Nicky has the Hallopeau-Siemens
(HS) subtype of Recessive Dystrophic Epidermolysis Bullosa, or, simply put,
RDEB-HS. He was never officially diagnosed with this particular subtype,
however, all the symptoms point to that form and that form alone.
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Nicky's skin
improved dramatically since I started wrapping him completely. The
new wounds are kept to a minimum and old wounds are covered with
creams and ointments to improve the skin.
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The HS form of RDEB
is the worse form of RDEB there is, he has a very severe type of this
as well. How do I know this? His index fingers were already contracting
by the time I noticed, when he was 8 months old. So... they probably started
before then. Webbing so early in life is a Hallmark of HS. With the other
forms the fingers are spared the webbing and contracting until kindergarten
or elementary school age, or even later if ever, it really depends on
the severity of the form. Nicky could never walk around without bandages,
he would blister up immediately, this is why he is bandaged from head
to toe.
Also, he suffers from Macrostomia (the tightening of all the mouth skin
and muscles and webbing of the inside of his mouth) and lingual adhesions
(his tongue is webbed to the bottom of his mouth). These two symptoms
so early in life are the characteristics of HS. The fact that he was also
throwing up blood by the time he was 11 months old (denoting major problems-blisters
and tearing up of the skin in mouth, throat and esophagus) and he
suffered
from growth retardation at such a young age as well, only confirms the
suspicions of a moderate to severe form of HS.
His throat dilatations so he can eat better and his gastronomy tube insertion
went very well. This was an incredible boost to his growth, and I am sure
it will be even better in the long run. He has seen a speech therapist,
a physical therapist and an occupational therapist. He is going to regular
school with a full time aide and he he loves his teacher.
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Nicky's leg
and foot and how it almost always looks. Actually I need to change
the pic and put a new one here... it looks much worse now... sigh.
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As always I wrap
his hands with 1 or 2 inch gauze and keep the hand moist with creams and
healing products.
He has no nails, they have been gone since his first birthday. His feet
look fairly normal, but they are definitely a weak spot for him. They
are only slightly webbed. No matter how much I wrap them, the ankles and
knees in particular are almost always in the healing process. He still
has toes, but only because I've kept one inch gauze in between them at times.
His hands are another
bad spot for him. The right hand is doing OK for the most part, the
index finger and the middle finger however, are bent. The left hand though, due to the fact
that it did not heal well from his first surgery, had to have another
surgery in the summer 2002. :-( I was not too happy about it, but I believe
quality of life for him is important, and having hands improves on his
quality of life in my view, especially since the computer is such an important
aspect of his life.... and always will be.
His wounds are similar to 2nd degree burns, and can and WILL scar.
Thanks to a variety of healing products, some areas are improving dramatically,
which is important because if they improve it will mean that not only
it is harder to hurt, but if he injures it again it will take less time
to heal.
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A Nasty blister
on his foot
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I wrap certain areas
of his body more aggressively, so they have a better chance at "staying"
healed. Constipation is now under control thanks to a variety of laxatives
in his tube... from Senokot to Milk of Magnesia, from Mineral Oil to Prune
Juice and prescription Miralax. They all work, but only if I rotate them.
He wears padding and gauze all over his body not only to protect his fragile
skin, but to give him more freedom of movement. He can do a lot more things
without being hurt. Under his normal clothes he looks like a mummy, that's
true, and I have been accused of 'over-wrapping' him, but this shows me
how some people do not understand the severity of the condition and the
importance of keeping him healed and let him be able to climb and even
jump without being hurt. Wrapping is GOOD. It's protection. Protection
and Prevention is GOOD, and nobody can convince me otherwise.
Nicky knows his limitations,
and knows he can get hurt, so his growth process, mainly his gross motor
skills, are following a different path than a normal, healthy child would.
Baths
and Bandage Changes
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Nicky completely
bandaged after a bath.
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Nicky
goes through 2 main routines a week that involve a bandage change and
at least one bath a week. He may have more bandage changes depending on
how bad his wounds are doing, I will change the wound leaving the rest
intact. Bandage changes are so traumatic for him that I do them only when
necessary. I've been incredibly lucky that Nicky's immune system is very
strong and I have yet to see an infection related to a wound that goes
haywire, or at least
one that spreads or makes him sick, knock on wood, hence the routine will
stay the same unless things change.
When
he was born I was told to change his bandages daily, and I did for the
first month or so, but then I realized that it was not necessary to put
him through that, especially since I had no help, so I decreased it to
every other day, and then twice a week once I was able to give him a entire
bath, and that is how it stayed.
