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

@annewhit

Colleen, she is not doing very well–has been out of school for four months. Are you able to give me an idea how experienced the Mayo pediatric GI docs are with SMA?

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Anne, I’m so sorry to hear this.
Unfortunately, I cannot answer questions about Mayo’s pediatric GI docs level of experience. But here is information about the Inflammatory Bowel Disease Center for Children including contact information. http://www.mayoclinic.org/departments-centers/childrens-center/overview/specialty-groups/ibd-center-children They will be able to answer specific questions, including expertise with SMA.

@hamiltl80 speaks very highly of the care she receives at Mayo in Florida. Any insights you can give Anne when looking for an experienced specialist?

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@annewhit

My 14-year-old daughter was recently diagnosed with SMA syndrome. We live in Salt Lake City and have an excellent children’s hospital nearby but I’m not sure they have much experience with SMA. Any suggestions for excellent doctors who treat children? I’ve heard about a John Alverdy in Chicago. Any thoughts on him or anyone else?

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Hi @malaki, What a relief it must be to have a diagnosis and treatment for your wife. How is she doing?

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@annewhit

My 14-year-old daughter was recently diagnosed with SMA syndrome. We live in Salt Lake City and have an excellent children’s hospital nearby but I’m not sure they have much experience with SMA. Any suggestions for excellent doctors who treat children? I’ve heard about a John Alverdy in Chicago. Any thoughts on him or anyone else?

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My daughter was diagnosed with SMA in December of 2015. We had a consult appointment with Dr. Alverdy in Chicago in January. Personally, I would not choose him as a surgeon. He only gave us a 50/50 chance of the surgery to correct her SMA to work. He left the room during our appointment to discuss my daughter’s case with her GI doctor at UofC. It was then decided that my daughter would be put on TPN for a period of time. I did not like his personality and his staff is rude. Not something you want to go through when your child is so sick.

We aren’t getting much satisfaction from her GI at UofC either. I don’t feel that the team is taking my daughter’s symptoms very seriously. Her doctors are hard to get a hold of and they never call back. My daughter had an episode of vomiting for 3+ hours a day for 5 days straight. She was so weak she couldn’t get out of bed. I e-mailed her GI 3 times in one day, then the next day and the next day. It took the doctor 2 weeks to get back to me about my concerns. Everything is “hurry up and wait” here. It takes months to get a test taken, and then another month to see the doctor to get the results; it’s ridiculous. She had a PICC line inserted and she is on TPN. At first she gained 7 pounds, but then lost 6 when she was so sick for 5 days. She is still on TPN, and has gained a little weight, but her pain, nausea and vomiting have not gone away.

Our to sheer frustration, I went on line and made an appointment request for Mayo Clinic in MH. My daughter’s appointment is April 4. I hope I get better results with Mayo Clinic than I have with the UofC in Chicago.

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Hi @katiesmom, welcome.
This is such a tough journey to see your child in such pain. I hope that you and @annewhit connect. She, too, has a daughter with SMA.

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I was diagnosed about 3 yrs ago with SMAS. The team at the hospital, in Austin, Tx, had never seen a case, had only read about it and pretty much said they weren’t exactly sure what to do. They said the fatty tissue that holds up the artery to the duodeum was gone so nothing was supporting it up and the circulation was being cut off like a water hose. I had lost so much weight, they thought if they could fatten me up, that the fatty tissie that holds up the artery would come back. After days with suction tube in stomach, IV diet and the vommiting under control, they released me saying i would probably need sugery some day.
I’ve had small bouts and discomfort since then nothing big until last night. There is a certain pain right where the ribs meet that ive had off and on my whole life that i now associate with smas and it is almost unbearable right now along with the vommiting, just like 3 yrs ago.
Do these flare-ups of pain and vommiting eventually pass like last time?
Im up to my neck in hospital bill collections and my credit is screwed, but i know my health is more important. I’ve never reached out to others with this syndrome before and i’m just looking for some advice from others that can relate. Thanks, Amanda

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@manzfield

I was diagnosed about 3 yrs ago with SMAS. The team at the hospital, in Austin, Tx, had never seen a case, had only read about it and pretty much said they weren’t exactly sure what to do. They said the fatty tissue that holds up the artery to the duodeum was gone so nothing was supporting it up and the circulation was being cut off like a water hose. I had lost so much weight, they thought if they could fatten me up, that the fatty tissie that holds up the artery would come back. After days with suction tube in stomach, IV diet and the vommiting under control, they released me saying i would probably need sugery some day.
I’ve had small bouts and discomfort since then nothing big until last night. There is a certain pain right where the ribs meet that ive had off and on my whole life that i now associate with smas and it is almost unbearable right now along with the vommiting, just like 3 yrs ago.
Do these flare-ups of pain and vommiting eventually pass like last time?
Im up to my neck in hospital bill collections and my credit is screwed, but i know my health is more important. I’ve never reached out to others with this syndrome before and i’m just looking for some advice from others that can relate. Thanks, Amanda

