Aidan:
Hello and welcome to the Ideal Nutrition Podcast. My name is Aidan Muir. I’m here with my co -host Leah Heigl and this is episode 131 where we are going to be talking about CSID, which is congenital sucrose isomaltase deficiency and we’re going to go over a little bit of a different format with this episode where I will be interviewing Leah, partly because this is a relatively rare condition. And while I have worked with clients in this position with CSID, Leah wrote a blog post, how long ago was it? Two years ago.
Over two years ago now. over two years ago that has generated a lot of clients. And she’s basically become the expert in this space because we just send every single one to her. And we because that generated so much interest because it’s a bit of a topic that not many people are speaking about. We want to do a podcast episode on it. But because Leigh is the expert on that, I wanted to interview her for this episode. So starting off, what is CSID?
Leah:
Yeah, so CSID is a rare genetic disorder that affects the digestive system, particularly the ability to break down certain kinds of sugars, specifically disaccharides. So disaccharides are basically two sugar molecules that are linked together and you need a specific enzyme to break those sugar molecules apart. The issue with CSID is there is this enzymatic insufficiency or deficiency where you actually don’t have the enzymes or you don’t have as much of those enzymes as other people to break down disaccharides.
So you get this overall like malabsorption of particular kinds of carbohydrates and sugars that can lead to symptoms like vomiting, diarrhea, bloating, gas distension, all those kinds of like gastrointestinal issues. Symptoms can very much differ person to person, but it does come from this malabsorption of these disaccharides, particularly your sucrose, maltose and isomaltose and then sometimes also lactose as well.
Aidan:
What causes it?
Leah:
So there are a couple of things that can cause it. It depends on if you have the primary CSID or secondary insufficiency. So your primary sucrase isomaltase deficiency, or I just call it SID or C -SID. So if you have this enzymatic insufficiency and you were born with it, it’s something where, unfortunately, you are just born with a lower ability to produce these enzymes, and that’s just something that you manage long -term. But there are cases where it’s not congenital, it’s not genetic, so you’re not born with it, but it does come from damage that happens to the small intestine.
Because that’s where these enzymes are produced so when there is any damage to the mucosal lining mucosal wall or the villi in the small intestine there is this potential that the enzymatic production is reduced from that and So many reasons why that can happen. They think that it definitely is linked to cases of Gastro, so after having like a bad case of gastro, sometimes people develop this condition that can alleviate over time as that’s repaired though.
Other reasons are SIBO. So small intestinal bacterial overgrowth can actually damage these cells as well on the mucosal lining of the small intestine. That’s a little bit of a harder one to tackle, a whole rabbit hole of its own. Maybe we do a podcast on that eventually. But also like unmanaged celiac disease. So you’re eating gluten when you have celiac disease, it can cause this issue.
Inflammatory bowel disease where you have a lot of inflammation in the small intestine specifically and like certain kinds of medications and stuff. So there’s so many things that can cause this insufficiency if you’re not born with it and it’s like you have to find that root cause to fix it.
Aidan:
Yeah. And since we wouldn’t really have stats on prevalence or anything like that, just in terms of your clients, what percentage would you estimate have had it from birth versus have secondary?
Leah:
That’s a really good question because sometimes it’s really hard for them to pinpoint when it started. So some people are like, yeah, I’ve dealt with this like since I was a kid. And it’s just something that was just not diagnosed and clearly was there in infancy. clearly did have somewhat of a failure to thrive as an infant. It just wasn’t like tested for.
But I feel like a lot of my clients are like, yeah, this just started happening one day. And they have no idea why, but it was like maybe a decade ago, so they can’t really pinpoint it.
Aidan:
Yeah. And what is the diagnostic process?
Leah:
So the diagnostic process is an endoscopy where they take a small bowel biopsy. So you need to take the biopsy to test for that enzyme production.
Aidan:
Yeah, and do most of the clients who come see you, have they gotten that diagnosis or is it something? Because there’s some other ways we could identify it, I’m assuming as well.
