Shirley, grandma
and caregiver of Jennifer, an RDEB patient, shares her hints and tips
and her knowledge about EB care with us.
Constipation
Hi all, I found
this article in a pamphlet put out by dEBra
International in their Nutrition section. Thought it might
be helpful:
Constipation in
babies and young children is often the result of an inadequate fluid
intake, due to a reduced intake of feeds and/or increased requirements
in hot weather. An EB sufferer with extensive blistering may have
fluid requirements considerably above normal.
Constipation can be aggravated by iron supplements. It may also
occur for no apparent reason. The frequency with which the bowels
are opened is less important that the degree of discomfort felt.
Provided the motions are soft and painlessly passed, it is not essential
that the bowels are opened every day.
For an EB baby, straining to pass even a moderately bulky motion
may cause pain and blistering of the delicate skin around the anus.
Fear of pain on passing further motions can quickly lead to withholding
the motion and before long a vicious cycle is set up as he becomes
more constipated and appetite is reduced.
Because regular bowel movements also depend on a regular intake
of food, a poor appetite and irregular feeds can lead to harder,
drier motions.
The importance
of preventing constipation cannot be overstated. Try to ensure a
generous fluid intake i.e. at least 150 ml per kg ( 2 - 3 ounces
per lb.) per 24 hours, for young babies who are not receiving fluid
from foods. If your baby refuses plain, cooled, boiled water, offer
well-diluted fresh fruit juice (i.e. 1 teaspoon juice diluted with
100 ml water) or give ready-to-feed baby juice diluted with an equal
volume of water.
If extra fluid makes no difference to the constipation, try adding
a teaspoonful of sugar to all baby feeds for several days. Alternatively,
try giving the diluted juice from a tin (can) of prunes or the water
in which dried prunes have been stewed. Once you baby is taking
solids, try to include fruit and vegetable puree daily. From about
nine months, offer wholegrain cereals such as Weetabix (English
cereal - don't know what the American version is), and from 10 -
12 months, include baked beans and sweet corn. The fiber in these
foods, combined with adequate fluid (about 100 ml per kg. (1 - 2
oz. per lb), will help to keep the motions soft and they will be
more comfortably passed. Unprocessed bran should not be given. If
constipation persists despite these measures, a gentle laxative
may be required. It is important to give this regularly as a preventative
measure rather than waiting until he is very constipated. Discuss
this with your doctor also.
Eye
Abrasions
Here is the information
I have learned from Jennifer's experience with eye abrasions. Please
discuss it with your doctor.
Jennifer has had
eye "blisters" for many years. Before she came with me they were untreated
and she has ended up with scar tissue on her left eye cornea. If this
isn't treated it can cause blindness. I took her to an optomologist
who just happen to be familiar with EB, as he worked at St. Jude's
hospital and said there were children there who had EB. We were very
lucky to have him. He did a thorough exam of Jenn's eyes. This is
what he told us:
EBer's have a great tendency to "dry eyes", as they are mucous membranes.
He prescribed "Isopto Homatropine". This is a drop that you put in
the affected eye, and it dialates the pupil and relaxes the eye taking
away the pain, and giving it a chance to rest and heal. They should
be in a dark room as the pupil is dialated and light will cause damage.
About five - ten minutes later eye OINTMENT should be put into the
eye to keep them very moist. We use Refresh Plus Eye Ointment. The
dosage - one drop three to four times a day - and the ointment as
often as needed, even if it is every hour. After the eye begins to
get better, use the eye drops, not the Isopto, again we use Refresh
or Celluvisc Lubricant Eye Drops (I like this better - it is a little
thicker) as often as necessary. When Jenn is on the computer I keep
a box of Celluvisc right next to the PC and remind her often to use
them, even if the eye feels good.
While she is going through the initial beginning problem it is VERY
painful, swollen, red and VERY feverish. I put a few ice cubes in
a clean white face rag and she holds that on her eye. This keeps the
fever down and she uses MANY ice cubes, as they melt fast from the
fever. Both of us dread this problem, as it is so painful for them.
I have no problem keeping her in a dark room, as she usually stays
in bed for the three days, and sleeps.... Dr. Tether said the reason
this happens is that EB will cause very dry eyes. When they sleep
and go through the REM eye movements, their eyes blink and that scratches
the eye lid and cornea, which cause the blisters. Also, as we all
know they sleep with their eyes open slightly. Also, I keep Jenn's
hair back with a twister, especially at night, cause a little piece
of hair that can get in the eye will also irritate the eye and cause
a blister.
Prenatal
Diagnose
There is now a test
that can be done to see if the baby has EB. Then if he/she does, you
will have to make a decision that of course none of us even want to
think of - to abort or not. You will need to contact Dr. Angela Christiano
- email addy: amc65@columbia.edu
to get the details. She is the Dr. who invented this test. She is
also very active in research for EB at a hospital in Philadelphia,
Pa. It has been done and it works. There is another EB mom who decided
to have more children and had this test done. Fortunately the baby
did not have EB and all went well. Good luck.
