Medical Care for EB patients

Bathing

Bathing can be a good way to cleanse wounds because you can add different things to the bath water. Some patients bathe while others shower or do a combination of both. These are some of the things people use in their baths to help with infections:

Aveeno Oatmeal bath – Aveeno Daily Moisturizing Bath with natural colloidal oatmeal is a natural cleanser and helps with dry itchy skin. This product needs to touch the skin, it will not help if the patient takes a bath with the bandages on.

Domeboro – Domeboro astringent solution helps dry out oozing, infected wounds. If you don’t want to dry out all of your wounds you can make a compress only for certain wounds.

Bleach – Use one capful for a normal size tub.

Chlorine – Hot Tubs/Spas are wonderful also and used often because of the helpful effects of chlorine.

Vinegar – I am not sure of the suggested amount to add to a bath so please consult a physician first.

Special Bleach and Vinegar Combination – I am also not sure of the suggested amount, and you should be very careful with this method, however I’ve heard it works wonderfully for infections.

Showering

Some patients cannot shower because it may be too painful to have the water hitting open sores. Some things that are helpful when showering are:

Shower chairs – There are different kinds of shower chairs, some with backs, some without, some are padded, some are not etc. They are useful so you don’t have to worry about having to stand up the whole time. They’re easy to clean. You can put a soft towel on it for extra padding also.

Adjustable shower head – This is a nice thing to have because it allows you to adjust the pressure of the water and how much you want to come out. Some even have a setting that mists you. They can be expensive depending on the one you choose but worth it.

Loofah – This may be easier to use than a bar of soap or constantly squeezing out liquid soap. You can just lather it up and gently wash areas or just squeeze soapy water over sores.

Preparing for a bath/shower

It’s easiest in most cases to set out all of the bandages, ointments etc. that you will be using before you bathe so it is ready for you when you get out. If you have long hair it’s best to put it up to avoid it sticking to wounds. A shower cap works well if you’re not planning to wash your hair.

Bathing – If you’re bathing, prepare bath with any solutions if necessary. You may want to put a soft towel on the bottom of the tub to sit on. A loofah also comes in handy for gently washing healed areas and you can also use it to squeeze water over sores to rinse.

Removing bandages – Some prefer to remove all bandages before getting in the tub, while some prefer to soak first to allow their bandages to be removed easier. Some also bathe with bandages on and remove them and re-bandage one at a time afterwards to avoid any trauma to the skin.

Blister Popping – It is definitely important to cut and drain any blisters, however wait until after the shower/bath because the blister fills up with water while bathing and that can make it larger and cause pain.

Soaps – Soaps aren’t necessary, however you can use mild, non-drying soaps such as Dove.

Washing Hair – Some prefer to wash their hair separately, on a non-bath/shower day. If the patient is prone to a lot of FLAKES, a shampoo/conditioner such as Head and Shoulder works wonders.

When You’re Finished

Getting in and out – It is important to have hand rails or something or someone for support to grab onto when getting in and out of the tub. Non-slip rugs and mats are important also.

Drying off – Remember to pat dry, do not rub. The bigger and softer the towel the better. Make sure the towel you are using is free of hair and tiny fuzz balls etc. because they can get stuck in wounds. You can also use a hair dryer or small electric heater to help dry off. Some use an electric heater to keep warm during bandages.

*****

With Recessive Dystrophic EB, some type of surgery or procedure is inevitable, whether it be a G-tube placement, hand surgery to release fingers, esophageal dilatation, dental surgery, blood draws, transfusions, etc. It all can be scary especially when most doctors and nurses do not know the specific things you can and can not do when treating an EB patient. The following is a checklist to remember some things to tell the doctors, and suggestions on how to do things in an EB friendly way without causing trauma to the skin.

Blood Pressure and Temperature

Blood Pressure – A child sized blood pressure cuff may be needed. The blood pressure cuff should NOT be placed directly on the skin. Put something soft underneath such as your sleeve, a washcloth, webril or cast padding. Also make sure they do not put it on too tight.

Taking Temperature – Some patient take their temperature under the tongue however many with RDEB have fused tongues so it must be taken another way. If you do get yours taken under the tongue I would make sure to not let them place the thermometer themselves to avoid them poking you too hard. You can also have them use an ear thermometer, but remind them to be very gentle, sometimes they push down on the ear too hard. You can also get it done under the arm with a regular thermometer, although that can tend to rub or stick to the skin.

