Need Awareness/ Prevention/ Research on Superior Mesenteric Artery Syndrome (SMAS)

Posted by mattis1best @mattis1bestyahoocom, Aug 16, 2011

“With only about 500 reported cases in the history of English-language medical literature, recognition of SMA syndrome as a distinct clinical entity is controversial, with some in the medical community doubting its existence entirely. SMA syndrome is also known as Wilkie’s syndrome, cast syndrome, mesenteric root syndrome, chronic duodenal ileus and intermittent arterio-mesenteric occlusion. It is distinct from Nutcracker syndrome, which is the entrapment of the left renal vein between the AA and the SMA.

SMA syndrome is estimated to have a mortality rate of 1 in 3. Delay in the diagnosis of SMA syndrome can result in fatal catabolysis (advanced malnutrition), dehydration, oliguria, electrolyte abnormalities, hypokalemia, acute gastric rupture or intestinal perforation (from prolonged mesenteric ischemia), gastrectasia, spontaneous upper gastrointestinal bleeding, hypovolemic shock, aspiration pneumonia, or sudden cardiovascular collapse from increased velocity of bloodflow in the SMA due to the reduced mesenteric angle.

Go here to this link to know about my fight http://rare-disease-day-belfast-2011.blogspot.com/2011/02/paula-mattis-is-real-super-hero-she.html ***Visit our sister group, by Samantha Mina: http://www.facebook.com/group.php?gid=170641319067&ref=ts Also go look and my group http://www.facebook.com/group.php?gid=129829963700139&v=info Superior mesenteric artery (SMA) syndrome is a very rare condition characterized by recurrent vomiting, abdominal distention, weight loss, and postprandial distress. The cause is compression of the duodenum between vessels and the vertebrae and paravertebrae muscles when the angle between the superior mesenteric vessels and the aorta is lower than 18°. The diagnosis is difficult and usually is made by exclusion. Conservative management is helpful at first; however, symptom recurrence would indicate surgical treatment. EVERY LIFE COUNTS Let’s get people the Public, Doctors start taking this more seriously and get Awareness/Prevention, researching possible causes and work towards early detection
Thanks, Paula

@gregh, Greg

So sorry for your frustration – I hope that her doctor in Atlanta is able to offer some encouragement. Do you think he could contact the doctor at Mayo in FL and make a personal referral/appeal for an appointment for your daughter? Might be worth a try.

Teresa

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@hopeful33250

@gregh, Greg

So sorry for your frustration – I hope that her doctor in Atlanta is able to offer some encouragement. Do you think he could contact the doctor at Mayo in FL and make a personal referral/appeal for an appointment for your daughter? Might be worth a try.

Teresa

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That is the plan right now. We have already identified all the info that the clinic will want from him to refer her. I know he will do whatever it takes or just about whatever we ask him to do. He is a wonderful doctor. The only doctor that believed her and didn’t give up on her. Ever. I will definitely advise you of the outcome.

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@gregh Good to hear Greg. This doctor in Atlanta sounds marvelous. It is hard to find doctors with that kind of compassion and intuitiveness.

We are wishing you and your daughter the best!

Teresa

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@hopeful33250

@gregh, Greg

So sorry for your frustration – I hope that her doctor in Atlanta is able to offer some encouragement. Do you think he could contact the doctor at Mayo in FL and make a personal referral/appeal for an appointment for your daughter? Might be worth a try.

Teresa

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Hi Greg,
I agree, that getting a referral from her physician in Atlanta is probably is a good course of action. I’m so glad that your daughter has both you and her doctor advocating with her.

You may also wish to discuss the frustrating experience you had to the Florida Office of Patient Experience. Please call them during weekday business hours at 904-953-2019.

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As an update, it was the Gastroenterology Department that she spoke with that couldn’t/wouldn’t help her. That is one of the reasons this has been so disheartening. I guess we all thought that when they told her to call for an appointment we were on our way to getting healthy. It got her hopes up, only to have them completely destroyed. And this isn’t the first time. She was in the hospital for three weeks last year. That was when we finally got a diagnosis and then she got her feeding tube. Unfortunately, the tube doesn’t seem to be the answer for her. The next procedure is the TPN I guess. Now, I know I’m not the sharpest knife in the drawer, but I would think that a diagnosed case of one of, if not the rarest gastro diseases around would be of interest to someone. At any rate, the appointment with her doctor here is this coming Tuesday. My wife and I both are going with her.

Fingers crossed……….

