Refractory Sprue

[GlutenWrangler]

As much as I wish it were not the case, a couple of my doctors think that I have it. Has anyone else here been diagnosed with refractory sprue? If so, have you received any treatment for it? Thanks,

-Brian

[Fiddle-Faddle]

You might want to PM Canadian Karen--I believe she was diagnosed with refractory sprue.

I'm sorry you're going through such tough times--I hope things improve!

[pugluver31902]

Funny, I was just going to post the same question. My doc said he wants to repeat the biopsy in a few months before he gives me the final diagnosis. So... if someone could shed some light on this, I would also be most interested!

[GlutenWrangler]

I'm going to re-post this thread in the "coping with" section.

[ravenwoodglass]

I googled refractory sprue treatments and came up with quite a bit. I also know someone who was diagnosed with this a couple of years ago. She is doing much better and had mentioned being on a specific drug. I will see if I can find out what helped her as soon as I see her.

Brian one thing I did learn by googling is that your doctor seems to have been doing the right thing. In severe cases it usually does require hospitalization and bypassing the intestines for nourishment for a while. The standard treatment used to be steroids, I don't know if your doctor has tried those yet or if they would be suitable. Many are able to achieve healing and there are some newer drugs and research available. You may want to consider printing some of what you find from reputable web sites and taking it to your doctor.

I hope you feel better soon and as soon as I get the chance I will see if I can do some research that is linkable.

[GlutenWrangler]

I guess the moderators weren't having it. But anyway, yes, one of my doctors prescribed prednisone, but I couldn't handle the side effects. Although it did give me more of an appetite. I really don't know how I will ever be able to eat at this point. Hopefully my doctors can figure out something.

-Brian

[ravenwoodglass]

I guess the moderators weren't having it. But anyway, yes, one of my doctors prescribed prednisone, but I couldn't handle the side effects. Although it did give me more of an appetite. I really don't know how I will ever be able to eat at this point. Hopefully my doctors can figure out something.

-Brian

Brian have they tried budesonide? It is a steroid type of med but is metabolised differently. Another treatment is cyclosporine but that is a immunosuppressant and your doctors may not want to use it. I wish I could find more. I hope they can figure out something to help you soon.

[ryebaby0]

My son was dx celiac and did not respond completely to a gluten-free diet, ended up in the hospital, was then also dx with something called Autoimmune Enteropathy. (If you google it, it will only scare you!) which is very rare in adolescents but less so (but still rare) in adults. He is treated with an immunosuppressant called ProGraf, under the strict supervision of Children's hospital at Pgh, and has been healthy for 4 years now. All immune-suppressing meds have serious side effects, and should only be used by physicians really familiar with them, but you might want to find yourself a GI practice that has heard of AE. There are pathological symptoms found on endoscopy, but it is mainly a "rule everything else out" diagnosis.

A doctor at Johns Hopkins told us that she personally did not believe there was "refractory sprue" , and these cases were misdiagnoses of other conditions/problems/gluten-free compliance.

Joanna

[GlutenWrangler]

The funny thing is that after I told the doctor that I couldn't take prednisone because of the side effects, he gave up on steroids and immunosuppressants. I figured he would try another drug with less side effects. So unfortunately I've never tried budesonide, or anything else for that matter.

Ryebaby, I've never heard of Autoimmune Enteropathy, so I'm glad that you told me about it. It is definitely something I can talk to my doctor about when I see her on the 27th. I know it's probably going to freak me out, but I'm going to google it to see how well it fits my symptoms. Thanks,

-Brian

[ryebaby0]

Boston Children's (I just read your signature and info today) has done a lot of pioneering work with immunesuppresion and GI issues, so you are in a good place to find people who are current. (GI docs, I swear, all eat lunch together whether they are Peds or Adult) You really don't want to take prednisone except in dire cases; it's soooo hard on you in so many ways, and many other drugs do a much better job anyway. Basically the theory on AE is that your body is attacking your GI system as it would a transplant. Children with AE are usually dx as babies through their inability to eat/digest/thrive and typically stay on TPN (iv nutrition) for what will be a very short life. Adults have a MUCH better prognosis so stay optomistic! My son had a long recovery but is a very healthy child 4 years later

