Nicky’s Wound Pics Part 2

This is part 2 of a series of posts I will make with wound photos that Facebook does not allow me to post. Post #1 is here.
I took some photos of Nicky’s wounds to be shared at the Hydrotherapy yesterday, June 18, 2012.  I just updated Nicky’s journal about how he is currently doing, it can be viewed here –>
http://www.caringbridge.org/visit/nickyz/journal

I picked 8 photos of his limbs, the knees/wrists are infected with Pseudomonas which we are dealing with. Overall we are pleased, he’s doing good with the combination products I use on his wounds and the heavy usage of Webril to prevent him from hurting himself by scratching, and create a padding that helps him do things he would not otherwise not being able to do… you know, like walking.

Nicky's Left Elbow. This elbow has significantly improved since last year thanks to the Polymem, it was even biopsied at Stanford.

Left Wrist has also significantly improved with the Polymem.

Right Knee and top

Left Knee

Left Knee

Right Knee

Right Hand and wrist

Right wrist

... and he STILL smiles at me despite it all. <3 <3 <3

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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Fecal Hoarding and Constipation

Written by an Anonymous RDEB Patient

I have had these issues since birth and have tried a myriad of medications that have been prescribed or suggested by prior physicians. The list includes mineral oil, senna products (liquid and herbal tea) ex-lax, milk of magnesia, dulcolax, docusate sodium, Metamucil, Miralax, Benefiber and many others that I don’t remember the names of. Ultimately, none of them worked in a manner that was consistent, or the side effects were to severe so outweighed the benefits, or they didn’t help at all.

For anyone who may not understand why constipation and fecal hoarding is such a prevalent problem for EB patients it is because our skin does not have the same elasticity that others do. Therefore, during a bowel movement, the skin of the rectum/anal area is stretched which causes blistering, tearing, splitting, shearing and detaching of many layers of skin. This is EXCRUCIATINGLY painful.

Generally, what happens is the fear and anxiety of having a bowel movement causes a behavioral habit called fecal hoarding which is when a patient deliberately avoids going to the bathroom when the body is trying to naturally expel the matter in an attempt to avoid the pain. What happens then is the matter becomes impacted and the intestines become stretched as more and more matter becomes impacted, thereby causing the size of the feces to become too large to be expelled naturally, as the skin simply would not be able to accommodate its passing. Because of the impaction, the matter becomes very hard and gas builds up in the body, causing painful cramping, as the gas cannot be released from the body, as it has no room to move past the matter.

At this point, the only thing that is going to alleviate the impaction is a series of laxatives, stool softeners and enemas. This is a painful and humiliating process that can sometimes take a few days to complete. The best way to keep this from happening is to make sure that you are ingesting enough fiber and water to allow the fecal matter to be soft so there is less pain when having a bowel movement.

What can also happen is some of the matter can become fluid and is able to slip past the harder matter so there are small bowel movements that are generally very messy but do help relieve some of the painful pressure of the matter that is bearing down on the inside of the intestines and rectum. This can give a false sense of security as one may think that they are having a bowel movement regularly; however, the case may be that only a small amount is being released during these loose movements.

It is a trial and error process of trying different medications or fiber supplements. Either they don’t help, or they help a little but not enough or you spend all your time running back and forth to the bathroom, an sometimes not quite making it there in time, which is humiliating for a person of any age.

When I was in my late 20’s I decided to try something different as I was frustrated by the lack of control I had over my bowel’s and it angered me that this skin disorder was making such a simple action so complicated. I decided to try a more natural approach of simply increasing my fiber and liquid intake. I began eating prunes and drinking more water every day, and it worked! After a day or two, I began having regular bowel movements that were very soft, and while it was still painful, it was not as painful or traumatic as I had been enduring.

After a few weeks, I got tired of prunes so I started looking for other foods or products that would have the same effect. The other benefit of eating foods high in fiber was that there were no side effects like when I was taking the medications.

Please remember that I am simply relaying information that helped ME. I strongly recommend discussing any diet change with your or your child’s doctor before making any serious changes. Also, continue taking medications until discussing any changes with the prescribing physician.

Below is a list of high fiber fruits and veggies and snacks

Please remember that FRESH fruits and veggies have much more fiber, vitamins and minerals than canned.

