Carbamoyl phosphate synthetase (CPS1)
is a rare genetic disorder caused by a deficiency in the Carbamoyl Phosphate Synthetase 1 enzyme.Patients are prescribed a protein-restricted diet to prevent high levels of ammonia in the body.
This App is designed to support families and children with CPS-1 on protein restricted diets.
What is CPS 1?
Carbamoyl phosphate synthetase (CPS1) is a rare genetic disorder caused by a deficiency in the Carbamoyl Phosphate Synthetase 1 enzyme. Read more on CPS-1 via this link.
The enzyme plays a key role in the first step of the urea cycle and is part of a group of disorders known as the Urea Cycle Disorders.
Ammonia is formed when the protein we eat in food is broken down. Protein is important for growth in children and health maintenance in adults.
As ammonia is very damaging in the nervous system, CPS-1 often results in both brain and liver problems.
The symptoms of CPS-1 usually appear in the first few days of life. These symptoms may include lethargy, loss of appetite and poor control of breathing rate or body temperature.
Some individuals with less severe CPS-1 may not experience these symptoms till later in life.
Why a diet?
Patients are prescribed a protein-restricted diet to prevent high levels of ammonia in the body.
People living with CPS-1 are prescribed their daily 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.
Calculating and tracking protein intake can be a very hard and troublesome task for those with protein-restricted diets.
We hope this App makes daily protein counting and meal planning easier for people following low protein diets.
For whom?
This App is designed to support families and children with CPS-1 on protein restricted diets.
This App is not a substitute for the health care provided by your medical team!