A Different Kind of Perfect: Writings by Parents on Raising a Child with Special Needs (Paperback)

A Different Kind of Perfect: Writings by Parents on Raising a Child with Special Needs (Paperback)

by Cindy Dowling (Author)

From Publishers Weekly

While all parents worry about their offspring, parents of children with special needs face a unique set of challenges that is served well by this touching collection. The conflicting emotions and frustrating dilemmas of raising special needs kids are well represented by more than 50 short essays; parents will find honest confessions that are wrenching, warming and probably familiar. “Why make a birthday cake when she can’t blow out the candles, make a wish or eat a piece of cake?” laments one mom in the section on “Depression”; another, in the “Love and Joy” section, is taken by surprise by her own happiness: “The sun is shining, the day is brand-new, my child is humming, and God is so good!” Divided at first into sections based on the emotional journey of parents of special needs children, essays on such topics as “Denial,” “Anger,” “Acceptance” and “Empowerment” give way to takes on “Marriage, family and friends,” “Spirituality” and “Laughter,” and include practical advice (“Don’t Always Trust the ‘Experts’”), philosophical musing (“The Kaledoscope of Our Life”), straight encouragement (“Dance Recitals Are Still Possible”) and life lessons (“We Take Nothing for Granted”). Though this text is not for parents who are expecting, it makes a valuable, readable, tear-jerking resource for parents raising a special needs child. ~
Copyright ~Reed Business Information, a division of Reed Elsevier Inc. All rights reserved.

Reviews

Compiled by a psychologist and two parents of special-needs children, the essays in this book reveal deeply personal issues. Through these sincere accounts we see both the strain on the parents and the potential of the children. Library Journal (starred review)

The honesty in this book took my breath away. There are so many different, compelling voices to listen to. The book embraces the entire family experience, as well as the whole grieving cycle.Susan Senator, author of Making Peace with Autism

This riveting book brings us the much needed voices of parents of children with disabilities, who speak with breathtaking honesty about their grief and gratitude, anger and exaltation, despair and laughter. It offers a wisdom that speaks not only to parents and families of children with special needs but to anyone who wants to learn how to live gracefully with disappointment and to transform lifes challenges into blessings.Miriam Greenspan, psychotherapist and author of Healing through the Dark Emotions his work.

Diet for Epidermolysis Bullosa

Diet for Epidermolysis Bullosa
For children over 1 year

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

Thank you Brenda for typing this for us!

Nutrition in 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

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.

Food is composed of many nutrients. These nutrients are usually categorized as:

Protein – Fat – Carbohydrate – Vitamins – Minerals

Fiber and Water – are also required to keep the body working well.

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.

The major nutrients are:

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.

SUGGESTIONS FOR FEEDING A YOUNG GROWING CHILD

On Waking – Unsweetened fruit juice or milk or weak tea.

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.

Mid morning – Milk or unsweetened fruit juice and or biscuit.

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

Bedtime – milk to drink

Vitamins A, D and C drops Available from child welfare clinics and chemists

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

Dietary Problems in Epidermolysis Bullosa

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.

Feeding Children with Epidermolysis Bullosa

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.

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.

Nutritional Supplements

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.

Chewing and swallowing difficulties

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.

Try some of the following:

Soups with added cream
Scrambled eggs, omelets, poached eggs
Cottage, cream or grated cheese
Sweet or savory pancakes and soufflis
Pouched or flaked fish in a sauce
Braised meat, shepherds pie, lasagna, spaghetti bolognaise
Vegetarian dishes using lentils and beans (good for constipation)
Milk puddings, stewed or pureed fruit, egg custard, milk jelly and yogurt

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

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.

Keep sweets and sugar to mealtimes where the presence of other foods will act as a buffer and reduce the bad effects of the sugar.

Give chocolate in preference to boiled or chewy sweets. Follow with a glass of milk or water to help rinse the mouth.

For snacks, give cheese, crisps, bread or plain biscuits.

Make sure your child has fluoride supplements if the local water is not fluoridated. Follow the advice given by the dentist about oral hygiene.

How to provide extra nutrients for a young child with Epidermolysis Bullosa

Sample menu

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.

Glossary of Nutritional Supplements

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

Nutrition for Babies with Dystrophic EB

BY: Lesley Haynes SRD

The latest version of this booklet is available in PDF format at the Debra International website. You can download it HERE.

