sma syndrome

When my son was 16 he had surgery and he had a sma obstruction at his duodenum. They bypassed the sma syndrome. Now in Canada the specialist won't talk to you unless you have a referral from gp. To get a referral for one question will not happen. Does the bypass of the sma mean it could still be a concern for him? I haven't been able to find anything online and the gp doesn't know.

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Is he having GI symptoms that could be ascribed to SMA syndrome? You don't have surgery (the treatment for SMA syndrome) unless you're having symptoms. If it were me (me living in the States), I would certainly go to a GI doc. Since you're in Canada, I guess the next best thing is to talk to your generalist about whatever GI symptoms your son is having.

I don't quite understand why you mean by "bypassed the SMA syndrome." Perhaps you mean that they re-routed the small intestine around the section that was being strangled by the superior mesenteric artery (the SMA in SMA syndrome)?

Your generalist would be helped greatly by a whatever notes (pre-op examinations and imaging, surgical notes, and post-op examinations and imaging) that you can provide him/her.

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Hi Thanks for replying. We have the surgical notes and it says they “... bypassed the sma syndrome obstruction”. My son is 21 and tired of dealing with doctors. He was very frustrated with the doctor and doesn't want to go back to him. I thought I would try and find out if someone might know if the sma syndrome could become a problem for him again or if it could be treated as just medical history. Having some knowledge is better than none.

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I'm so sorry. I don't know if it will become a problem in the future. This is a rare enough disorder in the first place that predicting the future is likely difficult, especially when combined with EDS. I suppose a new section of the small intestine could become entangled in the SMA, but I really do not know.

What I'm missing here is the reason why you're pursuing a GI appointment at this time. Frankly, my humble, non-physician opinion is that if your son, who is sick of seeing doctors right now is not having GI symptoms right now, leave well enough alone. If he begins to have GI symptoms, especially GI symptoms that seem like those of SMA syndrome, push the SMA syndrome angle in order to get an earlier appointment with a GI doc.

It seems like what you need is someone well versed in the Canadian healthcare system. That is not me.

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Thanks I am not looking for a gi, my son is gathering up his medical history and this was a surprise to me. The surgery was done as an emergency in the middle of the night when the doctor thought he had perforated something after spending almost 2 hours placing a feeding tube that afternoon. All we got told was his bowel was fine. The doctor never mentioned any type of repair being done. We only saw him very very briefly immediately after the surgery done to reassure us and once when the pain meds were not working. My son was discharged by a covering doctor. If he hadn't asked for a copy of the surgery notes he would have never known.

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Oh, geez! That's not cool.

This board--http://www.inspire.com/groups/agmd-gi-motility/discussion--might be a better place to post your question. They might also be able to help you better understand the surgery, though remember that surgeries can be slightly different based simply on an individual's anatomy and pure circumstance. In addition, technique varies somewhat country-to-country and over time.

My humble opinion is that, if he's not having GI symptoms or stomach pain, leave it alone. If he has GI symptoms or stomach pain, push hard to see a GI doctor. I hope that doesn't sound flippant. I really don't mean it to. I understand that not having answers can be scary.

Good luck!

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This a really interesting post! My 14 year old daughter was hospitalized this summer and diagnosed with SMA Syndrome and she also has EDS. She was the most popular girl in the hospital because we were told there wasn't any other documented case in medical history! Did your son have SMA obstruction or SMA Syndrome? I've been doing a lot of research because our local teaching hospital has misdiagnosed my child for a while now and I'm in the process of obtaining her healthcare elsewhere. Not only did they miss they SMA Syndrome for several years they misdiagnosed the EDS as well. She's been at home with a NJ feeding tube since the end of June and we will be going to NYC for surgery.

