What is Congenital Sucrase-Isomaltase Deficiency (CSID)?

by Valerie Polley | Jan 15, 2019 | 10 comments

I attended a three day GI conference at the University of Michigan in September. It was an amazing conference packed full of presentations from some of the best GI doctors and dietitians. One presentation was on a topic I had never heard of, so I was intrigued and ready to learn more about it.

​Congenital Sucrase-Isomaltase Deficiency (CSID) is a rare disorder that causes individuals the inability to digest certain sugars due to an absence or low levels of two digestive enzymes, sucrase and isomaltase. Both of these enzymes are involved in the digestion of sugar and starch. Table sugar is broken down into glucose and fructose with the help of the sucrase enzyme. Isomaltase is one of the several enzymes that help digest starches.

Both sucrose (found in fruits, and known as table sugar) and maltose (sugar found in grains) are disaccharides, which means they are made of two simple sugars. During digestion, intestinal enzymes break these down into simple sugars called monosaccharides. Sucrose=glucose + fructose and maltose=glucose + glucose. An individual with CSID has difficulty breaking down the disaccharides into monosaccharides that the body uses for fuel. These disaccharides are too large to be absorbed in the intestines by the microvilli (known as the brush border).

Starch can be a simple or complex carbohydrate. Simple carbohydrates have one or two sugar molecules, and complex carbohydrates have three or more sugars linked together.

Multiple enzymes are needed to properly digest starch. Digestion begins in the mouth with an enzyme in the saliva called amylase. Digestion continues in the stomach by further breaking down the food. In the small intestine, starch is processed by an enzyme called pancreatic amylase and converted into maltose and sucrose. The brush border contains numerous enzymes (sucrase, lactase, maltase) to continue the breakdown of digested food into smaller particles that can be better absorbed. The isomaltase enzyme breaks the bonds linking sugars into glucose, which can't be broken down by amylase or maltase. This is one of the final steps in the digestion of starch to glucose.

To sum it up, those with CSID lack the sucrase and isomaltase enzymes to properly break down the food to a simpler form, so the body can absorb it.

Without the proper enzymes, digestion can't take place correctly and individuals can have symptoms, such as:
-Chronic Diarrhea
-Abdominal Pain
-Abdominal Distention
-Excess Gas
-Weight Loss
-Constipation
-Acid Reflux
-Burping

So, what do you do if you suspect you have CSID?
Visit sucroseintolerance.com to take their quiz. On that site there is also an "ask your doctor" form to take to your physician. A sucrose breath test can help with the diagnosis. This can be ordered by your doctor or I can order a complimentary sucrose breath test to be sent to your home.

What if you have a positive breath test?
You will do a two-week elimination diet of sucrose and starch, followed by a gradual reintroduction of both. The reintroduction will help identify how much of these foods you can tolerate. Many like the guidance of a dietitian familiar with this deficiency to help them navigate through the elimination and reintroduction phases. Others also do the diet along with a test of the sucrase enzyme, Sucraid® (sacrosidase) Oral Solution. The sucrase enzyme can be purchased through One Patient Services, a subsidiary of QOL, Medical, LLC, the manufacturer of Sucraid® Oral Solution, at SucraidASSIST.com.

Please consult with your doctor regarding your symptoms and for a final diagnosis.

If you have any questions regarding Congenital Sucrase-Isomaltase Deficiency, please feel free to call or text me at 410-963-4782 or email at valerie@bluetreenutrition.com.

Spend any amount of time on social media these days, and you'd think protein is the answer to all your health concerns. Protein coffee, protein cereal, protein deserts…even foods that never needed protein are getting a boost.

Protein absolutely plays an important role in overall health. But when it comes specifically to gut health, fiber is the real MVP.

Why Gut Health Depends on What Your Microbes Eat

Your gut is home to trillions of microorganisms collectively known as the gut microbiome. These microbes, which include bacteria, viruses, fungi, and more, influence everything from digestion and metabolism to inflammation and immune function

But microbes need fuel to survive.

Unlike human cells, your gut microbes thrive on dietary fiber—not protein—as their main source of energy. When you eat fiber, your gut microbes ferment it and produce compounds that actively support health.

