Download What Every Dietitian Needs to Know about Genetic Sucrase

4/8/2017
Idaho Academy of Nutrition & Dietetics Annual Meeting – Boise, Idaho
What Every Dietitian Needs to
Know about Genetic SucraseIsomaltase Deficiency (GSID)
Disclosures
 Employed by QOL Medical, LLC
— QOL Medical is the manufacturer of a treatment to relieve the
Anne Boney, MEd, RD, LDN
Clinical Science Liaison QOL Medical, LLC
symptoms of Genetic Sucrase-Isomaltase Deficiency (GSID)
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Genetic Sucrase-Isomaltase Deficiency (GSID)
Outline
Introduction (Don’t Worry-This Is Not A Test)
 How many have heard of Genetic Sucrase-Isomaltase
Deficiency (GSID)?
#1 Quick carbohydrate overview – as it relates to
Genetic Sucrase-Isomaltase Deficiency (GSID)
#2 Why should I test for GSID? It’s so rare.
Prevalence
#3 Wh
Who should
h ld b
be screened
d ffor GSID?
Identifying the Patient
#4 What kind of diagnostic testing is available?
Diagnostic Tools
#5 How do I treat GSID?
Treatment Options
 How many are familiar with foods high in sucrose?
 What about foods that are high in starch?
 GSID is not something we learned about in school.
 GSID is not something most physicians are familiar with.
 But, there are patients suffering with GSID.
 And, I believe dietitians are in the perfect place to help identify
these patients, recommend further diagnostic testing, and
provide nutrition education to those with GSID.
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Genetic Sucrase-Isomaltase Deficiency (GSID)
Activities
Classification of Carbohydrates
 GSID comprises two activities1:
— SUCRASE



Sucrose = fructose + glucose
Hydrolyzes α-1,2- and α-1,4- glucosidic bonds
Digests
g
sucrose, maltose, and maltotriose
— ISOMALTASE



Maltose = glucose + glucose
Hydrolyzes α-1,6 linkages
Digests isomaltose, maltose, maltotriose, and limit dextrins
 GSID and maltase-glucoamylase (MGAM) are intricately
involved in sucrose and starch digestion2,3
5
5
1. Nichols BL, Adams B, Roach CM, Ma CX, Baker SS. Frequency of Sucrase Deficiency in Mucosal Biopsies. J Pediatr Gastroenterol Nutr. 2012b;55:S28-S30.
2. Lin AH, et al. Direct Starch Digestion by Sucrase-Isomaltase and Maltase-Glucoamylase. J Pediatr Gastroenterol Nutr. 2012;55:S43-S45.
3. Lee B, et al. Inhibition of Maltase-Glucoamylase Activity to Hydrolyze α-1,4 Linkages by the Presence of Undigested Sucrose. J Pediatr Gastroenterol Nutr.
2012;55: S45-S47.
Sucrose Digestion and Possible Clinical
Implications
Carbohydrate Digestion1
Starch
Glycogen
α-Dextrins
Glucoamylase
(Maltase)
Isomaltase
(Palatinase)
Maltotriose
Maltose
α-1,6
α-1,4
5
60
25
25
20
25
25
20
50
50
Sucrase
Membrane
digestion
TIME
Sucrose
Amylase
Luminal
digestion
95
Lactose
Sucrose
Pulls in excess
water
Numbers in circles
indicate percentage of
substrate hydrolyzed by
brush border enzyme
100
Glucose
Glucose
Glucose
Glucose
Glucose
Galactose
Glucose
Fructose
LOW ENERGY
LOW BLOOD
SUGAR
DIARRHEA
Glucose
1. Wahbeh GT, Christie DL. Basic aspects of digestion and absorption. In: Wyllie R, Hyams J, Kay M, eds. Pediatric Gastrointestinal and Liver Disease.
4th ed. Philadelphia, PA: Elsevier; 2011:11.
