Echo and Cardiology

What a day for sure, wow! My Cardiologists nurse who helped me arrange the Echo and Appt for today came in to the actual appt ahead of my Cardiologist and said to me "You must be exhausted after a day that started that early and ends this long" and she was so right! Thankfully I had only intermittent episodes of the headaches and other periods where I had headaches (pressure) but wasnt as bad as it can get so I wasnt as exhausted as I otherwise would have been. I got to the Cardiology clinic at 8:30 and the Echo took about 45-50mins total start to finish. Afterwards I hop, skipped and jumped (ok maybe just walked) :) across to the Curative building where the Genetics clinic is and where my weekly infusion is. After getting accessed this went till about 2:20 and then back to the Cardiology clinic for the Appt with my Cardiologist. (this way I could leave the Port accessed overnight for tomorrow's surgery vs having to de-access it to get the Echo pictures).

So the Echo looked about the same so far the heart muscle itself isnt showing signs of hypertrophy (weakening. thickening) but my Cardiologist knows the L sided filling pressures are very abnormally high and thus creating severe L atrial enlargement. This could be the cause of the chest pain and shortness of breath and skipped beats/arrythmias but the aortic stenosis and very abnormal aortic and mitral valves as a whole with leaking and stenosis also factor in. He is ordering a cardiac perfusion test which they inject dye in and watch how this goes through the heart and muscle of the heart as the person exercises and this will tell him if the symptoms are from not enough blood and oxygen is getting to any part of the heart. The test will also tell us if there is diffuse coronary narrowing which he said is very different than CAD in a non-MPS person and in the literature has rarely been reported to cause symptoms but he still wants to make sure it isnt the cause/addition to our issues. What I appreciate is that he factored in my joint and lung issues and left it up to me to do the test or not; he felt strongly it would guide us if we could safely push off doing the valve replacements but he also didnt want to cause a severe up-tick in joint pain for 3 or 4 days after at the expense of ruling out or in a cardiac issue. I just said that while I didnt exactly want to do the test I knew it would alos give us the best chance of knowing a different look at the cardiac disease progression and would tell us/give us a better picture of if valve replacement is needed sooner than we want or on the flip side if we can push it off as we want for as long as possible.

He is changing the medication I take and although i'll continue to take Lasix 40mgs (this was upped back in Jan) he is switching the diltiazem long acting which is a calcium channel blocker to bisoprolol a beta blocker for which we will start at a low dose. This will be done after this surgery tomorrow. He gave his blessing so to speak for the shunt revision surgery saying "you've had alot of surgeries in the past years and handled all of these reasonably well; while we dont know when your heart will act up and cause a problem with a surgery I think you will do pretty well." I appreciated the candid and upfront opinion and also how to him it isnt just 'cardiac issues' and instead is a whole person with many underlying issues and finding the best solutions for quality of life. He talked about the symptoms I am having and how until we do a valve replacements he probably isnt going to be able to get me feeling as good as he would like but also doesnt want to do anything to make matters worse.

I just appreciate that he is one of the docs I see who is very down to earth, very knowledgeable about MPS and looks at it from a 'whole person perspective' vs 'Im a cardiologist and this is all that matters to me." :)

I have to be at the hosptial at 5:30 tomorrow with surgery scheduled at 7:30. It will be as much a surprise to others as to me what we will end up doing with the shunt. :)

Erica