Webmaster note: Although this was written specifically for Dystrophic EB by Registered Dietitian Lesley, it has been pointed out to me that it is helpful for babies and toddlers of severe cases of simplex as well. Just FYI!

INTRODUCTION

Good nutrition is one of the most important, yet frequently underestimated, aspects of EB treatment throughout life. Although there is no special diet, which can provide a cure for EB, recent research proves that attention to diet can result in better growth, greater resistance to infection, improved wound healing and an altogether better quality of life. This is especially important in the first two years of life, because growth and well being in later years are greatly influenced by good weight gain from birth and by early experiences with food. This booklet describes the nutrients, which make up a normal balanced diet and emphasizes those foods, which are especially important in EB. This is followed by practical information specific to feeding EB babies up the age of about 18 months.

EB affects girls and boys in equal proportions, but for ease of reading, the baby is referred to as he throughout.

Why is nutrition so important in EB?

Good nutrition is important for all of us, whether we have EB or not, and this is especially so during periods of rapid growth e.g. infancy. Babies who do not have EB channel most of their nutrition into growth. The skin of EB babies is damaged very easily, and they need substantially greater amounts of food not only to permit normal growth, but also: -

To replace the nutrients lost through open wounds or lesions

To provide the extra nutrients necessary for rapid healing

To enable the body to fight or prevent infection in damaged areas of skin

Last, but by no means least, to feel well and enjoy a good quality of life.

All babies, including those with EB, have off days, e.g. due to teething or minor illness, when food intake is reduced. EB babies can also develop blisters in the mouth and throat, which make feeding uncomfortable and reduce food intake, sometimes considerably. For all these reasons, it is important to make the most of good days and try to give a nutritious diet as often as possible to compensate for periods of poor eating.

What is a nutritious diet?

A nutritious diet provides all that is necessary to keep the body fit and healthy. Food is composed of many nutrients, and these are usually categorized as:

Proteins Vitamins Fiber

Fats Minerals Water

Carbohydrates

Put very simply, most nutrients are used to satisfy the body’s need for growth and repair (normal wear and tear), and to supply energy (calories). This series of chemical changes which food undergoes in the body in order to maintain life is known as metabolism.

Requirements for growth and repair are relatively high during infancy, childhood and adolescence. In EB, when blistering and loss of body fluids require constant repair work, the metabolic rate (the speed at which metabolism takes place) is higher than normal and this necessitates a greater intake of nutrients.

A nutritious diet can be achieved with many different combinations of foods; in fact, the more varied the diet, the more likely it is to contain a good balance of nutrients.

How you can provide a nutritious diet for your EB baby

The following section explains the role of each nutrient and its importance in the EB diet.

Proteins

Proteins are particularly important during infancy and childhood to build strong, healthy body tissues. In EB, a high protein intake is also needed to help in wound healing throughout life.

The main sources of animal protein are meat, fish, eggs and dairy products e.g. milk, cheese and yogurt. Foods such as pulses (peas, beans and lentils), nuts and cereals contain vegetable protein. A vegetarian diet needs careful planning to ensure its adequacy for any child. Nuts (except for smooth nut butters) should not be given to children under five years in case they inhale them or choke.

Fats

Fats are the most concentrated source of energy in the diet. Some babies with EB may not be very physically active, but they often need a high-energy intake to allow the body to use protein effectively. If they do not consume enough energy from fats and carbohydrates, valuable protein is used as an inefficient and wasteful energy source. Those with small appetites or feeding difficulties should exploit the high energy content of fats and fatty foods by incorporating them frequently into the diet.

Butter, margarine, cream, oil, lard, suet and dripping are obvious sources of fat. Hidden sources are full cream milk, full fat yogurt, most types of full fat cheese (including fromage frais), ice cream, meat (especially when there is visible fat), eggs, oily fish (e.g. sardines, pilchards and salmon), avocados, nut butters and chocolate.

Carbohydrates

Carbohydrates comprise a large group of energy-providing foods some of which (cereals, breakfast cereals, flours, pasta, bread, potatoes, fruits and pulses) also provide fiber, vitamins and minerals. Other members of this group are useful just as a source of energy e.g. biscuits, sugar, sweets, glucose, honey, jam and syrup. Puddings and cakes are valuable principally for their energy content, but can also be valuable protein sources if they are made with eggs and milk products.