Leah:
Yeah, so we could use like an elimination style diet. And if you go on a low sucrose, low starch diet and you have like significant reduction symptoms, you can somewhat assume that it’s related to this. But I would say all of my clients who are seeing me for this condition, who have like sort me out for it, have had the biopsy and have been diagnosed, but there have been a couple of clients where like, let’s test for this. Cause it doesn’t seem to be like they come in with IBS and it’s, FODMAPS doesn’t work. They’ll say like food chemicals doesn’t work. Like we’re like, all right, what else could it be? And then maybe we, turns out to be this particular condition.
Aidan:
And like this is another question just in terms of prevalence and I assume you wouldn’t have stats on hand, but like one of the questions I’ve always wondered in relation to this is like, how common is it? Like if we say IBS affects like say 13% of the population, like how often is it going to be somebody in that say 13% or whatever, maybe a little bit higher. How prevalent would IBS be? IBS would be bit higher than that as well actually.
Leah:
Surely IBS would be a little bit higher. even when it comes to numbers, I almost disregard the prevalence numbers because I think it’s so under diagnosed. it’s like I’ve had so many clients that are like, I’ve dealt with this for decades and only just getting diagnosed. I can’t imagine how many people are out there with not being diagnosed and misdiagnosed as IBS and haven’t found any relief yet.
Aidan:
Yeah. So getting into the, I suppose the important stuff how to manage that, how to manage it. Take this however you will, like are there foods that need to be avoided or limited? Go general and then we’ll talk through your specific process, how to manage this?
Leah:
Yeah, go in general. think I’ll start with, I guess, the things that we want to in general terms. we’re specifically focused on limiting sucrose and maltose, or I use maltose and starch kind of interchangeably. Particularly limiting anything with added sugars. So you’re kind of general table sugar so things like soft drink confectionary all of that stuff, but also sucrose that is naturally occurring in fruits and vegetables and grains, etc. Then we also want to limit or avoid starch-based foods So again your grains your starchy veg things like rice oats pasta bread.
Over time, especially if you’re quite sensitive, this can become like a low carb diet. It’s not that you need to limit all carbohydrates, but there are so many carbohydrates that are disaccharides that you do want to limit to avoid symptoms.
Aidan:
Yeah. And we’ll talk through that as well. Talk us through your process. Like where would you start? How would you go? Like if you were working with somebody for like 12 weeks on this or whatever, like what would be the process?
Leah:
Yeah. So I guess at this point I have been working the space quite some time now and I’ve got this specific process that I go through. So typically we put someone on a relatively generally low carb diet. What I like to do is, especially if someone doesn’t know much about the condition, which a lot of the time they’ve been diagnosed for years and know very little about the condition or are still quite confused by it, we go, right, let’s go pretty much low carb. So we’re gonna go very, very little amounts of your starchy veg, really small amounts fruit, basically no grains. We may even, if they’re quite sensitive, we may even have to limit things like nuts and seeds to a certain extent.
So it becomes a very basic, like low starch, low sucrose, veg and meat, eggs, and maybe some low lactose dairy kind of diet. So we just reduce the disaccharides completely. And that usually is just generally a low-carb diet. Then step two is more of like, given that that had reduced all of their symptoms and most of 9 times out of 10, that’s all their symptoms are basically gone within a week and they’re feeling excellent because they don’t have this malabsorption happening. Then we go into more of a personalisation phase where we bring back some of this sucrose, some of this starch and we figure out, okay, what’s their tolerance? Because everyone is very different with the amount of sucrose and starch that they can tolerate.
A lot of the time your added sugars are always going to be pretty poorly tolerated, but things like your starchy veg that is also high in fibre, your whole grains like low GI whole grains that are also high in fibre can be somewhat tolerated. So we bring those back in small amounts basically until we get to a point that we’re like, no, we’re starting to get some like gas distension, some discomfort. Then we know that like that’s that person’s limit.