7
SUCRASE
Fermentation
100
100
Lactase
Product
H2O
COLON
Trehalase
Glucose + Fructose
H2O
Trehalose
8
Please see Sucraid® (sacrosidase) Oral Solution Important Safety Information on slides 37-39 and
Full Prescribing Information passed out at the beginning of this presentation. Sucraid® may cause a
serious allergic reaction.
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4/8/2017
Starch Digestion and Possible Clinical
Implications
#1 Why Should I Test for GSID? It’s So Rare
TIME
Starch
dextrins
Maltose
Glucose + Glucose
amylase – alpha glucoamylase – amylase -- isomaltase
Prevalence
MALTASE
COLON
+/- LOW ENERGY
Fermentation
Pulls in excess water
+/- LOW BLOOD SUGAR
GAS, DISTENTION
BLOATING, +/- DIARRHEA
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Let’s Talk Briefly about Prevalence…
Genetic Sucrase-Isomaltase Deficiency (GSID)
 Historical data (1960 – 1970) for Genetic Sucrase-Isomaltase
Deficiency (a genetically inherited autosomal recessive
disorder)1
 GSID originally thought to cause symptoms in autosomal
recessive (homozygous) inheritance only
 Recent studies have demonstrated carriers may also
present with symptoms1
— 5 – 10% in Greenland Eskimos
— 3 – 7% in Canadian native peoples
— 3% in Alaskans of native ancestry
— <0.2% in non-Hispanic whites
 But, look at what we have learned since then…
1.Nichols BL, Adams B, Roach CM, Ma C, Baker S. Frequency of sucrase deficiency in mucosal biopsies. J Pediatr Gastroenterol Nutr. 2012;55(Suppl 2):S28-S30.
1.Treem, WR. Clinical aspects and treatment of congenital sucrase-isomaltase deficiency. J Pediatr Gastroenterol Nutr. 2012;55(Suppl 2):S7-S13.
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GSID Genetic Prevalence Study
Genetic Results by Primary Symptom
Potential Etiologies of Sucrase Deficiency
Genetic Sucrase-Isomaltase
Deficiency (GSID) Genotypes
Congenital SucraseIsomaltase Deficiency
(CSID)
(Compound Heterozygotes)
GSID
Symptomatic Carriers
(Simple Heterozygotes)
Secondary Sucrase
Deficiency
Abdominal
Pain
Total
n= 375
n= 375
N= 750
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23
Celiac Disease
Primary Variants
(H t
(Heterozygotes)
t )
0
2
2
Bacterial
Overgrowth
Secondary
Variants
(Heterozygotes)
Compound
Heterozygotes
1
0
1
Crohn’s
Disease
Other
(e.g., blunted villi)
13
Diarrhea
Please see Sucraid® (sacrosidase) Oral Solution Important Safety Information on slides 37-39 and
Full Prescribing Information passed out at the beginning of this presentation. Sucraid® may cause a
serious allergic reaction.
Total
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10
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Rate
4.3%
2.7%
3.5%
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Frequency of Sucrase Deficiency in Mucosal
Biopsies Review of Disaccharidase Analyses1
N=27,875
Prospective Disaccharidase Testing in Children
with Recurrent Abdominal Pain1
Pilot Study N=28
9.3%
[VALUE]
Total Sucrase Deficiency (1.2 SD< Norm)
# of subjects
% of total
Low Lactase
15
53.6
L
Low
S
Sucrase
4
14 3
14.3
Low Maltase
5
17.9
Low Glucoamylase
4
14.3
Pan-Disccharidase
Deficiency (all 4 low)
3
10.7
All Other Results
1.Nichols BL, Adams B, Roach CM, Ma C, Baker S. Frequency of Sucrase Deficiency in Mucosal Biopsies. J Pediatr Gastroenterol Nutr. 2012; 55 (Suppl 2):S28-S30.
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1. Chumpitazi BP, et al. Yield of Prospective Disaccharidase Testing in Children with Recurrent Abdominal Pain. Gastroenterology. 2013;144(5 Suppl
1):S401-402.