Blood Draw and IV Placement

Before Drawing Blood – First make sure the person drawing the blood knows that absolutely no adhesive can be used on the skin so this means no band-aid afterwards. Also tell them to be very gentle when touching the skin because the skin can literally come off. Don’t be afraid to tell them when they’re being to rough! Tell them to gently dab the alcohol on the skin, do not wipe.

Drawing Blood – A tourniquet can be used ONLY if a soft material such as your sleeve, a washcloth or webril is wrapped around the arm underneath it. Often times a family member may be asked to gently squeeze the arm instead of using a tourniquet. Ask for a butterfly needle if they aren’t already using one, it is the smallest they have.

Removing Needle – Have them use a small piece of gauze over the site while removing the needle, then you can apply some pressure with the gauze until any bleeding has stopped.

Before IV Placement – Again, make sure they know that absolutely no adhesives can be used on the skin. If possible, ask for a 24 gauge needle, I believe it is the smallest IV they have but they can’t always use that one. Get materials ready to secure IV once it is in. Tell them to gently dab the alcohol on the skin, do not wipe.

Securing IV – There are different ways to secure an IV without adhesive. It’s up to you. First have them put a small piece of gauze underneath the IV so it doesn’t get pushed down onto the skin. I usually bring my own Conform wrap and they wrap around semi-tightly and tape the gauze to itself to secure it. Remember to tell them tape can be used only if it does not touch the skin. Another thing you can use is Coban. It sticks to itself but not the skin and can be used to secure an IV. The IV can still come loose so avoid too much movement or bending of the arm (assuming that’s where the IV is)

Removing IV – Gently unwrap or cut away gauze and make sure someone is holding the IV in place so it doesn’t jiggle too much and cause discomfort. Once it’s all unwrapped they can place a piece of gauze over the site and pull out the IV while you apply some pressure with the gauze until any bleeding stops.

Before Surgery

Anesthesiologist – If at all possible, speak to the anesthesiologist before the surgery to go over the Dos and Don’ts.

Eyes – Since the eyes are very sensitive to begin with and anesthesia can cause them to dry out even more, it is important to remember to put a lubricant in the eyes before hand and ask them to reapply more a few times during the surgery. Usually any gel type lubricant works. Also obviously remind them that the eyes can not be taped shut! A soft, damp cloth or vaseline gauze can be placed over eyes instead. If “blow by” oxygen is used, avoid having it blow across the eyes.

Lips – You may also want to put some ointment on the lips and if they’re working in the mouth for dental surgery or dilatation you may want to remind the doctors to apply more throughout the surgery to avoid blistering.

Bedding/Moving – Move by lifting, NOT sliding onto OR bed. To make the hospital bed and operating table softer, egg crate or sheepskin can be used. Use them as a hammock to lift onto another bed.

Versed – A drug called Versed can be given before surgery to essentially make you forget everything that happens. This is used mostly for children who are very upset, scared and anxious about the surgery and getting put to sleep. I’ve had this once and it did make me forget ever getting put to sleep, however the effects last even after surgery and most people will wake and up go back to sleep continuously for several hours until it wears off. It also makes you a bit “loopy”. But it is a lifesaver in some cases to ease the fear and anxiety.

Anti-Nausia – Medication is usually given to help reduce nausea and the chance of throwing up after surgery, although it may still be comman to be nauseous or throw up afterward.

Miscellaneous

Instruments, Gloves and Face Masks – All instruments placed into the mouth should first be generously lubricated with a water based lubricant. Gloves should be lubricated with vaseline whenever possible. Face masks should also be lubricated.

Heart Monitors/Leads/Probes – If monitors are needed FIRST cut off the adhesive portion of ECG leads, probes, pulse ox monitors etc. The monitors can then be secured using webril, koban or any other type of gauze or tucked under netting. Pulse ox monitors can also be clipped onto thumb or toe.

After Surgery

Eye Abrasions – Even when precautions are taken, it can be comman to wake up from surgery with an eye abrasion. You may want to have some eye medication on hand and you may want to have a patch or cloth to lay on the eye or wrap gauze around the head to keep a patch on. Keep the room dark if possible.

Rebandaging – If any bandages were taken off during surgery and not put back on you may want to have some bandages ready to rebandage right after surgery.

Cleaning Up – With dental/mouth surgery there may be dried blood etc. around the mouth. A washcloth wet with warm water can be used to gently clean it off. Ointment may need to be applied to the lips also.

