A rare genetic disease that can make healthy foods dangerous!

According to IsnaFor most people, eating a wide variety of fruits and vegetables is the cornerstone of a healthy diet, but for people with hereditary fructose intolerance, even a few bites of juicy watermelon or some sun-dried tomatoes in a salad can cause serious health problems.

It is not a rare disease, allergy or food sensitivity, but if not recognized and managed properly, it can lead to serious health problems.

What is hereditary fructose intolerance?

Hereditary fructose intolerance is a rare genetic condition that affects how the body handles the sugar fructose.

Fructose is not only in fruit. It is also found in honey, some vegetables, sweetened beverages, and many packaged foods such as cakes, cookies, sauces, and some breads.

Fructose can also be added during the processing of some meats (delicates and sausages) and dairy products (cocoa milk).

Sucrose (the sugar we use at our tables) and sorbitol (a sugar substitute often found in gum, toothpaste, and medicines) also contain fructose or are converted to fructose during digestion. This means that people with hereditary fructose intolerance also have an intolerance to these sugars.

People with this disease do not have the key enzyme “aldolase B” or (aldolase B) needed to break down fructose. This means that fructose accumulates in the liver, kidneys, and intestines, and this excess fructose can cause serious health problems such as seizures, coma, and in some cases, death from liver and kidney failure.

How common is this disease?

Hereditary fructose intolerance is transmitted to a person when both his parents have the gene. It is considered a rare disease that affects approximately 1 in 10,000 people.

It usually appears when babies start eating solid foods such as fruits, vegetables, or sweet baby foods that contain fructose.

In adults, hereditary fructose intolerance can be misdiagnosed as other conditions such as glycogen storage disease, eating disorder, or recurrent hepatitis.

Because of this overlap in symptoms, hereditary fructose intolerance in adults can go undiagnosed for years.

What is the difference between this disease and food allergy or sensitivity?

Hereditary fructose intolerance is significantly different from food allergy. A food allergy involves the body’s immune system reacting to a food, for example cow’s milk protein, as if it is harmful to the body, which can cause symptoms such as hives and blisters, swelling of the mouth, or difficulty breathing.

Hereditary fructose intolerance is also different from food allergies such as lactose intolerance or non-celiac gluten sensitivity. This disease does not affect the immune system, but it can still cause discomfort such as bloating, changes in bowel habits or stomach pain.

Hereditary fructose intolerance is a genetic disease that causes food intolerance and is not related to the immune system.

It is also different from fructose malabsorption, which was previously informally referred to as “dietary fructose intolerance.” It is a milder gastrointestinal disorder in which the small intestine does not absorb fructose well, causing symptoms such as stomach pain, bloating, and gas.

How to diagnose it?

Symptoms in infants and young children may include vomiting, unusual drowsiness or irritability, refusal to eat, and failure to gain weight.

Some children instinctively avoid sweet foods, which may mask the condition until late childhood or adulthood.

Symptoms in adults can include chronic stomach pain, fatigue, and unexplained low blood glucose (sugar) levels. Doctors may also notice subtle signs, such as a swollen liver, abnormal liver tests, or symptoms of fatty liver disease.

Confirmation of the disease requires genetic testing or specialized glucose (sugar) tolerance testing, but for many, the diagnosis comes only after years of confusion, frustration, and dietary trial and error.

How can it be managed?

There is no cure for hereditary fructose intolerance, but it can be managed by strict avoidance of fructose, sucrose, and sorbitol. Reading labels on food packages becomes essential for everyday life, as even sauces, medicines and toothpaste can contain these sugars.

People suffering from this disease should observe the following:

• Avoid consuming all fruits, juices, canned fruits and other fruit products.
• Avoid cereals containing added sugars, honey, dried fruits or sweet flavorings. Pasta, rice, and other plain grains like quinoa or buckwheat are generally safe, but avoid flavored or prepared varieties.
• Most vegetables are safe to eat, except sweeter ones like peas, corn, beets, onions, pumpkin, sweet potatoes, carrots, and zucchini.
• In the case of bread, there is nothing wrong with bread without added sugar or sweeteners.
• Regarding desserts and dairy products, you should also avoid sweet desserts or flavored yogurts. Natural yogurts are usually fine, but be careful with plant-based milks like almond milk, which often have added sugar.
• In the case of protein, red meat without sugar or flavored, chicken, Turkey, fish, beans and lentils, eggs and tofu are usually safe, but processed meats such as sausages and prepared meats or marinated meats should be avoided.
• Care should also be taken with sauces and condiments, as many contain hidden sugars or sorbitol. In this case, you can use homemade versions using safe raw materials.

Awareness is important

If someone avoids certain foods, or if they feel sick after eating fruit, don’t assume that they have a problem or have an eating disorder, as they may have “hereditary fructose intolerance.”

More awareness of this rare disease can mean early diagnosis and better support for affected people.

For parents, paying attention to a child’s sudden or severe aversion to sweets, frequent vomiting or slow growth can be an important clue.

For doctors, considering hereditary fructose intolerance as a possible cause of unexplained digestive problems, low blood sugar or liver changes can make a significant difference in patients’ lives.

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