Shortly after
Christmas one year, we received a phone call asking us if we were
interested in caring for two little boys with a very rare disease
that affected their skin. We are caregivers to medically fragile
children and like to care for those with 'challenging' conditions.
The oldest, Corey was 14 months and the younger brother, Alex, was
only five weeks old and not yet five pounds. They had Recessive
Dystrophic Epidermolysis Bullosa: Hallopeau-Seimens subtype, a rare
double recessive genetic disease that affected the integrity of
the collagen VII area's in the skin. This meant that any shear pressure
on their skin would cause it to lift causing blisters to form or
for the skin just to peel off at the lamina densa level or in other
words at full depth of skin. The fibrils that hold the skin to the
flesh are made of Collagen VII are greatly compromised if not non
existent. When the blisters form in an area of damage, they fill
with more and more liquid and keep growing larger and larger since
the anchoring fibrils were not there to hold the surrounding tissue
together.
We said that
we would take them but had no idea of the 'challenge' we had accepted.
They were both hospitalized in Packard Children's Hospital at Stanford
for ''wound management''. Both children were on morphine for pain
and in very poor condition. Corey had an absence of skin on about
75% of his body, his toes were webbed over completely and his fingers
were encroaching his palms they were so badly mittened and contracted.
What everyone failed to tell us was that no one expected them to
live long enough to make it back to the next clinic appointment.
Which turned out to be a good thing in the long run. We got them
and only saw future. We did not concentrate on death, but rather
on life! It was a year before we were told this expectation. When
their little sister, Brandi, was born the next year, she also joined
the family.
First
Care
Care instructions
were few and supplies were hard to come by at first. We were told
to soak Corey every day in a whirlpool bath that had bleach and
salt added. We were told not to try to wrap too much. Not to try
to wrap to protect them, that they would get blisters anyhow. That
the fewer bandages would give them more room to move as the wrapping
would get in their way.
For some reason,
we just could not get connected with the insurance and suppliers.
The insurance that covers them will and does pay for all their supplies,
but putting the three parts together was a frustrating challenge.
But we persevered and prevailed! In the meantime we learned to stretch
a bandage many ways.
Alex was released
from the hospital a few days before Corey as his wounds were not
as infected and serious. We had to stop at another hospital for
our daughter to have some tests before we went home. I was holding
this tiny scrap of humanity and noticed he had what looked like
a chunk of raw liver in his mouth. Alex had formed a huge blood
blister in his mouth and when it broke, it was impressive. This
was our first blister and it was a good one! This was not what babies
are supposed to be all about.
On the way home
from the hospital the day we picked up Corey, he threw up in the
car. We thought that it was car-sickness. We should have been so
lucky! He had a bad case of flu with all the things that are inherent
with it. It was impossible to keep him clean on either end. It is
bad enough to have any baby get the flu, but one who is wrapped
up like a mummy is really impossible to keep clean. No just stripping
off the soiled clothes and washing the baby here. All the bandages
had to be changed too. A two-hour job. Only he was not lasting even
that long between bouts of nausea and diarrhea. He finally dehydrated
so badly that the hospital near us could not find a vein to put
the IV in. And this was made worse by the fact that every nurse
they called in was frightened by his EB. Finally, in desperation,
the Dr. put it in his jugular and they taped it to his neck in spite
of my protests. They then transported him down to Stanford for another
weeks stay.
The same week,
Alex, who was almost big enough to see came down with pneumonia.
And of course the technicians in the radiology lab, who I warned
against handling him wrong, took all the skin off his feet and a
goodly part of his upper body. I was very upset and vowed that this
would never happen again as I would be a very involved and vocal,
even physical, guardian angel from then on. And I have been.
This was our
first week's introduction to EB. We were impressed by our experiences
and worried about the boys. As challenges go, this was a first rate
one. It would take everything we could give it. And then some!
Things
we observed and the evolution of how we care for them:
The boys were
in extreme pain during bath time. They constantly developed new
blisters during the process. Especially on areas like the heels
and bottom. Picking them up, Corey with his mass of open wounds
and Alex, so tiny and easily damaged, was very, very difficult when
they were completely naked and that warm, wet skin seemed to be
even more fragile. So, rather than completely stripping them and
doing a full bath daily, we began to wash and rewrap smaller areas
like the upper body, using a small tub (one or two gallons) of water
with bleach (Dakin solution) and salt added. Using pieces of Kerlix
as a washcloth and running the water over the wounds that didn't
fit in the tub with a turkey baster or the gauze squeezed out over
them. Also, we keep a spray bottle with a gentle spray full of bleach
water to spray on small areas quickly. After we wash, medicate and
rewrap the area, say the arms and torso, we then go on to the next,
lower body and feet, and repeat the procedure.
We noticed that
whenever an area started breaking down, it continued to break down.