[GlutenWrangler]

I was hoping that my doctor would try another drug in prednisone's place, but he just gave up on that kind of treatment for some reason. As much as I hope that I don't have Autoimmune Enteropathy, it would be nice to have a more definite treatment plan. Right now I'm not getting any useful treatment. My doctors say they want me off of TPN asap, but nobody seems to know how to get me to that point. Boston is definitely one of the best places to go for health problems. Thanks for the information about Boston Children's. I was thinking about going to Massachusetts General Hospital for this, considering it is the 4th best hospital in the country. I was happy that they put me on TPN at Beth Israel Medical Center, but they didn't offer any treatment to improve my condition. But going back to Boston is definitely something to think about. I just don't want to get my hopes up for nothing again. But thanks for all the info! I've got a lot more to think about now

-Brian

[Fiddle-Faddle]

Did they try tacrolimus? Open Original Shared Link

Adult Autoimmune Enteropathy Treated Successfully with Tacrolimus

Abstract

Background: Autoimmune enteropathy is a life-threatening, chronic disease of the small bowel mucosa, which generally responds well to steroids. Treatment requires long-term immunosuppression, and steroid-sparing treatment strategies are desirable. Azathioprine and cyclosporine A have limitations, however alternatives have not been described in adults. Case Report: We present the case of a 54-year-old male patient with autoimmune enteropathy who responded initially to a standard treatment with steroids, but was dependent on 30 mg prednisolone. Medical treatment was changed to tacrolimus after renal function deteriorated under treatment with cyclosporine A. Under this regimen, small bowel histology normalized and the clinical condition is stable after 2 years of introduction of tacrolimus. Conclusion: This constitutes the first report of effective treatment of adult autoimmune enteropathy with tacrolimus, a substance with a similar mode of action to cyclosporine, but with fewer side effects and improved bioavailability.

Copyright

[GlutenWrangler]

Scary stuff. I should mention this to my doctor. Maybe it's something she will try.

[Fiddle-Faddle]

It seems to me that you should seek out doctors who have SUCCESSFULLY managed patients with refractory sprue, no matter how far away. If they are experienced with RS, they won't hang up on you if you call them from wherever you are, they'll know that you're not faking or crazy. They might be willing to speak with your current doctor, or they might want you to come to them. Either way, see if you can get a hold of a copy of your medical records so you can fax it to the RS doctor.

The docs listed in this study are in Germany, but maybe they can clue you in to other RS ocs here in the US.

[ryebaby0]

Tacrolimus, or FK506 (its trial code) are both names for the drug that is branded as ProGraf, i.e. what my son has been taking. Most AE patients, and many transplant patients, are on ProGraf. It is a med discovered/produced in Japan and is VERY expensive (but in insurance formularies) . My son was (briefly--about a week) on TPN until they put him on ProGraf, at which point his symptoms improved steadily. He was weaned from prednisone entirely.

Cyclosporine is not as popular as an immune-suppressant, because it has some troubling side effects (not that Prograf doesn't, mind you, but TPN is no picnic either). Prednisone is not practical for long-term use, and I can't think of the IBS steroid but it takes time to build up in your system and we didn't have time (although they started him on it, until a doctor who had worked at Boston Children's suggested FK) . My son was 9 when diagnosed; he's 14 and has regular blood tests to check his liver, kidney function; we are cautious about germs and germy people but otherwise he leads a normal life.

Anyway. Boston hospitals pioneered the use of ProGraf in the 80's, and I doubt that any reputable GI associated with the larger beantown hospitals is unfamilar with it's applications. As I said, endoscopy reveals AE pathology fairly conclusively. We drive a 6 hr. round trip to my son's doctors, and it has been more than worth it.

[num1habsfan]

They thought for awhile there I may have. One of the specialists I'm waiting to go back to see again knows it's not Celiac causing my constant severe pains in the lower right side. Anything I eat, regardless of gluten-free, leaves me in pain (most of you know this whole case already, just reminding those who dont know ). It could also be Crohn's or Colitis (and I am currently on a Crohn's diet besides gluten-free).