So my goal is always to not have someone on this unnecessarily restrictive diet where they’re avoiding these foods forever, but essentially finding that personal tolerance. Then as part of that, we’ll also bring back your other carbohydrates that are non -sucrose, non -starch related. So we could look at different kinds of sweeteners. So using things like glucose syrup, dextrose powder, fructose, because those are all monosaccharides, so they’re just like a single sugar molecule instead of two combined together. We can use those freely without malabsorption. So again, it’s not a low carb diet, it’s a low disaccharide diet, but obviously that’s quite complex.
Aidan:
Can you use those sweeteners off the bat, like in the elimination phase?
Leah:
I often do, the person is at a point where they can tell the difference between different carbohydrates. Sometimes that education process takes a really long time. So we go through things like label reading and figuring out what sweeteners they can have and what ones cause symptoms. When someone comes to me and they’re like constant diarrhoea, unintentional weight loss, vomiting every time they eat, they just want symptom relief. So I usually don’t bring in initially unless that person has done heaps of research and has some of an understanding of the difference.
Aidan:
I feel the same when I’m working with somebody on a low FODMAP diet when like, so I talk about lactose, I will just treat it, I won’t go through the nuances of being like, hard cheeses have almost no lactose or whatever because I’m like, that’s so much to chuck in while also introducing FODMAPs as a concept off the bat as well.
Leah:
100 % at this point most of the time people because as if you Google CSID like you will get some stuff that comes up but the internet is actually not particularly helpful and health professionals at least in Australia are pretty unaware of CSID so I find there’s a lot of people that just have very like limited knowledge about this condition so try to start simple and build and like usually I’m working with people for a few months so like we had time.
Aidan:
Yeah, digestive enzymes do you use them?
Leah:
Yeah, digestive enzymes can be really helpful. So there’s two main brands that I know of that you can use for the CSID. The first one isn’t available in Australia, which sucks. And that’s Sucrade specifically for the digestion of sucrose, but it is available in the U .S. Apparently really effective.
Aidan:
Yes, it’s not in Australia. You’re right.
Leah:
Yeah, no, it’s not available in Australia. And I’ve like tried to find ways to get it in Australia. But like, was like, we need to like import this. that would be really handy. Because yeah, it’s like meant to be a really, really good option. And I was talking once to someone that did live over in the US that had this condition and they were like, yeah, sucrate is like, awesome, really, really effective. But yeah, can’t get it here. But something that we can get is a product called starch away by intolerant. So that does act on both your starches and your sucrose.
In my experience, starch away is really good, but it is less effective for sucrose and it is for starch. So like whilst you might get away with like, let’s say having like something with like rice in it and having the starch away, works quite well. If you’re going to have sucrose, you still kind of need to limit that to a certain extent, particularly sucrose as like just an added sugar. So it does seem to be more effective on those like more starchy foods.
Aidan:
Yeah. With other sweeteners, is there anything else to watch out for like say sugar, alcohols or anything like that?
Leah:
Yeah, I think like when you go onto a CSID friendly diet and you are trying to avoid added sugar, you go for a lot of the keto low carb products that are artificially sweetened. And if this is like monk fruit or stevia or something like that, it doesn’t seem to be a big issue. But if it’s something like your sugar alcohols like sorbitol or maltitol, that can cause diarrhea.
Regardless or like just stomach upset regardless of if you have CSID or not So sometimes people like I ate like all these protein bars and then I got diarrhea and we look at the ingredients and we’re like well, it’s sweetened with like maltitol and it has that like if consumed in excess will cause a laxative effect They’re like well, yeah, would be why.
Aidan:
Other stuff anything gut microbiome related like you kind of touched on that a little bit early but like anything we need to worry about particularly like another variable like on the low carb diet like that could play a role as well.