For years my daughter has had intermittent GI problems, mainly pain and vomiting, and I was told it was abdominal migraines. I'd take her back when these episodes would recur and I was treated as an overprotective parent. We finally lucked out when the last ER doc finally diagnosed her correctly and she spent several days in the hospital and we were told to follow up with GI. The GI doc's treatment plan was "trial and error"... we could remove the feeding tube to see how she did but she lost the weight she gained. Thankfully we just stopped the feedings rather than pull the tube. She still has right sided abdominal pain and poor appetite so we do both tube feeding and oral diet. She has a healthy build and no major weight loss but is having problems gaining and maintaining weight. What we were told was that if she didn't have the surgery that the episodes would come back. I was advised that surgical intervention if conservative treatment didn't work was the best route to take but that surgery wasn't guaranteed to resolve the problem. After years of going through these episodes and no real progress post hospitalization we're hoping the surgery will be successful and she can go back to being a typical teen again.

If symptoms aren't present then it sound like your son is doing well but I would advise that you, or rather your son get a complete copy of his history and medical records. I can't stress the importance of this! I'm not a doctor but I've worked in the medical profession for several years and one of the most important things you can do is have a complete understanding of all of your health conditions as well as the records. Never assume that one doctor is communicating with another!

My post is just our personal experience, not meant to be any kind of medical advice.

Good luck to you and your son!

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Hi, Thanks for your post. My son had a complete obstruction but the doctor didn't clue in to my son's condition until complications occurred after they placed the tube in. They thought his bowel was ruptured when his stomach blew up as hard as a rock and he went in for emergency surgery and this is when they found the SMA syndrome blockage.

Like I said in my previous posts, when my son turned 21 and he decided he needed a better understanding of his health history and when he went to the doctor the doctor told him the blockage occurred because of T3's he was taking for the stomach pain he was experiencing and then he photocopied the surgical report sent by the surgeon. My son didn't even realized the error his gp made until when he returned home and I read the report which stated very clearly he had a complete blockage due to SMA, T3's were not even mentioned in the report. The other mystery is the surgeon never addressed if his bowel had been rupture, I guess we would have to go after the surgical record of the operation. We don't understand why we were not given a more complete account of things in the hospital.

The doctors never suspected EDS at this time and my son had a difficult recovery which the doctor's and nurses felt I was encouraging because I refuse to let him cry in pain when his pain meds were due. Had we known about EDS things would have probably been treated differently. They claim he should not have had the amount of post op pain that he had and when I questioned why he was not on antibiotics the surgeon became angry that I would second guess his treatment. My son's recovery was so difficult because he had a pretty bad infection inside in his abdomen, the wound that took over 12 weeks to clear up with us going to the hospital as an out patient for 10 days. If had he been put on antibiotics when my instincts told me he needed to be on them he would not have had such a long difficult recovery.

I didn't know that SMA could reoccur after surgery, it's been difficult trying to find information which is in layman terms, maybe this is why I haven't heard of it.

My son's weight didn't stabilize until after he was completely healed and once he did he put the weight back on quickly. The worse part of all of this is my son doesn't have very pleasant memories of this time and it cost him an extra year to graduate because he went undiagnosed for so long and the doctors refused to take his symptoms seriously and only decided to take action after he lost over 40lbs in a 6 month period. Believe me when I say we went looking for answers a lot and it wasn't until he lost the weight that the doctors decided to be proactive and put the feeding tube in. If he hadn't had the complications from the inserting the feeding tube I am not sure how long we would have waited for a proper diagnosis.

I now understand you really are the only one who can advocate for your health, the doctors are so overwhelmed that you become just another body in their office for that short 10 minute appointment. If you are a parent who advocates for your child and stands up or questions the treatment your child receives you become an overprotective parent who they try to dismiss. In our case the doctors we saw even managed to convinced my ex that I was being overprotective by listening to my child. I am glad I was because who knows how long he would have suffered had I not been. I agree with you about keeping copies of the medical history I am amazed how often reporting mistakes are made by the doctor misquoting the medical history or forgetting to write down suggestions they have made to you and then these mistakes are compounded as it is passed along to other professionals.

