Homocystinuria (HCY)
is a genetic disorder caused by a deficiency in the cystathionine beta-synthase enzyme.Patients are prescribed a protein restricted diet to prevent elevated levels of homocysteine and methlymalonic acid.
This App is designed to support families and children with HCY on protein restricted diets.
What is HCY?
Homocystinuria (HCY) is a genetic disorder caused by a deficiency in the cystathionine beta-synthase enzyme. Read more on HCY via this link.
The amino acid methionine (Met), commonly found in a normal diet, is converted to homocysteine. The enzyme cystathionine beta-synthase is then responsible for converting the homocysteine to cysteine (Cys).
Met is an important amino acid that makes up protein in foods. Protein is important for growth in children and health maintenance in adults.
Decreased cystathionine beta-synthase enzyme activity results in the build up of homocysteine and methylmalonic acid.
Symptoms of HCY may include: downward dislocation of the lens, increased risk of strokes and developmental delay.
Why a diet?
Patients are prescribed a protein restricted diet to prevent elevated levels of homocysteine and methlymalonic acid.
People living with HCY are prescribed their daily protein restriction by their physician or dietitian. In particular, patients restrict their Met intake. Each person’s daily goals change and need ongoing adjustment based on each person’s nutritional needs.
Since nutrition labels on foods do not list Met content of foods, it is very difficult to track their daily dietary intake.
We hope this App makes daily protein and tyrosine counting and meal planning easier for people following low protein diets.
For whom?
This App is designed to support families and children with HCY on protein restricted diets.
This App is not a substitute for the health care provided by your medical team!