All carbohydrates are important in the EB diet. The sweet ones (biscuits, sugar, etc.) should be included with, but not instead of, the less sweet ones (cereals, potato etc.). (See also section on Sugar and tooth decay).

Fiber

Fiber or roughage is the part of food which is largely unabsorbed as it passes through the digestive system. Although it has little food value, it is very important in assisting normal bowel function and helps to prevent constipation. Fiber is found in wholegrain breakfast cereals, e.g. Weetabix, porridge, bran flakes & muesli, whole meal bread, whole meal flour, whole meal pasta, brown rice, pulses, dried fruit and the flesh, leaves, skins and pips of fruit and vegetables. Those with mouth and throat blisters can find high fiber foods difficult to chew and swallow. Citrus fruits (oranges tangerines etc.) and tomatoes may irritate the mouth if it is blistered or sore. A high fiber diet is bulky and filling and consequently can be low in energy, as less total food is eaten. (See section on Constipation).

Vitamins

Vitamins are nutrients, which are essential for growth and health. For most people, if a sufficiently varied diet is eaten every day, their intake of vitamins is satisfactory. Several vitamins exist and each has a specific function in the body. For example: -

Vitamin A is found mainly in liver, carrots, milk margarine and butter. Dark green, red and yellow vegetables contain a substance called retinal which can be converted by the body to vitamin A. Vitamin A is needed to maintain healthy skin and eyes.

The B group of vitamins occurs in dairy foods, meat, eggs, bread and cereal products and potatoes. Different members of the group have different functions e.g. promoting the efficient use of energy from carbohydrate foods, maintaining healthy blood and skin and aiding protein metabolism.

Vitamin C is found mainly in fruit, especially citrus fruit and some vegetables, e.g. green vegetables and potatoes. Vitamin C is important in wound healing and helps the body to absorb iron.

Vitamin D helps to build strong bones and teeth. It is found in butter, margarine, oily fish, evaporated milk, eggs and liver, but the richest source is fish liver oils. The action of sunlight on the skin produces vitamin D in the body.

Those with EB often have difficulty eating normal amounts of food. Also their vitamin requirements are probably higher than for non-sufferers. For these reasons, vitamin supplements are often prescribed. Excessive intakes of some vitamins can be harmful. You should always ask for dietetic advice regarding the most appropriate supplements of your child.

Minerals

Minerals, like vitamins, are essential for health and growth and a well balanced diet usually provides adequate amounts for normal requirements. Minerals of special importance in the EB diet are iron and zinc. Iron is needed to keep the blood healthy and to prevent anemia. Supplementary iron is often needed in EB to replace losses from skin lesions. Baby milks contain iron and some manufactured baby foods such as rusks and savory meals are fortified with iron. The main sources of iron in the diet are meat (especially liver, kidney and corned beef), bread and fortified breakfast cereals.

Zinc is vital for rapid wound healing. It also has an essential role in many complex metabolic processes. In EB, the healing process is often continuous and zinc supplements are frequently required. Zinc is found in a variety of foods, particularly protein foods such as mat and dairy products.

Iron and zinc supplements can be prescribed. Ask for dietetic advice as to the need for, and best type of supplement for your child.

Calcium, with vitamin D, builds healthy bones and teeth. It is also needed for normal muscle and nerve functioning and blood clotting. Calcium is found in milk and milk products such as cheese and yogurt. Breast and formula milk supply plenty of calcium and many EB children receive enough calcium from milk and milk products, so extra supplements are not usually needed.

Water

Although often not considered as a nutrient, water is essential to life. The kidneys regulate body water, and babies need sufficient water from breast/formula milk or as a separate drink, to avoid becoming dehydrated. Babies cry with thirst as well as hunger, so offer plain cooled, boiled water between feeds. An adequate fluid intake is also important to avoid constipation. (See also section on Constipation).

What about breast-feeding?

Human milk is a unique food perfectly suited to most babies to promote optimal growth and development. It also contains antibodies, which contribute to the baby’s defense against infection. Breast-feeding may also lessen the baby’s risk of allergy. It is quite possible to breast-feed a baby with EB, provided that this results in normal growth. If you baby has mouth blisters, this does not necessarily rule out breast-feeding. The presence of mouth blisters often puts mothers off breast-feeding more than babies.

Here are a few tips which mothers of EB babies have found helpful: -

Put the baby to the breast often and let him suckle as long as he wishes.

