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Isovaleric Acidemia (IVA)

is a genetic disorder caused by a deficiency in the isovaleryl-CoA dehydrogenase (IVD) enzyme.

Patients are prescribed a protein and Leu restricted diet to prevent high levels of Isovaleric acid.

This App is designed to support families and children with IVA on protein restricted diets.
What is IVA?
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Isovaleric Acidemia (IVA) is a genetic disorder caused by a deficiency in the isovaleryl-CoA dehydrogenase (IVD) enzyme. Read more on IVA via this link.
IVD is responsible for the break down of isovaleric acid that comes from an amino acid called leucine (Leu).
Leu is an important amino acid that we need to eat from protein in food. Protein is important for growth in children and health maintenance in adults.
Decreased IVD enzyme activity results in very high levels of isovaleric acid.
Symptoms of IVA, which usually appear within the first few days of life, include: poor appetite, vomiting, and lack of energy. If the Isovaleric acid continues to build up, it could lead to seizures.
In some cases, patients are supplemented with carnitine and glycine (Gly).
Carnitine binds to isovaleric acid to form a less harmful substrate for the patient.
Gly also binds with isovaleric acid to form isovalerylglycine that can be safely removed from your body through urine.
Why a diet?
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Patients are prescribed a protein and Leu restricted diet to prevent high levels of Isovaleric acid.
People living with IVA are prescribed their daily protein and/or Leu restriction by their doctor or dietitian. These daily goals often need to be changed based on each person’s nutritional needs and age requirements.
Since nutrition labels on foods do not list Leu content of foods, it is very difficult for families to track their daily dietary intake.
We hope this App makes daily protein and Leu counting and meal planning easier for people following low protein diets.
For whom?
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This App is designed to support families and children with IVA on protein restricted diets.
This App is not a substitute for the health care provided by your medical team!