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.
My journey started long time ago when i was not Diagnosis it took 3-4 more years for me have a diagnosis and they Sadie I did not have it even with the test saying I did so then I went and got a 2ND opinion at John Hopkins with a new gastric Dr is name is Dr. Gerard E. Mullin (gastroenterologist) I had a pick line in before I seen Dr. Gerard E. Mullin gastroenterologist then from there Lot's of test then he referred me to Dr. Michael R. Marohn, (laparoscopic gastrointestinal surgeon) after June We where going to have my surgery done for august 13Th before that date came I was back in the hospital at john hopkin with SMA SYNDROME and pancreatitis for 2 weeks but it was OK I got to have the surgery for august 13th before I went for surgery I had a feeding tub put in they took that out when he did the surgery for the SMA SYNDROME and also took out my appendix, my gallbladder and also took an biopsy of my livery wich was OK. I will be seeing Dr. Michael R. Marohn and another Dr on Wednesday 29Th. More things have been going. Paula Mattis
My journey started long time ago when i was not Diagnosis it took 3-4 more years for me have a diagnosis and they Sadie I did not have it even with the test saying I did so then I went and got a 2ND opinion at John Hopkins with a new gastric Dr is name is Dr. Gerard E. Mullin (gastroenterologist) I had a pick line in before I seen Dr. Gerard E. Mullin gastroenterologist then from there Lot's of test then he referred me to Dr. Michael R. Marohn, (laparoscopic gastrointestinal surgeon) after June We where going to have my surgery done for august 13Th 2010 before that date came I was back in the hospital at john Hopkins with SMA SYNDROME and pancreatitis for 2 weeks but it was OK I got to have the surgery for august 13Th before I went for surgery I had a feeding tub put in they took that out when he did the surgery for the SMA SYNDROME and also took out my appendix, my gallbladder and also took an biopsy of my livery wich was OK. I will be seeing Dr. Michael R. Marohn and another Dr on Wednesday October 29Th 2010. I seeing the Dr had blood work done and then I went to get a MRCP test done because they think now I'm have something going on with my pancreas. I go back to see the Dr on November 3rd 2010 to see about the blood work and the MRCP test I had on October 13Th 2010.
I'm in pain, vomiting, diarrhea all the same things I had befor my smas surgery and on TPN for month and 3 weeks with no weight gain, I cant go to my Dr's Intel I have insurance there are so many test to be done this has all been on hold for 2 months now. I had very good insurance from my job but dont have that any mor...e. The GI sade I cant go back to my job ever that in my condesion I cant do my job ever. My GI sined my disabilty retirement and I got an low estmated of retirement benefit of 88.00 a month only but wating for the state to give me amont then I will be in retirement. So sad I loved my job with spacel ED kid's those I worked with.
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January 8 at 11:42pm Need to see my GI sometime tomorrow because johns Hopkins called him and Sade that the TPN is not working I'm not gaining any wight and my symptoms have not improving. I have to get a high amount of bills Intel I can have insurances johns Hopkins Sade to the GI. So I'm waiting for the GI sucutery to call me back to see... if I see the GI tomorrow are not but Johns Hopkins Sade to the GI that he need to late them know if I need not to be on TPN any more are not, if thees test need to be done asap are go into hospital.
Also need a ride up to the GI.
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January 19 at 4:42pm My GI appointment was something else. I go to johns Hopkins for an Upper GI and small bowel on Monday the 31st then see my Dr on Tuesday Feb 1st and go to umc to get MRCP with secretin enhanced. I also have to see surgical consult at umc.
I have a narrowing from the surgery so they may have to go back in also have things going on with pancreas. My Dr Sade today I my not be well for the rest of my life because of both things going on I may be on TPN long term.
Samantha Mina Much love to you too, Paula! Thank you for always being positive despite all you are going through. I am sorry you are experiencing ongoing problems from your surgery.
Everyone: It is important to note that many of the patients who do not fe...el relief post-op are struggling with significant issues other than their SMAS that contributed to the condition's development in the first place. It is necessary to identify if a co-morbid condition, root cause, or even a consequence/complication of SMAS is present and address that issue as well, because intestinal bypass surgery will only treat the SMAS and nothing else.
Everyone, let us keep Paula in our prayers as she battles ongoing post-op problems. She will have a surgical consult soon, to discuss the possibly narrowing of her anastomosis. She also has to see a cardiologist. She had an Upper GI on 1/31, she saw her doctor yesterday. She is going to UMC to get MRCP (enhanced with secretin) her ongoing pancreatic issues on Thursday at 3:15pm.
***Visit our sister group, by Samantha Mina: http://www.facebook.com/group.php?gid=170641319067&ref=ts and her Group http://www.facebook.com/group.php?gid=129829963700139&v=app_2373072738&ref=ts#!/group.php?gid=129829963700139My journey started long time ago when i was not Diagnosis it took 3-4 more years for me have a diagnosis and they Sadie I did not have it even with the test saying I did so then I went and got a 2ND opinion at John Hopkins with a new gastric Dr is name is Dr. Gerard E. Mullin (gastroenterologist) I had a pick line in before I seen Dr. Gerard E. Mullin gastroenterologist then from there Lot's of test then he referred me to Dr. Michael R. Marohn, (laparoscopic gastrointestinal surgeon) after June We where going to have my surgery done for august 13Th 2010 before that date came I was back in the hospital at john Hopkins with SMA SYNDROME and pancreatitis for 2 weeks but it was OK I got to have the surgery for august 13Th before I went for surgery I had a feeding tub put in they took that out when he did the surgery for the SMA SYNDROME and also took out my appendix, my gallbladder and also took an biopsy of my livery wich was OK. I will be seeing Dr. Michael R. Marohn and another Dr on Wednesday October 29Th 2010. I seeing the Dr had blood work done and then I went to get a MRCP test done because they think now I'm have something going on with my pancreas. I go back to see the Dr on November 3rd 2010 to see about the blood work and the MRCP test I had on October 13Th 2010.
