Deadly Risk Found in New Remsima Formulation for Rare Disorder Patients

A critical warning has been issued for patients with hereditary fructose intolerance (HFI) regarding a new formulation of the medication Remsima. The updated intravenous version contains sorbitol, a substance that can trigger deadly reactions in those with the rare metabolic disorder. While other forms of the drug remain safe, the change has raised urgent concerns for affected individuals and their doctors.

Hereditary fructose intolerance is a genetic condition caused by a deficiency of the enzyme aldolase B. First documented in the 1950s, it prevents the body from breaking down fructose, sucrose, and sorbitol. Without treatment, exposure to these substances can lead to severe vomiting, seizures, and even catastrophic organ failure.

Symptoms usually appear in infancy, with hypoglycaemia and metabolic crises posing immediate risks. Diagnosis now relies on genetic testing, as older methods like breath tests could be dangerous. Management requires a strict lifelong diet avoiding all sources of fructose and sorbitol. The new intravenous liquid Remsima contains 45 mg of sorbitol per millilitre, making it completely unsafe for HFI patients. Even tiny amounts administered this way can cause life-threatening complications. However, the original powder form of Remsima—free from sorbitol—remains a safe alternative. Prefilled syringes and pens for subcutaneous injection also pose no risk, despite containing sorbitol, as they bypass the immediate metabolic pathway that triggers reactions.

The warning highlights the need for careful medication selection in HFI patients. Doctors must now verify the formulation of Remsima before prescribing it intravenously. For those affected, the original powder version and subcutaneous options provide safe alternatives without compromising treatment.