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Retrospective Study of 963 Symptomatic Children
Undergoing Upper Endoscopy and Disaccharidase
Testing1
#2 Who Should Be Screened for GSID?
Identifying
y g the Patient
1. Cohen SA. The clinical consequences of sucrase-isomaltase deficiency. Molecular and Cellular Pediatrics. 2016;3;5.
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Checklist for Considering GSID
 Reported Symptoms:
 Possible Diagnosis:
- Chronic diarrhea
- Viral/bacterial gastroenteritis
- Abdominal pain
- Parasitic diarrhea
- Abdominal distension
- Lactose intolerance
- Excess gas
- Celiac Disease
- Excoriated buttocks
- Inflammatory Bowel Disease
- Vomiting
- Crohn’s Disease
- Failure to thrive
- Ulcerative Colitis
- Weight loss
- Cystic Fibrosis
- Constipation
- Irritable Bowel Syndrome
- Acid reflux
- Genetic Sucrase-Isomaltase
- Burping
Initial Presentation of GSID in Infancy1
 May not present until ~ 6 months - 1 year
— Breast milk and standard infant formula (CHO source is lactose)
are tolerated
— First illness; antibiotic (high in sucrose)
— Introduction of sucrose and/or starch
— Formula, cereals, fruits, vegetables, and/or juice
 Often misdiagnosed as allergy or intolerance
to cow’s milk or soy protein
— Changing baby formula may actually worsen
symptoms and delay diagnosis
Deficiency
1. Treem WR. Congenital sucrase-isomaltase deficiency. J Pediatr Gastroenterol Nutr. 1995;21:1-14.
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Think about the Toddler with Chronic Diarrhea…
Think about the Toddler with Chronic Diarrhea…
 Started with diarrhea around 6 months of age
 Reports no sweets and no nuggets or fries from McDonald’s
 Hospitalized with vomiting and diarrhea
 Typical intake at 3 y/o
B: ham, egg
Sn: potato chips or corn chips
L: eats meat off sandwich
D: mac and cheese
Drinks: Peptamen Junior®
 Susceptible to infection, URIs
 Allergy testing all negative
 Tried Alimentum®, EleCare®, Neocate® Splash,
Splash Peptamen Junior®
 Endoscopy with small bowel biopsies for disaccharidase assay
— Low-sucrase
— Low-glucoamylase (maltase)
— Normal lactase
— Normal mucosa
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Think about Your Older Patients Diagnosed with
IBS – Could It Be GSID?
#3 What Kind of Diagnostic Testing Is Available?
 You’ve tried medications and various diets
 Patient finds some relief with Low FODMAPs* diet
— But still has lingering symptoms: diarrhea, abdominal pain, gas, and
bloating
Diagnostic
g
Tools
— And you believe they are compliant with the diet
 Consider testing for GSID (see #4 for diagnostic options)
Sucraid® (sacrosidase)
 Consider a trial of
Oral Solution therapy
and diet modification (see #5 for treatment options)
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
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“Gold Standard” Diagnostic Test:
Disaccharidase Activity Assay
#3 Diagnostic Options for GSID
 Diagnostic Tests:
— Upper Endoscopy – Small Bowel Biopsy - Dissacharidase Assay
“Gold Standard”
Normal Range
— Trial Prescription of Sucraid® (sacrosidase) Oral Solution
(µmol glucose/min/g protein)
Prescription/order forms on Sucraid.net website
 Tests That Aid in Diagnosis
— Sucrose Hydrogen Methane Breath Test (SHMBT)
QOL Medical offers this test free of charge through
Commonwealth Labs
— Genetic Testing (buccal swab or saliva)
Genetic Test Kits can be ordered free of charge from
Sucraid.net
Deficient
(µmol glucose/min/g protein)
Sucrase
29 0 – 79.8
29.0
79 8
<25
Maltase
98.0 – 223.6
<100
Palatinase
4.6 – 17.6
<5
Lactase
16.5 – 32.5
<15
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
25
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Pan-Disaccharidase Deficiency
Sucraid® (sacrosidase) Oral Solution Therapy
 Do not discredit a Disaccharidase Assay where all 4 enzymes
are deficient (pan-disaccharidase deficiency)
 If other more common GI disorders have been
ruled out, it may be warranted to prescribe
Sucraid® as a one to two week trial to see if the
patient responds.