What's the best way to test for this, though?? Is colonoscopy the only solution??

~ Lisa ~

[GlutenWrangler]

I'd like to know how they would differentiate between Refractory Sprue and Autoimmune Enteropathy. I have had multiple endoscopies, and AE has never been mentioned. So I guess that would mean that I don't have it. Especially since my last endoscopy was done in Boston at Beth Israel.

I should try to find a specialist in Refractory Sprue. It's just tough to be taken seriously by celiac specialists because I don't have DQ2 or DQ8. I've read about some places who won't even accept someone as a patient unless they have one or both of those genes, which is ridiculous. But it's definitely worth a shot.

-Brian

[Fiddle-Faddle]

Is it possible that they are one and the same? Like IBS and celiac, or (sometimes, maybe always) fibromyalgia and celiac, etc.?

[GlutenWrangler]

Well the histology of both shows villous atrophy, and both do not respond to dietary changes, so it seems possible that they are one in the same. But at the same time, you have to develop celiac disease first, in order to have refractory sprue. And you have to eat gluten to develop celiac disease. So if someone who is predisposed to refractory sprue never eats gluten, they will never develop celiac disease, and thus, never develop refractory srpue. While someone who is predisposed to autoimmune enteropathy will develop it no matter what they eat. I could be wrong, but I think it makes sense.

-Brian

[Fiddle-Faddle]

I understand what you mean, but since very few people in Europe and the Americas have NEVER eaten gluten, how can they rule gluten out as THE cause of autoimmune enteropathy? And even in Asia, where the gluten load in food is relatively tiny compared with that in the West, there is still gluten-containing soy sauce.

[GlutenWrangler]

It's true that just about everyone alive has consumed gluten in some form. But it is possible that Refractory Sprue is gluetn-dependent, and Autoimmune Enteropathy is not. It's just tough to make that determination.

-Brian

[ravenwoodglass]

Well the histology of both shows villous atrophy, and both do not respond to dietary changes, so it seems possible that they are one in the same. But at the same time, you have to develop celiac disease first, in order to have refractory sprue. And you have to eat gluten to develop celiac disease. So if someone who is predisposed to refractory sprue never eats gluten, they will never develop celiac disease, and thus, never develop refractory srpue. While someone who is predisposed to autoimmune enteropathy will develop it no matter what they eat. I could be wrong, but I think it makes sense.

-Brian

That does make sense. Here's something else to throw into the mix. Since in the US many doctors are of the opinion that without the 2 celiac genes that have been 'discovered' you can not be celiac how many of the AE folks might really be celiac? I was pronounce to have many serious autoimmune disease that I was told I would have to just learn to live with. It was celiac period that was causing the problems with my entire body. Would AE have been the next misdiagnosis for me if it wasn't for that very elderly, very savvy, non-pharmacopic allergist?

[GlutenWrangler]

That makes a lot of sense. Doctors are notoriously bad at diagnosing celiac disease, never mind refractory sprue. So I'm sure there have been a lot of cases where people have been diagnosed with AE when they actually have RS, and vice versa. I think it all depends on what the doctor is looking for, and how they interpret a patient's medical history. Either way, I'm sure both diagnoses are never the first thing on a doctor's mind.

-Brian

[Guest celiac mum]

Just seen your message about refactory sprue i hope everything is going ok . I have two children aged 5 and 6 with it . We are all finding it hard, although i don't really understand it all

[GlutenWrangler]

I'm so sorry to hear about your children. Refractory Sprue can be a devastating illness. So your children didn't improve at all on a gluten-free diet? I improved a little bit, but then it just went back downhill again. I'm just so surprised that your children's doctor has settled on a Refractory Sprue diagnosis while they are still so young.

I'm not doing that well right now. I just found out that my kidney function has dropped to 35%, and it's possible that it is due to the Refractory Sprue. How are your children doing? I wish them the best!

-Brian