I am sure your daughter will have an easier time with her surgery but make sure the doctors understand she will have more post op pain because of her EDS than an average patient who has the same surgery and this will make her recovery more difficult and a little longer. Good luck to you both and please let your daughter know she isn't alone, there is at least one other person who has gone through the exact same challenge. You lucky because you are one up on me, you understand what is happening, we bounced around in the dark and didn't get closure until 5 years later. It has helped my son a lot to know he is not alone, especially being a young person with health problems when very few young people have the same challenges and therefore they don't relate to their experiences.

This forum has been a god send and I thank everyone who takes the time out of their very busy lives even when things seem overwhelming to post knowledge, questions and experiences.

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Thank you so much for your reply! I know what you mean about the frustration with doctors and treatments. For years I was told my child's pain was psychosomatic, I was even accused of Munchausen by proxy! After seeing a geneticist for something unrelated we BOTH were diagnosed with EDS. While it was a frustrating process to find a diagnosis, once we did all of the pieces fell together.

I'm STUNNED they let your son lose so much weight first! My daughter started with bronchitis, then was so tired she slept 18 hours a day, lost 3 pounds, and when the vomiting started again we went to the ER because it was the first time she said her right side hurt. I thought it was her appendix. This spanned a period of three weeks. The exam was negative for her appendix but in the process she vomited green on the doctor. If not for the bilious vomit we would have been sent home. When he saw the green she was sent for a CT and we were stunned to see how large her stomach was and nothing was passing the duodenum. That's when she was admitted and we finally got the answer as to the episodes she had been having since birth.

I can't thank you enough for posting your story! Is it okay if I tell our new team about your son's case? Just that while not published, there is another patient with both syndromes? Your son's doctor didn't research or publish the case and I do not want them to erroneously think my daughter is the only case.

While I'm not thrilled with my local healthcare I'm thankful that the people at Columbia are really working hard to get together a top notch team of specialists for us. I've learned to not only research the diagnoses but the physicians as well. Knowing she has EDS has changed everything. We know what is happening and why and what to do to preserve future health as well.

We're lucky to that the high school she just started is really going out of their way to work with us! After the surgery she'll be homebound for at least a couple of months and the school will send her work and a tutor to the house a couple of times a week. This will decrease her risk of infection and also from getting sick from othere students.

I too am stunned to to see how different records can be from reality. I always have a written list of dates of diagnoses and symptoms and everything and give it to doctors to help keep facts straight. Only sometimes has it worked.

I'm feeling really positive about our new docs as they call me regularly to work on scheduling so that when we go for the consult we meet everyone who will be involved on the same day and they are clearing significant schedule time for us. We're lucky to that the PCP I found a year ago is generous with his time as well and we see him usually for an hour every visit!

I realize how lucky we are and that our tenacity has paid off.

SMA is so rare, and until I found you, unheard of in a patient with EDS. I will have a much better idea of long term post op prognosis after our consult and will let you know what they say. Two of the team member are surgeons- one a vascualr surgeon and the other a pediatric general surgeon with a specialty in thoracic and abdominal surgery. Both have done reseach and published papers regarding problems with the SM artery. They will be publishing our case as well. My hope is that by bringing light to the possiblity that SMAS might be correlated with EDS. One never likes to suffer or see their child suffer but if there is a chance we can help other people I am more than willing to do so!

I'm so glad I found this forum! :)

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Thanks for posting this story.

This brings to mind my niece's situation: she was close to dying from pulmonary embolisms a few years ago due to a congenital condition called May-Thurner syndrome. It interesting that this is also a 'structural' defect that involves vascular issues in a non-vascular EDSIII family. See http://en.m.wikipedia.org/wiki/May-Thurner_syndrome

It seems also to be something that would not be suspected in a younger person ... until a pulmonary embolism is caught with no indication of coagulation issues. Even operations wouldn't 'catch' it because cheat xrays aren't done pre-op until an older age ... which is at the end of the age ranges in which this occurs. Age is what probably also delays dx in SMA Understandably, tests can't be done w/o reason (unless one is wealthy enough to afford concierge medical attention... available at all major heathcare facilities that want to identify major donors and justify allocating times in support services calendars..... Ugh, I'm going to stop before I go ballistic on that one).