Throwing Up – Anti-nausea medicine can be given before surgery but it is very comman to still be nauseous and even still throw up. So having something nearby at all times to throw up into is important.

Specific Procedures/Surgeries

Barium Swallow – Be sure to pad the table with blankets or sheepskin etc. You can use a straw to drink the barium.

Dental Surgery – Because RDEB patients generally have very small mouth openings, dental surgeries can be difficult. Make sure all instruments going into the mouth are generously lubricated and the mouth and lips should be continually lubricated throughout the surgery. If a lot of teeth need to be pulled or worked on, it may be best to do it in stages (multiple surgeries).

Blood Transfusion – Blood transfusions are generally a simple procedure although they can take several hours depending on how much you are recieving. Read the IV Placement information above for more information.

Iron Transfusion – Premedicating with Benadryl may be important, they will do this at the hospital. Reactions of iching, hives or swelling is not too uncomman when getting an iron transfusion. Be sure to tell the doctor of these kind of reactions. Read the IV Placement portion above for more information.

Epo Shots – Epo shots can be given the same way as anyone else. Usually they are given on the thigh or stomach. Instead of wiping the alcohol onto the skin, gently dab.

EKG – Since the sticky monitors can not be plced directly on the skin, for an EKG you can have them first cut pieces of gauze, such as Conform, and get them wet. Apply them to the areas the monitors will be placed and put the monitors directly onto the wet gauze, making sure the sticky part does not touch the skin. Someone may need to hold each monitor in place. Dry gauze will not work, they need to be damp.

Helpful Words for New Parents

Taken and adapted for EB from the Down Syndrome for relatives page website

I cannot stress enough how important it is for family to support the new parents at this time. This is a frightening time for the new parents and they often wonder how their family will treat the new baby. Showing how much you love them and their new baby will help to alleviate these fears; “gently” pick up the baby, fuss over the baby, play with the baby. Sometimes you may not know the right thing to say or what you say, is in fact, the wrong thing.  The following suggestions are based upon the input of many parents of children with EB and Down Syndrome.

Things NOT to say
These are the things that parents have said really upset or angered them:

“I’m sorry”, “Poor___” (either you or the baby) or any form of pity.
Pity is not what parents want or need. What they need is love and acceptance of their baby.

“God gives special parents special children” or any variation.
The new parents probably don’t feel very special right now. Also, some parents may be a little mad at God. Trying to make them feel better with words like these might be appreciated by some parents and not by others. It is best to avoid this.

“Do they know how serious it is?” or any variation.
This is a demonstration of a lack of knowledge about Epidermolysis Bullosa. Some parents may be angry and want to reply with, “How serious is it? Well, every part of his body can and will blister… he can die at anytime… is that serious enough?”

“You are handling this better than I could.”
This is an invitation for the new parents to say something like, “No, you would be wonderful.” Suddenly, the conversation has switched to you instead of the parents and their new baby. Plus, you don’t really know how the new parents are handling it, do you?

 Things TO say
These are the things parents have found comforting or made them feel good:

“Congratulations.”
They just had a baby! What better response to show that you love them and their baby than to say congratulations. It made us feel like ‘normal’ parents when someone said that to us. If the hospital allows it, a bottle of champagne could be greatly appreciated.

“He/She looks just like you.”
Or similar comments you would have said to the parents if the baby would have been born healthy. This tells the parent that you can see the child behind the disability.

Friends and family who actually ‘did’ something like read about the disability (or find information on the web!)
This really means something to the new parents. It shows love and concern for the baby.

Offer to baby-sit.
It is a fear of the new parents that their family will not accept the new baby. Especially with babies with EB, most people are afraid to hurt the child. By saying something like, “Well, when are you going to let me baby-sit?” you are showing the new parents that you want to be part of the baby’s life. This will be a great relief to them. Of course, due to the fragile nature of and EB child, you will probably never baby-sit this child, and it’s important that you do not get offended by this. But knowing that you care enough to offer means a lot.

“He/She will do fine.”
The new parents are probably pretty worried. They might not know much about EB, and they may be concerned about possible medical problems. Having a positive attitude will rub off on them. They don’t need pessimism or negativity from their loved ones.

“We’ll all learn from him/her.”
This is another good way to show that you intend on being part of their lives. After all, how can you learn from their new baby if you are ashamed of him/her? Their new child will be an opportunity to learn about love, acceptance, and respect for the disabled.