 In general, as people age, they are more likely to have a
lactase deficiency:
— Congenital lactase deficiency (in infants) is rare
 Order/prescription forms are available at
www.sucraid.net
— Approximately 65 percent of the human population has a reduced
ability to digest lactose after infancy1
— It makes sense that an older child and certainly an adult could
have both a lactase deficiency and Genetic Sucrase-Isomaltase
Deficiency
— QOL Observational Study of 49 Sucraid® patients; 25% also had a
lactose intolerance
 Please see Sucraid® Important Safety
Information and details on accessing the Full
Prescribing Information in slides 37-39
 Treat for both GSID and lactose intolerance
1. NIH – US National Library of Medicine Website. https://ghr.nlm.nih.gov/condition/lactose-intolerance#sourcesforpage. Accessed March 20, 2017
27
Please see Sucraid® (sacrosidase) Oral Solution Important Safety Information on slides 37-39 and Full
Prescribing Information passed out at the beginning of this presentation. Sucraid® may cause a
serious allergic reaction.
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
28
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4/8/2017
Sucrose Hydrogen Methane Breath Test (SHMBT)
Sucrose Hydrogen Methane Breath Test (SHMBT)
 A noninvasive, functional test to aid in identifying and
diagnosing patients suffering with Genetic Sucrase-Isomaltase
Deficiency (GSID)
 Prior to the SHMBT, patients are instructed to discontinue
certain medications (probiotics, antibiotics) and follow a lowcarbohydrate diet one day before the test
 After a 12-hour overnight fast, the patient starts with a baseline
breath sample
sample, drinks a sucrose solution
solution, and completes a
series of breath samples (test is ~3 hours in all)
 SHMBT kits are available to healthcare professionals, including
dietitians, for patient distribution at no charge (through QOL
Medical, LLC)
— Patients with GSID may experience GI symptoms due to the
consumption of the sucrose solution and may prefer to take the
test at home
— Both hydrogen and methane gas production are measured
 Mal-digested sucrose results in an increase of these gases, due to
bacterial fermentation
 Test results will be sent to the physician’s office within 48 hours
of receiving the completed test kit
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#4 How Do I Treat GSID?
Genetic Testing
 Genetic Testing – no charge for the cost of
the kit or testing
 Order genetic test kit from www.sucraid.net
Treatment Options
p
 Buccal
B ccal sswab
ab
 Test kit sent out to LabCorp
 Detects 37 known mutations
 Can help rule GSID in, but may not
necessarily rule it out
31
Please see Sucraid® (sacrosidase) Oral Solution Important Safety Information on slides 37-39 and
Full Prescribing Information passed out at the beginning of this presentation. Sucraid® may cause a
serious allergic reaction.
32
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4/8/2017
Patient Identified!
What Are the Treatment Options?
