain (seriously) it would be to have to trek out there. I can remember the 2 times I had surgery in MN which was at least semi close enough my Parents came and then they went home from the 1 surgery for a day or 2 (about a 5hr drive 1 way) and my Dad came back to get me. That was likely one of the more memorable drives for him given a huge snow storm cropped and 5hrs turned in to far longer on his way to MN.
I have friends in AZ of it comes down to it. A now eine really!
It will suck having to break in a new Neurosurgeon if this new guy coming to UW works out but I also love my Nurses and Team on D6-4 (Neurosurgery at UW) so am praying. I can always do w/ Dr.Bragg like I do with my Genetics dr, see her maybe once a yr but in mean time just have to pray the new guy really is a nice guy and interested in complex cases with underlying genetic dx.
Ironically I ran in to my Neuro Endocrine Dr (she works with the Neurosurgery Team at FMLH, where I get my wkly ERT infusion and where my 1st Neurosurgeon was) and 1st thing she asked me when she stopped me (I'll admit I was in my own world, on a mission to go home so not paying attn when she walked up to my heading to the elevators:) ) but literally 1st thing she asked was "have you found a new Neurosurgeon yet " which we talked some and she comments along the line "We'll why don't you just see her in Phx?"
I am just praying Weds's ♡ procedures go well, we can figure this shunt stuff out soon and overall the new/former Neurosurgery issue works out best however it's supposed to work out.
Will try to update later in the wk after Weds's Heart stuff.