Allow plenty of time so that neither of you feels rushed.

If our breasts are very full, express a little milk first so that the baby does not choke when the

milk comes down. Make sure that the baby is properly latched onto the nipple and not askew.

Mouth blisters usually burst during suckling. If not, burst them in the usual way with a sterile

needle. If your baby’s mouth is too sore for him to suckle, or if he tires easily, you might consider

expressing your milk and feeding it from a dropper or spoon.

The EB Nurse Specialist, your Health Visitor and your local National Childbirth Trust (NCT) Advisor will also be able to provide practical advice and oral support. It helps enormously to talk to someone who has been through the same experience, so contact the DEBRA office and ask to be put in touch with another mother.

What if you don’t breast feed?

Because many EB babies have increased nutritional requirements, breast milk alone may not be enough to promote satisfactory growth.

If this is the case, the dietitian will discuss with you the best feeding plan for your baby. It may mean giving him some bottle-feeds in addition to your breast milk, or it may mean your stopping breast-feeding and giving fortified feeds instead. (See section on Weight gain and fortified feeds).

If you choose not to breast feed, for whatever reason, do not feel that you are letting your baby down. He can derive very satisfactory nutrition from one of the baby milk formulas, which have been manufactured to resemble closely human milk.

If your baby is bottle-fed and his mouth is very sore, sucking may be made more comfortable by enlarging the hole in the teat. Do this with a needle, or make a small crosscut with sharp scissors. Sterilize the teat before use. Watch that the faster flow of milk does not cause coughing or choking. Softer, flatter teats e.g. Milupa Orthodontic teats and other specialized teats are available. The dietitian, EB Nurse Specialist, Health Visitor or NCT Advisor can give you further information.

Weight gain and fortified feeds

The best gauge of any baby’s progress and development is increased weight and length, and your baby should be weighed regularly to ensure that he is thriving and growing normally. This will probably mean weighing on alternate days in hospital, and weekly to start with once home. If possible, this should be done on the same scales and without dressings or clothes. Alternatively, weigh the baby with dressings and at the next bath time or dressing change, weigh the old dressings and subtract the difference.

If your baby’s weight gain is a little slow, the dietitian may advise you to make up his feed in a concentration greater than the usual dilution of one scoop of baby milk powder to 30 ml (1 fluid oz.) of water. This will give a feed richer in all nutrients but in the same volume of fluid as before. This is called fortifying the feed. It is safe, provided that it is done under medical or dietetic supervision and reviewed regularly.

An example of such a feed would be: -

4 scoops of baby milk such as SMA Gold or Premium (The usual dilution is 3 scoops), plus 100 ml (3-4 fluid oz.) cooled, boiled water.

If your baby needs more energy (calories), this can be provided in the form of a specially manufactured supplement of carbohydrate and/or fat mixed with the baby milk.

The most commonly used carbohydrate supplements are called glucose polymers.

Brand names include: Caloreen, polycal, Maxijul and Polycose.

An example of a feed incorporating a glucose polymer would be: -

4 scoops of baby milk

plus 1 scoop glucose polymer (using baby milk scoop)

plus 100ml cooled, boiled water.

Calogen is the brand name of a fat supplement in the form of an oil and water emulsion.

A combination of glucose polymer and fat is available in two forms, Duocal (a powder), and liquid Duocal. All these energy supplements can be obtained on prescription from your general practitioner (GP). Always ask your dietitian for advice regarding their suitability for your baby, and directions for their use.

Keep in contact with your dietitian so that she can check the adequacy of your baby’s feeds regularly, assess the need for supplements and liaise with your GP.

Fruit juice and other fluids

Babies do not normally need any fluids other than breast milk/formula milk to nourish them and boiled water to quench any extra thirst. Fruit juices and herbal infusions are not an essential part of a baby’s diet and the feeding of a sweet-tasting fluid may suppress the baby’s appetite for feeds. Babies often cry because of thirst as well as hunger, so, offer your baby cooled, boiled water in between feeds, especially in hot weather. If he is thirsty, water is the perfect drink.

Insufficient fluid intake can cause and aggravate constipation. If you feel that your baby needs more fluid, but he refuses water, offer ready to feed baby juice diluted with and equal amount of water or 1 teaspoon of fresh fruit juice plus 100ml cooled boiled water, (See also section on Constipation).