Infant Diet Guidelines for Patients Diagnosed
with GSID
 Diet alone (low-sucrose and low-starch)
 Sucraid® (sacrosidase) Oral Solution - enzyme replacement
therapy for the treatment of the genetically determined
sucrase deficiency which is part of congenital sucraseisomaltase deficiency (CSID)
Available on:
CSIDcares org
CSIDcares.org
— Although Sucraid® provides replacement therapy for the deficient
Sucraidassist.com
sucrase, it does not provide specific replacement therapy for the
deficient isomaltase
Sucraid.net
 Combined diet and Sucraid® therapy
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
33
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
34
Infant Diet Guidelines for Patients Diagnosed
with GSID
Infant Diet Guidelines for Patients Diagnosed
with GSID
 Formula
 Starting Solids
— Breast milk
— Start solids as soon as age appropriate; don’t delay
— Standard infant formula (lactose-based)
— Abbott Nutrition RCF® no added carbohydrate
— Start Sucraid® (sacrosidase) Oral Solution therapy when
soy-based
soy
based formula



starting solids
Add dextrose or fructose
Add water
Provide mixing instructions

Sucraid® has been tested in clinical trials with children 5
months of age and older
— Start with low-sucrose, low-starch, pureed vegetables, fruits,
and then meats; do not start with cereals
35
Please see Sucraid® (sacrosidase) Oral Solution Important Safety Information on slides 37-39 and
Full Prescribing Information passed out at the beginning of this presentation. Sucraid® may cause a
serious allergic reaction.
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
36
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4/8/2017
Sucraid® (sacrosidase) Oral Solution
Indication/Safety Information
Sucraid® (sacrosidase) Oral Solution
Indication/Safety Information
INDICATION:
IMPORTANT SAFETY INFORMATION (cont’d.):
 Sucraid® (sacrosidase) Oral Solution is an enzyme replacement
 Sucraid® may cause a serious allergic reaction. Patients should stop
taking Sucraid® and get emergency help immediately if any of the
following side effects occur: difficulty breathing, wheezing, or swelling
of the face. Care should be taken when administering initial doses of
Sucraid® to observe any signs of acute hypersensitivity reaction.
reaction
therapy for the treatment of the genetically determined sucrase
deficiency, which is part of congenital sucrase-isomaltase deficiency
(CSID).
IMPORTANT SAFETY INFORMATION:
 Although Sucraid® provides replacement therapy for the deficient
 Adverse reactions as a result of taking Sucraid® may include worse
 Do not use Sucraid® (sacrosidase) Oral Solution with patients known to
 Before prescribing Sucraid® to diabetic patients, the physician should
abdominal pain, vomiting, nausea, diarrhea, constipation, difficulty
sleeping, headache, nervousness, and dehydration.
sucrase, it does not provide specific replacement therapy for the
deficient isomaltase.
consider that Sucraid® will enable sucrose hydrolysis and the
absorption of those hydrolysis products, glucose and fructose.
be hypersensitive to yeast, yeast products, papain, or glycerin
(glycerol).
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Sucraid® (sacrosidase) Oral Solution
Dosing and Administration
Sucraid® (sacrosidase) Oral Solution
Indication/Safety Information
IMPORTANT SAFETY INFORMATION (cont’d.):
 Sucraid® must be kept refrigerated
 The effects of Sucraid® have not been evaluated in patients with
 Dosing:
secondary (acquired) disaccharidase deficiency.
— 1 mL (28 drops) if ≤ 15 kg (≤ 33 lbs)
— 2 mL (56 drops) if > 15 kg (> 33 lbs)
 DO NOT HEAT SOLUTIONS CONTAINING SUCRAID®. Do not put Sucraid®
Sucraid®
in warm or hot fluids. Do not reconstitute or consume
with fruit
juice since the acidity of the juice may reduce the enzyme activity of
Sucraid®. Half of the reconstituted Sucraid® should be taken at the
beginning of the meal or snack and the other half during the meal or
snack.
 Mixing:
— Mix Sucraid® in 2 – 4 ounces of milk, water, or sucrose-free, starch-
free formula
 Do not mix in hot beverages; do not heat after mixing
 Administer Sucraid® with every meal or snack
 Sucraid® should be refrigerated at 36°F-46°F (2°C-8°C) and should be
— Take ½ at beginning of meal/snack and other ½ during meal/snack
protected from heat and light.
 FULL PRESCRIBING INFORMATION was provided at the beginning of this
presentation and can be accessed online at sucraid.net/pi.pdf.