“We will always be here to help.”
Another very good way to show that you are going to be there. Let the new parents know that you intend on being part of their lives. But don’t just say it without doing nothing afterwards, actually plan how you’re going to be there for them, otherwise they are just meaningless words, and the new parent will resent it.

 FOR DOCTORS/NURSES

Disability Etiquette: Some Do’s and Don’ts-from the Parents’ Perspective
Tips to Make the Office Visit Better for Everyone

The following suggestions have been compiled from parents of children with special health care needs across the country:

DO respond positively to our children in your initial interaction. Tell us they are cute, sweet, cool, that they are wearing a neat hat. Let us know that you value them even if you can’t cure them.

DO ask questions of our children, even if the child can’t speak. Show your recognition and respect. Don’t worry; his parent will find a way to facilitate the communication.

DO look at our children when you are talking to them. Please make every effort to establish a personal connection.

DO get down to our child’s level. For example, if our child is in a wheelchair, kneeling or sitting down will help make him feel more comfortable.

DO realize that sometimes parents are very tired of telling their story over and over. Even though you may have your own personal medical interest, don’t ask if you don’t need to know. Be understanding if the parent shows frustration at telling the child’s story yet again.

DO notice and be attentive to brothers and sisters. They have grown up in a situation where their sibling, by necessity, has received lots of extra attention. They don’t understand why they shouldn’t be just as entitled to a sticker or balloon.

DO be aware that our grief and sadness may recur at any time and often does at transitional times-beginning pre-school or high school, the day of the senior prom, an anniversary of another loss. It’s never resolved entirely.

DON’T ask questions or raise issues that could be painful for us (and our child) in front of our child. If you need to understand more about the birth history, for example, find a way to talk separately with the parent. You should never assume our child doesn’t understand or doesn’t have feelings.

DON’T refer to our children by their diagnoses (e.g., “EB kid,” “Downs” kid); it’s a good habit to develop no matter with whom you’re talking.

DON’T judge parents; we are doing the best we can often under difficult circumstances. If we are impatient or rude, it may be that we are under particular stress.

DON’T be surprised if we need instructions, procedures or explanations repeated several times, especially if the information is complicated, upsetting or unexpected. We are often thinking about a million other things and we are trying to synthesize what you have to tell us at the same time.

 Silva, TJ, Sofis LA, Palfrey JS. 2000. Practicing Comprehensive Care: A Physician’s Operations Manual for Implementing a Medical Home for Children with Special Health Care Needs. Boston, MA: Institute for Community Inclusion/UAP, Boston

Guidelines for parents

Guidelines for parents on the play and development of the child with Epidermolysis Bullosa 

This is the transcript of a little booklet distributed by DEBRA UK by Joyce Stebbings
Thank you Brenda for typing this for us!

 INTRODUCTION

As parents with a child who has just been diagnosed, as having Epidermolysis Bullosa it is difficult at first to look forward to the future. If that child is to develop as normal a life as possible then we have to encourage them every step of the way. Each new milestone achieved is as important to the child as it is to the parent. We need to show the child that it can be fun to learn.

BABIES AND TODDLERS

A newborn baby needs to feel safe in his surroundings. Cuddling and picking up an EB baby is not the same as with any other baby. We have to take precautions to protect him such as removing all jewelry before handling in the early years. If you want to hold him close, it is easier to have him lying on a pillow or foam wedge. In doing this he is protected from being scraped against any buttons etc. on clothing. Babies love to hear the sound of peoples’ voices talking to them, especially mum’s. They are not aware of what is being said to them, but the peaceful tone being used has a somewhat calming effect. It is also important to share eye contact at this time. It can make them fell more secure if they know someone is actually standing there.
Extra precautions have to be taken at this time with toys. Check for sharp edges, and that the toy will not make the baby too excitable. Vigorous movements will cause damage to exposed skin.
We must also remember the importance of stimulation. This encourages the baby to use all of its given senses. A bright colorful environment is ideal with lots of pictures and mobiles. Musical toys can be used during dressing changes for a calming, soothing effect.
Baby gyms are a good idea if the baby does not have to stretch too far to reach it.
As the child grows older it is a little easier to find suitable toys. Telephones are a great favorite and benefit them intellectually especially their language skills. Bath books are also good because they are soft and easy to carry. Another favorite is the video recorder. This can not only be used as a fun item but also as an educational back up. Because of the length of time dressing changes may take it is a good idea to use videos for distraction play. It will help keep the child amused and reasonably happy during a somewhat distressing time. Story and music cassettes are also helpful if a video recorder is not available.
A playpen is a good idea if you feel that you need a rest from continually keeping watch over your child. Pad the base with sheepskins, and put soft interesting toys within easy reach and tied on for easy retrieval by the child. This is also a time when they want to be continually on the go. Watch out for sharp edges on the furniture. Extra padding on the elbows and knees may be essential as protection. Some falls and scrapes will be inevitable during this time.
The tongues of children with Dystrophic EB are often shorter than normal and can be slightly fused, so the mouth tightens. Mirror work can encourage the child to make funny faces and achieve maximum mouth mobility. Fun games like trying to lick jam off the nose or blowing bubbles can help. Singing is very useful to as it can be done with others and requires wider mouth opening than speech. Some things, which can be made enjoyable, do not seem like such a chore to the child.