Leah:
Yeah, typically like the last once we when I work through my process, we’re like, okay, symptom management is like the first thing we try to do. Then we be we would find your individual tolerance and, you know, figure out what a more open diet looks like for and then the third part of my process is always trying to nourish your gut health and generally find a way to make sure we’re taking care of our gut microbiome whilst we’re limiting these like higher fibre foods. And because that can become an issue over time, especially if someone is literally just doing small amounts of veg and meat. Like we’re thinking like there’s not a lot of plant-based food variety there. So then how do we build this in over time?
Because what I found is people who live with this condition they get such a restricted diet over time, so they’re not really sure how to manage it. And then that in itself creates issues with FODMAPs because they’ve cut out all these foods or they’ve gone through FODMAP elimination diets thinking they had IBS. So not only are they dealing with the CSID, but they’re also dealing with co -occurring intolerances that could be reduced by focusing on the gut microbiome.
So at that point, it gets quite complex. But I’ve worked with people that were like so restricted, not only from CSID, but other intolerance perspective and this third aspect of focusing on gut health made such a huge difference to being able to open up their diet even further. And then also with that secondary like CSID, if we are nourishing that gut health, then potentially that could help repair some of that small intestine damage and reduce symptoms from condition in like a roundabout way. So yeah, I think it’s definitely a focus that needs to be had.
Aidan:
Yeah, with other conditions, say lactose intolerance, like sometimes if we have other foods alongside the lactose or even examples like higher fat foods alongside the lactose, because it slows down the process of lactose going through the body and then getting digested in the small intestine and also potentially large intestine.
There’s a few little things like that we can do to improve the absorption and digestion of lactose. Does that also apply with CSID as well?
Leah:
100%. I absolutely like two of the biggest recommendations that I give in that first consult is A), to chew your food really well because digestion starts in the mouth and then two, pairing any starches or sucrose-heavy food with fats and fibre to slow digestion.
So even like those doing those two things can help you tolerate a higher amount of starches and sucrose in your daily diet and meaning you don’t have to restrict quite as much. So it can be a game changer.
Aidan:
For sure. So in prepping for this, is there anything else that came up that I haven’t asked about or anything like that that you think would be useful to cover?
Leah:
I actually think you’ve done a really good job. I thought you were going to miss that last one on the chewing well and like slowing down digestion stuff. To be honest, I think the only thing I would add to this is that dealing with sucrose isomaltose deficiency. I really think you should work with somebody. think it’s, you’re to get so much more benefit in working for with like a dietitian that is informed in this space. Because even when I first started practicing in this it was really, it was really hard to navigate. Also all those nuances around nourishing your gut health, maybe reversing the condition if possible, looking at different tolerance, like opening up your diet to other sweeteners and things like rather than just following this like low carb diet. I just think it’s so worth working with someone.
Aidan:
Yeah, I think so too. And that’s also a message I’m pretty consistent on. Like I never want this to be an ad or anything like I’m really big on putting out good content and helping where possible. And that’s what I hope this podcast is, this episode is like, if you had CSID and you were listening to this, I hope you got some value out of it. But if you hear me talk about other situations such as the low FODMAP diet, I try so hard to put out so much information for free that it’s possible to manage it yourself. That is my goal. If you Google the low FODMAP diet, Ideal nutrition, you’ll see a very comprehensive blog post that has pretty much everything that I think you need to know to get started and everything like that. But even in that case, I’m like, I’ve never seen anyone do the low FODMAP diet well without working with a dietitian. I always chuck that out there, as an ad to just be like, that’s me honestly speaking the truth. Like I’ve just never seen that. And I’ve seen heaps of people have really good success while working with a dietitian. I would wager the same applies in this space as well.
Leah:
A hundred percent. Even when it comes to the complexity of like label reading and knowing what some products actually are, like that can be really confusing without a lot of nutrition knowledge and backgrounds. So I think it can be very helpful.
Aidan:
Perfect, well this has been episode 131 of the Ideal Nutrition Podcast. As always, if you could please leave a rating and review, that would be greatly appreciated.