Glutaric Acidemia type 1 (GA1)
is a genetic disorder caused by a deficiency in glutaryl-CoA dehydrogenase (GCDH) enzyme.In addition to prescribed riboflavin, patients are prescribed a Lys and protein restricted diet to prevent high levels glutaric acid, 3-hydroxy (OH)-glutaric and glutaconic acid.
This App is designed to support families and children with GA-1 on lysine restricted diets.
What is HCY?
Glutaric Acidemia type 1 (GA1), is a genetic disorder caused by a deficiency in glutaryl-CoA dehydrogenase (GCDH) enzyme needed to break down lysine (Lys) and tryptophan (Trp). Read more on GA1 via this link.
Lys and Trp are important amino acids that we need to eat from protein in foods. Protein is important for growth in children and health maintenance in adults.
GCDH is an enzyme that helps break down glutaric acid, 3-hydroxy (OH)-glutaric and glutaconic acids
Patients with GA-1 may or may not have any symptoms at birth and appear normal until illness occurs. This illness may present with symptoms such as vomiting, ataxia, seizures, brain bleeds, and macrocephaly.
An oral dose of riboflavin, a vitamin, is prescribed to help increase any remaining function of the GCDH enzyme.
Why a diet?
In addition to prescribed riboflavin, patients are prescribed a Lys and protein restricted diet to prevent high levels glutaric acid, 3-hydroxy (OH)-glutaric and glutaconic acid.
People living with GA-1 are prescribed their daily Lys and protein 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 Lys content of foods, it is very difficult for families to track daily dietary Lys intake.
We hope this App makes daily Lys counting and meal planning easier for people following low Lys diets.
For whom?
This App is designed to support families and children with GA-1 on lysine restricted diets.
This App is not a substitute for the health care provided by your medical team!