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
39
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4/8/2017
Treatment Plan for Previously Discussed Toddler
(GSID Confirmed by Biopsy)
Formula Options for Our Toddler…
 Start Sucraid® (sacrosidase) Oral Solution therapy
FORMULA
 Modified diet/elimination (sucrose and starch)
 Consider changing formula to RCF® (Abbott) or KetoCal®
(Nutricia)
PROTEIN SOURCE
CARB SOURCE
FAT SOURCE
Portagen®
(Mead Johnson)
Sodium
caseinate
Corn syrup
solids, sugar
MCT, corn oil
% of Kcals
RCF®
(Abbott)
14%
Soy protein
isolate
46%
None
40%
Safflower oil, soy
oil, coconut oil
% of Kcals
KetoCal® 4:1
(Nutricia)
% of Kcals
14%
Milk protein
(casein, whey)
8.2%
0
Corn syrup solids
86%
Vegetable oil
3.1%
88.7%
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
41
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A Comparison of Other Elemental Formulas
Often Trialed, But Failed…
FORMULA
CARB SOURCE
FAT SOURCE
casein
Similac
hydrolysate
Alimentum®
(Abbott)
EleCare® (Abbott) amino acids
sugar, modified
tapioca starch
safflower oil, MCT,
soy oil
corn syrup solids
amino acids
corn syrup solids
amino acids
maltodextrin,
sugar
safflower oil, MCT,
soy oil
vegetable oil,
canola oil,
safflower oil
coconut oil,
canola oil,
sunflower oil
MCT, soybean oil,
canola oil
Neocate
(Nutricia)
Jr®
Splash® (Nutricia)
Peptamen, Jr®
(Nestle)
PROTEIN SOURCE
hydrolyzed whey
protein
maltodextrin,
sugar, cornstarch
Child or Adult Diet Guidelines for Patients
Diagnosed with GSID
 Begin Sucraid® (sacrosidase) Oral Solution therapy
 Elimination Diet (2 weeks) – eliminate the culprits
— Sucrose
— Starch, maltose
— Others
 Symptom improvement is generally seen within these first
two weeks
 Induction Diet – establish tolerance to sucrose and starch
— Sucrose (Sucraid® may allow for a nearly “normal” intake of
sucrose)
— Starch tolerance will vary significantly (Sucraid® does not
replace isomaltase)
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
43
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4/8/2017
Child and Adult Diet Guides
Menu Guide
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
45
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
46
Why Do Patients with GSID Need to See a
Registered Dietitian?
Why Do Patients with SI Deficiency Need to See
a Registered Dietitian?
 Sucrose and starch modifications are difficult to understand,
making the diet hard to follow for newly diagnosed patients:
 Continued…
— Many patients may have other diagnoses and diet
restrictions to contend with:
— Food labels do not provide sucrose and starch content


— Most nutrient databases do not have complete sucrose and
starch data


— People do not think in terms of sucrose and starch; they are more
Lactose intolerance
Wh t or gluten
Wheat
l t
sensitivity,
iti it Celiac
C li
Di
Disease, or food
f d allergies
ll
i
IBS/low FODMAPs diet
Milk protein intolerance
— They may be given a list of what NOT to eat, but do not
likely to think in terms of sugars and carbohydrates, but even those
terms are not always familiar to the general population
know what they CAN eat!
 Improper diet modifications and restrictions increase risk of
nutrient deficiencies
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Nutrition Topics to Cover with Patients
 What is sucrose?
 What is starch?
 What are acceptable
alternatives to cow’s milk?
 How much sucrose/starch is
too much?
 How much sucrose/starch is
not enough?
 How to read a food label?
Grams of Sucrose per Meal/per Day?
 Compliance with a sucrose-free diet is very difficult
 What artificial sweeteners
are acceptable?
 Is gluten-free low in starch?
 Following an elimination diet, sucrose can be increased by
~0.5 g/day or more
 What about 100% fruit juice?
 Add back sucrose via fruits and healthier “sugar”
g
foods first
 What about FODMAPS, SCD?
