Starting Solids When Your Baby Has Hereditary Fructose Intolerance (HFI): A Parent’s Guide
If you’ve recently learned that your baby has hereditary fructose intolerance (HFI), you may be feeling a mix of emotions. You may be feeling relief about having answers, but also concern about what feeding your little one will look like moving forward.
Feeding is such a central part of parenting, so it’s natural to want to get it right. The good news? With early diagnosis, proper management, and the right support, babies with HFI who follow an HFI-safe diet have a great prognosis and often live healthy, long, thriving lives while enjoying many safe and delicious foods. In fact, the babies in my private pediatric nutrition practice who have HFI tend to eat healthier, more balanced diets than those without!
Understanding Hereditary Fructose Intolerance (HFI)
Imagine biting into a crisp apple or drizzling honey into your tea—simple pleasures for most of us. But for someone with hereditary fructose intolerance (HFI), these foods can cause serious harm.
HFI is a rare genetic condition, affecting about 1 in 20,000 people in the United States. It occurs when the body lacks the enzyme fructose-1-aldolase, which is needed to break down fructose. Fructose is a naturally occurring sugar found in fruits, some vegetables, and many sweetened products. Without the enzyme fructose-1-aldolase, fructose builds up in the liver and kidneys, which can lead to low blood sugar, vomiting, jaundice, and seizures. If left untreated, it can cause more serious complications like liver or kidney failure.
HFI is often confused with fructose malabsorption, but these conditions are not the same. Fructose malabsorption is far more common and less severe than HFI. While people with fructose malabsorption can often tolerate varying amounts of fructose, those with HFI must completely eliminate fructose, sucrose, sorbitol, and sucralose from their diets.
Signs and Symptoms of HFI
Symptoms of HFI often appear when an infant is first introduced to solid foods or infant formulas containing fructose or sucrose. Common signs include:
Vomiting
Abdominal pain
Jaundice
Low blood sugar
Poor growth or “failure to thrive”
Irritability, sweating, or seizures after feeding
Diagnosis and Early Detection of HFI
Historically, HFI was diagnosed only after infants showed severe symptoms, once they began eating solid foods or consuming certain formulas. Today, however, an earlier and safer diagnosis is possible. Genetic testing now allows for the identification of HFI even before symptoms appear. This early identification will enable families to make safer dietary choices right from the start.
***It is important to note that fructose tolerance testing should never be used as a diagnostic method for HFI, as it can cause life-threatening reactions.
With a carefully managed diet and guidance from a registered dietitian, individuals with HFI can expect a normal life expectancy and healthy development.
Preparing Babies with HFI for Solid Foods
Timing for Starting Solids
Just like with any baby, solid foods can typically begin around 6 months of age when the baby shows signs of developmental readiness, which include:
Sitting up with minimal support
Having good head and neck control
Reaching for food, grasping larger objects, and bringing them up to the mouth
Showing interest in eating
Choosing HFI-Safe Ingredients for Infants
The goal when introducing solids to a baby with HFI is to provide nutrient-rich foods that are free from fructose, sucrose, sorbitol, and sucralose. Many wholesome, savory foods fit perfectly into an HFI-safe plan. These include:
Meats
Grains
Eggs
Dairy
Some vegetables
All of these can support growth while keeping your baby safe.
Understanding Sugar Types: Sucrose, Fructose, Sucralose, and Sorbitol
Lifelong avoidance of any foods containing the following four types of sweeteners is the cornerstone of treatment for individuals with HFI:
Fructose: Naturally found in fruit, honey, and some veggies.
Sucrose: Common table sugar, which is a combination of glucose and fructose.
Sorbitol: A sugar alcohol often used as a sweetener.
Sucralose: An artificial sweetener made from sucrose.
Starting Solids With HFI
What is Baby-Led Weaning?
Baby-led weaning (BLW) is a popular feeding approach that encourages self-feeding and exploration. Rather than spoon-feeding purees, parents offer soft, finger-sized foods that babies can pick up and explore independently. This helps babies build motor skills, independence, and positive associations with food.
For babies with HFI, BLW can be an empowering and joyful experience. It just requires being a bit more mindful about ingredient safety.
Do HFI Babies Have to Choose Between BLW and Puree Spoonfeeding?
Many families find that combining purees with baby-led weaning offers the best of both worlds when starting solids with HFI. This flexible approach allows you to spoon-feed purees like green beans while letting your baby explore finger foods such as scrambled eggs or strips of lightly toasted, sweetener-free bread.
Blending these two methods helps your baby build confidence with textures and flavors while incorporating plenty of feeding flexibility into your plan, allowing you to keep your baby’s meals free from fructose, sucrose, sorbitol, and sucralose.
Getting HFI Babies Started with Solid Foods
Here are some important BLW tips to keep in mind, before you begin:
Make sure your baby can sit upright with good control and minimal support.
Use a sturdy high chair with a food rest and safety straps.
Serve small amounts, about a tablespoon of each food to start.
Stay close, observe throughout, and let your baby set the pace.
