GSD Type III, VI and IX
- nutrition Wyboston Lakes
11.10.2015
Urike Steuerwald
Tórshavn / Hannover
[email protected]
Where does energy come from?
Sources? (where from) Function? (what for)
• sugar / carbohydrates
– Sources: bread, cereals, potatoes, rice, pasta, sugar,
vegetables, fruits….
– Function: nutrition of brain, sugar = “fast” fuel
• fat
– Sources: oil, butter, cheese / meat / fish
– Function: fuel for muscle, transport of vitamins
• proteins
– Sources: milk, meat, fish (dried fish: 80%!), chicken, turkey,
soy, tofu, beans, nuts
– Function: growth, muscles, 2nd line energy-source for brain
Storage of nutritional components
• Carbohydrates
– Stored as glycogen
– In liver and muscles (and nerve cells)
– If glycogen storage are filled up: stored as fat!
• Fat
– In adipocytes (fat cells) and muscle cells
• Protein
– In muscles
Aims of dietary treatment
• to prevent hypoglycemia by continuous supply of
exogenous glucose (polymers)
• a generous amount of protein to prevent too much
protein breakdown for gluconeogenesis
• to prevent and improve biochemical abnormalities
as hyperlipidemia and hypercholesterinemia
• to support an optimal physical condition,
muscle development and growth
• to minimize liver seize
• to be able to achieve a good quality of
life; a live as normal as possible

Treatment of GSD Type III, VI and IX
Goal: Provide energy for the muscles without
overstoring glucose as glycogen
Minimum amount of
cornstarch or
carbohydrate
possible to avoid
hypoglycemia
High protein
diet with 3-4
grams/kg/day
(in type VI and IX: 2-3g/kg/day)
Adjust dose based upon home glucose,
ketone,
and urine myoglobin monitoring
Principles of diet in GSD III, VI and IX
 Many small meals
 Max 30g of carbohydrates per main meal
 Max 5g of simple sugar per meal/snack
 Slowly absorbable carbohydrates
 Blood sugar 4.1 – 5.5mmol/L
 High protein food (3-4g/kg
 Bedtime snack
(low glycemic index)
(pre- and postprandial)
body weight/day, resp. 2-3g/kg)
 Uncooked corn starch UCCS
 Supplements (vitamins, calcium, iron)
Many small meals
• Why?
– Stable blood sugar (before AND after meal)
(4.1 – 5.5 mmol/L or 75-100mg/dL)
– How can this be achieved?
continuous supply of exogenous glucose
(polymers like Polycose® and cornstarch)
and / or FREQUENT MEALS
0-6 years: every 2-3hours, >6 years: every 3-4 hours
Max 30g of carbs for mean meals
• Why?
– Too high amounts of carbohydrates lead to
overweight!
• What happens with surplus carbs?
– Will be stored as glycogen and fat
15grams carbohydrates per serving
Max 5g of carbs of simple sugars
• Why?
– Blood sugar goes quickly up!
• What happens if more simple sugars are
consumed at a time?
– High insulin production, often higher than
appropriate for amount of sugar; thus reactive
hypoglycemia 1-2 hours after meal
• Risk for hyperinsulinism / type 2 diabetes
– Body becomes “deaf” to insulin!!!
Glycemic index 1
BS (mmol/L)
Insulin
5
► HUNGER!!!!
Food 1
Ketones
Time
Glycemic index 2
BS (mmol/L)
Insulin
Insulin
5
Food 1
Food 2
Ketones
Time
Glycemic index GI
• Tells you how fast blood sugar is going up
• High GI: not good!
Blood sugar is risking quickly, lots of insulin are produced.
Thus sugar is driven into storages (fat and glycogen) and
blood sugar levels falls. Counter regulation starts:
breakdown of protein, mobilization of fat, ketone body
production. Hunger! (see slide “Glycemic index 1”)
• Low GI (<50): ok
Blood sugar gets very slowly up, little or no insulin is
produced. Blood sugar level remains stable for a long
period. (see slide “Glycemic index 2”)
Simple sugars: how can you check?
Simple sugars: how can you check?
Don‘t adapt
carbs and
sugars for
YOUR amount
of food!
Other measure to slow down
absorption of food =
to get a low glycemic index:
• Fat and protein if consumed together with
carbs will impede absorption of
carbohydrates
• Thus: milk product (which contain
carbohydrates, fat and protein) lead only
to a slow increase of blood sugar
Why high-protein food?
• Protein can be transformed into glucose
and excreted into blood by the liver, thus
serving as „energy rescue line“ for the
brain.
• To prevent usage of muscle protein for this
pathway, add extra protein to your diet.