 Sucraid® (sacrosidase) Oral Solution will cover a significant
amount of sucrose in the diet, but tolerance is variable
and depends on the individual
 How do I meet calorie
needs?
 Where do I buy fructose and
dextrose?
 What about lactose,
fructose?
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
49
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Grams of Starch per Meal/per Day?
Nutrient Data for Sucrose and Starch
 Although Sucraid® (sacrosidase) Oral Solution provides
replacement therapy for the deficient sucrase, it does not
provide specific replacement therapy
for the deficient isomaltase; starch tolerance is highly variable
 Sucrose and starch information are not found on food labels
 “Cracker
Cracker Test”;
Test ; adding 1 Saltine cracker/day (2 g starch per
day)
 University
y of Minnesota Nutrition Data System
y
for Research
(NDSR) has complete sucrose and starch data
 Ann McMeans, RD suggests 120 grams/day may be tolerated1
 Total carbohydrates, total sugars, and fiber are on Nutrition
Facts Label
 Sucrose and starch data are not a part of most nutrient
databases; USDA has limited information, but adding to it
 The type of starch may allow more grams per day
Total CHOs – total sugars – fiber = starch
 Chewing food well – exposing to amylase longer
 Type(s) of sugar and starch sources are listed on the ingredient
label, but patients must learn to be ingredient detectives
 Some take over-the-counter “starch enzymes”
1. McMeans AR. Congenital Sucrase-Isomaltase Deficiency: Diet Assessment and Education Guidelines. J Pediatr Gastroenterol Nutr. 2012;55(2):S3739.
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
51
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4/8/2017
QOL Observational Study1
QOL Observational Study1
 49 subjects on Sucraid® (sacrosidase) Oral Solution
% of Total CHOs
— >3 months on therapy
AVG for All Subjects
(%)
Typical American
Diet (%)
12 – 39
49
Maltose
1–2
N/A
Sucrose
11 – 14
N/A
— Age ranged 1 – 45 years
 24-hour dietary recalls; three consecutive days
% of Total Kcals
Starch
AVG for All Subjects (%)
DRI (%)
CHO
42
45-65
FAT
39
25-40
PRO
18
5-35
1. Boney A, Elser H, Silver HJ. Relationships Between Dietary Intakes and Persistent Gastrointestinal Symptoms in Patients on Enzyme Treatment for Genetic
Sucrase-Isomaltase Deficiency. In: World Congress of Pediatric Gastroenterology, Hepatology and Nutrition; October 5-8, 2016; Montreal, Canada.
1. Boney A, Elser H, Silver HJ. Relationships Between Dietary Intakes and Persistent Gastrointestinal Symptoms in Patients on Enzyme Treatment for Genetic
Sucrase-Isomaltase Deficiency. In: World Congress of Pediatric Gastroenterology, Hepatology and Nutrition; October 5-8, 2016; Montreal, Canada.
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
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QOL Observational Study1
Tolerance to Sweeteners
 Sweeteners “tolerated by most”
 Top Five Sucrose Sources:
— Dextrose and Fructose
— cake/pie, donut, ice cream, frozen desserts, espresso/chocolate
— High Fructose Corn Syrup
milk
— Aspartame (NutraSweet®)
 Top Five Starch Sources:
 Sweeteners “tolerated by
y some”
— pizza, pasta, potato, brown rice, bread
— Acesulfame K (Sweet One®)
— Agave nectar (primarily glucose and fructose)
— Equal® (aspartame based)
 Top Five Maltose Sources:
— Honey (primarily glucose and fructose)
— breakfast cereal, chips, pancake syrup, tortilla, CLIF Bar
— Saccharin (Sweet’N Low®)
— Increased maltose associated with increased GI symptoms
— Stevia®
— Sucralose (Splenda®)
— Sugar alcohols
1. Boney A, Elser H, Silver HJ. Relationships Between Dietary Intakes and Persistent Gastrointestinal Symptoms in Patients on Enzyme Treatment for Genetic
Sucrase-Isomaltase Deficiency. In: World Congress of Pediatric Gastroenterology, Hepatology and Nutrition; October 5-8, 2016; Montreal, Canada.
