Download Protein substitute - PKU

Protein substitute - PKU
Questionnaire about the use of protein substitute in PKU patients
* Required
Introduction
Dear Colleagues,
We are writing to you for your help once more.
We are writing the European PKU guidelines and we would like to understand your prescribing practices
for the amount of L-amino acid supplement you prescribe in PKU. We need to know what factors
determine your prescription dose, if you consider the amount of natural protein in your calculations, and
the types of L-amino acid supplement you prefer to use.
We would therefore be grateful, if you would take a few minutes to complete this short survey on line.
This data will be very helpful to us and will be important in the guidelines writing process in the nutrition
section.
Thank you for your help.
With best wishes,
Anita MacDonald
Francjan van Spronsen
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Demographic information respondents
Name
Role/Position *
One answer possible.
Physician
Dietitian/ Nutritionist
Nurse
Other
Centre/Hospital *
Please state the full name.
Country *
One answer possible.
Armenia
Denmark
Ireland
Austria
Estonia
Italy
Belgium
Finland
Latvia
Bosnia &Herzegovina
France
Lithuania
Bulgaria
Germany
Luxembourg
Croatia
Greece
Netherlands
Cyprus
Hungary
Norway
Czech Republic
Iceland
Poland
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Portugal
Slovenia
Ukraine
Romania
Spain
United Kingdom
Russian Federation
Sweden
Other
Serbia
Switzerland
Slovak Republic
Turkey
Which PKU patients do you currently manage? *
One answer possible.
Children
Adults
Children and Adults
What is the number of patients with PKU in your centre? *
Could you indicate the number of patients per (age) category? *
One answer per row possible.
None
1-10 persons
11-20
persons
21-30
persons
>30 persons
<1 year
1-10 years of age
11-16 years of
age
>16 years of age
Maternal patients
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Protein substitute - Dosage
1. What is the TOTAL dose of protein equivalent (PE) (including natural and from protein
substitute) you give to the following (age) category of patients with PKU? *
One answer per row possible.
Not
Applicable
<1 PE
g/kg/day
1 - 1.5
PE
g/kg/day
>1.5 - 2
PE
g/kg/day
>2 - 2.5
PE
g/kg/day
>2.5 - 3
PE
g/kg/day
>3 PE
g/kg/day
Infants <1
year of age
1-3 years of
age
4-10 years of
age
>10 years of
age
Maternal
patients
2. What is the dose of protein equivalent (PE) you give to the following (age) category of patients
with PKU from PROTEIN SUBSTITUTE ONLY? *
One answer per row possible.
Not
Applicable
<1 PE
g/kg/day
1 - 1.5
PE
g/kg/day
>1.5 - 2
PE
g/kg/day
>2 - 2.5
PE
g/kg/day
>2.5 - 3
PE
g/kg/day
>3 PE
g/kg/day
Infants <1
year of age
1-3 years of
age
4-10 years of
age
>10 years of
age
Maternal
patients
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3. What factors do you consider when prescribing the dose of protein substitute in PKU? *
Multiple answers are possible
Age [A]
Adherence issues [G]
Blood phenylalanine control [B]
Side effects [H]
Weight [C]
Costs [I]
Growth rate of child [D]
Local guidelines/recommendations [J]
Overweight/ Obesity [E]
Not applicable
Natural protein tolerance [F]
Other:
4. Which factors are most important when prescribing the dose of protein substitute in PKU? *
Please rank your TOP 3 most important factors. Choose the letters next to potential answers.
1)
2)
3)
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Protein substitute- Inefficiency factor
5. Do you consider a maximum level of protein intake when determining total protein requirements
in PKU? *
One answer possible.
Yes
No
Don't know
6. If YES, on what do you base this maximum level of protein intake, e.g. WHO/FAO/UNU (2007)?
Please explain.
7. Do you allow an inefficiency factor for utilization of L-amino acids when prescribing for protein
substitute in PKU? *
One answer possible.
Yes
No
Don't know
8. If YES, what % of additional L-amino acids do you prescribe (over and above WHO/FAO/UNU
2007 safe levels of protein intake) to allow for an inefficiency of utilization of L-amino acids?
