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Marfan's-update on cousin

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I wrote a while back on my cousin's wife's hospitalization from severe chest

pain. She is in her 40's and has Marfan's. My parents saw her and found out

a little more of what is going on. Apparently the walls of the aorta are

quote: " separated from the top (breast bone area) to the groin " .

She is going to need surgery. There are only two hospitals in the country

that do it. One in the East and one in Texas.

I know Sue Ginley wrote to me on dissecting aneurysms, but now I can't

locate it. My parents are wanting some more information on this.

My cousin's wife has only one sister left living, so our family is her main

support system.

Until her surgery she can't do anything around the house.-absolutely no

lifting, etc. Mainly personal care and that's about it.

Does anyone have any info or web sites, that I could go to for info? My

parents and aunt are very concerned and would like to know what's in store

for her, success rate of these surgeries, etc.

thanks in advance for any information.

H.

HEDS

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,

I am so sorry to hear that this situation has escalated. I am sure you are

all terrified.

An aortic aneurysm is the dilatation (widening or bulge) of a portion of the

aorta, usually at a weak spot in the aortic wall. The aorta is the largest

artery in the body. It carries all the blood that is pumped out of the heart

and distributes it, via its many branches, to all the organs of the body.

The aorta projects upwards from the heart in the chest and then arches

downwards, travelling through the chest (the thoracic aorta) and into the

abdomen (the abdominal aorta). The normal diameter of the abdominal aorta is

about one inch.

Dissecting aortic aneurysms are classified according to anatomical location:

Type A involves the ascending aorta; Type B originates in the descending

aorta. Acute aortic dissection is often fatal within one month of onset.

Surgical treatment may be delayed in aneurysms involving the descending

aorta until the blood pressure has been controlled and edema and friability

of the aorta are diminished. The usual course of treatment for an aneurysm

of the ascending aorta is immediate surgery. The surgical procedure for

either type is aimed at either repairing the intimal tear or removing the

affected portion of the aorta. This may be done by suturing the separated

aortic layers back together or by removing the damaged section of the aorta

and replacing it with a synthetic graft. If the operation is successful and

the patient survives the first few weeks after the operation then the

outlook is quite good so long as the blood pressure is carefully monitored

and controlled. Marfan's is a factor in this mix which weakens the

structure of the blood vessels however, it is a grave situation indeed.

Please let me know how she is doing and how you are.

Jill

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,

Is the Tx Hospital St Luke's??? We have one of the

best cardiac centers int he world there. My neighbor

across the street is a cardiologist at St Luke's too

boot.

=====

Love, C " Cass' Momma "

HEDS

Houston, TX USA

__________________________________________________

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Hi ,

Well, Jill said it pretty well. I will continue praying for her and for a

full recovery as it sounds if she is in the right hands, and a graft takes

well, she will be all right. Please let her know we are praying for her on

our list as well.

Love, Sue Ginley

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Jill,

Thank you for the information. I'm glad I waited to call her.

Dad mentioned that she was on blood pressure medication also,(she's been on

one-a beta blocker) and has to be extremely careful. Now I understand the

implications of what is going on.

My parents went down to see her, and how she was doing, and they both are

understandably really anxious about what is going on. With this info, I now

know more how to be of support.

It's not going to be easy, knowing they have an 8 year old daughter with

Marfan's too. All of this has been so hush-hush over the years. One, the

family didn't want to accept the fact I have EDS, much less the fact that my

cousin's wife has Marfan's. That's why there has been the secrets on any

info. I did have a really nice visit with her last summer-lots of

EDS/Marfan's talk, and mom said she's looking forward to talking to me.

To be honest, I've held off, not knowing what would be said and having so

little to prepare myself emotionally to handle it.

It's a poor reason to not call some one that needs to talk more now that at

anytime, especially to some one that understand what it's like to have a

collagen disorder and is a family member.

Thanks again for the support, and the information.

Subject: Re: Marfan's-update on cousin

> I am so sorry to hear that this situation has escalated. I am sure you

areall terrified.

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,

Dad didn't say which hospital, but that it was one of the two best cardiac

hospitals. I'll let you know what I find out, after I call her.

~~

From: " C. " Subject: Re: Marfan's-update on cousin

> Is the Tx Hospital St Luke's??? We have one of the

> best cardiac centers int he world there. My neighbor

> across the street is a cardiologist at St Luke's too

> boot.

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,

Now more than ever your cousin needs your support and understanding, even if

it is just a listening ear. You will not know all the right things to say,

but don't let that stop you from calling and just talking. I can't tell you

how many of my old friends don't talk to me anylonger, and I know it is

because of all the lifethreatening things I have gone through. It hurts to

think they can't put their own feelings and fears aside to be a friend. To

be very honest I consider all my new EDS friends to be my closest friends

now. They are truly the only ones who can understand what I am going

through, who I can joke about morbid things, and not have them think I am

weird. So go ahead and call her, she needs you now more than ever.

Love, Sue Ginley

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