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Tolerance to Sweeteners
Starch Ingredients That May Not Be Tolerated
 Sweeteners to “Avoid”; Sucrose:
 Limit dextrins
— Beet sugar
 Maltodextrin
— Brown sugar
— Cane juice, cane sugar
 Modified tapioca starch
— Caramel
 Glucose polymers
— Coconut sugar
— Confectioner’s sugar
 Maltose
— Date sugar
— Maple syrup
— Brown rice syrup
— Molasses
— Corn syrup solids
— Raw sugar
— Sucrose
— Malt
— Sugar
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Fruit Juice
Differences Between Elimination Diets
Low FODMAPs
JUICE
(4 fluid ounces)
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SUCROSE
STARCH
GLYCEMIC
INDEX
FRUCTOSE
GLUCOSE
MALTOSE
LACTOSE
Dairy
No (some cheese)
Low Sucrose,
Low Starch
Yes
JJ APPLE
1.6
6
0
39
7.1
7
3.3
3
3
0
0
Fat
Yes
Yes
JJ GRAPE
0.8
0
50
8.2
5.9
0
0
Protein (meats)
Yes
Yes
LIBBY’S PEAR
0.3
0
64
10.7
7.3
0
0
Starch/grains
Yes (selected)
No
ORANGE JC, fresh
5.0
0
46
2.8
2.6
0
0
Fruits
Yes (low fructose)
Yes (low sucrose)
PINEAPPLE JC
1.9
0
46
4.8
5.8
0
0
Vegetables
Yes (selected)
Yes (low starch)
PRUNE JC
0.3
0
29
10.1
7.0
0
0
“Sugars”
APPLE-GRAPE DRINK
7.4
0
67
3.3
3.7
0
0
No fructose;
No sucrose;
Yes (sugar, syrup, jelly) Yes (fructose, dextrose)
TOMATO JUICE
0.3
0
33
1.9
1.6
0
0
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Case Study – RD Intervention
 4-year-old female presented to RD with IBS for education on low
FODMAPs diet
 Patient showed some improvement on diet, but still with GI symptoms
— FODMAPs does not restrict sucrose and starch
 RD recalled our conversation about SI deficiency and Sucraid®
(sacrosidase) Oral Solution therapy
 RD probed and thought perhaps this could be “one of those patients”
 RD recommended the MD order a trial of Sucraid®
 After 10 days on Sucraid®, BMs normalized and all other symptoms
improved
— This child’s symptoms began around 1 year of age
— She was 4 years old before GI symptoms resolved
— Genetic testing was negative
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
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Allowed or Not Allowed on Elimination Phase of
A Low-Sucrose, Low-Starch Diet
Apple
Olives
Cracker
QOL Medical, LLC Offers a Variety of Services to
Healthcare Providers and Their Patients
One Patient Services – SucraidASSIST™
 Nurse Case Manager
Banana
Tomato

Eggs


g cheese
Cottage
Nuts
Blueberries
Gluten-Free
 Insurance Specialist
 Dietitian
Di titi
 Peer Coach
Grapes
p
Butter
Green beans
Websites:
Black-eyed peas
 SucraidAssist.com
 CSIDcares.org
 Sucraid.net
Yogurt
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not tolerated by most
tolerated by most
Point of contact for all needs (patients and clinicians)
Prescriptions, forms, paperwork, tracking
All questions
1-800-705-1962
Please see Sucraid® Important Safety Information on slides 37-39 and Full Prescribing Information
passed out at the beginning of this presentation. Sucraid® may cause a serious allergic reaction.
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Thank You!
Questions?
Anne Boney, MEd, RD, LDN
1-800-705-1962, ext. 1001
1-919-832-0404
[email protected]
Thank you for the opportunity to share this presentation with you today!
SUC17.1019b 04/2017
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