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

Bandaging and Severe RDEB ~My Story~

by Silvia Corradin

My story on how I came to care for my son started, as with all RDEB babies, when he was born. Since he was c-section he came out perfect except for some problem with his mouth. The nurses sucked all his skin out of his mouth when they did the routine suctioning of the liquid in his mouth when he was born, but we did not know it at the time. It wasn’t until the next morning that the pediatrician came into my room to tell me that they had to put Nicky in NICU in an incubator because of the problem with his mouth. They thought it was either a “Staph Infection”, “Herpes” or some rare skin disorder called “Epidermolysis Bullosa”. By then, in fact, he had started blistering all over the place, not just his mouth. The Neonatologist had a dermatologist on his way from the Children’s Hospital in Phoenix where he always worked on Tuesday from his office in Tucson. When the dermatologist arrived it was around noon, he told me that he had seen Nicky and did some sort of test with the eraser from a pencil and he wrote down the name “Epidermolysis Bullosa”. He was fairly sure that is what he had but did not know what form.

Nicky, 17 months old

After he left, the nurse took me down to NICU in a wheelchair and then left me. I was stunned to see that my baby had been put in isolation. They had to put an IV on him because he could not eat since his mouth was in such a poor shape, and he was attached to a heart monitor and other things. He was in an incubator to keep him warm, had no clothes on him aside some bandages and the diaper. They were keeping the tubes attached to his tiny body with gauze. They could not use tape. The tape had already damaged his tummy, and the hospital bracelets that they had put on his ankles had done some serious damage as well. I held him for what I thought was an eternity, the blisters on his little body were horrible, and I had never seen anything like it. On the right side of his head there were a lot of little red marks where the skin had torn off. The nurse told me he got those spots when I was trying to push him out, he kept banging his head onto my bone. The heart monitor they had put onto his head when his heartbeat had fallen caused other spots on his head.

I went back to see him later on with my mom and my mother-in-law, and it was there that my husband called me in tears. Earlier he had come to see us, and I had given him the name of that strange skin disorder. Now he had had the time to research the Internet about it, and he was completely devastated. He told me that he would most likely die from this. The Neonatologist comforted us a lot, and told us that EB is something that could be lived with. Another nurse told us that he had seen a newborn with EB before that did not survive, but that was in much worse shape than Nicky and that, unlike him, this baby’s wounds never healed. We did not know what to think.

It was apparent from the very beginning that on spots where the nurses had bandaged his body he was not blistering anymore and he would not be able to rub his hands causing blisters on his hands and face if they were in mittens. Therefore I had my husband bring clothes and other things to put on him so the skin would not be so exposed. We had to learn this on our own because the Dermatologist told nobody how to properly care for our son, not even that the blisters needed to be punctured so they wouldn’t grow. Because he failed to tell us this, Nicky was in real bad shape. Both elbows were completely skinless, and so were his ankles, the top of one hand, several fingers, one of his knees, the big toe on his right foot, several spots on his legs, and his chest.

The dermatologist came back the following week, and because the blisters were not being popped Nicky was in absolute horrible shape; he talked to us for a while and managed to really depress us. Over and over again he did not want to give us any hope for his survival. He told us that we would be in week after week to treat infections, if not worse, and totally painted an extremely bleak picture for us. Afterwards he took us step by step on how to care for his wounds taking extreme care in doing so.

When we went back to see him at Phoenix Children’s Hospital a couple of weeks later, he brought in a lady, Heather, who had lost a son from Junctional Herlitz EB a few months earlier to help us and teach us proper wound care. When Heather saw our son, she immediately knew he could not possibly have HJEB, and that he was going to be OK. She showed us photos of her son and we could definitely see a difference. Heather was wonderful and told us how important wound care was; showing us exactly what she did to help her son. I never forgot it.

