EB Info World

This is the transcript of a little booklet distributed by DEBRA UK called
"Diet for Epidermolysis Bullosa".

Children with the more severe forms of EB have problems consuming an adequate food intake. This is caused by a combination of factors, namely the increased need for nutrient requirements and difficulties with eating and swallowing.
The purpose of this is to explain these factors, and to offer some practical solutions to the problems and difficulties. For those with no specific problems it will provide advice about normal, healthy eating.

A balanced diet provides all the food that is needed to keep the body fit and healthy and a positive attitude to food and mealtimes will help encourage your child to eat a balanced diet.

The various nutrients are used as 'fuel' to satisfy energy requirements or 'building blocks' to satisfy the body's needs for growth and repair. Energy requirements will vary depending upon the physical activity; a lot of activity requires a lot of energy. Even those who take no exercise need energy to maintain normal body functions, such as breathing and digestion.

The need for building materials also varies. Requirements are relatively high for growing children and following blistering and loss of body fluids, when 'repair work' is underway. Repair work also requires energy, so fuel too needs to increase in these situations.

Proteins
Proteins are essential constituents of all living cells and are, therefore necessary for the growth and repair of the human body.

Fats
Fats provide a large proportion of energy in the diet. 35 - 40 % of energy in normal diets is, in fact, provided by fats. They also form part of the essential structure of all living cells. Fats are only 'bad' foods if taken in excess of the body's energy needs.

Carbohydrates
Carbohydrates are the major energy source of the diet. 45 - 50 % of energy in normal diets is provided by carbohydrate. They also form a part of the structure of all living cells.

Vitamins
Vitamins are essential for health and the normal functioning of the body but are only required in very small amounts. They can be thought of as specialist 'building blocks'. Without vitamins many of the body's basic processes, such as burning fuel or building new tissue, will not work. Each vitamin has a different but very specific function, for example, vitamin C is important in wound healing, Concentration in foodstuffs vary a lot and some foodstuffs are more vitamin rich than others. Oranges, for example are well know to be rich in vitamin C.

Minerals
Like the vitamins, minerals can be thought of as specialist 'building blocks' essential for normal body processes. There are approximately twenty essential minerals. Each has a different function, for example, iron is an essential component of blood. Some minerals, such as zinc and copper are called trace elements because only very small amounts are required.

Fiber
Fiber or roughage is the part of food that passes through the body without being absorbed. Although it has no energy value, it is nonetheless very important and provides bulk to the diet, assists normal gut and bowel function, and helps prevent constipation.

Most foods are a mixture of all or some of these nutrients. For example, bread contains all of them, and meat contains protein, fat, vitamins, minerals and water. A few foods provide only one or two nutrients. Sugar, for example, is all carbohydrates, and oil is all fat. Nonetheless each of these foods contributes to our energy needs and is important in the total diet.

A balanced diet can be achieved with many different combinations of foods. There is no one food which is essential for health, or indeed, which provides all the essential nutrients, except breast milk for babies and infant formula feeds. The very best way of ensuring your child receives a balanced diet is to include al wide range of foods.

The following foods are good sources of:
Protein - Carbohydrates - fats - Vitamins - minerals and Fiber

A balanced diet would contain foods from each group every day:
Protein - Meat, poultry, fish, eggs, cheese, milk, beans, lentils
Fats - Butter, Margarine, oil, cream, fatty meats, oily fish, eggs, cheese.
Carbohydrates - Bread, pasta, breakfast cereals, cakes, biscuits, potatoes, sugar.
Vitamins and minerals - Meat, fish, eggs, milk products, fruit, vegetables, cereals.
Fiber - Wholemeal bread, wholemeal flour and pasta, wholegrain breakfast cereals, wholegrain biscuits, brown rice, vegetables, lentils, haricot beans, kidney beans, and other pulses, fresh and dried fruit.

Breakfast - Wholegrain cereal e.g., Weetabix, porridge oats, full fat milk. Wholemeal bread or toast with butter or margarine, peanut butter, honey jam or marmalade. Milk to drink.

Lunch - Meat or chicken or fish or lentils. Sauce or gravy. Potato or rice or macaroni. Vegetables. Or 'made up' dish e.g., shepherds pie, fish pie, macaroni cheese, casserole or lasagna. Fruit, ice cream custard, yogurt or milk pudding. Unsweetened fruit juice or water.