However, when fiber intake is low, your gut microbes are forced to rely on other sources of fuel, including protein. And that shift can change which metabolic byproducts are produced in your gut. And spoiler alert—that's not always for the better.

What Happens When You Eat Fiber

Fiber is a type of carbohydrate that resists digestion in the small intestine. Instead of being absorbed, it travels to the colon intact. 

This is where the magic happens. Your gut microbes ferment fiber, producing a variety of different compounds. Some of the most important byproducts of fiber breakdown by your microbiome are short-chain fatty acids (SCFAs).

SCFAs are incredibly beneficial for overall health. Here are just some of their benefits:

  • Nourish colon cells
  • Strengthen the gut barrier
  • Reduce inflammation
  • Support regular bowel movements
  • Maintain a healthy gut pH
  • Protect against several diseases

In other words, fiber doesn't just help you poop—it actively supports the structure and function of your gut.

The Different Types of Fiber (And Why They All Matter)

You might be surprised to learn that not all fiber works the same way. Soluble fiber, which dissolves in water to form a gel-like substance, slows digestion, helps regulate blood sugar, lowers cholesterol, and, in some cases, acts as fuel for your good gut microbes. The fiber that can act as fuel for your gut microbes is called prebiotic fiber, and you can think of it as fertilizer for your gut microbiome. 

In contrast, insoluble fiber does not dissolve in water, but adds bulk to stool, helping promote gut regularity and speeding up the transit of waste through your digestive tract.

Both types of fiber are needed for a healthy gut, but soluble fiber is typically "gentler" on digestion and can be especially helpful for people with IBS.

What Happens When Protein Reaches the Colon

Protein is essential for muscle repair, immune function, and hormone regulation, and most of it is digested and absorbed in the small intestine. However, when large amounts of protein (especially protein from animal products) reach the colon, your gut microbes break it down through fermentation. Unlike fiber fermentation, protein fermentation produces compounds that can negatively affect gut health.

Byproducts of protein fermentation can include ammonia, hydrogen sulfide, phenols, and indoles. In large amounts, these compounds have been linked to gut barrier irritation, increased inflammation, unfavorable changes in gut microbiome composition, and a potential increase in colorectal disease risk.

This doesn't mean protein is harmful, but it highlights that a high-animal-protein, low-fiber diet shifts microbial activity toward less beneficial metabolic pathways. In other words? The extremely popular carnivore diet is not a good choice for gut health. Overall, it's best to focus on plant-based sources of protein, which studies show can improve gut microbiome composition. 

Why Fiber Matters More Than Protein for Gut Health

From a gut health perspective, fiber has several advantages over protein:

  • Feeds beneficial microbes
  • Produces anti-inflammatory compounds
  • Supports stool consistency and transit
  • Improves microbial diversity

Most people meet or exceed the recommended protein intake, but fall dramatically short on fiber intake (some studies suggest that as many of 95% of people in the United States don't reach the recommended fiber intake).

The National Academy of Medicine recommends that women aim for around 25 grams of fiber per day, while men should target about 38 grams per day.

Simple Ways to Increase Fiber Intake

Increasing your fiber intake doesn't require a complete diet overhaul. Small, consistent changes tend to work best, especially if you have a sensitive digestive system.

Instead of removing foods from your diet, focus on adding fiber where you can or switching to higher fiber options:

  • Toss canned beans or chickpeas into salads
  • Add chia or ground flax seeds to your yogurt
  • Include at least one plant food at every meal
  • Switch to whole wheat or fiber-enriched pasta
  • Choose rolled oats instead of sugary breakfast cereals
  • Cook and cool starchy foods like potatoes, rice, and pasta before eating to naturally increase resistant starch (a type of prebiotic) content

To avoid digestive problems, increase your fiber intake slowly by 2-3 grams per day, and ensure you're drinking plenty of fluids to keep things moving smoothly. To increase the diversity of fiber you're providing to your microbes, aim to eat 30 different plant foods (which include fruits, vegetables, legumes, nuts, seeds, herbs, and spices) per week.