I'm in pain, vomiting, diarrhea all the same things I had befor my smas surgery and on TPN for month and 3 weeks with no weight gain, I cant go to my Dr's Intel I have insurance there are so many test to be done this has all been on hold for 2 months now. I had very good insurance from my job but dont have that any mor...e. The GI sade I cant go back to my job ever that in my condesion I cant do my job ever. My GI sined my disabilty retirement and I got an low estmated of retirement benefit of 88.00 a month only but wating for the state to give me amont then I will be in retirement. So sad I loved my job with spacel ED kid's those I worked with.
See More
January 8 at 11:42pm Need to see my GI sometime tomorrow because johns Hopkins called him and Sade that the TPN is not working I'm not gaining any wight and my symptoms have not improving. I have to get a high amount of bills Intel I can have insurances johns Hopkins Sade to the GI. So I'm waiting for the GI sucutery to call me back to see... if I see the GI tomorrow are not but Johns Hopkins Sade to the GI that he need to late them know if I need not to be on TPN any more are not, if thees test need to be done asap are go into hospital.
Also need a ride up to the GI.
See More
January 19 at 4:42pm My GI appointment was something else. I go to johns Hopkins for an Upper GI and small bowel on Monday the 31st then see my Dr on Tuesday Feb 1st and go to umc to get MRCP with secretin enhanced. I also have to see surgical consult at umc.
I have a narrowing from the surgery so they may have to go back in also have things going on with pancreas. My Dr Sade today I my not be well for the rest of my life because of both things going on I may be on TPN long term.
Samantha Mina Much love to you too, Paula! Thank you for always being positive despite all you are going through. I am sorry you are experiencing ongoing problems from your surgery.
Everyone: It is important to note that many of the patients who do not fe...el relief post-op are struggling with significant issues other than their SMAS that contributed to the condition's development in the first place. It is necessary to identify if a co-morbid condition, root cause, or even a consequence/complication of SMAS is present and address that issue as well, because intestinal bypass surgery will only treat the SMAS and nothing else.
Everyone, let us keep Paula in our prayers as she battles ongoing post-op problems. She will have a surgical consult soon, to discuss the possibly narrowing of her anastomosis. She also has to see a cardiologist. She had an Upper GI on 1/31, she saw her doctor yesterday. She is going to UMC to get MRCP (enhanced with secretin) her ongoing pancreatic issues on Thursday at 3:15pm.
With only ~500 recorded cases in history, a demographic of 0.013% and a staggering mortality rate of 1:3, this dangerous gastro-vascular disorder remains largely unrecognized and misunderstood in the medical world. But, we believe that it still deserves attention and research because EVERY LIFE COUNTS. This group is for patients, their support networks and all who want to advocate awareness for this life-threatening syndrome.
ReplyDeleteSee the Discussion Board's on facebook at this link http://www.facebook.com/group.php?gid=129829963700139&v=app_2373072738&ref=ts#!/group.php?gid=129829963700139"Roster of Doctors / Surgeons Who Can Treat SMA Syndrome" if you are looking for an MD closest to your home who can perform the gold-standard surgical treatment for chronic/severe SMA Syndrome, the "laparoscopic duodenojeunostomy." If you find a doctor/surgeon who is not on the list and would like to share, feel free to post a reply. The "Frequently Asked Questions (FAQs)" thread can hopefully answer some common SMAS inquiries.
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FACTS ABOUT SMA SYNDROME:
* SMA Syndrome was first discovered in 1861 in patients at autopsy. Although it is no longer terminal in this day and age, it still has a mortality rate of 1:3 (mainly because SMA Syndrome is an equivocal diagnosis of exclusion that is often missed, or reached only after the malnourished patient has passed the point of no return).
* SMA Syndrome is one of the rarest disorders known to medical science. Currently, it is recognized by the Canadian Rare Disease Foundation, but not by the US National Organization for Rare Disorders (NORD). (Too rare for the rare disease organization!?)
* Recognition of SMA Syndrome is controversial, with many in the medical community still doubting its existence or its validity as a distinct
February 3, 2011 8:40 PM
ReplyDeleteteri22399 said...
Hi, I'm another one of Paula's sisters. When Paula first told me about her problems, I knew she needed help fast. She had many people telling her she was faking to get out of work or to get attention (even doctors) but I can assure any doubters, this is real. You can't fake the pain she goes through. You can't fake the malnutrition. You can't fake the lack of energy. I have taken Paula to nearly all of her appointments. I was there when she was officially diagnosed. You could see the weight of the world come right off her shoulders that day. I was there for her surgery. We're devastated that she is still having problems. I feel that if her first doctor didn't spend years telling her she had bad heart burn, things would not be as bad as they are now. The syndrome has affected her entire body and every aspect of her life. Paula's always been skinny, but never malnourished. She always had energy and she was always happy. She liked working and took pride in whatever she did, even back in the days when she worked in a video store. Since this problem Paula can't do much of anything. She doesn't have the energy to do anything. She's been forced into early retirement. Sitting too long puts her in pain. Standing for too long gives her pain. Even laying down is painful, so it's a fight for her to even sleep. When people ask me what she's going through, I say imagine what it would be like if everything you tried to eat turned your stomach and intestines into knots and came back up. Imagine what it would be like to slowly starve to death. Let's hope people and doctors start taking this more seriously, start researching possible causes and work towards early detection.
Thanks for sharing this interesting and valuable information.
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