Infections
and Wound Dressings
A friend, Nancy has
twins, 21 yrs, Katie and Kelley and shared this with me. Since it
is for the good of all I want to share it also.
Regarding infections:
Nancy suggested it is better to rotate topical antibiotics - gentamycin
for about 3 wks to a month, then bactroban, and areas with a lot of
discharge/seepage silvadene. She feels it confuses the bad bacteria,
and I agree.
Some of the antibiotics you may want to mention to your Dr. are:
Cipro, Cephalexin, Rifampin, Sulfa, Minocyclene, Augmentin,
Vancomyacin.
Cipro does not work for Jennifer. Some of these may not work on your
EBer. A bath soak with epsom salts - half a quart size container to
a tub of water. When there is a very stubborn infection that does
not want to respond, try a vinegar bath - about a gallon of vinegar
in a VERY full tub of water. Thanks to Nancy for this info.
For those of you
anticipating Apligraf surgery. Jennifer had this done, and it worked
about 70 % - better than nothing - right. Of course it will slough
off as normal skin does, and then the bad gene will produce more bad
skin - a catch 22. Here are some other options I have learned about:
We are planning to use one of these new options when the next skin
graft is necessary - which appears to be soon - on her chest... :
Oasis, Cook Manufacturing Company, Andy Cron, General Manager, 800-468-1379,
EXT 3456, Fred Roemer, V.P.,800-457-4448 EXT 204. Mention Gary Cummings
from Winfield Labs referred you - I will tell you about that in a
minute.
There is a Silicon Gel Sheeting called Duo Dress manufactured to put
over scar tissue to make it more soft and pliable, and possibly invisible.
The only information I have regarding this is: Mark Dillon, President,
BioMed Sciences, www.Silon.com
I am not too familiar with this. It was mentioned to me by Gary Cummings
and I have not had a chance to research this as yet. When I do, I
will get back to all of you.
Of course there is the Ortec product called CCS (Composite Culture
Skin), which FDA has just approved for use in the U.S. That is the
product I am leaning toward for the next graft Jenn has.
I have been introduced to a wound contact dressing called N-terface
this summer. I find it as good in a lot of ways as Mepitel, and cheaper.
I am not saying Mepitel is not the best, it is just when you can't
afford it you take the second choice. They make several products and
if you contact Gary Cummings, President, Winfield Laboratories, 800-527-4616.
They make the N-terface and also Breakaway, which is a pad to go over
the N-terface to absorb the excess seepage. Gary will send you samples
I am sure. Please tell him I referred you. They are also working on
a "vest" to cover a much larger area for those who need it. It is
new, being introduced at the next meeting in California next week
or two. He is sending me a sample. It is manufactured in China and
is VERY inexpensive. These products work for Jennifer very well.
I just feel it important to share this with you all - it may work
for your EBer also. Please let me know.... As most of you know Jennifer
is just ending the Accutane Study at Chapel Hill - our last visit
is Oct. 1 - 3. 2000. The Study will end in November. The results will
follow a bit of time later as some are not finished for sometime.
It does appear that FDA will approve the 5 year study. Soon as I can
I will post from the results from the start of the Study in March
to the end.
Blisters
on the bottom
For Blisters on the
bottom I suggest using a product called MEPILEX. It does have a sticky
side which goes onto the wound. It will not stick to the wound, but
will around the "good" skin. It comes off very easily with no damage.
It will absorb the "seepage", and can stay on until it is completely
saturated. If it becomes saturated it will come off by itself. Instead
of wrapping between the legs like a "vest", we now use HYAFIX. It
is the only type of tape we can use. It will come off very easily
if you take a 4 x 4 (non-sterile), saturate it will alcohol and ease
the tape off. The little bit of alcohol will not hurt the skin, just
don't get it on the wound. Place the MEPILEX on the wound and surrounding
area about 1", place about 2 - 4 x 4's (sterile) over that, take the
HYAFIX tape and criss cross it over the 4 x 4's, just enough onto
the "good" skin to hold it in place. Leave it on until the seepage
comes onto the 4 x 4's. You can also put your topical antibiotics
onto the MEPILEX. Just keep it away from the outer edges so it will
stick to the skin. If you put the anti close to the edges the MEPILEX
will slide off the wound. The MEPILEX will act like a cushion and
protect the wound. Try it, you'll like it... :-) Also, for those of
us who do not use MEPILEX, or prefer to use something else: in the
past we have also used VISCOPASTE (this is gauze saturated with zinc
oxide, and also MEPITEL. In this case, we cover the MEPITEL and/or
VISCOPASTE with a sort of sponge called ALLEVYN, before we put on
the 4 x 4's. This absorbs the exudate. This is also a good method.