Please indicate the percentage.
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Daily practice
9. Which type of protein substitutes do you use in your clinic? *
Multiple answers are possible.
L-amino acids supplemented WITH vitamins and minerals
L-amino acids supplemented WITHOUT vitamins and minerals
Glycomacropeptide (GMP)
Large Neutral Amino Acids (LNAA)
10. If you use more than one type of potein substitutes, which is the MOST commonly used
protein substitute in your clinic? *
One answer possible.
L-amino acids supplemented WITH vitamins and minerals
L-amino acids supplemented WITHOUT vitamins and minerals
Glycomacropeptide (GMP)
Large Neutral Amino Acids (LNAA)
11. What % of your patients on treatment for PKU take a protein substitute? *
Please indicate the percentage.
12. How often do you routinely recommend protein substitutes throughout 24 hours in PKU to
children OVER 1 YEAR of age? *
One answer possible.
1 time per 24 hours
2 times per 24 hours
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3 times per 24 hours
4 times per 24 hours
5 times per 24 hours
6 times per 24 hours
7 or more times per 24 hours
13. What influences frequency of dosage in protein substitutes in your clinic? *
Multiple answers are possible.
Age of patients
Lifestyle of patients/ caregivers
Metabolic control
Patient adherence
Not applicable
Other:
14. Do your patients routinely take protein substitutes containing a trace amount of
phenylalanine? *
One answer possible.
Yes
No
Don't know
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15. If NO, what is your barrier for NOT taken protein substitutes containing a trace amount of
phenylalanine?
Please explain.
16. Do you routinely recommend extra water with protein substitutes? *
One answer possible.
Yes
No
Don't know
17. If YES, why do you routinely recommend extra water with protein substitutes, e.g. transition
phase, taste?
Please explain.
18. Do you routinely recommend more concentrated protein substitutes? *
One answer possible.
Yes
No
Don't know
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19. If YES, why do you routinely recommend more concentrated protein substitutes, e.g.
compliance?
Please explain.
20. Which of the following do you monitor in PKU? *
Multiple answers possible.
Gastrointestinal upset e.g. abdominal discomfort/constipation
BMI/growth
Renal function
Nutritional biochemical markers
Not applicable
Other:
21. Do you routinely calculate the vitamin, mineral and trace element intake of all your patients
with PKU? *
One answer possible.
Yes
No
Don't know
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Protein substitute- Format
22. What format of protein substitute do you MOST prefer for children aged 1-3 YEARS? *
One answer possible.
Powder made into a liquid
Powder given in a spoonable/gel format from a spoon
Ready to use liquid
Powder added to food
Not applicable
Other:
23. What format of protein substitute do you MOST prefer for children aged 4-10 YEARS? *
One answer possible.
Powder made into a liquid
Powder given in a spoonable/gel format from a spoon
Ready to use liquid
Powder added to food
Chocolate or biscuit style bar
Not applicable
Other:
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24. What format of protein substitute do you MOST prefer for children aged 11-16 YEARS *
One answer possible.
Powder made into a liquid
Powder given in a spoonable/gel format from a spoon
Ready to use liquid
Powder added to food
Chocolate or biscuit style bar
Tablets
Not applicable
Other:
25. What format of protein substitute do you MOST prefer for children aged OLDER THAN 16
YEARS *
One answer possible.
Powder made into a liquid
Powder given in a spoonable/gel format from a spoon
Ready to use liquid
Powder added to food
Chocolate or biscuit style bar
Tablets
Not applicable
Other:
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26. What format of protein substitute do you MOST prefer for MATERNAL patients *
One answer possible.
Powder made into a liquid
Powder given in a spoonable/gel format from a spoon
Ready to use liquid
Powder added to food
Chocolate or biscuit style bar
Tablets
Not applicable
Other:
Thank you for participating
Please send your questionnaire back to:
Postal address:
Dr. Anita MacDonald
Birmingham Children’s Hospital,
Dietetics Department, Inborn Errors of Metabolism
Steelhouse Lane, B4 6NH, Birmingham, West Midlands
United Kingdom
OR
Email address:
[email protected]
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