FEED PKU
PORTFOLIO
Background
What is it?:
Feed PKU is a mixture of amino acids Phenylalanine free indicated
in the dietary management of:
✓ Phenylketonuria
✓ Hyperphenylalaninemia
Category:
Food for Special Medical Purposes
Reimbursed
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Phenylketonuria - PKU
• What is it?: PKU is an inborn error of metabolism that results in decreased metabolism of the
amino acid phenylalanine (Phe). It is due to mutations in the PAH gene which results in low levels of
the enzyme phenylalanine hydroxylase. This results in the build up of dietary Phe to potentially toxic
levels.
• Incidence: PKU affects about 1 in 10,000 - 25,000 newborns.
• Estimated number of Patients:
– Spain: 4,000
– Italy: 5,800
– Portugal: 1,000
• Diagnosis: newborn screening (heel prick), biochemical analysis.
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Phenylketonuria - PKU
• Symptoms:
If a child is not diagnosed and a Phe restricted diet is not introduced, then Phe levels in the blood will
increase.
Toxic levels of Phe (and insufficient levels of Tyr) can interfere with infant development in ways which
have permanent effects. The disease may present clinically with seizures, hypopigmentation, a "musty
odor" to the baby's sweat and urine, microcephaly, hyperactivity, learning disabilities, mental
retardation, etc…
• Treatment:
PKU is not curable. However, if PKU is diagnosed early enough, an affected newborn can grow up with
normal brain development by managing and controlling Phe levels through DIET, or a combination of
DIET and MEDICATION.
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DIET in PKU
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DIET in PKU
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Maternal PKU
• Pregnancy & Maternal PKU is not a contraindication for
pregnancy, however, mothers with PKU must strictly adhere to
the PKU diet and consistently maintain phenylalanine levels in
the 2‐6 mg/dL range.
• Hyperphenylalaninemia during pregnancy may result in intra
uterine growth retardation (IUGR), microcephaly, mental
deficiency, and congenital heart disease.
• Twice weekly phenylalanine level monitoring is recommended
in pregnant women with PKU.
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Why Feed PKU range? COMPLIANCE
• Taste:
– Neutral: Feed PKU Star → no taste / no change of food taste
– Orange: Feed PKU Avant & Plus → common & easy taste
• Low Osmolality: Feed PKU 1: 360 mOsm/l
Feed PKU Gel: 480 mOsm/l
– Easy dissolving even in low liquid volumes, Feed PKU Star: 590 mOsm/l
soups
or purées, no lumps. Feed PKU Plus: 610 mOsm/l
Feed PKU Avant: 790 mOsm/l
• Convenience :
– At home or take away individual sachets
– Feed PKU Gel allows liquid or purée texture, ideal when introducing solids
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Background Feed PKU
What is it?:
Feed PKU is a mixture of amino acids Phenylalanine free indicated in the dietary
management of Phenylketonuria & Hyperphenylalaninemia
Indication:
Feed PKU is a mixture of amino acids free from phenylalanine, enriched with long chain
fatty acids and carbohydrates and supplemented with vitamins, minerals and trace
elements according to the current recommendations of daily intake (R.D.I). for the
nutritional management of PKU and Hyperphenylalaninemia.
Category:
Food for Special Medical Purposes
Reimbursed
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Foods for Special Medical Purposes
• Foods intended for the dietary management (under medical
supervision) of individuals who suffer from certain diseases, disorders or
medical conditions
• These foods are intended for the exclusive or partial feeding of people
whose nutritional requirements cannot be met by normal
foods.
• There is a specific legislation for FSMP ruling:
– Composition
– Minimum & Maximum levels of vitamins and minerals
– Labelling
– Promotion, advertising, marketing (specifically prohibited to make nutrition
and health claims on foods for special medical purposes, in order to ensure
legal clarity and avoid inappropriate promotion of the products
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FEED PKU
PORTFOLIO
A product for each age range
PKU Maternal
≥ 6 months 3 yrs ≥15 yrs
0 yrs 1 yrs 14 yrs
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From 0 to 1 year Feed PKU 1
100g contains:
Eq. Prot. 11g
508 Kcal
Tyr: 10,91g
Feed PKU
100g contain:
Portfolio
Eq. Prot. 42g
From 6 months Feed PKU Gel 339 Kcal
Tyr: 4,04g
From 1 to 14 months Feed PKU Star 500g
100g contains:
Eq. Prot. 31,1g
422 Kcal
Tyr: 2,5g
Feed PKU Star 40sachets
From 3 to 14 months Feed PKU Plus 500g
100g contains:
Eq. Prot. 31,1g
412 Kcal
Tyr: 2,5g
Feed PKU Plus 40sachets
From 15 months, adults Feed PKU Plus 500g
& Maternal PKU 100g contains :
Eq. Prot. 45g
331 Kcal
Tyr: 4,6g
Feed PKU Plus 40sachets
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Why Feed PKU range? COMPLIANCE
• Taste:
– Neutral: Feed PKU Star → no taste / no change of food taste
– Orange: Feed PKU Avant & Plus → common & easy taste
• Low Osmolality: Feed PKU 1: 360 mOsm/l
Feed PKU Gel: 480 mOsm/l
– Easy dissolving even in low liquid volumes, Feed PKU Star: 590 mOsm/l
soups
or purées, no lumps. Feed PKU Plus: 610 mOsm/l
Feed PKU Avant: 790 mOsm/l
• Convenience :
– At home or take away individual sachets
– Feed PKU Gel allows liquid or purée texture, ideal when introducing solids
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Feed PKU
OPPORTUNITIES
✓ Leverage our engagement with Clinicians
✓ Samples in dispensing hospitals to get new patients
✓ “Taste loyal” patients not willing to change formula
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THANKS