Grip water can be given for colic according to the manufacturers’ instructions. Colic can be caused by swallowing air while feeding, especially if the hole in the teat has been enlarged. Check that the bottle is held so that the contents always fill the teat, and gently wind the baby regularly during feeding, by gently patting his back (do not rub), or rocking him over your knee.

Introducing solids

Weaning is the process during which babies learn to graduate from sucking to biting, chewing and swallowing progressively more solid foods. Every baby is an individual, and arrives at this stage in his own time, but by about four months of age he should be ready to try something new. The EB baby is no different in this respect, although a sore mouth or tongue may mean that he takes a little longer to become accustomed to changes in flavor and texture.

From the start of weaning and over the next few years, eating habits are established for life. It is very important to set the right foundations by offering foods on which an appropriate diet can be built. Babies and young children learn by copying those around them and are greatly influenced by other people’s reactions to foods, their likes and dislikes. So try to set a good example yourself; it is well worth the effort! Mealtimes should be enjoyable social occasions, so whenever possible, feed your baby in the company of others and make food fun.

Your own nutrition is important too. A new baby is very demanding and you will be better able to cope with the less easy days if your health is good. This will depend to a large extent on eating well and trying to fit in some relaxation.

Weaning foods can be introduced to the EB baby in jus the same way and at the same time as for other babies. At first, solids should supplement milk feeds, not replace them. If your baby is having fortified feeds, his appetite for solids may be small. Do not worry if this is the case; as long as you are offering a range of appropriate foods, his nutritional needs will be met. As your baby gets older, he will eat larger amounts of solids and drink less milk. However, milk will continue to provide a valuable source of nutrients, and he should continue to drink at least 600 ml per day after he has been weaned.

What to offer, when and how

There is a suggested plan for introducing solids at the back to this booklet. Use this as a guide rather than a strict timetable. Let your baby progress at his own pace.

Weaning solids can be home-cooked or commercially prepared.

Do not cook with, or add salt to home-cooked food.

Ensure that home-cooked food is lump free by pureeing it in a food mill (mouli) or liquidizer; do not sieve food as this reduces the fiber content.

Use a spoon especially designed for baby feeding. It can be made of strong plastic or metal and should have no sharp edges.

Always give solids from a spoon; don’t add them to bottles.

Choose a time of day when your baby is most hungry and when you have time to relax.

Offer solids before your baby is too hungry for the next feed. Give some milk first to satisfy him if he is crying.

Begin by offering a little cereal at one feed, e.g. 1-2 teaspoons of baby rice mixed to a smooth paste with breast/baby milk.

Do not be disappointed if your baby refuses new foods at first. It takes a little time to get used to new flavors and textures.

Gradually increase solids according to your baby’s appetite by offering cereal or pureed fruit or vegetable (apple, pear, carrot) at a second feed.

After about three weeks, introduce pureed meat or stage 1 commercial savory baby meals.

Between 6 and 9 months, babies gradually learn to chew, and foods need to be more lumpy. Fruit and vegetables should be mashed, meat minced or stage 2 or Junior foods given. A little salt can be added in cooking.

Finger foods should be encouraged now, even if your baby has dressings on his hands. Babies need to touch food and feed themselves even if it is a messy business and means an extra quick dressing change!

Do not give sharp or rough foods which may scratch the mouth and gums try rusks which melt in the Mouth e.g. Farley, also ripe banana, soft pear or peach, cooked carrot, potato, Swede.

Never leave any baby alone to eat in case he chokes.

When can cow’s milk be introduced?

Ideally, all babies should receive either breast milk or an infant formula until one year of age. Many non-EB babies are switched to cows’ milk at about six months. However, EB babies with their higher requirements should continue to take the recommended infant formula, fortified if necessary, (see section on Weight gain and fortified feeds), for at least the first year and possibly longer.

A follow-on milk, designed to bridge the gap between infant formula and cows’ milk may be suitable after 6 months. Skimmed and semi-skimmed milk are unsuitable because they are low in fat and therefore low in energy.

Yogurt and fromage frais made from pasteurized cows’ milk may be given from about nine months. Choose the full-fat, sugar-containing varieties of these products, as the energy content is higher than the low calorie types.