A month later we finally received the diagnose of RDEB and the Dermatologist emphasized how Nicky’s wounds, because they are deep, needed to be healed with moisture. A dry cell is a dead cell he explained, and could not heal back to “normal” and that is where scars come from.
In the beginning we had not bandaged all the wounds because they were not scarring and healed much faster, but as time went on it became apparent that moisture was indeed much needed, even though it slowed down the healing time considerably. One particular instance etched in my mind was when Nicky got a rather large wound on his tummy when he was 6 months old-it healed really badly because it was almost completely air dried, and it was absolutely awful. It looked so completely different than the normal skin surrounding it, it was an eye sore. Not only it looked bad, it was also much weaker than normal skin. It was only a matter of a few weeks that the complete area (and only that area, not the normal skin surrounding it) was a new wound. This time I made sure it healed with a ton of moisture and covered and protected, and although it took a while to heal, the skin looked a million times better, and, most of all, 13 years later, the wound has never re-opened!

As Nicky started crawling and moving more and more, it became very obvious that he needed more and more protection. At first it was just the hands, feet and elbows, and then his knees and more and more as time went on. It was apparent that the areas that had never gotten blisters in the first place were getting much tougher than the areas that had wounds before-those areas were much easier to get a wound on, so it was important to us to keep him as healthy and wound-free as possible. After we finally saw an EB specialist it became even more important to us to keep him bandaged because he stated that the #1 killer of RDEB children was infection, that many children are anemic due to the blood loss and, finally, the threat of cancer in non-healing areas was particularly upsetting to us, making us realize how important it really was to keep the baby as wound free as possible. One particular eye opener came a few months later. We were visiting Stanford for a routine check-up and we were able to meet a few other parents with RDEB children, and one in particular, the late and much loved Sheri Coil, decided to do an impromptu bandage “class” showing the parents how to wrap hands, armpits, torso, legs and feet. It was amazing. The things I learned that day I still use today. What was most impressive is how little damage little Brandi had on her body due to the heavy bandaging, and while at the time I thought she was “overwrapping” (a term I was referred to regarding my bandaging Nicky later on), at the same time I was amazed on how little wound mass this child had. I know some people feel that heavy bandaging means the child cannot move, but I beg to differ. It is because of the bandaging that Nicky is even able to walk and even run a little. It is because of the bandaging that Nicky is able to bump on things a little bit and be a little rougher without fear of getting hurt. It is because of the bandaging that he is not afraid of people touching him and hugging him. Sheri showed me how active her 3 RDEB children were despite the heavy bandaging and I became a believer.

At the time, however, I was not able to start the heavy bandaging because the insurance was not covering any of my son’s supplies, so I had to wash and re-use bandages because they were so expensive and we simply could not afford them. Hence I tried my best to cover what I thought were his worse spots, but with time I had to do more and more because of the level of activity my son had.
I did not start wrapping the torso, for example, until one day when Nicky was 1.5 years old. He was wearing a white t-shirt that day and I will never forget how one moment the shirt was completely dry and after I turned around, the shirt was completely soaked. At first I was shocked, not knowing how he got all wet, until I took off his shirt for the biggest eye sore ever. He had a blister, the size of my hand, which went from the front of his chest, and covered the entire side and went all the way to the back. To date, that was the biggest blister he ever had, and the last he had on his chest, because I’ve been heavily wrapping it ever since.