It was fragile and very susceptible to damage. Those fibrils were
broken and did not reform well. As the children got older, the skin
areas that had never broken down, were stronger and not near as
easily damaged as those areas previously blistered. And that when
a blister was growing, it would stop dead where ever there was a
bandage. So the wheels started turning. If the bandages could stop
blisters from growing bigger where they would stop them, could they
prevent the blisters from forming at all. Could this preventative,
or prophylactic, bandaging keep that fragile and oh so tender baby
skin in tact for a while and maybe the skin and fibrils, what few
there were, might become stronger with aging. Thus began our journey
of discovery, to find every possible way to wrap all the body successfully
to protect it from the fast lane of breakdown.
The OT's and
PT's were determined to find splints and gloves that would prevent
some of the contractures from happening in their hands. We used
everything we could think of to help. Plastic splints that were
heat formed. Foam ones. All sorts of them. They were less than worthless
and some even caused more pain and damage than they would have prevented.
We were assured
that the burn glove web-spacers would prevent any more web space
lost. In theory it sounded great. Reality for us was they were a
no- go! They were hard to fit properly on these tiny little hands.
Remember that my kids were losing web space from birth. And if they
didn't fit right and keep those web spaces very snug, they couldn't
do any good. They were stiff and the Velcro closures were bigger
than the gloves! The burn garments are made from a very stiff latex.
In trying to put it on fragile EB skin, it was causing a lot of
shear pressure that was doing damage. A true case of the cure was
worse than the illness. Any glove type product was a problem as
it would do damage if it was snug enough to do a good job. I finally
decided to find a way to wrap the gloves on them instead of pulling
them on. So the hand wrapping technique
was developed.
When 'designing'
our wrap-on glove, we had two areas of concern or criteria that
had to be met. One was that it must protect the hand and fingers
from damage as much a possible and the other was that it keep the
web spaces intact. We needed about a one inch bandage to wrap around
those tiny fingers. The suppliers we were using assured us that
no-one made a one inch roller gauze bandage. So we cut 2" soft-cling
in half to make 1". The next year we found 1" Conform
at the hospital where Brandi was taken after her birth and changed
suppliers!
The rest of our
bandaging techniques just grew with the kids. We shared ideas with
other parents and played with different ideas and products. We are
ever-changing as the kids grow and as the blistered areas of concern
move around, but all our wrapping is basically as we show you in
the Wrapping the ABC Way pages. We encourage you to be creative
to suit your own ideas and needs. This is just what works for us
and is somewhere to start.
The kids –
after five+ years of being bandaged this way… are doing well.
Corey still has ten fingers with very little lost web space. They
are a bit crooked and are going to be straightened fall of '99,
but are still doing well, in spite of the fact that they are very
fragile and the skin in tissue paper thin. I have no doubts that
they would have been completely webbed in again within months had
they not been wrapped. The wrapping also gives him a lot of protection
for that so fragile skin and enables him to do so much with them
that he could not do if they were not wrapped. He has gone from
being 75% with out skin to 15%to 20%. His feet, shins, hands and
elbows are almost always broken down, but the area reduces every
year.
Alex maintained
well with almost no breakdown, once we got him healed up, for some
time – until he got the itchies. Then, the bane of EB reared
it's ugly head and he scratched himself from maybe 5% of breakdown
to over 75% of his body without skin. He was a mess. A regimen of
special creams (first our own concoction and then the Alwyn cream),
and oral drugs (Claritin in the am and Atarax in the pm) has cleared
almost all of this up, but has taken a year and has left him scarred.
We had to do some very heavy wrapping to try and keep those persistent
little fingers from doing constant damage. He was far more tenacious
than we were. I cannot imagine what he would have looked like if
he wasn't wrapped at all. Any area he could reach was constantly
blistered. But he is doing well now. His hands are exactly where
they were when we started wrapping them fully at about six months,
slightly crooked with a very small amount of lost web space.
And "Baby
Princess Brandi" (as she calls herself) is amazing. She was
a mess when we got her home from the hospital. A friend, who happened
to be the attending neonatal nurse at her birth, told me that one
could just stand and watch the blisters coming out all over her.
This nurse was the home care nurse that came to our home when we
first got the boys and gave us so much support and help, so she
had an excellent handle on EB. She was able to tell the doctors
right away all about Brandi. They sent her to a children's hospital
for care that knew nothing about caring for an EB child. I have
never understood why she was not transferred to Stanford, just a
few miles further. We did not get the final word that Brandi was
ours for two weeks and could not care for her until then. As soon
as we got the ok, we went to the hospital and started wrapping her.
None too soon, either. As soon as her pediatrician there watched
us wrap her and tell all about EB, she released her to us. Thank
goodness! We had her healed up in about two weeks and has been wrapped
ever since. Her feet are fragile, as they keep getting stepped on
and find their way under bike tires frequently. And she got the
dreaded itchies on one arm, which is hard to heal. She has had blisters
on her elbows after falls and small ones on her hands as she persists
in pulling off the finger wraps. (She looks like she has about fifteen
fingers all the time as the wrapping stays in the shape of a finger)
She takes out her knees pretty often from falling. Other than that,
she has had virtually no breakdown. And is a very active child.
She is even taking ballet lessons. We credit all to her being wrapped
from the start.
God
may not take away the darkness, but he will guide us through it.
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Silvia | Sleeping
Angel Web Design | Last Updated: Tuesday, December 11, 2001