Towards the end of the first year

By 9-12 months, many family meals can be suitable for the EB baby, provided that they are of an appropriate consistency and not too spicy. Citrus fruits, e.g. oranges or tangerines, tomatoes or sharp fruit juices may cause discomfort if the mouth is blistered, cool foods may be more acceptable at such times. Foods which are likely to cut or scratch and blister the mouth or throat must be avoided e.g. crisps, hard crusts and sharp chips, toast; crackers etc. If you give fish, check very carefully that all bones have been removed. Be careful with hard pieces of fruit e.g. apple, which may cause choking.

Encourage a good, balanced diet with the emphasis on protein and energy. If your child has a small appetite, offer three small meals a day with nutritious snacks between meals. Milk continues to be an important source of many nutrients, so do not give less than one-pint (600 ml) daily of the recommended milk formula. Many babies prefer to take milk from a bottle rather than a cup or teacher beaker. By all means, try to wean off the bottle but don’t force the issue if it risk a significant reduction in milk intake.

Can’t eat, won’t eat?

During the forthcoming months, normal events such as teething, toddling, minor illness, food fads and hunger strikes will interfere with feeding. These are all part of normal development, but parents who appreciate the importance of nutrition, they can be difficult times. Children rapidly pick up parental worries about feeding, so if your child is having an off day, try not to transmit your anxiety and never force feed.

A well-nourished child will be better able to cope with periods of poor food intake, so gradually accustom your child to extra protein and energy in his diet as routine, rather than waiting until an episode of poor eating when they are less likely to be accepted. Add extra protein and energy to the diet in any of the following ways: – Add 1-2 teaspoons of sugar to breakfast cereals, yogurt, and stewed fruits.

Add 2-3 teaspoons of cream or evaporated milk to mashed potato, sauce, yogurt, custard, mousse, jelly.

(Single/whipping cream or evaporated milk can be frozen in ice cube trays for later use).

Add a knob of butter or margarine to hot vegetables.

Spread butter, margarine, cream cheese or smooth nut butter liberally on bread, soft toast and plain

sweet and savory biscuits.

Top bread or biscuits with jam, honey, lemon cheese or chocolate spread.

Mix any of these spreads into milk puddings or yogurt.

Add grated cheese or cream cheese to mashed potato, baked beans, spaghetti and scrambled eggs. Serve white or cheese sauce with fish and vegetables.

Add 1-2 teaspoons of jam, honey or lemon curd to custard or rice pudding.

If your child requires a pureed diet, use soup, milk or white sauce to mix with it.

Water will dilute the food making it taste bland and reducing the nutrient content.

Do not sieve pureed food as this reduces the fiber content.

Constipation

Constipation in babies and young children is often the result of an inadequate fluid intake, due to a reduced intake of feeds and/ or increased requirements in hot weather. An EB sufferer with extensive blistering may have fluid requirements considerably above normal. Constipation can be aggravated by iron supplements. It may also occur for no apparent reason.

The frequency with which the bowels are opened is less important than the degree of discomfort felt. Provided the motions are soft and painlessly passed, it is not essential that the bowels are opened every day.

For an EB baby, straining to pass even a moderately bulky motion may cause pain and blistering of the delicate skin around the anus. Fear of pain on passing further motions can quickly lead to withholding the motion and before long a vicious cycle is set up as he becomes more constipated and appetite is reduced. Because regular bowel movements also depend on a regular intake of food, a poor appetite and irregular feeds can lead to harder drier motions.

The importance of preventing constipation cannot be overstated. Try to ensure a generous fluid intake i.e. at least 150 ml per kg (2-3 oz per lb) per 24 hours, for young babies who are not receiving fluid from foods. If your baby refuses plain, cooled boiled water, offer well diluted fresh fruit juice (i.e. 1 teaspoon juice diluted with 100 ml water) or give a ready to feed baby juice diluted with an equal volume of water. If extra fluid makes no difference to the constipation, try adding a teaspoonful of sugar to all baby feeds for several days. Alternatively, try giving the diluted juice from a tin of prunes or the water in which dried prunes have been stewed. Once your baby is taking solids, try to include fruit and vegetable puree daily. From about 9 months, offer whole grain cereals such as Weetabix, and from 10-12 months, include baked beans and sweet corn. The fiber in these foods, combined with adequate fluid (about 100 ml per kg, 1-2 oz per lb) will help to keep the motions soft and they will be more comfortably passed. (See earlier section on fiber). Unprocessed bran should not be given.