Another bandaging area that I had to learn how important it was is the hands. When Nicky was a baby I was told his hands could “web” but I was never told about the “contracting”, or, in laymen’s terms, the curling up and webbing of the fingers into the palm. I was pretty consistent in keeping the palm or back of the hand bandaged if he got hurt, and made sure that if he got a wound in between fingers I kept the fingers moist and apart. But it wasn’t until the visit with the EB specialist that he pointed out to me how his index finger, at barely 8 months of age, was already curling up. I could not believe it; my 8 month old baby was already disfigured. The pediatrician sent us to a hand therapist and physical/occupational therapists to see if we could get a handle of hands and prevent further damage, and while the hand therapist made some splints to wear at night, the physical/occupational therapists basically showed me the importance of straightening those fingers every single day. Another mom sent me some gloves made of Lycra that were elastic, so that the tips of his fingers could be free. Since I still felt as if bandaging each finger was overkill, I thought this was the best course of action. Well, I was wrong. The splints were so incredibly hard to put on Nicky, we quickly gave up. There were times they were doing more harm than good. While the gloves really helped in protecting the hand, they did not help in keeping the fingers apart or straight because there simply was not enough tension. Lastly, the straightening of the fingers worked, but without a way to keep the fingers apart, the fingers were becoming useless anyway because they were webbing together. By the time Nicky was 2 years old his right hand (and he is right handed) was so badly webbed he would no longer use it. The fingers were webbed together and somewhat curled and Nicky was now doing everything with his left hand, which was in a little better shape than the right one. Something had to be done, and soon. After much research, talks to various Doctor and internal debates, we decided to go ahead and do the surgery, and that we would start aggressively doing the hand bandaging immediately after surgery. While the surgery and the recovery is something I rather forget because I had never seen my baby in so much pain, on the other hand, he came out with a hand from this procedure. I have been wrapping his fingers individually and the palm/back of his hands with Vaseline gauze first, to retain the moisture, then I cover with 1′ gauze. The surgery on that hand was now about 12 years ago, and while the right hand’s fingers are a little curled up at this point, he can still use them well, and there has been no loss to web space. The hand can probably hang in there for a few years to come without any surgeries, and even so, we’ll most likely opt for a “partial” surgery, just to straighten a couple of fingers.
The left hand benefited from having aggressive bandaging early on, but it was already is fairly poor shape, and a surgery became a need when Nicky was 3 years old. Unfortunately the recovery from this surgery did not go well and the fingers were not straight afterwards, I blame myself for it, I was working full time as a single mom and was not able to tend to this hand as much as I should have. Because this was, after all, his left hand, I pretty much “let it go” and it was unbelievable how fast the fingers webbed and curled into the palm of his hand. I was still keeping them apart with gauze, but he basically had no fingers to speak of. The only good thing that came out of that surgery was that the thumb was released and he could use it well now. I was always planning to have another surgery on that hand and tried to get the right Drs involved this time, which took time, but I did not seriously and aggressively look into it until after Nicky started kindergarten and he saw the other children’s hands. To see my son devastated because his hand was different than his peers and asking me if the doctors could straighten his hand was more than I could bear.

Just before his 6th birthday I was finally able to get him in to get a hand surgery with a Dr at Stanford and, by far, this was the best experience with a hand surgery yet, he wore a cast and took a lot of pain medicine and he did quite well. While Nicky himself told me he did not like the surgery or recovery, he is so happy he has a hand! Future surgeries, if any, I will leave up to him. It’s his hands, his pain to endure.

Now that Nicky is 14 years old, he himself understands how important it is to get him wrapped, and he even asks me for MORE bandages in certain areas. If for some mistake I miss an inch of his body it would be blistered up next time I unwrap him, so he’s become extremely aware of the benefits of bandages and how much they improve his life. There are many parts of his body, such as his back, that haven’t blistered at all in years and the skin there is absolutely perfect and normal, even has some little hair growing out. His chest is also looking fairly good considering some of the problems we had early on. The hardest and most wound prone areas are his entire legs, ankle and toes, his hips, his armpits, elbows and hands. Even so, he has very little scarring elsewhere, which is a miracle of the bandaging, since I know how severe he is and how bad he would be if I was not bandaging him. The fact that he’s also not so active surely makes a big difference, but he does scratch a lot, hence some of the heavier bandaging to prevent him from hurting himself aside the anti-itch medicine he receives everyday.

To parents of severe RDEB children, don’t let anybody tell you that you are “overwrapping” your child. There is no such thing. Every child is different and needs to be protected. Perhaps some children deal with it better being underwrapped, and if it works for you, good for you. Bandaging, in my son’s case, has improved his life dramatically. He can still do everything he wants to do and more, because he feels protected, and his wounds at times only account for maybe 20-25% of his body, and mostly because some take a while to heal. New wounds are now fairly rare, and are mostly accounted for by his itching that we are trying to get under control.

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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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.