Prunes  – Peaches  – Cantaloupe  – Watermelon  – All green veggies
– Plums – Nectarines – Grapes – Grapefruit – Honey Dew Melon –
Apricots – Apples – Pears – Oranges – Mandarin – Oranges

  1. Salad (darker lettuce and baby spinach has more fiber, vitamins and minerals than iceberg lettuce)
  2. Pretty much ALL forms of BEANS are high in fiber (they are effective although they do create more gas)
  3. Whole wheat bread (Brownberry whole wheat has 3 grams per slice and is the best tasting whole wheat I’ve found…It has a sweet taste and is yummy : )
  4. Weight watcher brand muffins and dessert cakes (generally found in the bread aisle)
  5. Slim fast brand muffin bars
  6. Polaner all fruit with added fiber
  7. Shredded wheat cereals (Fiber One brand has a shredded wheat cereal with a higher fiber content than regular brands)
  8. Oatmeal (several brands have many flavors with more fiber or added fiber)
  9. Fiber One pop tarts
  10. Fiber One brownies
  11. Boost Plus supplement shakes

For infants and those that are not crawling or walking, it is helpful to massage the abdomen as well as pushing the legs up towards the abdomen area (as if riding a bicycle). This helps move the fecal matter through the intestines. If the matter is firm or hard it can hurt as it moves through the intestines (cramping) as the tissue is sensitive. It best to limit milk intake as it does not provide as much hydration as water or juices. You can water milk down or add ice to it and add a touch of sweetner to make it taste better. I recommend using stevia or Agave Nectar sweeteners as they are all natural, do not raise blood sugar levels or cause chemical reactions (mood swings, hyperactivity etc…)

It is imperative that you increase your water intake (fluid) if you increase your fiber intake as the body pulls the water into the intestines to allow the fecal matter to soften. If you don’t drink enough water then your body is not hydrated enough to complete this process and the matter will become harder.

Below is a photo of the rectum of an RDEB patient. It depicts the trauma caused to the rectal area by the stretching of the skin from passing fecal matter. There is a permanent tear in the rectal wall from repeated ripping of the tissue during bowel movements.

 

Nicky’s Wound Pics

I struggled a lot to make this, the first post on this site particularly for Nicky’s wounds. In the past I’ve been victimized by many to do so, even from EB patients and other parents, Facebook nearly deleted my account for posting photos (you can read about that HERE), *but* I always feel as if we’re “sanitizing” EB for mass consumption, particularly RDEB since its wounds are rather deep and nasty, and showing photos of Nicky all bandaged up I never felt served the purpose it needed to serve, SHOWING the reality of EB.

So, from time to time, I will start posting wound pics as I take them. More wound pics for the Recessive Dystrophic form can be seen HERE

I picked three photographs that I took just the other day. One shows the ankle, one the top of his foot and one a knee. It’s important for me to say a few things about these pictures:
1. Nicky is perfectly OK and has *always been* perfectly OK with me posting these. Go ahead, ask him!
2. His knee and ankle are his worse spots by far. His ankle was damaged at birth from those hospital bracelets and while it always healed up to the time he was 5 or 6, it’s been several years now that no matter what we do and what products we use, it may heal partly but it will blister up again.

NIcky's ankle

The top of his foot-hasn't healed in ages. We use Polymem on it now and it seems to help a lot!

Nicky's knee

I also wanted to say that even though Nicky’s wounds are not much worse per se than when he was smaller, there are more of them and take much longer to heal or they do not heal at all. They are also much more painful to him, whose every step is an agony, who cannot walk straight because his knees are too painful to straighten. Lately his ankle has made it so it’s even MORE painful to flatten his feet. The toll having EB has taken after 15 years it’s something I truly cannot describe.

Next time I will post photos of his arms/hands. Thank you for keeping your comments kind. 🙂

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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EB wounds offensive to Facebook?

Apparently the daily life of my son is offensive to Facebook and whoever ‘reported’ this photo to Facebook as a violation of the statement of rights and responsabilities. Huh?

No, this photo isn’t pretty. That’s the point of posting it, isn’t it? To SHOW what EB is really like. To show the suffering EB patients endure. We’ve seen much, much worse photos on facebook, from gory, since it was just Halloween, to photos of drunk, disgusting partying sexy people, yet a photo of a child suffering gets removed?

It all started during EB Awareness Week, which is the last week of October; people are used to seeing EB patients wrapped in bandages, so they have no idea what goes on underneath the bandages. So, I posted wound pics I’ve taken over the years. Many of these pics had been on my profile for years without as much as a peep. The first photo they removed last week was over 2 years old. This one I posted several months ago.

