Our PA is going to have a girls day in with us today. She’s going to do art with us, paint our nails, wash our hair, bake, and give us a makeover. She said because we don’t get to get out very often’ she wants to treat us. We are so excited! Yay!
Ray
genetics appoint TW medical
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So, we had our genetics appointment on Wednesday. It was a good appointment. They had found two variants of uncertain significance. One of them was for Sticklers Syndrome, and the other one for blood vessel and muscle wall problems. The geneticist said that these variants didn’t explain my symptoms.
She is going to do a chromosomal array test to test to see if there are any deletions or duplications in my chromsomes. If that test shows nothing, she will do whole-exome sequencing. She also said that we get swelling in our ankles because of what’s called third spacing. This means that because of the connective tissue. She says that the fluids leak out of the blood vessels because they are weak and the fluid leaks in to my other tissues. So, it’s not that my body is going into fluid overload, it is because of my connective tissues.
Hope this update was interesting.
Ray
update on port placement
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Hello everyone,
So, yesterday, we went to Chicago to get our new port and our new feeding tube button.
We got Propofol for sedation. We didn’t get enough that we needed a breathing tube, but we got enough to be what they would consider sedated enough.
We told them how EDS affects anesthesia, and they said they know. Well, apparently, they didn’t. The med took forever to kick in, and we felt everything except getting the feeding tube replaced. We felt them putting the catheter in our neck into our jugular vein. The surgical glue on the right side where they took out our other port looks like a kindergartener took the bottle and just squirted the glue out. We are ore today.
Ray
update cental line
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Hello everyone,
So, our port is starting to be a problem. Our skin is eroding over the port, because the nurses at the infusion clinic are accessing it in the same place every time. The hole is supposed to close after the needle comes out, but it is not and is becoming a permanent wound. Our port is accessed 24/7, and so we don’t really get that much of a break. We do daily IV fluids through the port. Our GI who ordered it, doesn’t feel comfortable giving us any other option s besides another port. We haven’t even had this one for a year yet, and these are supposed to last for years. One reason our port is not lasting as long is because of our connective tissue disorder. We need 24/7 access. WE are going on the 19th to get the port removed and a new one put in on the other side. Our GI referred us to another doctor to get a second opinion, but that doctor doesn’t take our insurance. He doesn’t have anyone else to refer us to. Our primary care doctor wont’ refer us to anyone either because he’s not the one who ordered the port to be placed. We need a line like a Hickman, but we can’t find anyone to get another opinion. Thanks for reading this far.
We also found out that our feeding tube isn’t too long, the balloon just didn’t have enough water in it. It had 1.5 ML of water in it, and it is supposed to have 8 ML in it! No wonder it was too long!! We got a cream called Silvadine cream to put on it, and that seems to be helping with the irritation. Thanks for reading.
Ray
TW Medical port and feeding tube
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So, tomorrow, we have to go to Chicago to get our port and feeding tube site looked at. Our port is looking red and irritated because the nurses keep accessing it in the same place each time. They have a dime-sized area to work in. We might be allergic to the dressing, the needle or the skin prep. Also, our feeding tube stoma site is stretching which means there is space between the tube and our skin, so if we eat anything orally it drains out of our stoma and even if we don’t, the stomach acid leaks around the tube to! It burns away the good skin, and the tube is in the crease of a scar. It was the only place the radiologist could place it without a surgeon doing it. They told us to pack a bag in case they have to replace our port or do something with the tube. We are also going to see if we can talk to the palliative care team there to see if we can get into palliative care to get better pain meds or pain management as we don’t have any now, and EDS is so so very painful!!!! Thanks for reading if you’ve read this far.
Have a good night everyone. We will be up at 5:30 in the morning.
Ray
medical update
Hi everybody. We had our appointment with our POTS specialist yesterday. We found out that we have autonomic neuropathy, inappropriate sinus tachycardia, vasal vagal syncope and POTS. We found this out in reading our chart. Plus our doctor’s PA told us that the doctor suspected an autonomic neuropathy. Our hands and feet often fall asleep doing nothing. It also affects the temperature of our hands an feet to.
Just more diagnosis to add to pour list.
Ray
update on gastroparesis
It is gastroparesis awareness month!!! I am doing better with my feed rate now. Yay!!! I’m at 45 ML per hour now. I’m on a new medication called Promethizine. It is working.
Hope everyone is well!!
RAy
TW Medical ER and ⠝⠁⠥⠎⠑⠁
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So, our local GI is no longer prescribing our Zofran. The liquid, pill form, and the orally disintegrating tablets don’t work, so I was trying to get IV Zofran. We had to go to the ER to get nausea meds and fluids yesterday. My PCP won’t prescribe it either. Nor will my team at Northwestern. The ER gave me a script for liquid Promethizine. We were so tired when we got home yesterday that we just crashed. We are nauseous 24/7 except for the few minutes when we’re not running our feeds when we have to change our bag. I hate gastroparesis!!! Our motility not only affects our stomach, it affects our colon to. Ugh!!
Ray
FROM AMELIA
Vacation in California
So, We are in California. We are having fun. We are moving here in September. Yay!!! Can’t wait!
We are going horseback riding today. Yay!
Ray
my multiple life 