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 bodys 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 babys defense against infection. Breast-feeding may
also lessen the babys 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 babys 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 dont 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 babys 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 babys 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 babys 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 babys diet
and the feeding of a sweet-tasting fluid may suppress the babys
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 peoples 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; dont 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 babys 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 cows 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 dont force the issue if it risk a significant
reduction in milk intake.
Cant eat, wont 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.