I have had comments on this website from people assuming the wounds EBers get are like rashes, or bruises. Nothing could be further from the truth. Nicky has RDEB-Recessive Dystrophic Epidermolysis Bullosa, which means that when he gets a blister, the blister is deep. So deep in fact that it tears away the entire epidermys and even part of the dermis, leaving 2nd degree burn-like wounds. It’s horrible, it’s painful and it’s lethal. Children born with the more severe forms of EB live in cosntant pain, full of procedures and bandages. Some with the Junctional Herlitz form rarely survive to see their first birthday. Those with Recessive Dystrophic are always battling infections and a great amount of them die from skin cancer.

There is no cure.

There are a handful of organizations on the verge of finding a treatment, if you do NOTHING else today, share the link of this page to someone who you think might help. The organizations to donate to for a cure are either the EBMRF (http://ebkids.org) or DEBRA (http://debra.org)

The posting on Facebook that explains more of what the ramifications to me were for posting this picture and to comment is here: http://fb.me/13PkgqMHE

Yes, my second warning, my second wound pic they deleted, which forced me to remove all the wound pics from my profile.

Maybe Facebook should stop policing EB families and start donating to find a cure. Who agrees with me?

UPDATE 11/9/2011
I wanted to take a moment and thank everyone for the kind words. One thing I must clarify, however, is that while some feel Facebook is not at all to be “responsible” since it’s all ‘automatic’, actually that is not true.
A lawyer friend looked into this ‘Rights & Responsabilities’ issue and apparently the way this works, FB only looks at the photo once someone complains. If they deem the photo worthy of removal they can remove it and their decision is FINAL. There is no recourse. Basically if you sign up with an account with FB you agree to their rules, and if you don’t like the rules, too bad.
From now on clinical (wound) photos will only be available on my THIS (ebinfoworld.com) website, which is a site I PAY FOR, so I have rights. Because of my twitter campaign about this yesterday and this morning, many people are now aware of EB, so at least something GOOD comes out of Facebook’s abscence of a heart. {{HUGS}}

Mom to 3 boys, 1 in heaven, 2 on earth. My first son Alex (whose demise is most likely EB related) was stillborn at full term. After a miscarriage, I had my second son Nicky, who has the Recessive Dystrophic form of Epidermolysis Bullosa. My youngest son, Connor, is 100% healthy, and I never, ever take it for granted. I am an author, photographer, graphic artist, webmaster, blogger and more.

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Hints From Grandma Shirley

Grandma Shirley holding Nicky

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…. another thing that we have found as far as this is concerned has to do with bedding because it can be quite uncomfortable to sleep. We have found that babies sleep better on a sheepskin blanket.

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 4×4 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

Hand Wrapping Instructions

By Lorraine Spaulding

The following illustrations are an example of a hand wrapping technique found to be helpful in the protection and preservation of RDEB involved hands. The instructions are for very small hands and some adjustments probably need to be made for larger hands. Lorraine also have some suggestions if anyone is having trouble with the thumb pulling in.

Please note: these instructions may seem overwhelming at first, but after a little practice and as your child gets used to the process, it becomes a worthwhile routine.

Keep in mind the positive results and don’t get discouraged!

Note from the webmaster: this is a graphic intense page. I recommend waiting for it to load and then print it out.

WATCH THE VIDEO OF THIS DEMONSTRATION AT THE STANFORD WEBSITE:

http://dermatology.stanford.edu/gsdc/eb_clinic/eb-videos.html

All illustrations are of the left hand.

Step 1 is an optional application of Vaseline Gauze to the palm of the hand. Use a 3″ X 9″ piece of Vaseline Gauze. Cut about 2″ off one end, then slit lengthwise about a 4″ length, about 1″ down from the top edge.

Wrap around hand with 1″ strip between thumb and index finger.

This provides some resistance from retracting.

Fingertips can be left exposed to some degree, so that tactile sensation can be experienced.

At this point it may be necessary to start a 2nd roll of 1″ gauze. Make one overlap and continue.

Views 10-19 Front of Hand

Spaces in palm bandaging will be covered by criss/cross bandaging of web spaces.

Web spacing can be randomly done. The point is to cover any open areas of the palm while

gently pulling a wrap between each web space

Be very careful to never wrap or pull too tight!

Be sure to have slight tension in web spaces.

