Evaluation of PKU Sphere in Maternal PKU

Study Results

Results pending

The study team has not published outcome measurements, participant flow, or safety data for this trial yet. Check back later for updates.

Basic Information

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Recruitment Status

COMPLETED

Total Enrollment

12 participants

Study Classification

OBSERVATIONAL

Study Start Date

2019-07-29

Study Completion Date

2025-10-07

Brief Summary

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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.

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Detailed Description

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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.

Conditions

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Maternal Phenylketonuria

Study Design

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Observational Model Type

COHORT

Study Time Perspective

PROSPECTIVE

Study Groups

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PKU sphere

PKU sphere (an FSMP) as per individual requirements determined by a dietitian.

PKU sphere

Intervention Type DIETARY_SUPPLEMENT

PKU sphere is a Food for Special Medical Purposes (FSMP). This product is for use in the dietary management of phenylketonuria (PKU).

It is a powdered, low phenylalanine medical food containing a balanced mix of GMP, essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals, and DHA.

It is available in 27g sachets providing 15g PE and contains 28mg Phe and 35g sachets providing 20g PE and contains 36mg Phe.

Interventions

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PKU sphere

PKU sphere is a Food for Special Medical Purposes (FSMP). This product is for use in the dietary management of phenylketonuria (PKU).

It is a powdered, low phenylalanine medical food containing a balanced mix of GMP, essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals, and DHA.

It is available in 27g sachets providing 15g PE and contains 28mg Phe and 35g sachets providing 20g PE and contains 36mg Phe.

Intervention Type DIETARY_SUPPLEMENT

Eligibility Criteria

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Inclusion Criteria

* Diagnosis of phenylketonuria (PKU) or hyperphenylalaninemia (hyperphe) which requires dietary management during pre-conception and/or pregnancy.
* Aged 16 years and above.
* Following dietary management advice pre-conceptually and/or during pregnancy, aiming for phenylalanine levels of 120-250µmol/L.
* Prior positive PKU sphere taste test completed as part of routine care.
* Chosen to take part or full requirement of protein substitute as PKU sphere pre-conceptually and/or during pregnancy.
* Willingly given, written, informed consent from patient.
* Participant is, in the opinion of the investigator, able to participate and can comply with the study protocol.

Exclusion Criteria

* Conception of pregnancy without commencement of phe-restricted diet and blood phenylalanine not maintained within target range by 10 weeks' gestation.
* Patients with known soya, milk or fish allergies / intolerance.
* Intake of pegvaliase or large neutral amino acids within 30 days prior to screening visit.
* Patients who are currently participating in, plan to participate in, or have participated in an interventional investigational drug, food or medical device trial within 30 days prior to screening visit.

Minimum Eligible Age

16 Years

Eligible Sex

FEMALE

Accepts Healthy Volunteers

No