EXPERIMENTING WITH FOOD

When any child starts solids there are always initial problems in discovering what he likes, how to feed him, and the conflict of preventing him from making too much mess whilst allowing him to experiment with food. You have the added problem of not causing more blisters and keeping the bandages clean. However, it is necessary for your child to explore new textures and tastes. This might involve getting in a mess but a compromise is needed. It is possible to buy aprons with sleeves for such occasions.
Star off with soft foods such as mashed potatoes or banana, then progress into slightly harder textures. If the skin is too blistered around the mouth it may be necessary for you to feed your child for a while to prevent more damage. The types of food your child is able to eat will become clear. There might be some food he finds difficult to chew or swallow. Feeding himself is the first step towards independence. This will begin confidence building. Small thin baby spoons are better than the wide metal ones you can get for older children.
As these children often suffer from sore mouths and throats due to blistering, in addition to teething, minor illness and food fads, their food intake can become affected. Children quickly pick up parental anxiety on feeding so on bad days parents should try no to transmit such anxiety to the child and never force food. Sometimes just looking at the size of the plate is off putting to the child. Food can be made fun by making funny faces and different colored milkshakes can tempt the child, even a small mouthful is better than nothing.

SCHOOL AGE CHILDREN

Parents should be encouraged from early days to let their EB child mix with others. It is tempting to want to protect him and argue that he will be injured too easily but it is vital to aim for as normal an upbringing as possible. Friendship and conversation, fun and laughter need to counter balance physical pain and irritation.
Mental ability and knowledge will have great value in future job prospects, if physical activity has to be curtailed. The aim should be to produce well balanced, well informed, well-rounded personalities rather than one that is introspective and nervous or embittered. At the age of three or four organized play groups in the home or at a center are the next stage. The parent can initially stay with the child to settle him in – show staff how to handle him and watch for hazards, but parental involvement should be gradually withdrawn after a few sessions.
Most children go to mainstream schools but a few do better in schools that can meet their special requirements. In both types, integration must still allow for the restrictions imposed by the condition. Time must be allowed for the child with EB to achieve a result rather than leave an activity incomplete or have some – one unnecessarily helping them. It must be remembered that children with EB can become exhausted quite easily, especially if they are anemic. However, it must be assessed whether your child is genuinely tired, unable to cope, or is using the excuse of exhaustion to opt out. The aim is for as normal a life as possible.
A great many activities that nay other child can achieve can be achieved by a child with EB as well. Sometimes a minor adjustment may be required. The ability to participate may change form day to day, but it must be remembered that exclusion can cause as much damage as participation. For example, if your child has to sit out a PE session, the stiffness incurred can actually increase the need for physiotherapy or exacerbate existing contractors. Adapting the lesson so that the child with EB can participate is better than him not trying. This encourages participation and avoids feeling of being “left out”. If the teacher has any concerns it might be a good idea to consult the physiotherapist.
When a child goes to mainstream school, many local authorities provide a support worker to be with him during the school day. The worker can help the child with any dressings that may need changing, be there to help during break times, and so on.
Special equipment may be required for an individual EB child. Wherever possible normal equipment should be modified. This will help the child avoid becoming segregated from other children. Items that are too narrow to hold can be enclosed in foam or melolin to make them more usable. Foam tubing is available in various colors through your local occupational therapist/physiotherapist, or if you prefer it, a wide range of pens and pencils are available which are designed for easy holding. They are triangular in shape, fairly thick, and are called hand huggers.
If surgery is planned, try and make modifications to equipment in advance so that the child doesn’t miss out on activities.