Suggest wrapping additional 1/2 roll of bulky bandage around arm from the wrist to the upper arm. (Held by Tube Gauze) Provides padding of forearm and elbow.

Wrapping Hands & Arms The ABC Way

By Sheri Coil – with  the assistance of Brandi

The following is the way we wrap the hands (and arms while we are so near) to reduce the mittening deformity that RDEB causes.

After cleansing and debriding I use a lot of Alwyn cream or what ever else I have used before it came along.  The open wound or skinless areas get Mepital first, then Vaseline gauze.

We have had excellent luck with the Mepital preventing sticking bandages. The oozy serum stuff can drain right through it to be absorbed by outer gauze.  And the creams and ointments can get back to the wounds.  The  Vaseline puts up a further barrier to sticking.

The gauze starts on the top of the hand.  I use Kendall conform 1″.  You can see the Mepital under the Vaseline gauze.  Please note that the wounds on this arm are all self inflicted from scratching. The areas she doesn’t scratch are very clean.  I used this area to demo on because it needs the works as far as under gauzes

The gauze then goes around the thumb a couple of times, until it is wrapped and then  around the hand, over the top and around the first finger,  around it til wrapped, and around the hand to the next finger…..

After the fingers are wrapped, I go around the hand and come up through each web space.   Two going around the first way and then  an extra half wrap around the wrist to make the last one cross over the first two.  Clear as mud, huh?  Ggg

Then I wrap a two inch Kendall  Conform bandage over the wrist, around the hand one loop and then through the thumb space and on around the arm on up Coming back down the arm, I pad the armpit, elbow and anywhere bad and roll the bandage right over it.  I use Kerlix for that padding, cut to what I need.  I just roll the bandage on.

No pulling, but not loose.  It just needs to support the skin, not cut off circulation or cause mis-shaping over time.

I  use tube gauze over everything.  I do not like to use tape at all.

Here she is, all ready to go! 🙂 Our Dancing Queen 🙂

Here is the Video of the wrapping technique recorded in January 1998

Common Misconceptions about EB

Through the years of running this website I’ve been asked questions about Epidermolysis Bullosa that follow in the category of misconceptions. People believing some things regarding this condition that are false. This is not only from strangers, friends and family, but from the medical community, as well as actual EB patients and parents. I’ve asked other parents and patients to give me their input of what the most common misconception about EB is that they have encountered and that ishow I came up with this page. I add to it regularly when new misconceptions arise.

Here are the most common ones in an effort to educate everyone about EB. Please note… I am posting most of these questions verbatim, exactly as they were asked.

Q.Is EB caused by the parent in any way? By their use of illegal or legal drugs? By them using too much sweet-and-low in their coffee or anything of sort?

A. Epidermolysis Bullosa is a GENETIC condition, much like Down Syndrome, Cystic Fibrosis or Tourette Syndrome.The gene that causes EB was finally found in 1993. Before then there was a lot of speculation as stated in the question, and much fault was put on the parents. EB per se was first written about in the late 1800s, before then surely patients would die and nobody knew why. EB has been around forever because it is an equal opportunity disorder, being equally present in ALL RACES and GENDER. Parents have absolutely NO responsibility and are not ever to blame over how it manifests, as it is in our genes, much like we have blue eyes or blonde hair.
In brief, some forms of EB, such as Recessive Dystrophic and any form of Junctional, are indeed a gene that is recessiveand it only shows up if mom AND dad are both recessive carriers, healthy carriers. But the baby must inherit BOTH to have the condition. If he/she only inherits one he/she will be a healthy carrier like mom and dad. Hence two healthy carriers have a 25% chance of having a child with EB.
Dominant Dystrophic and Simplex are ‘flukes’ at conception, much like many other conditions, such as Trisomy 18, and once the patient has it, it is then a dominant condition, hence the patient has 50% of giving it to their children.

Q. Can antibiotics CURE EB?

A. EB is not an infection, it is a genetic defect. No antibiotic could cure EB, much like an antibiotic cannot cure Cystic Fibrosis or Down Syndrome.

Q. There must be a way to prevent EB, isn’t? What precautions can parents take?

Because EB is not a disease, but a genetic defect, there is nothing that can truly be done to prevent it. In most cases, the parents are oblivious they are even carriers, and since the DNA is so tricky regarding this, they can’t even be tested for it. In the cases where this is a ‘fluke’ at conception, well, that certainly can’t be prevented.

