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…
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.
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.
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.
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.
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.
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.
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.
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.
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. 🙁
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.