Hereditary Fructose Intolerance (HFI) Support Australia
Our Story
This organisation was born out of my own journey with HFI. It's a space created for individuals and families affected by HFI to find guidance, reliable information, and a sense of community. You're not alone in this. We’re here to help you navigate the challenges of living with HFI—together.
What is HFI?
Hereditary Fructose Intolerance (HFI) is an inherited metabolic condition caused by a deficiency of the enzyme Aldolase B, which is normally found in the liver, kidneys and small intestine. Someone with HFI is born with it. Symptoms don’t generally appear until a baby is introduced to solid foods.
Resources
We offer a continually expanding collection of resources to support you and your family in managing HFI. From diet guides and safe product lists to metabolic support, genetic testing clinics, and more—our goal is to provide practical tools and trusted information every step of the way.
What is Hereditary Fructose Intolerance (HFI)?
Hereditary Fructose Intolerance (HFI) is an inherited metabolic condition caused by a deficiency of the enzyme Aldolase B, which is normally found in the liver, kidneys and small intestine. Someone with HFI is born with it. Symptoms don’t generally appear until a baby is introduced to solid foods.
An Aldolase B Deficiency
In a healthy liver, fructose is broken down in a stepwise process:
1. Fructose → Fructose-1-phosphate (by the enzyme fructokinase)
2. Fructose-1-phosphate → split into usable sugars (glyceraldehyde + DHAP) by aldolase B
But in HFI, aldolase B is missing.
Even small amounts of fructose, sucrose (which breaks down into fructose + glucose), or sorbitol (which gets converted to fructose) can trigger this cascade. It’s not just about discomfort — it’s a toxic reaction.
How is HFI treated?
There is no treatment as yet, but someone with HFI can stay healthy and well as long as they stick to a diet free of Fructose, Sucrose and Sorbitol. This diet is our lifesaver, but is often misunderstood, even by dieticians. The common and generalised concept of a "healthy" diet is poisonous for individuals with HFI.
What is the difference between Hereditary Fructose Intolerance (HFI) and Fructose Intolerance / Malabsorption?
HFI is a Genetic Metabolic disorder
Fructose Intolerance / Malabsorption is a Digestive issue