If constipation persists despite these measures, a gentle laxative may be required. It is important to give this regularly as a preventative measure rather than waiting until he is very constipated. Ask for specialist advice about this.

Sugar and tooth care

Dental caries or tooth decay occurs when bacteria in the plaque around the teeth react with the sugars in food to produce acid. The acid dissolves the tooth enamel and the resulting caries may cause pain and teeth may need to be filled or extracted.

Sugar is present as an ingredient in many foods as well as in the sugar bowl. The number of times sugar-containing foods or drinks are taken is as important as the overall amount consumed. Sugar at mealtimes seems to be less damaging to teeth than sugar on its own; e.g. a bar of chocolate or a sticky bun eaten with a savory snack or main meal is less harmful than the same foods eaten on their own.

In EB, the teeth can decay partly due to their structure, but mainly because blistering and scarring of the tongue can reduce its ability to cleanse the teeth in the normal way. Also, it is recognized that many EB sufferers need high-energy intakes and this cannot be achieved without the consumption of considerable amounts of sugar and frequent meals and snacks.

Compromise is possible and if sugar is used sensibly, its benefits as a high-energy food can be exploited, whilst at the same time minimizing the likelihood of tooth decay. Here are a few important points: -

Babies should not normally have sugar or syrups e.g. rose hip syrup, ribena or fruit squash added to their bottles and dinky feeders should never contain anything other than plain boiled water.

Dummies are usually inappropriate because of the danger of blister formation but, if used, they should never be dipped in honey, jam or sugar.

Suitable drinks are water, baby milk, cows’ milk, and diluted fresh fruit juice. Tea and coffee are unsuitable for babies. Lemonade, coca-cola, cordial, squash etc. should be avoided because of their sugar and acid content both of which are harmful to teeth.

For snacks, try to give cheese, sandwiches filled with smooth nut butter, Marmite, cheese spread or meat/ fish paste, or savory biscuits, which melt in the mouth.

It is unwise to give sweets and foods with a high sugar content (e.g. chocolate biscuits) to babies under one year as this may encourage a liking for these foods to the exclusion of more nutritious items. If they are offered, they should always be restricted to mealtimes when the presence of other foods in the mouth partly neutralizes the acid produced.

It is better to finish a meal with a savory rather than a sweet food.

Even young children with only one or two teeth should see a dentist, preferably one familiar with EB. Good dental hygiene is essential and the dentist can give advice on appropriate cleaning techniques, mouthwashes, fluoride supplements etc.

Living with Epidermolysis Bullosa

For more information or to Order the book, simply click on the title.

Living with Epidermolysis Bullosa (Paperback)

Compiled by Silvia Corradin, edited by Brenda G.

Living with Epidermolysis Bullosa has first-hand accounts written by parents and patients who are living or have lived with EB, and was compiled to help people in general understand how it is like to truly live with every form of Epidermolysis Bullosa; from the milder Simplex variants, who are nonetheless not simple to live with, to the more lethal, namely Junctional, whose patients usually succumb to before their first birthday. The stories you will read are from proud parents, or patients and their struggles and how they are coping. Some stories are sad, some are encouraging, and everything in between.

This book is also available directly from the Publisher and also as a paperback at both Amazon & B&N and is also available in the iBookstore in iTunes, and for the Kindle.

EB and Dental Health

By Tim Wright, D.D.S., M.S. Department of Pediatric Dentistry

Thank you Brenda for typing this for us!

Individuals with EB may have teeth with severely malformed enamel (enamel hypoplasia) and/or dental caries depending on the EB type. The enamel is usually normal in simplex and dystrophic EB types. Generalized enamel hypoplasia is typically limited to Junctional EB. Rarely individuals with non-Junctional EB types may have generalized enamel hypoplasia. Rampant dental caries occurs in Junctional EB partly because of the enamel hypoplasia. Dental decay also is frequently seen in patients with severe recessive dystrophic EB. This excessive dental caries results from severe soft tissue involvement which leads to dietary changes (soft and high carbohydrate), increased oral clearance time (secondary to limited tongue mobility and oral scarring), and creates an abnormal tooth/soft tissue relationship. Oral involvement also reduces the ability to practice preventive measures directed at reducing caries.