Be patient. Mess and exploration are part of the process!
Remember, the majority of your baby's calories will still be coming from breast milk and/or infant formula at this stage, so the goal is exploration, not volume.
10 HFI-Safe First Foods for Baby-Led Weaning
These early foods are free from fructose, sucrose, sorbitol, and sucralose and can be prepared safely for self-feeding. Each supports growth and development while keeping your baby’s nutritional needs and safety in mind.
Soft-scrambled eggs: A great source of protein and choline for brain development.
Cooked, trimmed green beans: Steam until soft and coat with butter for flavor and fat.
Plain, full-fat yogurt: Unsweetened and creamy, it offers probiotics and calcium.
Full-fat ricotta cheese: Mild, soft, and rich in calcium and protein.
Cooked chicken or turkey: Choose darker cuts of meat for more tender pieces that are easier to swallow and richer in iron; serve as a drumstick on the bone, after removing the skin, gristle, and any protruding, smaller bones.
Soft, flaky fish (salmon): Packed with omega-3s for brain and eye health
Cooked, unsweetened oatmeal: Iron-rich and easy to scoop with hands when thickened slightly.
Lightly toasted bread (made with no added or natural sugars): Top with a thin layer of butter or plain, unsweetened nut butter.
Mashed avocados (if tolerated; per Stanford, UCLA Genetics): Full of heart-healthy fats and a smooth texture for self-feeding.
Mozzarella cheese: Easy to handle and a good source of protein and calcium.
Permitted and Not Permitted Foods for HFI
Permitted foods for Individuals with HFI:
Plain meats (chicken, turkey, fish, beef)
All fruits (fresh, dried, canned) and fruit products
Eggs, cheese, yogurt (unsweetened)
Honey, syrups, molasses
Plain grains (rice, oats, barley, wheat)
Sweetened cereals, breads, or baked goods
Unsweetened nut butters
Foods NOT Permitted for Individuals with HFI:
Processed foods with sucrose, fructose, sucralose, and sorbitol
Butter, oils, and plain fats
Fruit juices, sodas, or sweetened beverages (including milk)
Vegetables low in fructose (green beans, spinach, zucchini)
High-fructose veggies (corn, peas, carrots, sweetpotatoes)
For a more complete list and individualized guidance, consult a registered dietitian who specializes in HFI.
Infant Formula for HFI Babies
Considerations and Ingredients
If your baby is formula-fed, it’s critical to avoid formulas sweetened with fructose or sucrose. Instead, choose formulas that are lactose-based or made with corn syrup solids and/or maltodextrin (which contain glucose only, not fructose) as these are safe carbohydrates for babies with HFI. Paying close attention to ingredients ensures your baby stays nourished and safe. If you have questions about formulas, always consult a dietitian who specializes in HFI.
Transitioning HFI Babies From Formula to Solids
Transitioning to solid foods can and should be a gradual process. Start slow, and watch to ensure your baby is tolerating new foods. Keep a food log to track intake and reactions, and communicate regularly with your dietitian to ensure balanced nutrition.
Introducing potential allergens early (starting at around 6 months of age) and consistently should be a part of the process of starting solids, particularly for babies with HFI, so that their diets don't end up having to be further restricted due to food allergy.
Potential allergens like peanuts, eggs, dairy yogurt, almonds, other tree nuts, excluding cashews, soy, wheat, fish, and shellfish should be introduced early and often to help prevent food allergies from developing. See below for resources to help you work through this process, step-by-step.
As your HFI baby transitions off formula and/or breast milk, it will be important to supplement your child with nutrients that they may not be able to get from food alone, due to their dietary restrictions, such as vitamin C. Talk with a registered dietitian who specializes in HFI to find supplements that do not contain unsafe ingredients, are third-party tested, and that will help fill specific potential nutrient gaps.
Resources and Support for Patients
Raising a child with HFI can feel overwhelming at first, but know that while it might feel like it sometimes, you are not alone. Connecting with others, staying informed, and working closely with a knowledgeable dietitian can make all the difference.
Safe and Simple Food Allergy Prevention: For help navigating baby-led feedig, starting solids, and introducing allergens, check out my book, full of baby-friendly, allergy-safe recipes and strategies. Note that you will need to modify many of the recipes and foods to make them HFI-safe, as this book was not written for babies with HFI. However, it contains a lot of valuable information for you about starting solids, introducing allergens, preventing food allergies, and planning your baby’s meals.
HFI Support Network: Find real stories, family experiences, and community guidance.
Cleveland Clinic: A comprehensive HFI overview for families.
Connecting with HFI Support Groups
Online and local support groups can provide encouragement, practical advice, and updated product recommendations.
The Bottom Line
Starting solids when your baby has HFI can feel intimidating, but with careful planning, expert guidance, and a focus on minimally processed, safe foods, your baby can thrive. You don’t have to navigate it alone. Work with a registered dietitian who specializes in HFI to ensure your baby’s diet supports growth and safety.
Thank you to my Dietetic Intern, Dr. Bryant O’Leary, for his contributions to this post.