I started
giving Nicky a bath when he was almost 7 months old, when he could sit
up well, before then I gave him sponge baths. When he was little I used
to give him a bath in an inflatable tub to make sure he did not bang and
blister his feet or hands, but starting at about 3.5 years of age he did
fine in the bath with just a towel underneath for softness. Just
recently I stopped putting a towel in the water due to the fact that his
towel would get stuck to butt wounds, it is okay because he no longer
hurts himself unintentionally. I
put a little bleach in the water to 'kill' whatever bacteria he may have
brought into it as well.
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Just one instance
of Nicky's big blisters.
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I usually
change his bandages one limb or body part at the time... as he's getting
older I can even have him completely naked while I change his bandages,
but he's NEVER completely naked while taking a bath-it hurts too much.
Depending
on the progression of the wound at hand, I cover it with either Mepitel
(if fresh), Xeroform (if looks like it might be infected), or just Vaseline gauze if practically healed or healed
if the skin is red, until the redness disappears. Also depending on how
fresh the wound is, I may use Aquafor (or triple-antibiotic ointment if
I think there is a possibility of infection), Zinc oxide (40%) or a greasy
cream if the wound is healed and just needs some extra emollient to help
the skin regenerate properly. I then cover with gauze. The feet get extra
padding on the ankle, bottom and toes with kerlix gauze or webril, and so do the
elbows, knees and armpits. Every inch of Nicky's body is covered. Feet
and upper thighs are also covered with coban for extra strength and protection.
Toes also get 1" gauze in between them to prevent webbing (well,
not always!). Hands
are wrapped the "ABC way", a method perfected by the late Sheri
Coil.
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Nicky's wrapped
right hand
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Basically
what I do, is first soak the hand in warm water with vitamin oil. This
helps emolliate the skin which is usually pretty tough. Then, I take it
out, dry it, and cover it with very greasy cream or just cream that is
made to help scar tissue or sore skin. I gently massage each finger to
cover it with the cream, then I gently pull each finger back and count
to 10. At this point I take some Vaseline gauze and cover the entire palm
and back of the hand-you can make a slit on it to anchor it at the thumb.
Then I cover each finger with Vaseline gauze (1 in). At this point I take
some 2" gauze and wrap the palm and back of the hand, always making sure
gauze goes in between the thumb and index finger not only to anchor it,
but to pull the thumb away. Then I take some 1" gauze and roll it around
each finger, always anchoring it at the wrist after each finger is wrapped.
Always making sure the gauze is going in the direction away from the palm,
to promote straight fingers. After that I go around and make sure I go
between each web space with gauze. I use netting to keep it all in place
as you can see from the picture.
This not only
prevents new blisters and wounds on the hands, but keeps the hands from
webbing and contracting. I then cover legs and arms with stretch net tube
gauze-which I dyed for fun :-)
Wounds
and Blisters
If you've
read the Surgery Tales page, you saw
the heartbreaking surgeries and pain Nicky goes through, but unfortunately,
the surgeries are only a minimal parts of the wounds to recover from,
and Nicky, just like any other RDEB child, has had many awful wounds to
recover from. The worse one was when mommy ‘accidentally’ stepped
on his hand when he was crawling just after his first birthday. The skin
of his entire hand came off and it was the most awful and heart wrenching
experience I have ever had the misfortune to endure, and I felt like the
most horrible mother in the world to cause this much pain to my child.
His hand never looked the same after that.
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A nasty blister
on Nicky's armpit
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But Nicky
is no stranger to wounds as large as the palm of my hand-if not more,
anywhere on his body. He has had these on his chest, armpits, knees, elbows,
ankles, bottom, feet and even a huge one in his private parts, which was
horrendous to deal with, since he would scream anytime he went pee-pee.
He has
also had big large blood blisters the size of his tongue on top of his
tongue which were making it hard for him to breathe... on one instance
he was practically choking to death if something was not done-he was barely
4 months old when I put him on top of me with a flashlight and a needle
trying to pop the monster in the back of his throat. It was horrible and
heart wrenching at the same time.
And who can forget the many times he choked up blood? To say it was awful
it’s the understatement of the year.
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A double whammy,
knee and elbow. Ouch!
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All in
all, Nicky is doing quite well now. Because I wrap him like a mummy his
skin is always protected from any bump or scratch. I have seen kids with
less severe forms of EB look worse than Nicky simply because they do not
have this extra layer of protection in the form of gauze.
By having him protected this way, not only his wounds are kept to an absolute
minimum, but his skin literally looks normal now in many areas because
it can STAY healed. Generally his wounds only account to maybe 35-40%
of his body. It is getting much worse as time goes by. 3 years ago it
was maybe 20%.
Nicky
is an extremely bright, happy little boy, who looooves his momma. His
favorite thing is hugging and kissing.