TEENAGE YEARS

Some may think that this is a long way off but for most EB children this is and important time of their life. It is important that during their younger years they do get the right nutrition and are encouraged to keep mobile, doing the appropriate exercises given by the physiotherapist. At school most of the EB children will have access to computer if they find it difficult with their writing skills. If planned by the school, exams can be done with the use of computers. It is important however that they do not become dependent on them, as this would discourage them from using their hands to write with.
If at any time they feel they are the only ones with difficulties like this talk to them honestly. Be positive about the things they can do. This can sometimes be painful. If you feel that you are finding it hard to cope, don’t hesitate in asking for help. Maybe it would help if they could contact with others who are facing, or who have faced, the same problems. Teenagers often find it easier to talk to their peers rather than family. This is normal so don’t feel rejected.

SELF ESTEEM

Although some children may find it easy to cope others may not. You as parents can look for any change in behavior or you might overhear something the child says. This can be worked through with the help of a child psychologist, play specialist (if your child is in hospital), or teacher.
These people are in a good position to help children build self esteem by making them feel special and introducing them into joint activities with other children e.g. board games such as “all about me”, where everyone expresses and talks about how they feel, to encourage them to feel good about themselves and let them know there is always someone to talk to.

DISCIPLINE

Children with EB can only grow and develop to their full potential with the constant support and guidance from their parents. This may be a daunting thought but don’t wrap them in cotton wool, let them experience what the world has to offer and develop from it.
It is very difficult for the extended family and friends no to over compensate for the affected child. Under this pressure it is very hard for you not to do likewise. It is important that you have a positive attitude and expect the most for, and of your child.
All children need boundaries set and some sort of discipline for them to grow in a secure, structured framework. If discipline is not introduced early the child may think he is exempt from punishment and this could cause more problems. Children can feel insecure if they are out of control. Physical discipline is not advisable. It can however take the form of losing privileges, treats or being sent to their room for a period. If there are problems at school, discuss ways to approach discipline with the teachers.

A POSITIVE THOUGHT

The best source of information can only come from other parents with EB children who have gone through it all before you. Their constant support for each other is unquestionable, as is that of the professionals who also work with EB children.

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.

Basic 101 Care

This page was put together with the aid of a little booklet distributed by DEBRA UK called “Care and Management of Children with Dystrophic Epidermolysis Bullosa”.

Because of the rarity of EB, many parents have not been helped by the medical community, and hospitals have even injured these children by applying tape, bracelets, sucking the liquid from the mouth… (only proceeding in taking all the skin off also in the process) on these fragile newborns. The following are BASIC 101 care needs and knowledge for an EB child… something that, unfortunately should go without saying, but every parent wishes they would have known (and wishes the doctors would have known as well) when their child was born…

Blisters

POP THE BLISTERS!!!!!!! Yes, you can’t get anymore basic than this. These blisters have a tendency of increasing in size incredibly fast, and since they leave behind quite a remarkable wound, the smaller the blister, the smaller the wound. Leave the roof on the blister because it will protect it. Do not just put a hole on the blister, make sure to tear it so there is no chance for it to re-fill. Sterile needles can be used or scissors.

Lifting the child

Before handling any child with EB, remember that friction will cause blisters and skin damage, but direct pressure will not (unless this is a Simplex DM child). Children of any age who suffer from Epidermolysis Bullosa must NEVER be lifted from underneath the arms, as painful blistering will always result and this area is notoriously difficult to dress and to heal.

Bathing

Naked children are very vulnerable, for this reason most babies are sponge bathed once a day until they can sit well on their own. At that time the child can be immersed in water on a piece of foam or soft tub, which prevents damage from the child kicking the tub and such. To pick up this child use arms instead of hands, as the children get older they should be encouraged to climb in and out of the bath themselves in order to avoid skin damage caused by lifting them. There is no need to give these fragile babies/children a bath every day, water dries the skin too which is bad news for any EB patient.

Wound Care

Gauze should NEVER be put over a raw wound… NEVER! When the gauze is taken off it will be stuck to the wound and it will be not only painful getting it off, it will also take off the healing taking place. Use either a non-stick pad such as Telfa, Mepital or Vaseline gauze (put extra Vaseline as needed). Tape can be used to secure the wound as long as it does not have any chance to come in contact with the skin, however, using tube gauze is far preferable to tape. It is a good idea to pad these children’s entire body to PREVENT new blisters. Remember, is far less painful to sweat than to have open wounds.

