Maternal Phenylketonuria Clinical Trial
Official title:
An Observational Study to Evaluate Metabolic Control and Dietary Management in Women With PKU Taking PKU Sphere During Pre-conception and/or Pregnancy
This observational study aims to recruit females, aged 16 years and over, with phenylketonuria (PKU) or hyperphenylalaninemia (hyperphe) following dietary management advice pre-conception and/or during pregnancy, who are willing to take PKU sphere as part of their dietarty management.
The aim of this study is to observe dietary management and metabolic control in women taking PKU sphere pre-conceptually and/or during pregnancy. To do this the following data points will be captured: - Blood spot phenylalanine and tyrosine levels to measure metabolic control - Changes to dietary management and any adaptations arising from incorporating PKU Sphere - Compliance/adherence of patients to their recommended amount of protein substitute prescription. - Tolerance of PKU sphere with respect to gastrointestinal (GI) symptoms and pregnancy associated nausea and vomiting. - Acceptability of concurrent protein substitute(s) (if applicable). - Nutritional status and weight management. - Routine standard of care data on the final pregnancy outcome and postpartum. ;