Q. If the slightest touch hurts the skin, how come bandages don’t? I don’t get what it means by “touch” or”contact.” How do EB patients lie in bed, sit down, hugged, carried,etc?

A.The answer is two-fold, because it really depends on the form of EB.

With Simplex EB, indeed bandages “might” cause more harm than good. The wounds are superficial after a slight bump, and bandages cause sweat that can cause more blisters. This, of course, if the wound is rather small. If a wound is large it may need to be bandaged anyway to promote healing. In simplex, and especially with babies, a small pressure of the skin will cause a blister, bandaging does not press the skin, hence does not usually cause blisters.

In RDEB (Recessive Dystrophic) the blisters are from friction, not pressure, hence the bandages actually help getting less blisters because they act like protection against the normal scratches and bumps that would tear up the skin immediately.

Patients can lie in bed, but they usually have to have very soft sheets or padding of some kind. Sitting is okay, but a lot of patients do need some extra padding on a hard chair. Patients can be hugged gently. Patients will need to be carried in a way that does not cause any kind of friction, absolutely NOT under the armpit for example, and more cradled like babies, without using hands but arms under the buttocks or legs.

Q. Do you use antibiotics all the time to treat infections?

A. Unfortunately if the body is given too many antibiotics all the time, such as bactroban, it can build a resistance to it. Hence it is vital to make sure the patients develops a strong immune system to fight infection on their own and not always rely on antibiotics of any kind. Antibiotics should be used sparingly and onlyi f they are really needed to ensure that when they are indeed needed, they will WORK!

Q. Are EB blisters like rashes, red areas or sores of Eczema or Psoriasis patients? Is EB just perhaps a more severe form of these conditions?

A. No. EB blisters are indeed wounds, not rashes orsores. In the cases of RD they are second degree burn-like wounds-the kind they hospitalize people for. Eczema and Psoriases are NEVER, EVER to be confused with EB. Even in theire xtreme cases they are vastly different from EB, and never as serious. It needs to be understood that the blisters normal people get are not the kind of blisters that EB patients get. They are much, much more severe.The protein that acts as a glue between the layers of skin is not produced or not enough of, causing the skin to peel off. Leaving 2nd degree burn-like wounds on RD patients.

Q. Won’t wrapping the hands cause children with EB to be very behind for their age group with using their hands?

A. The first thing to understand that is very important is that it is cruel to “expect” any child with EB with their fragile hands (and this is most especially for those children with the more severe forms of EB) to be able to do all the things a normal child does. I am not saying here to ‘not let the children try things’ , what I am saying is ‘do not expect’, big difference. Their little hands hurt very easily, and they “might” (depending on severity)never be able to do even a fraction of what healthy children can do, so, as advice to parents, it’s always suggested to throw out the window the notion that your child with severe EB can even remotely have normal hand use and to expect your child to be behind in this area. Having said this, things vary differently from form to form. With the more severe forms of EB it is more important for the child’s hand to be protected and safe than worry about development-children WILL learn to do things, adapt andd evelop at their own pace and figure out a way to do things.
There will never be a need to wrap or protect the hand of a child with a mild form of EB at all (such as DDEB, Simplex andJ unctional non-herlitz), but it is essential with severe forms, and especially so with the disfiguring form, which is Recessive Dystrophic.The hands of children with Recessive Dystrophic, if not wrapped, WILL web and contract, becoming unusable or close to it as time goes on, needing painful reconstructive hand surgeries to restore at least some function. Hence, with RD, the wrapping serves more than protection from wounds orcovering wounds, it literally is a form of physical therapy, because by using slight pressure the fingers are kept apart and straight. The gauze also serves by giving the patient’s fragile skin a little more strength in pulling and pushing.

Q. Do people need to keep their distance from EB patients so they won’t catch it?

A. EB is not a disease or an illness like Chicken Pox or HIV. It cannot be ‘caught’ by being close to a patient, much like youc annot ‘catch’ Down Syndrome by hugging them or sharing a drink with them. EB is a birth defect, the defect lies in the DNA, in the genes. It is perfectly safe to hug and kiss an EB patient, EB is not contagious.

Q. How can twins be born one with EB and one without if EB is a genetic condition?

A. While that is true for identical twins (meaning, if one identical twin has the condition, the other one, by virtue of the DNA being identical, must have it as well), that is not always the case for fraternal twins. In fraternal twins it is very possible to have babies where one has a genetic condition like EB, and one not. Just like one baby can be born with down syndrome, and the fraternal twin be perfectly healthy. A recessive condition has a 25% chance of developing, and it’s ar oll of the dice each pregnancy/baby.