Diapers

This one will depend on the child, but most kids do fine with Huggies Supremes or Ultratrims, use a size bigger if necessary to make sure they are not too tight. Cloth diapers are the only alternative. You may need to put Vaseline around the waist and leg areas to decrease friction, or cut the elastic around the diaper.

Nutrition

Children with EB have an increased requirement for nutrients as they are attempting to grow without diverting some of the nutrition into wound healing. Unfortunately, there are many factors (including blisters in the mouth) which impair eating in these children and heroic efforts must be made to supply the correct balance. For babies, use the powder formulas and increase the dosage of powder to make a more caloric meal-this tip came straight from Stanford. If your child cannot use powder formulas for whatever reason, try using extremely HOT water for mixing first, if not, use liquid concentrated formula, but check with the pediatrician first for dosage. For children over 1 year old, PediaSure seems to be the best bet.

Crawling and Walking

Children with EB often crawl and walk later than other children. This is because they tend to be cautious because they are hurting and/or are afraid of falling. However, once mobile, they quickly gain confidence. Each child develops different, so as one EB child might walk early, one other may walk very late, as normal children do too.

Eating Difficulties

Often babies with RDEB have a sore mouth, because the skin inside the mouth can blister the same way as the rest of the skin. Most children with Recessive Dystrophic develop “microstomia”, a small mouth opening, causing problems with putting food into the mouth. Fusion of the tongue to the floor of the mouth, makes it difficult to move the food to the back of the mouth for swallowing. Although teeth “can” be (not for all kids unfortunately) structurally normal, they are prone to decay in view of the necessity for a high calorie diet, poor access for teeth cleaning, and often a reluctance on the part of the child as the mouth is sore. The “labial sulcus”, the space between the inside of the lips and the lateral surface of the gums, is often lost as a result of scarring, this leads to a reduction in the circulation of saliva and adds to the problems experienced when chewing and swallowing.

Swallowing difficulties

Some children experience recurring blistering of the esophagus. Unfortunately, these blisters tend to heal with scarring in the same way as blisters on the external skin. This can cause strictures, which are narrowed areas of the esophagus. These strictures can be severe, and sometimes a semi-liquid or liquid-only diet can be taken. Many children will choke on a blister to pop it, but be reassured that it is the esophagus and not the trachea that is blistered, hence breathing is NOT impaired.
Side note from a patient with RDEB: some patients have had problems with breathing later in life due to scarring close to the esophageal flap.

Webbing and Contracting

Most children with Recessive Dystrophic EB, particularly the ones with the Hallopeau-Siemens subtype have a tendency of webbing and contracting on the fingers and toes. Webbing means that the fingers can become fused together, contracting meaning they will contract toward the palm. Despite meticulous care, some children require plastic surgery to divide the fingers and to restore function of the hand. This happens because of too much scar tissue prevents the growth of the hand.

Constipation

This has been described as being the worse complication of all. It may start somewhere around the child’s first birthday and it takes just one hard stool to blister the anal margin, and the cycle of holding back will begin. Even a young baby will soon learn to avoid opening his bowels and so avoid the pain. A combination of laxatives, increased fiber in the diet will help, but the dosage will need to be regularly reviewed and adjusted.

Anemia

Regular blood loss is the main cause of Anemia in children with RDEB. Iron supplements and a diet rich in iron, fiber but *especially* protein can help.

Care of the Eyes

In many children with RDEB the surface of the eye can blister in the same way as the external skin. Blisters on the eye are often caused by the infant or child rubbing the eye or by a foreign body traumatizing the conjunctiva. Child should immediately be taken by an optimologist and given a local antibiotic. The eye must never be forced open for medical examination as further damage will be done. Apply the ointment to the corner of the closed eye and it will melt and run in. Scarring may develop in the eye which can impair vision. 🙁

Cancer

A complication of Dystrophic EB seen in adults is the appearance of skin tumors called “Squamous Cell Carcinoma”. If these are identified quickly they can be removed before metastatic spread occurs.

For New Parents

For New Parents
I put together this page with the aid of a leaflet distributed by DEBRA UK called “Dystrophic Epidermolysis Bullosa Initial Information for Parents”. The leaflet this information is taken from is specific about caring for newborns of the “Dystrophic” form of Epidermolysis Bullosa, however, most of the information is valid for the other forms as well.