Dinner - Ham, egg, cheese, baked beans, liver sausage, sardines or peanut butter. Wholemeal bread or toast with butter or margarine. Custard or yogurt and/or fruit. Milk to drink

Fluoride drops (If the local water supply is not fluoridated) Available from chemists.

Children wit severe EB usually find eating painful and difficult. Problems arise because they have increased requirements for protein and energy due to the blistering and fluid losses, and yet they cannot eat enough.

Blistering Mouth and Gums - Difficulty Swallowing - Dental Disease
A small mouth opening and / or an immobile tongue

All contribute to making eating a painful process and result in a poor food intake. An inadequate intake leads to nutritional deficiency, which is made worse by increased requirements for protein caused by blood losses from open blisters.

Pain on passing motions also contributes to constipation, and this is aggravated by an inadequate dietary fiber intake.

A vicious cycle is created and the constipation makes the child feel apathetic (listless) and uncomfortable. The effect of this is to put the child off his food and this results in a reduced food intake which make the constipation worse.

The effects of nutritional deficiency are many, and include growth retardation in children, poor wound healing, anemia and increased susceptibility to infection. A further vicious cycle may be established from iron treatment which also aggravates the child's constipation resulting in further reduction in food intake.

Not all children with EB will experience all these problems. The most common nutrition problems are:

Dysphasia (Difficulty in/with swallowing)
This is a common complication of dystrophic EB of the recessive types, but is not restricted to them. The problem is caused by a number of factors. The scarring process in dystrophic EB leads to contraction of the mouth, fixation of the tongue and narrowing of the throat. In addition dental decay and blistering of the mouth and throat make it very difficult for children to chew and swallow. So eating becomes a very slow, tiring, painful process and anorexia (poor appetite) is very common.

Anemia
Anemia is a major problem in dystrophic and Junctional EB. It is caused by an inadequate dietary intake of iron together with a chronic loss of blood through skin, mouth, esophagus (throat) and anus. Severe anemia causes children to be tired and listless.

Dental Decay
Dental problems are common in children with severe dystrophic and Junctional EB. The teeth are prone to severe caries (decay) for two reasons: a high sugar intake and chronic mouth infection and gum disease together with blistering of the gums makes cleaning teeth painful. In dystrophic EB scarring and fixation of the tongue aggravates the situation by making normal cleaning impossible and allows residual food to stagnate around the tooth/gum margin.

Good nutrition is essential for everybody but for children with EB, it is crucial to minimize the complications of the disease. A well nourished child will have wounds which heal more rapidly, blisters which are unlikely to become infected, a good chance of achieving the best possible growth and reduced risk of anemia constipation and dental caries.

It may not be possible to eliminate all of these problems, particularly in the more severe forms of the disease, but we believe that good nutrition can help reduce these problems. Attention to the diet from an early age is essential. Much time is given to learning wound dressing routines and time is needed to learn about nutrition.

The main problems to be confronted are:
1. Poor appetite
2. High nutritional requirements.
3. Pain and difficulties with eating.

Nutritional intake can be improved in many ways. Some helpful tips will be given later. The complex nature of the disease means that often in solving one problem another is created. For example, some children with dysphasia require a smooth diet but straining the food to remove the lumps reduces the fiber content, thus increasing the likelihood of constipation. Likewise, although adding sugar to the diet increases the energy content, it also provokes dental caries.

The following are intended as guidelines for feeding children with EB (more specific ideas for meals will be given later):

Meals should be happy occasions for parents and child. Be firm but gentle, give praise and encouragement when food is eaten but never scold or force-feed when it is refused.
Your child is likely to have a small appetite. More food may be consumed by giving 3 small meals and 2-3 snacks a day rather than 3 large meals a day. Do not allow mealtimes to linger, it is boring and tiring for the child and frustration for you. Set a time limit on meals and snacks.
Give a limited number of distinct meals and snacks, and avoid continuos eating through the day. This will help your child to develop and appetite and also stimulate the gut, thus reducing the likelihood of constipation.
Tempt small appetites with attractive food. Cut sandwiches into small animal shapes, make jelly animals, give drinks from cartons with bendy straws. Use colorful crockery and only put a small amount of food on the plate. The child can always ask for more. Use colorful food for pureed diets, such as peas, baked beans and carrots and serve each item separately on the plate.
Use high energy foods, such as cheese, milk, cream and butter to increase the energy content of the diet
Blistered mouths can be very sensitive to high temperatures. Do not serve hot food; serve food warm or cold. Cold food and drinks may be particularly soothing when the mouth and throat are sore. They even make swallowing easier.
Adjust the texture of the food to suit the state of the mouth and throat. Soft or pureed foods are easier to eat when the mouth and throat are blistered. Scratchy foods, such as hard toast and crisps, may actually cause blisters. They should be avoided in children whose mouths blister easily
Acidic food and drink, for example, citrus fruit, may irritate a sore mouth and throat.
To compensate for bad days, make the most of days when the mouth is not sore and the appetite is good.
Involve your child as much as possible. Teach him about nutrition from an early age and allow him to help in the planning and preparation of his meals
Do not forget to give him the vitamin and mineral supplements that have been prescribed. Give only the dose that has been recommended. Excess vitamins and minerals can be dangerous.
If, after reading this, you are still concerned about your child's diet, ask your doctor to refer you to a dietitian.