The Bottom Line

Protein is essential for overall health, but when it comes to gut health, fiber plays the starring role. It feeds your beneficial gut microbes, strengthens the gut linking, and produces anti-inflammatory compounds that help keep your digestive system running smoothly. Meanwhile, diets high in protein but low in fiber may encourage metabolic byproducts linked with less favorable gut outcomes.

If your priority is improving gut health, the evidence consistently points in one direction. Eat more fiber, and your gut microbes will thank you. If you're struggling with increasing your fiber intake, a dietitian can help. Click here to get in touch with us at Blue Tree Nutrition and learn about how we can support your journey to better gut health.

10 Comments

  1. Edy Trujillo

    Valerie,
    I have suffered with an unhealthy gut for years and much likely due to my bad eating habits, I’m slowing improving. Y food choices but get discouraged easily when I can’t pinpoint the cause of my problems. Reading your article makes me think I have CSID. Can I do the complimentary hydrogen test? Thank you

    Reply
    • Valerie Polley

      Edy,
      There can be many reasons for gastrointestinal issues. I will email you at Remeeedy@gmail.com so we can talk more about it.
      Valerie

      Reply
  2. Robin

    I had an endoscopy last Sept. It said CSID. I haven’t really received much more help to live with this beast. I’m on the toilet more than I’m not. I weigh 92 lbs and sad at my body appearance. Saw Rochester Mayo Dr and he is making me take the breath test AND a fructose test too whenever they get in touch with me, because he thinks the biopsy was wrong last Sept. I’m a true mess. I know I do have Sucraid and it seems to help some, if I remember to take it.

    Reply
    • Valerie Polley

      I am sorry you are dealing with this. I see clients with varying degrees of SID. Some have SID with other things on top, such as IBS. Once you get the results of the breath tests, then you can go from there. There are dietitians that can help and I am happy to help as well depending on what state you live in. I would try to use the Sucraid now to see if it gets control of some of your symptoms. You are welcome to reach out to me at valerie@bluetreenutrition.com or 410-963-4782.

      Reply
  3. Vanessa

    Does anyone know of any CSID cookbooks?

    Reply
    • Valerie Polley

      I have never been asked this question, so I did some searching. I found one on Amazon called A Place to Start Without Sugar and Starch. It is for those with CSID. That is the only cookbook I found. Pinterest has some ideas as well. This looks like something that is lacking, but could be very helpful as more individuals are diagnosed with it.

      Reply
  4. Andrea

    My 13 year old daughter was diagnosed last July with CSID and lactase deficiency. She is on sucraid and lactaid. She lost 20 pounds the first few weeks when cutting many sugars and saw improvement. Now she’s back to cramping, bloating. Where can I find directions on how to do the 2 week elimination diet? Any help will.be appreciated. She is very frustrated and sad about her condition.

    Reply
    • Valerie Polley

      I am sorry your daughter is dealing with this. I know it can’t be easy for any of you. I am not sure what you mean by the 2 week elimination diet. Is that for SID or something else? Some individuals with SID also deal with other issues, such as IBS. I would need to evaluate what she is eating now and go from there. I am happy to help out or find another dietitian closer to you. You can reach out to me at valerie@bluetreenutrition.com or 410-963-4782.

      Reply
  5. Christin Firestine

    I live in western nc do you see clients with CSID tele- health or can you recommend someone near me that went to that conference about CSID. Not many are educated in that field.

    Reply
    • Valerie Polley

      I am sorry I am just seeing this now. I have no idea why it didn’t alert me. Every state has different licensure laws and NC is one of those states, so I can’t legally see someone from NC.If you have not found someone there is a great dietitian in your state that can help. Her name is Erin Skinner and I am including a link her website.
      https://www.empowerednutrition.health

      Reply

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Hello, I am

Valerie

My name is Valerie Polley. I am a Indianapolis-based registered dietitian and owner of Blue Tree Nutrition. I consult with clients both local and far away.
I have a bachelor’s degree in nutrition from Purdue University and I have been practicing for 20 years.
I thoroughly enjoy helping clients through their gut health journey. I see a range of GI issues including, but not limited to celiac disease, IBS and SIBO. I also specialize in the FODMAP elimination diet.