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Just a quick update…..had our appointment with the doctor yesterday. She is down a couple more pounds and they are scheduling her to get a TPN. Her doctor got all the information I had about the referral and said he was going to get his assistant on it immediately. So we will wait to see what happens. Thank you for the info…..I will update as we get new info. Maybe it will help someone else…..

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Thank you, @gregh. We are confident that sharing your daughter’s journey is going to be a means to help and support others on theirs.

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@gregh

Just a quick update…..had our appointment with the doctor yesterday. She is down a couple more pounds and they are scheduling her to get a TPN. Her doctor got all the information I had about the referral and said he was going to get his assistant on it immediately. So we will wait to see what happens. Thank you for the info…..I will update as we get new info. Maybe it will help someone else…..

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Hello @gregh

So glad that your doctor is able to assist you! Thank you for the update – we look forward to hearing from you again.

Teresa

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@sharronbz

My son had a Ladd’s procedure to re-arrange his intestine away from the SMA, yet more than 6 years after the surgery he still has daily pains. 2 years ago he got spinal cord pain pacemaker that helps him a little. I wonder if you heard of other SMA patients that their pain continue post surgery?

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Hi @sharronbz,

I came across this journal article about Ladd’s Procedure, https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3073800/ which states, “Adhesion-related obstruction is the most common and significant postoperative complication [7]. Adhesion-related obstruction is mostly happening within the first postoperative year but can occur at any time.”
I was wondering if bowel obstruction has been ruled out as the cause of pain for your son?

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Hi I am just seeing this and wondering if anyone has told you this is could be a vascular disease? My superior mesenteric artery and celiac artery were compressed by my diaphragm. Is that what you daughter has going on with the SMA? My GI dr at Mayo really was of no help. My vascular surgeon was excellent. They problem is that along with the vascular issues, I truly believe that it causes all other GI symptoms. If you look at MALS topics and groups you may see a lot of similarities. Well good luck to you, I understand your frustration. I have had 3 surgeries and diagnosis and still not sure of other GI symptoms. You think you find an answer, but then there is something else….Hang in there

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@gregh

Just a quick update…..had our appointment with the doctor yesterday. She is down a couple more pounds and they are scheduling her to get a TPN. Her doctor got all the information I had about the referral and said he was going to get his assistant on it immediately. So we will wait to see what happens. Thank you for the info…..I will update as we get new info. Maybe it will help someone else…..

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Hello @gregh

I was thinking about your daughter and wondering how she is doing now. How is she doing with the TPN? Was she able to get an appointment at Mayo?

If you are comfortable providing us with an update, we would appreciate hearing from you.

Teresa

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Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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@gregh

Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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That’s great @gregh! I am curious if you don’t mind me asking what is TPN. I have been through so many GI drs and tests and never heard of it, or maybe the abbreviation. I posted on Jan. 11 about the possibility of it being vascular and MALS. It is very rare and overlooked. I feel for your daughter so young. Most of the MALS patients are female and young so just a thought. Well good luck to you. You can try calling the nurse of the department and tell them you had a call for an appointment. Maybe that may help better then just going through scheduling. I got through to my dr. once by saying he called and left a message and operator put me through. He did, but otherwise they don’t list numbers. You are not lying since you were given an appointment “time”. Just a thought. I understand your frustration. I wish you the best

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@gregh

Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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Here is the link. A lot better than me trying to explain it. She is hooked up 14 hours a day. We’re just lucky that her employer allows her to work from a home office. Any more questions feel free to ask. I will respond as best I can.
https://en.m.wikipedia.org/wiki/Parenteral_nutrition
Thank you,

Greg

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@gregh

Just wanted to give a quick update on my daughter Shelby. We still haven’t gotten in at Mayo. However she had her TPN pick line put in about three weeks ago. And while it’s early in the process, Shelby has put on about 6 pounds. Doesn’t sound like much to most folks, but to the people on this board they know how important it could be. We had a Sunday family dinner about two weeks ago……….it was the first time in I don’t know how long that I watched her clean her plate. Fried chicken, mash potatoes, biscuits and gravy. Her spirits have been better too.

Like I said, it’s early, I know that. But I’m looking for good anywhere I can find it. We have an appointment with her Gastro on Friday. So maybe there will be more to share.

Thank you so much for thinking about my Princess.

Greg

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@gregh Hello Greg:

Thank you so much for the update! I’m glad to hear that she is doing better. Yes, I believe that a 6 pound gain is worth celebrating!!
Please keep us posted on the progress of your Princess, we are all in her corner and cheering her on.

Teresa

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