The following ideas are intended to help overcome specific problems that may affect your child.

Small Appetite
The problem most likely to be caused by a small appetite is an inadequate intake of energy and protein. There are two main ways of increasing the energy and protein content of the diet without actually having to eat larger meals.
(A) Use everyday foods to increase the protein and energy content of the diet.

Use gold top (jersey) milk if it is available. It has more fat and hence more energy than other milks. Use it for drinks, on cereal and for cooking. Alternatively, use red or silver top (full cream milks) but avoid skimmed or semi-skimmed, which have lower fat and, therefore, a lower energy content. Try to give at least 1 pint of milk daily.
Fortify milk with dried skimmed milk powder. Add 5 level tablespoons of dried skimmed milk powder to 1 pint of fresh milk, to increase the protein content. Keep this in the refrigerator. Use throughout the day, on cereal, for drinks, making sauces, puddings, jellies and adding to mashed potato. This also ensures that your child has 1 pint of milk daily.
Add grated cheese to mashed potato, baked beans, spaghetti, minced meat, meat loaves, omelets and scrambled eggs. Add cheese sauce to fish and vegetables, such as cauliflower and broccoli. Use pieces of cheese for snacks or as cheese spread on biscuits or bread.
Give eggs as omelets, scrambled, fried or boiled. Also add raw before cooking, to meat loaves or homemade beefburgers. When making pancakes or cakes use and extra egg. Add chopped hard-boiled eggs to fish pie or sprinkled on vegetables.
Add cream to soup, sauces, puddings and milk shakes.
Spread butter thickly on bread, toast and biscuits (even sweet biscuits). Add to vegetables and mashed potato.
Add lentils, noodles and beans to casseroles and homemade soups.

(B) Supplement the diet with high-energy drinks. Use these drinks as a replacement for meals if your child is not eating. Offer them in-between meals in addition to normal meals in order to increase the protein and energy content of the diet.

Fortified milk shake 200 mls (7oz) cows milk. 1 brickette or 1 large tablespoon ice cream 3 level tablespoons skimmed milk powder Flavoring or milk shake syrup Whisk together and chill If the flavoring is omitted, this may be used for cereals	Yogurt drink 1 small (5oz) carton yogurt 3 teaspoons honey 1 banana 150 mls (5oz) orange juice Liquidise together until smooth
Juice shake 100 mls (3 = oz) prune or orange juice 2 teaspoons brown sugar mix together and chill this is good for constipation	Banana Shake 150 mls (5 oz) cows milk 1 dessert spoon double cream 3 level tablespoons skimmed milk powder 1 banana or 60g (2oz) tinned fruit 2 heaped teaspoons sugar Liquidise together and chill
Fruit drink 100 mls (3 = oz) evaporated milk 100g (3 = oz) tinned fruit in syrup Liquidise together until smooth	**Use gold top milk if possible, Otherwise silver or red top, but not Skimmed or semi-skimmed milk. Only use skimmed milk powder As indicated in the recipes where The aim is to increase the protein Content of the drink.

There are many nutritional supplements available in liquid or powder form. Each product varies in composition, some being complete meal replacements. Others are supplements of carbohydrate and/or fat, which can be added to other food and drinks to increase their energy content. Some are commercially available 'over the counter' from chemists and others are prescribable.

A sore mouth and narrow throat means that some children with EB can only eat soft or pureed foods. Such diets can become boring if you rely on soup and ice cream but with a little imagination and effort, they can be appetizing and nutritious.

If your child requires a pureed diet, make the food look appetizing and give lots of variety.

