In many forms of EB, blisters will form with the slightest pressure or friction. This may make parents hesitant to pick up and cuddle young babies. However, a baby needs to feel a gentle human touch and affection, and can be picked up when placed on a soft material and supported under the buttocks (bottom) and behind the neck. A baby with EB should never be picked up under the arms.
A number of things can be done to protect the skin from injury. These include:
Caring for Blistered Skin
When blisters appear, the objectives of care are to reduce pain or discomfort, prevent excessive loss of body fluid, promote healing, and prevent infection.
The doctor may prescribe a mild analgesic to prevent discomfort during changes of dressings (bandages). Dressings that are sticking to the skin may be removed by soaking them off in warm water. While daily cleansing may include a bath with mild soaps, it may be more comfortable to bathe in stages where small areas are cleaned at a time.
Blisters can become quite large and create a large wound when they break. Therefore, a medical professional will likely provide instructions on how to safely break a blister in its early stages while still leaving the top skin intact to cover the underlying reddened area. One technique is to pat the blister with an alcohol pad before popping it at the sides with a sterile needle or other sterile tool. The fluid can then drain into a sterile gauze that is used to dab the blister. After opening and draining, the doctor may suggest that an antibiotic ointment be applied to the area of the blister before covering it with a sterile, nonsticking bandage. To prevent irritation of the skin from tape, a bandage can be secured with a strip of gauze that is tied around it. In milder cases of EB or where areas are difficult to keep covered, the doctor may recommend leaving a punctured blister open to the air.
A moderately moist environment promotes healing, but heavy drainage from blister areas may further irritate the skin, and an absorbent or foam dressing may be needed. There are also contact layer dressings where a mesh layer through which drainage can pass is placed on the wound and is topped by an outer absorbent layer. The doctor or other health care professional may recommend gauze or bandages that are soaked with petroleum jelly, glycerin, or moisturizing substances, or may suggest more extensive wound care bandages or products.
The chances of skin infection can be reduced by good nutrition, which builds the body's defenses and promotes healing, and by careful skin care with clean hands and use of sterile materials. For added protection, the doctor may recommend antibiotic ointments and soaks.
Even in the presence of good care, it is possible for infection to develop. Signs of infection are redness and heat around an open area of skin, pus or a yellow drainage, excessive crusting on the wound surface, a red line or streak under the skin that spreads away from the blistered area, a wound that does not heal, and/or fever or chills. The doctor may prescribe a specific soaking solution, an antibiotic ointment, or an oral antibiotic to reduce the growth of bacteria. Wounds that are not healing may be treated by a special wound covering or biologically developed skin.
Treating Nutritional Problems
Blisters that form in the mouth and esophagus in some people with EB are likely to cause difficulty in chewing and swallowing food and drinks. If breast or bottle feeding results in blisters, infants may be fed using a preemie nipple (a soft nipple with large holes), a cleft palate nipple, an eyedropper, or a syringe. When the baby is old enough to take in food, adding extra liquid to pureed (finely mashed) food makes it easier to swallow. Soups, milk drinks, mashed potatoes, custards, and puddings can be given to young children. However, food should never be served too hot.
Dietitians are important members of the health care team that assists people with EB. They can work with family members and older patients to find recipes and prepare food that is nutritious and easy to consume. For example, they can identify high-caloric and protein-fortified foods and beverages that help replace protein lost in the fluid from draining blisters. They can suggest vitamin and mineral nutritional supplements that may be needed, and show how to mix these into the food and drinks of young children. Dietitians can also recommend adjustments in the diet to prevent gastrointestinal problems, such as constipation, diarrhea, or painful elimination.
Surgical treatment may be necessary in some forms of EB. Individuals with the severe forms of autosomal recessive Dystrophic EB whose esophagus has been narrowed by scarring may require dilation of their esophagus for food to travel from the mouth to the stomach. Other individuals who are not getting proper nutrition may need a feeding tube that permits delivery of food directly to the stomach. Also, patients whose fingers or toes are fused together may require surgery to release them.