GLYCOGEN
GLYCOGEN
X Type VI and IX (liver)
Type IIIB (liver)
X Type IIIA (liver and muscle)
Gluc-6-Phos
Gluc-6-Phosphate
Glucose
Uric acid
Fructose / Galactose
Lactate
Protein
(Muscle)
Pyruvat
Fat
Acetyl-CoA
Ketone
Adapted from: Fernandes J. & Pikaar NA. Arch Dis Child. 1972 and proposals by David Weinstein and Terry Derks
Prevention of extensive breakdown
of muscle (-protein)
• When is muscle broken down?
Low blood sugar / hypoglycemia
• What is protein used for in low blood sugar?
For gluconeogenesis
(production of sugar from protein)
• How can you avoid breakdown of muscle?
Extra carbs (e.g. during exercise) / eat extra protein
High-protein food: more effects
• Protein can enhance muscle protein synthesis
• Protein can replace some of the carbohydrates
thus reducing risk for extra storage of glycogen
High-protein food: what?
• Question:
Which kind of protein do you use / recommend?
• Answer:
Milk protein (low fat milk) and lean meat, chicken,
turkey; fish (especially dried fish) is a perfect source of
protein! (but not tuna fish, too much mercury, therefore
max once per week).
Low fat curds (Quark, skyr) and low fat cheese, cottage
cheese, and so on.
Soy beans and soy “meat”! Tofu. Pulse have a high
protein content.
Nuts, peanuts: in 50g are 13g of protein, but be careful:
also 25g of fat!
High-protein food: how much?
• Type IIIA:
3-4g per kilo body weight per day,
about 1/3 to 1/4 during night time
• Type IIIB, VI and IX:
2-3g per kilo body weight per day
Late-night/bedtime snack
• Slowly released carbohydrates and protein
• Examples:
– bread and dried fish/dried meat
– yoghurt and flakes
– Uncooked cornstarch and protein powder
Uncooked cornstarch = UCCS
Who?
How much?
How often?
UCCS: who?
– In older children (>6 years) not always UCCS is
necessary during daytime. Regular meals
containing slow releasing carbohydrates (low
glycemic index!!) might be enough (interval
between meals not longer than 3 to 4 hours).
– In this age group (>6years) it might be enough to
use UCCS before bedtime.
– But careful: during growth spurt/puberty many
children/youngsters NEED cornstarch e.g. after
breakfast and at 2pm for optimal metabolic control
(plus at bedtime)
– Type VI and type IX, too, do best with two doses of
UCCS during daytime and one at bedtime
UCCS – how much?
• Differs between individuals
• Check with your doctor and dietician!!!
• Usually start with about 1.0gram per kilo body
weight per dose
• Tolerated from the age of 6 months on, but has to be
introduced very slowly (1gram per dose)
• If blood sugar falls 1-2 hours after UCCS:
cave hyperinsulinism!!
• Measure: REDUCE cornstarch dose/carbohydrates
UCCS/cornstarch during daytime?
• If you use a lot of cornstarch during the
day, one can expect troubles from the
gastro-intestine: for example flatulence.
• Therefore it is preferable to eat „normal“
during daytime (every 3 to 4 hours with
slow releasing carbohydrates).
• Control metabolic situation!!! (blood sugar,
ketone bodies)
UCCS/Glycosade at bedtime
• (New) guidelines:
ADD protein (0.5 to 1g/kg body
weight) to cornstarch at bedtime.
• Thus, for 13 year-old: at night time: about
1g/kg carbohydrate and protein 0.5-1g/kg.
• In adults, max. 60g of UCCS
Late-night snack / night feeding
• Slow releasing carbohydrates and protein
• In some cases and in young children
continuous gastric drip feeding
• Drip feeding contains glucose (polymers)
based on basal glucose requirement
protein.
Pump: who?
• Young children
• Those with need for many meals during
night time
• Those with short fasting tolerance
• How? PEG (pericutane endoscopic gastrostoma)
• Risks of continuous night feeding?
Overdose of carbohydrates?
• Increased amount of glycogen in
–liver
–muscle
–heart (!!!) and
–nerves
• obesity
• type 2 diabetes mellitus / insulin resistance
Vitamin supplements
• Vitamin D
• Other vitamins / supplements only if
shown to be low
Principles of diet in GSD III, VI and IX
 Many small meals
 Max 30g of carbohydrates per main meal
 Max 5g of simple sugar per meal/snack
 Slowly absorbable carbohydrates
 Blood sugar 4.1 – 5.5mmol/L
 High protein food (3-4g/kg
 Bedtime snack
(low glycemic index)
(pre- and postprandial)
body weight/day, resp. 2-3g/kg)
 Uncooked corn starch UCCS
 Supplements (vitamins, calcium, iron)