Use colorful foods e.g., carrots, baked beans, and peas, puree each item separately. To save time, make in bulk, freeze in ice cube containers and just thaw enough for individual meals.
Puree the family meal. Lasagna, spaghetti bolognaise, casserole can all be pureed, and will taste more interesting than plain meat and boiled potato. It will also make the child feel a part of the family if he is eating the same food as everybody else.
Use soup, milk and sauces as the liquid to puree foods. If water is used it will dilute the mal, making taste bland and also reducing the energy content
Show your child the food before it it's liquidated, so that he can see what he is eating.
The thermomix 3300 is an excellent liquidiser which heats and liquidates at the same time

It is available from:
The Barbel Marketing Co.
Stanton Old hall, Stanton-in-Peak,
Matlock, Derbyshire.
Telephone number: Matlock (0629) 733632

Financial assistance to purchase the thermomix may be available from the DHSS if you are receiving income support or family credit. Ask your social worker if you are eligible.

Constipation
The effect of constipation is underestimated. Constipation can make children feel listless and uncomfortable and can severely reduce the appetite. Everything possible should be done to establish a good fluid and fiber intake together with a regular daily routine to try and prevent the vicious cycle of constipation - apathy - poor appetite - constipation.

Include plenty of cereal fiber in the diet. Use wholegrain breakfast cereals, such as weetabix, shreddies, bran flakes, wholemeal bread, wholegrain biscuits, such as digestive, brown rice, wholemeal spaghetti and wholemeal flour for cooking (or at least a mixture of half whoelmeal, half white).
Give plenty of fruit and vegetables. Prunes and figs are particularly affective
Drink plenty of fluid.
Avoid sieving food to remove lumps as this will remove the fiber (pureed foods retain their fiber content).
Give distinct meals snacks and avoid continuous snacking throughout the day. This will help to create a strong desire to pass motions.
Try to establish a daily routine, which allows for going to the toilet at a set time.
Despite all your efforts your child may still require laxatives. If this is the case it is still important to include fiber in the diet, as this will minimize the amount of laxative required.

Anemia
Anemia is a common problem in children with EB. Providing a diet rich in iron will help to reduce the likelihood of anemia developing.

Include red meat as often as possible. If your child has difficulties chewing try minced meat, sausages and beefburgers.
Give liver, kidney or black pudding at least once a week. Disguise it if necessary by mixing together with minced meat.
Try liver pate spread on bread or toast.
Haricot beans, lentils, kidney beans and other pulses are all good sources of iron and also very cheap. Many are available in tins and do not need soaking overnight. Mix into casseroles and soups. Baked beans are good on their own or with bread/toast.
Other good sources of iron include bread, fortified breakfast cereals, dark green vegetables, dried fruit, egg yolk, cocoa and chocolate.
Some children with EB show have a good iron intake still develop anemia and require iron supplements. Do not feel you have failed if this is the case with your child.

Tooth decay
Sugar and sweets will contribute to dental caries if given in excessive amounts. However, sugar is a high-energy food useful to increase the energy intake, without increasing bulk.

Breakfast
Cereal - Choose high fiber cereal sprinkled with sugar, use fortified milk.
Toast - use wholemeal bread, butter liberally while still warm and add jam, honey or peanut butter.
Milk - Use gold top milk fortified with skimmed powder and milk shake flavor.

Lunch
Shepherds pie - add milk, butter and cheese to potato.
Mix liver and lentils with minced beef
Cauliflower - serve with white sauce.
Rice pudding - add cream
Drink - give sweetened fruit juice

Mid morning and afternoon snack
Milk - give high energy milk
Biscuits - choose a digestive biscuit spread with butter and cheese spread.

Dinner
Baked beans on toast - add grated cheese and knobs of butter.
Use wholemeal bread and spread thickly with butter whilst still warm.
Yogurt - add cream and chop in fresh fruit.
Bedtime drink - Make with fortified milk and flavor with horlicks.

Sources of Carbohydrate, which may be added to food and drinks to increase the energy content. Caloreen - Roussel
Maxijul - S.H.S.
Polycal - cow and gate
Polycose - Abbott
Sources of fat, which may be added to food and drinks to increase the energy content.
Calogen - S.H.S.
Source of carbohydrate and fat, which may be added to food and drinks to increase the energy content.
Duocal - S.H.S.
High-energy drinks which can be used as meal replacements or as supplements to normal meals.
Build up - Nestle
Complan - Crookes Healthcare

Enrich
Osmolite - Abbott
Pediasure

Fortimel - Cow and Gate
Fortisip
Fresubin - Fresenius

Liquisorb - Merck

Some of these products are available on prescription. Ask your dietitian which would be appropriate for your child