Because dental caries can form rapidly in individuals with recessive dystrophic and Junctional EB, dental examinations should begin by 1 year of age and be conducted at least twice a year. If caries becomes a problem then more frequent visits (4 times a year) are indicated for preventive treatments and examination. Individuals with mild EB can be treated much as any other patient. The dentist should, however, be made aware of any history of mucosal fragility and oral blistering since dental therapy can precipitate oral lesions even in mildly affected patients. Many dentists are not familiar with EB and the patient or parent must help educate the health care team. An altered approach to treatment may be required in individuals with enamel hypoplasia or rampant caries, extreme fragility of the mucosa and/or the presence of microstomia (a decreased oral opening size). Individuals with severe soft tissue involvement requiring multiple restorative and/or surgical procedures are often best managed with general anesthesia.

Preventing tooth decay is most challenging for individuals with severe mucosal involvement. In patients prone to oral blistering, oral hygiene may best be accomplished with a soft bristled, small headed toothbrush. Many small headed children’s toothbrushes are available, some of which have special grip handles that may be helpful to individuals with hands involvement. Running the bristles under hot water prior to brushing makes the even softer. Parents need to brush children’s teeth until about the age of 6 or 7 years because children lack the manual dexterity to properly clean their teeth. Parents should be very careful not to damage the gums or make the brushing experience negative and unpleasant. It is important, however, that the teeth be cleaned at least once a day preferably just prior to bedtime.

Be sure and use a fluoridated toothpaste. In small children a pea size amount is adequate to deliver the fluoride to the teeth. For individuals prone to developing cavities there are special high strength prescription fluoride toothpaste. Strongly flavored toothpaste (mint) may be irritating to the individuals with severe oral involvement, however, there are numerous non-mint flavors available. Bubble gum flavor is a big hit with children (and some adults). In addition to the systemic fluorides that we get in the water or alcoholic fluoride rinses available for the EB patients that are sensitive to strong flavoring agents and alcohol. Non-alcoholic rinses with greater amounts of fluoride are available by prescription. Chlorhexidine mouth rinses (an antibacterial rinse) also ma assist in controlling dental caries, however, sensitivity due to the high alcohol content can be problematic. This may be overcome by swabbing it directly on the teeth. Chlorhexidine rinse is a prescription item.

There are a variety of fluoride treatments applied by dentists. The most common treatment consists of placing a 1.23% acidified gel in a tray that is held in the mouth for 4 minutes. The high concentration of flavoring agents and acid nature of the gel make it unacceptable to some EB patients. Milder flavored neutral sodium fluoride gels are available that may be less irritating to fragile mucosa. Recently, a high concentration fluoride varnish has become available in the United States offering a wonderful option for delivering maximum fluoride protection to the teeth of individuals with even the most sensitive mucosa. This varnish is simply painted on the teeth by the dentist.

The diet constitutes major difficulty in caries control, and due to the complex systemic nutritional demands of individuals with sever EB types, this may be best managed with the assistance of a dietician. The effects of any diet planning should be considered with regards to dental health and tooth friendly foods (cheese, vegetables, fresh fruits) eaten as much as possible. Be careful of the less obvious cavity producing foods such as highly sweetened breakfast cereals, raisins and dried fruits. Cavity producing oral bacteria can ferment carbohydrates from a wide variety of foods. Other helpful hints are to rinse the mouth or drink water after eating if brushing is not possible. Bottle or breast feeding infants at bedtime can result in nursing caries after the teeth are present (1 year of age). If nursing continues after 1 year great care should be taken not to give the bottle while the infant is going to bed. Virtually any bottle fed liquid, except water, can cause rapid tooth decay of the baby teeth.

Individuals with even the severest forms of EB can now maintain their natural dentition providing them the ability to chew, eat a healthy diet and have a pretty smile. In the not too distant past, dental extraction was considered the treatment of choice for individuals severely affected with EB. Now we are able to prevent tooth decay, restore malformed enamel and help produce good alignment of the teeth. The future looks even brighter as dental prevention continues to improve, new dental materials are stronger and more esthetic than ever and new technologies, such as dental implants, continue to improve. Dental health for all EB patients has become a reality and that is really something to smile about.

EB Info World

Supporting families dealing with Epidermolysis Bullosa.

Monthly Archives: November 2010

A Different Kind of Perfect: Writings by Parents on Raising a Child with Special Needs (Paperback)

Diet for Epidermolysis Bullosa

Nutrition for Babies with Dystrophic EB

INTRODUCTION

Living with Epidermolysis Bullosa

EB and Dental Health