Anyway, Thot I would make a possible connection here.

As for the MD: it irritates no end with how quickly non-proven reasons are quickly depended on... Dare I call it mere, biased opinion-- when someone is in pain. Sometimes you gotta to okay hard ball with them. Be brave and tell them that you wil run a test and ignore your son's pain as long as 1) they supply the science or study that says this is appropriate action to take and 2) that the doctor contracts, in writing, that your son's pain is psychological and that all pain meds should be withdrawn.

They quickly understand they would no leg to stand on in a lawsuit. After that, you can suggest a trial period and a second opinion. Learn who they suggest (feel safest with for that) and RUN in the opposite direction for a competing, healthcare provider who wouldn't mind one upping the doc for better care.

Your son was traumatized by the pain and loss of such an event.... and you by being an observer. He's got to count his blessings and understand that the MDs did not let him die. While they were helping him, they may have been negligent to his pain needs or need for prompt, complete care. HE MUST UNDERSTAND THAT THIS DOESN'T NECESSARILY MEAN THIS IS GOING TO HAPPEN AGAIN. This is the problem when one has chronic health issues. A general fear of 'what next' sets in. Thus, any lack of attention to the painful aspects of an event seem possible in the future. But the fear is not realistic or well founded. Courage becomes I increasingly more difficult to muster. Health and strength are an cornerstone of confidence and chronic health issues makes for a poor quality cement to repair it with.

My suggestion: help yourself and your son make this a chapter... not the whole book. He needs to understand thd details and delineate how lax treatment lead to ill feelings and how the actual problem was ameliorated. Then, move on. Gather all records for health history sake and put them on a USB (with passworded files) or possibly on a phone or cloud if you have that available to you.

If you get to that point, you can put it in context and move on.... hopefully. It would be good to free up his thoughts to the great possibilities ahead him even if it may mean a few bumps in the road. He would have better shock absorbers if he k ew hd could get by this one.

I hope this is helpful.

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Hi there...

Unchatfrais please don't hesitate to inform your doctor about my son's connection to EDS and SMA. I am glad they are writing your daughter's case up. There just isn't enough information out there so any information we can get circulated the better off future patients will be. If I can be of any further assistance please let me know.

Ehdosomething thanks for the suggestions we are working on doing just what you suggested. To be honest I have never consider shopping for a doctor, the medical system in Canada is very different and I live in Northern British Columbia which means you take what you can get. The good thing is I am moving to a larger community and need to find a doctor so I am going to give this shopping around idea a try. I am hoping with a fresh doctor it will mean a fresh start.

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I'm putting this here so it hopefully won't draw too much attention yet. One of my student's parents is an experienced nurse going back for her PhD in nursing. I had been doing too much, had missed a massage appointment, and had to sleep for 16 hours straight to get back on track, messing up one of my sessions with her daughter. I explained to her the whole collagen, pain, blood pressure, cortisol, adrenaline thing. She got it immediately even though she hadn't ever heard of EDS. She's now thinking of doing her dissertation on EDS. I suddenly realized that there is a huge crop of people who are out there just begging to do research on EDS--to answer our questions, including this one on SMA recurrence. MD/PhD, PhD, MPH, MSW and all sorts of other students are looking for areas that are understudied. The death knell to a dissertation or master's thesis is picking a topic that has been overstudied already.

By the way, this same nursing student (a mature woman of 50 and experienced oncology nurse) suggested I got before congress to demand all sorts of things. She's a mover and shaker. I sort of what to create a list of topics for PhD and masters candidates to pick from. This SMA business would be on there.

Thoughts?

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Sounds good to me. Getting information out to the medical field is the only way to help all future and past patients. If it makes a good thesis topic than I would add it.

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OldTexan,

Don't you think our "frequent flyer" status contributes, albeit anonymously, to the health care economy??? LOL

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