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.