Q. I saw on TV a show about a child with EB who got skin grafts and she is now basically cured! Why can’t all patients get this skin graft?

A. Dateline NBC had a special in conjunction with People Magazine about Medical Miracles in the summer of 2001. The child that received this treatment, Tori Cameron, was the first EB child to receive a skin graft called Apligraf in an effort to heal her extensive wounds. Tori suffers from the Simplex, Dowling Meara form of EB, which can be quite severe at birth. Because she was the first patient to receive it, and it worked in closing her wounds, she made national news and appeared in shows such as Headline News, Extra and Dateline. Apligraf has since been used in many patients in an effort to close their wounds.
2 things are important to know:
1. Tori’s form of EB dramatically improves with age regardless of any skin graft.
2. Apligraf only has about a 50% success rate, but all it does is close the wound, it does not cure EB because skin grafts cannot alter the patient’s DNA unless they are made to do that specifically. The patient can and will eventually re-blister in the spot where the Apligraf was placed. Stanford is working on skin grafts which are made to specifically replace the patient’s skin, and in that case they would indeed ‘cure’ that area where the skin graft will be put, but the treatment is a few years away at best.

Q. Does EB spontaneously appear at 3, 4 or 5 years of age? Could it be a side-effect from vaccinations? My girlfriend is 45. She says her EB showed up when she was 5 years old.

A. Patients are definitely born with EB. EB is a genetic disorder, and to say that it didn’t “show up” until the patient was 5 years old is like saying that the baby was born healthy and then at 5 years old all of a sudden he/she had Down Syndrome! It just cannot be. Maybe your girlfriend was very mild before and then something triggered it to make it worse or does not have EB at all. No vaccination could ever cause this, because vaccinations do not alter the DNA. The vast majority of patient’s EB is diagnosed immediately or when it starts to show which is usually within the first few months of life. In some babies it does not show right away because the baby still has some of mom’s good fluids in its system. There is only one form of EB (aquisita)that all of a sudden shows up in later years (there is one documented case of a patient being in his 30s, usually it is much later, 60s+) when the body becomes weak and stops producing enough of the protein, but that is very rare.

Q. There must be a lotion or a cream that can heal that right up! I bet that products for sensitive skin would heal EB!

A. Creams and Lotions can help in the healing, but because this is a genetic defect, they will not get rid of the condition per se. EB cannot be cured by creams and lotions just like Sickle Cell Disease cannot be cured this way. Genetic Conditions can’t be cured with any sort of cream unless they can alter the patient’s DNA.

Q. Why does bleach in baths helps EB patients?

A. Bleach Baths help because they kill germs that cause infections. Infection is the #1 killer of EB patients.

Q. If you starve a baby long enough, she will nurse or eat.

A. While this might be true for healthy children with no mouth involvement, children with EB of any form can and will blister and have raw areas in their mouth, throat and esophagus making it extremely painful to eat. Doctors not familiar with EB have told parents not to force their babies to eat, that if they get hungry enough they would suck and swallow their milk. These babies are in too much pain to actually eat, and they can and will act hungry and will keep dropping weight. It is in these instances that a G-tube becomes a life savingoption. The point is, don’t starve your babies! Get help.

Q. I heard that a high protein diet causes blisters. Since EB patients need to be on high protein diets, could it be that that is the reason why they blister?

A. Patients lose so much blood and nutrients from their wounds, they need to get high protein from anywhere they can find. Wounds can cover most of an RD patient’s body, hence the body needs to make it up somehow. No high protein diet would ever cause any symptom that is anywhere close to what RDEB patients endure

Q. Don’t Herbs and Plant treatments help cure EB?

A. During the time that it was not known what caused EB (before it became known that it is a genetic condition) patients were often treated and maltreated, put through every herb and plant treatment known. Nothing ever worked. Whether herb or plant treatments can somewhat help EB symptoms has never been fully proved, surely they can’t hurt, but it is an impossibility for these to cure EB unless they can alter the patient’s DNA.

Q. Can EB disappear, I mean, completely disappear with age?

A. EB can’t per se disappear because the mutation lies in the genes. EB is not a virus or a disease, it’s a genetic mutation, and the genes don’t mutate by themselves. There is an extremely rare form of EB called ‘transient of the newborn’ where it does go away around the child’s first birthday, but the patient’s DNA was not altered per se, the child simply had a late start on producing the missing protein.

