UPDATE-Mid Aortic Syndrome/Surgery Tomorrow!

Well, the doctors are coming to a conclusion I have a very rare disorder called Mid-Aortic Syndrome. about 200 reported cases around the world. We are still waiting on genetic testing to come back for Marfan Syndrome..

My surgery will be performed tomorrow to correct my celiac artery, they will be placing a stent with a major risk of an emergency bypass being performed.. There are only 3 cases reported of this relieving the pain, but at least it's a chance.

Hope everyone is having a reduced-pain day!
~Kayla

Sorry to hear this news...

I see in your signature that you already had the celiac artery reconstructed...so did that fail and they are doing this again?

I have to say that if it were my daughter (being that I am old enough to be your mom)...I wouldn't want such major surgery done so quickly as I would research this more and consult with Drs. who actually deal with this particular syndrome...The Boston Children's hosptial has it's own center dedicated to this...There are other treatments that can be done first to see if they work. I would be wanting a video conference with the Drs. there to see what they suggest..

And I'm also concerned about you having so many surgeries back to back and having so much trouble with this hospital and Drs/Nurses not giving you the pain control you need.

May I ask what tests they did these last 24 hours that they came up with this syndrome?

And because this is so rare...I'm thinking that this Dr. hasn't done this surgery before for this as I think you said you are in a more local/county hospital.

Anyway...just trying to figure all this out as it seems they are coming up with a different diagnosis every few days with you so that is a big concern.

Post Edited (Snowbunny21) : 11/20/2012 11:36:52 AM (GMT-7)

You have been through so much in your life already, i feel so bad for you. I will be praying for you sweetie.

I'm not a doctor. This is my point of view from my own research into my own symptoms.

I'm in the process of getting tested for a similar disorder, Ehlers-Danlos syndrome. Marfan's has some of the same symptoms, like many surgeries don't heal properly, especially on major blood vessels. Both are among the collagen related syndromes that can occur, they are genetic, and there's not a cure. The issue is, if they keep working on the same artery, it's eventually not going to hold. With EDS, stents and even colonoscopies are contraindicated, as the risk of unexpected rupture of the large blood vessels and intestines are high. If it's a collagen related condition, your body produces poor quality collagen, the stuff that holds everything together. It makes up most of your blood vessels, skin, connective tissue, ligaments, tendons, intestines, you name it.

The only way to know that is the genetic test. Rooting around in there if it's not an emergency before knowing if the disorder is present could very well make the situation worse.

If I were in your hospital bed, knowing what I know, and if my life was not in immediate danger from an aneurism or a ruptured vessel, pain or not, I would wait for the genetic testing to come back, and rule some things out, like EDS or Marfan's, since most of those have collagen disorders have specific genetic markers, and carry a higher risk of complications during and after surgery.

Or, at an absolute minimum, I would bring up the possibility of EDS or a collagen disorder with the surgeon him/herself so they know up front to handle those large caliber blood vessels with extra care. At least until the genetics testing is in.

I'm pulling for you hun.

Well, I've been working with this surgeon for a year now. He's well known and has performed miracles. People fly in from all over the country just to have him as a surgeon so I trust him with my entire life as does my mom. He knows my arteries extremely well and since he did my bypass is very familiar that they are so abnormal from seeing them up close in person.
Anyways, I can't really explain the whole story as I need to get sleep since surgery will be at 8am & it is currently 9pm here.. But this seems to be a very good option for me right now & with uncontrolled 8-10 pain despite the dilaudid, myself & family see this surgery as a sort of emergency. I've been in the hospital for 3 weeks because of the pain & am ready to go home.. Yet this will be the 4th surgery in these 3 weeks, I believe this is what I need.
Thanks for all the concern and prayers. It really does feel great knowing I have people who support and worry for me outside of my family.

I'll update as soon as I feel up to it after the surgery!
~Kayla

Kayla,

Just want you to know that you are in my prayers, I hope this surgery helps with your pain.

Know we are thinking about you.

Hugs,
Barbara