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Epidermolysis Bullosa (EB) is the name given to a group of genetically inherited skin disorders, all characterized by the fragility of the skin, and a tendency for the skin to blister when subjected to friction.
There are many different types of EB, but the three main types are simplex, Junctional and dystrophic.
Each of these types are like a completely separate disease. It is not possible for one type of EB to change to another type.

Your baby has the dystrophic type of EB.
Dystrophic EB can be inherited in 2 different ways:

1. Dominant dystrophic EB occurs when one parent actually has EB and has skin which is fragile and blisters easily. There is a 1:2 chance whenever that parent has a child, that the child will inherit EB.

2. Recessive dystrophic EB is always a shock to parents as neither parent suffers from the disease themselves, both are what we call healthy carriers. Every time two parents who are carriers of EB have a baby there is a 1:4 chance that the baby will be affected by EB.
If only one parent is a carrier, then there is no risk that the baby will be affected. Unfortunately, at present there is not a test available to detect carriers, wen we only know parents must be carriers if they have an affected child.

As a general rule, dominant dystrophic EB is a milder condition than recessive dystrophic EB.
There are many variations in the severity of dystrophic EB. The condition of the baby at birth is not an indication of the severity of the disorder.

Many babies with dystrophic EB are born with denuded areas of skin, typically over the feet and lower legs. This is thought to have been caused by the baby kicking in the womb and knocking the feet together. Further problems can result from the birth, and handling immediately after birth.

With the application of dressings, the feet will heal, usually over a period of several weeks.

There is a tendency for the toes to fuse together, even if the dressings are done with great care, and dressings have been put between the toes. This will not affect the child’s ability to walk. Toe nails and finger nails are often lost when blistering occurs under the nail bed. These do not always re-grow.

You will quickly learn how to handle your baby without damaging the skin, but remember that however careful you are, the baby will always have some blisters.

The best way for you to pick up the baby is by gently rolling him away from you, placing one hand under the head, and the other under the bottom, allow the baby to roll back onto your hands and lift, rather than sliding from the cot.

For friends and relatives it can help to place baby on a thin cushion and let them lift from underneath the cushion.

You will soon become expert at handling and will be able to handle the baby without thinking through every step. Although you may want to protect baby and want to be the sole carers, remember to teach relatives and close friends how to look after your child. It is important to be able to leave your child feeling confident that he or she is well cared for so you can go out for the evening or in the event that you yourself are unwell.

Clothing
At first, a soft babygrow is ideal. As the child becomes older underclothes can be worn inside out to prevent seams from rubbing, Always remember to remove labels which can rub and cause blisters.

Car Seats
A normal car seat can be used, but wrap a soft cloth or muslin under the straps to prevent rubbing on the face. Do the same with indoor baby chairs and pushchair straps.

Feeding
Often babies with dystrophic EB have a sore mouth, because the skin inside the mouth can blister in the same way as the rest of the skin. This does not usually affect feeding as babies, but can cause problem with older children.

If bottle feeding, it helps to wet the teat with cooled boiled water as a dry teat can stick to the blisters.

It is important that the baby has plenty of calories and other nutrients as some of the goodness will be diverted into wound healing and the rest is needed for growth.
If necessary the dietitian can add supplements to the feeds to make them richer. Watch out for constipation which is very common in all of those who have EB. The skin around the bottom may blister, causing soreness, and the baby will be reluctant to pass a stool. Again, the dietitian can advise on the prevention and management of constipation.

Crawling and Walking
Children with Dystrophic EB often crawl and walk later than other children. This is because they tend to be cautious because they are anxious that they will feel sore. However, once mobile, they quickly gain confidence.

Scarring
In dystrophic EB there is a tendency for the blisters to heal with scarring. Physiotherapy can help to prevent such scars from causing reduced mobility. In the very severe forms of dystrophic EB some children develop contractures of the hands and may need corrective splintage and plastic surgery.

Dental Care
The teeth in dystrophic EB are normally formed, but extra care must be taken with oral hygiene as blisters in the mouth can make it difficult to keep the teeth clean.

A day at a time
In the first few weeks with your new baby try to take one day at a time, and not to rush ahead thinking about years to come, as no one can predict at this stage how EB will affect your child in the longer term. Most children will not develop all of the complications, and many are only mildly affected.

Prenatal Testing
If you wish to have more children, there is a test available in pregnancy to determine whether the baby is affected by dystrophic EB. The test is either in the form of chronic villous sampling after the 10th week of pregnancy, or a skin biopsy from the baby in the 15th week. A blood test from both parents and all their children is required to determine suitability for the earlier test.