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Hello Amanda,
I’m sorry you are suffering again with symptoms from SMA. My 19 year old daughter had surgery for this. I took her to an ER due to weird uncontrollable vomiting when she stood up. she was admitted and they diagnosed her with SMA. They scheduled laparoscopic surgery for her. She was in hospital for 1 day and it fixed the problem. I don’t know why they wait with some people for so long to surgically fix this. She feels much better and no more vomiting. There were some doctors not agreeing on treatment. One said to put in feeding tube one said to do surgery right away. She was of normal weight, so feeding tube seemed like it would not help in the long run. The surgery was done at the American Center which is part of UW Health in Madison WI. No more issues with SMA. I hope you can get some good treatment for the SMA syndrome. I was so pleased with the way my daughters problem was handled and fixed.

Sue

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I am reaching out to anyone that knows any Doctors that help with SMA Syndrome. I was just dx with it and I need some treatment plan to get me to stop throwing up.

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@danamarie18

I am reaching out to anyone that knows any Doctors that help with SMA Syndrome. I was just dx with it and I need some treatment plan to get me to stop throwing up.

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Hi @danamarie18, welcome to Connect! I moved your post over to this thread about SMA in hopes that you’ll be able to skim the discussion and find some helpful information. Please meet @mattis1bestyahoocom, @hamiltl80, @sbraudrick13, @candymarie72, @jobones94, @smfjf101, @malaki, @annehwhit, @katiesmom, and @manzfield.

I’m not sure where you’re located, but if you look at one of the comments from @colleenyoung earlier in this thread, she noted that Mayo Clinic in Minnesota has been recognized as the best Gastroenterology & GI Surgery hospital in the nation for 2015-2016 by U.S. News & World Report. There’s a complete listing of all the Gastroenterology & GI physicians here http://www.mayoclinic.org/departments-centers/gastroenterology-hepatology/doctors

To Request an Appointment, visit http://mayocl.in/1mtmR63 to find the contact phone numbers or use the online form.

After your diagnosis, did your doctor discuss next steps or treatment options with you?

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Since my last post in April, I have been doing much better even though I have not been to see a medical professional.
I have completely changed my diet. I pretty much live on smoothies, yogurts, oatmeal, soups..so more or less a liquid diet. I also eat very slow as to not overload my stomach. This might not work for everyone, but it has helped me tremendously with the vomitting and could provide some kind of ease until you get to a doctor or in between visits. My heart is with you! Amanda Field

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@hamiltl80

I also have this condition and we need to raise awareness. I have had this condition for around 18 years before it was correctly diagnosed but by the time they found it I was admitted to the hospital with 5 days of testing resulting in surgery to help fix the condition. I also have a Facebook page that is updated with the events that I have gone through and am going through. Each person who has this has a little different set of symptoms and circumstances they go through. Please visit all our pages and learn more about this condition, you could save someone who has this condition.

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I was just diagnosed with superior mesenteric artery syndrome a little over a week ago in the hospital. I was down to 102 pounds and ended up getting very sick which led to my ER visit and diagnosis. Currently, I am on 24 hour tube feeding and only allowed to consume full liquids. It is very hard to find accurate information on the syndrome so I was very blessed to find this site. I also noticed that there is no support group on Facebook so I started one and was hoping that we could get connected on there so we were able to raise awareness and help support each other. If you search this condition on Facebook, the closed support group page should come up.

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@malaki

wife test show SMA syndrome looking for surgeon at mayo clinic because so rare.any suggtions
she is 73 yr old.suffering for 2 year just now found cause.

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My mother was diagnosed with this at the beginning of this year. Her local doctors chose conservative therapy with some success. She is now back in the hospital with a 20 lbs weight loss and we are looking at trying to get her to Mayo under the care of Dr. Cangemi. I have heard he is the best at treating SMA. Any advice or words of wisdom?

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@malaki

wife test show SMA syndrome looking for surgeon at mayo clinic because so rare.any suggtions
she is 73 yr old.suffering for 2 year just now found cause.

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Hi @tracys, welcome to Connect! Mayo Clinic in Minnesota has been recognized as the best Gastroenterology & GI Surgery hospital in the nation for 2015-2016 by U.S. News & World Report. You can find a complete listing of all the Gastroenterology & GI physicians here http://www.mayoclinic.org/departments-centers/gastroenterology-hepatology/doctors

To Request an Appointment, visit http://mayocl.in/1mtmR63 to find the contact phone numbers or use the online form.

I’m tagging @ej9718, @smfjf101, @jobones94, @mattis1bestyahoocom, @malaki and @sbraudrick13 to see if they have any words of wisdom.

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