Nutritional Aspects in ATTR-Amyloidosis

Anna Hüsing-Kabar

Medizinische Klinik B für Gastroenterologie und Hepatologie

Universitätsklinikum Münster

2ND EUROPEAN ATTR AMYLOIDOSIS MEETING FOR PATIENTS AND DOCTORS 02.09.2019

Is there a special diet for ATTR-Amyloidosis?

• There is no special diet for ATTR-

Amyloidosis

• Taking care of nutritional aspects

helps to reduce gastrointestinal

symptoms and is able to improve

quality of life

• A balanced diet can help boost your

mood, give you more energy and

improve your overall feeling

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Symptoms of hATTR-Amyloidosis

• Polyneuropathy

• Carpal tunnel syndrome

• Impairment of visus

• Cardiac symptoms such as dysrhythmia and myocardial

• ……

• Gastrointestinal symptoms caused by

autonomic neuropathy (early satiety,

nausea, vomiting, dysmotility of the

bowel, constipation, diarrhea, abdominal

distension, etc.)

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Bloating and abdominal distension I

Often caused by excessed intestinal gas, which is produced by microbiota from carbohydrates and fibres

General recommendations to reduce intestinal gas:

•Do not drink liquids at mealtime, if you do, drink slowly and in small quantities (water or water/juice

mixes without gas)

•Avoid carbonated drinks (e.g. soft drinks)

•Avoid stress and during meal times without swallowing air

•Avoid chewing gum

•Eat slowly and chew your food well

•Switch to several small meals throughout the day instead of three large ones

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Bloating and abdominal distension II

Avoid nutrients causing gases:

• Pulses/Legumes (beans, lentils, peas)

• Vegetables like olives, peppers,

asparagus, raw vegetables

• Naturally high-fat meats like goose or

duck and higher-fat animal parts in

general (e.g. skin)

• Fried or battered food

• Carbonated beverages

• Unripe fruits and dried fruits (soft prunes

etc.)

• Cabbage (red cabbage, broussels sprout)

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• Leak, allium, onions, garlic, wild garlic

• Mushrooms

• Spicy meals

• Products rich in sugar (sorbitol,

fructose)

• Yeast based pastry, warm bread,

grain products

• Dairy products

Constipation I

Avoiding Constipation:

• Daily recommended intake of water is 1,5 -2 liters per day,

depending on your weight (30 ml/kg/KG) and renal/cardiac function

Liquids may include water, juices or water/juice mixes, soup,

tea, and other beverages, no alcohol

Black tea steeped for 3 minutes stimulates the digestive

system

• Try to do physical exercises daily

• Try buttermilk, yoghurt and quark

• Ask your doctor or pharmacist for side effects of your medication:

some analgesics cause constipation (opiates)

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Constipation II

• Avoid low fiber diet, if possible (cave: abdominal distension), increase dietary fibers slowly

Eat vegetables every day (some raw: carrot, pepper, tomato ...)

Eat raw fruit if possible with skin (pear, apple, peach, berrys ...)

Prefer wholemeal bread, pastry and cookies, brown rice

Eat dried and dehydrated fruits (e.g. plums, apricots)

• In case you do not tolerate high fiber nutrients: try dietary supplements containing e.g. flagseed,

psyllium or wheat bran

• If you develop gases by using wheat bran, it may be caused by insoluble dietary fibers; then prefer

psyllium husk or just reduce the amount

• In case that changes of diet, adding fibers, drinking sufficient and doing excercises are not beneficial,

ask you doctor for help, in some situations laxatives may be indicated

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Diarrhea I

In case of diarrhea ask your doctor for differential diagnosis: is it due to a gastrointestinal infection (stool

tests, blood test, further diagnostics) or is intestinal hyperactivity and motility caused by ATTR?

Recommendations in case of diarrhea:

• Drink liquids in small amounts during the whole day (fluid balance)

• Avoid alcoholic beverages: sparkling wine, whiskey etc.

• Prefer still water, black tea (long time brewing of > 10 min to release tannic acids), camomile tea, peppermint tea

• Grated apple or banana puree (pectines bind liquid and thicken digestive tract content), bananas contain potassium

(compensates electrolyte loss)

• Prefer white bred or rusk (easily digestible carbohydrates)

• Try slightly salted crushed rice (the mucilagenous substances bind water) for example with steamed vegetables

(carrots, zucchini)

• Potatos are also rich in potassium and often well-tolerated

• In case of steatorrhea talk to your doctor

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> 10 min.