The VISCOPASTE was especially effective. Zinc is good. There is another
product called BREAKAWAY, that has a built in padding. It is also
good. The diaper will also hold the bandage in place. Also, if it
gets wet or dirty, just change the 4 x 4's, that is of course if the
MEPILEX is dirty also. Gee, I don't mean to insult your intelligence..
Well I hope this helps. When Jenn was a baby and until she came with
me in 1997, she had this problem. Now she has the scars to prove it...
We do keep a nursing ointment (we call it BLUE GOO) on the scars if
they look like they are starting to breakdown, and it works....
Jennifer's
Experience w/Anemia
Jennifer had been
going to Chapel Hill, NC to the EB Registry as she was on the pilot
study for Accutane. We go every three months via Angel Flights. They
do thorough lab reports on her each visit, and she is seen by Madeline
Weiner and Dr. Jo-David Fine. In June, 2001 we all noticed that Jenn's
RBC (red blood count) was really dropping. At that time her Hematocrit
was about 26, which is low. Originally it had been at 32, which was
do-able. Normally the Hematocrit should be between 35 - 45. However,
in EBers trends are very important. For example, when Jennifer's Hematocrit
was at 32, she did very well, but the Hematologist wanted it higher
as there is a "leak in her bucket". Will explain that later.
We got in touch with
her doctor here in Indialantic, Fl. Doctor suggested we have her kidneys
checked. Kidneys produce the erythropoietin which stimulates the bone
marrow to make the red cells. The kidney doctor did a thorough exam
and found the kidneys are functioning properly but we needed to see
if they were producing the Erythropoietine. He referred us to a Hematologist
and we saw him sometime in July. When they drew blood the first time
her Hematocrit was down to 23. Very bad. The Hematologist ordered
a "mid-Line" and she was given intravenous iron (ferritin) every day
for two weeks. A mid-line is actually a line that is introduced through
the blood vessel and goes up the arm to the shoulder area. A pic-line
is the same thing, except it goes past the shoulder area and down
close to the heart. With a pic-line you also need an x-ray to be sure
the line is not too close to the heart. This is all done on a out-patient
basis. You should not have them draw any blood from the mid/pic-line
as it will clog up the line and cause a blood clot. This is not a
blood transfusion. However, a blood transfusion works for some
EBer's;
in this case it was not what Jennifer needed. This was done at the
I.V. Therapy Lab. At the end of two weeks they drew blood again, and
the Red Blood Count (RBC) had gone up, slightly, but not enough. The
Hematologist then prescribed Procrit Injections (Erythropoietin) to
be given - I believe this was done once a week, with blood drawn every
two weeks. Procrit is a pharmacological erythopoietin and is usually
used in patients with depressed bone marrow function. This is not
the usual cause of anemia in EB patients and a hematological work-up
should be done prior to treatment with this drug. Other causes of
anemia will not be corrected with Procrit and there are risks involved
with this treatment. Jennifer's RBC and Hematocrit was coming up and
the doctor suggested we continue once a week with the Procrit Injection,
with blood drawn once a month to keep checking the hematocrit and
rbc. Jennifer's hematocrit is now at 37.5, which is good. There is
a pronounced difference in Jenn's energy level and her skin is really
looking good. Jennifer still gets Procrit every Friday, and we see
the Hematologist and have blood drawn once a month. A great description
of why Jennifer may need the Procreit for a long time - maybe not
every week - but every two weeks or once a month - picture a large
bucket full of liquid, with a tiny pin-hole leak in the bottom of
the bucket. If you do not take measurements to keep the bucket full,
eventually you will empty the bucket. EBers bleed constantly - as
their wounds always have some seepage, being blood or some other liquid,
usually blood. So it is very important to keep that "bucket full".
:-)
As we all know, each
EBer is different. This was extremely beneficial for Jennifer and
that is why we want to pass it on. It may be a good thing for your
EBer. Anemia is very prominent in EB and it is so important to keep
up their Red Blood Count and Hematocrit.
Anemia is caused
by a various number of different causes. First you must establish
what is the reason for the Anemia through a Hematologist, and then
follow the advise of that doctor.
Cradle
Cap
We soak the scalp for 15 minutes
with Acidic Solution, and it consist of 8 O.Z of saline and 2 O.Z of White
Vinegar. And then we put the Olive Oil on the scalp and below is how we do that.
We bought Olive Oil and I part Jenn's hair where I can see the areas that are dry and
I take a 4x4 sterile pad and puts some Olive Oil on it and rub it GENTLY to massage the Olive oil in
her head & hair. We put it in my hair every day. If you want it to work leave the Olive oil in the hair for about One day to Two days.. Check for Order, this will give you an idea when to wash the hair or if you can leave it for another day.. If you smell an order you NEED TO WASH hair, and then put the Olive Oil in the hair again.. The scabs that form with come off, but you need to help me a little bit.. If it doesn’t come off will just a LITTLE force then leave it and put Olive Oil on it.. This method has worked for me and I am sure that it will hopefully work for you
all.
Love to all Grammy
Shirley