Diarrhea II

Avoid intake of:

• Milk and dairy products for some days

• Raw fruits and for one week vegetables, vegetables and raw vegetables: tomato, cucumber, cabbage

• Spices like pepper, curry, peppers

• Fried potatoes, fried meat and fish

• Almonds, walnuts, hazelnuts, pistachios, peanuts, nuts

• Wholegrain foods

• Sugar-added drinks, such as soft drinks and sparkling water

• Fermented beverages such as wine and beer and alcoholic drinks (wine etc.)

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Nutrition counselling/ Nutritional therapy

• In case of gastrointestinal symptoms cuased by ATTR, nutrition

counselling might be helpful

• Ask for specialists in nutrition

• Learn how to deal with hATTR Amyloidosis associated

gastrointestinal symptoms (individual approach)

• Especially in case of unwanted weight loss, altered fluid balance and

long lasting obstipation, diarrea etc. caused by hATTR Amyloidosis

nutritional therapy might me indicated

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Aims of Nutrition counselling and nutritional therapy

• Analysis of inidividuals digestibility of nutrients

• Ensure sufficient supply of (micro)nutrients

• Support of body function/ organ function

• Improve immunological status

• Improve nutritional status

• Avoid weight loss

• Ensure muscle strength

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Nutritional therapy

• Preparation of a food diary

• Documentation of physical activities

• Find out which groceries/food is digestible for you

• Analysis of food diary and professional suggestions (clinical

nutritionists)

• Monitoring of nutritional status initial and in follow up visits

• Counselling includes partner, depending on who prepares the meals,

who goes food shopping etc.

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Monitoring of nutritional status

• Laboratory measurements:

Electrolytes, liver enzymes, Vitamins, trace elements and

micronutrient serum levels (Vitamin A, B1, B2, B6,12, D, zinc,

selenium, folic acid etc.)

• Bioelectrical impedance analysis

Phase angle, BCM, ECM/BCM

• Medical history+ physical examination

Edema, skin colour, rhagades, skin ulcer, etc.

BIA (Bioelectrical impedance analysis)

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Possible interventions in case of

malnutrition…..

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Powders, sip-feed products and creams

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• Enriching your meals

with powders

• Add sip-feed if

necessary

• Cooking with sip-feed

or creams (soups

e.g.)

• Try savory variants

instead of sweet ones

if you prefer hearty

meals

Samples of Sip-feed, powders and creams available at our center

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Parenteral Nutrition

• In case oral supplements and addititional sip-feed do not reach

sufficient nutritional status, parenteral nutrition might be initiated (e.g.

in advanced disease stages); Cave: fluid balance depending on

renal/cardiac function.

Vitamins and trace

elements will be added

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Parenteral Nutrition (J.E., 06.10.2011, 33kg/1,62m)

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Parenteral Nutrition (J.E., 30.04.2012, 43,2kg/1,62m)

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Nutritional Management

Pharmacy

Family doctor

Nutritional medicine

Homecare-

services

Nursing

service

Medical expert centers

Summary

Nutrition should be adapted to individual gastrointestinal symptoms

Contact a clinical nutritionist/ dietician for individual consultation

Monitoring of nutritional status (BIA, symptoms, lab)

Supplements of vitamins and trace elements if necessary

Considering of supplementary food in case of gastrointestinal symptoms

followed by weight loss

Discussion of parenteral nutrition in advanced ATTR disease stages with your

doctor/nutritionist in case of progressive malnutrition

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For further details…

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Available at the desk of the

FAP e.V. on level 1

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Contact

Dr. Anna Hüsing-Kabar

Universitätsklinikum Münster

Med. Klinik B für Gastroenterologie und Hepatologie

Diabetologie/Ernährungsmedizin

Tel.: 0251-83- 47661

Survey of patients with ATTR amyloidosis –

Understanding of ATTR patients needs

and challenges

Your experiences matter.

Please take the survey available at the

desk of the FAP e.V. on level 1!