Q. There must be a typical diet for EB patients, isn’t there?

A. There is no typical diet for EB patients, because every patient is different. Those whose mouth is sore with wounds can only eat usually mushy liquid fluids, those whose throat and esophagusis damaged, they get the tube. Most others can eat whatever they want with caution, of course.

Q. Why do patients get EB?

A. … for the same reason why you have blue eyes or brown hair, or get Down Syndrome instead of Sickle Cell Disease, or why some women get Breast Cancer at 30 and some at 60, and some never get it. There is no rhyme or reason, sometimes is just bad luck, or something inthe genes that is incorrect… every human being is a carrier of at least 7 potential gene defects lying dormant in their system, waiting to be passed on to the next generation. Some get lucky and don’t pass it, some however…

Q. I bet there are no books about EB.

A. For books about EB please refer to the Books Category on this website.

Q. Do patients recover from EB?

A. There is no way anyone could ever recover from EB. Patients with the simplex form improve in the first years of life, and then again somewhat improve after puberty, but that’s all. Think about it like this. Do your blue eyes turn brown later in life? No. That is because it’s in the genes. It is impossible for a patient to lose their blue eyes like it’s impossible for them to lose their EB or their Down Syndrome. As far as if the condition gets worse or improves with age, that depends on the form. Simplex improves with age, Recessive Dystrophic gets worse due to the constant breakdown of the skin and severe scarring.

Q. Nobody cares about EB because is so rare, I bet there is no one looking for a cure.

A. Stanford is working on Gene Therapy for RDEBat the moment with success. So is a Dr in Italy. The University of Minnesota has started Bone Marrow Transplants on young RDEB children and some have been successfull, particularly those that have a sibling donor. There are injection therapy trials all over the world. There is hope.

Q. Does a skin area damaged by the EB get deep red and sore and itch and be hot to touch at one moment, and then maybe an hour later, be more faded, a more normal skin temperature, and maybe just itch a little?

A. A blister on an EB patient is a wound. Is not a red area that an hour later is more faded. A wound is a wound and needs to be treated as such. On RD patients it’s actually a second degree burn-like wound.

Q. Do all EB patients have Allergies?

A. Not all EB patients have allergies. It just depends if they are predisposed for it. I know many EB patients who are not allergic to anything, it just depends on their genetic make up like normal people.

Q. EB patients cannot be out in the sun, right? I bet they blister in the sun.

A. EB Patients do not sunburn easier or faster than anyone, wowever, a bad sunburn would be surely quite more painful and severe than for you and me.

Q. Why do patients die of EB?

A. EB patients don’t die from EB. They die of infections, anemia, organs that are weak because of lack of nutrition or secondary damage or skin cancer. All of these are side-effects of EB.

Q. Are all forms of EB considered ‘Lethal’?

A. Any condition that is labeled ‘Lethal’ (also known as TERMINAL) implies that, upon diagnose, the patient will die from the condition sooner or later. The only two forms of EB that are considered lethal are Junctional-Herlitz and other Junctional forms (such as Pyloric Atresia) and RDEB. Junctional babies usually die before their first birthday, while RDEB patients live a painful life that can range from 0-30+ years in most cases, although there have been patients that have lived longer. The causes vary, usually ranging from severe Anemia, Infection and Skin Cancer.
Yes, other forms of EB ‘can’ be lethal too, but not by definition, as in the vast majority of cases the patient has a normal lifespan. Some old textbooks and Doctors will say that babies born with a rather severe form of EB Simplex called Dowling Meara have a 25% chance of dying their first year of life, but the validity of that statement has become questionable in recent years due to advances of antibiotics and wound care. The National EB Registry lists EBS-DM patients as having onlya 1.44% chance of dying at any age.

Q. All EB patients can die of Cancer, right?

A. Yes and No, it depends on the form. While there is only a minute chance of Simplex patients to develop Cancer (1 in 100 in their lifetime), with RDEB patients, in most cases, it is unfortunately not a matter of IF, but a matter of WHEN.
I found this very interesting page with Cancer in EB patients statistics you mightwant to check out: http://www.med.unc.edu/derm/nebr_site/cancer.htm
Basically, up to age 40, there are virtually NO instances of SCC (Squamous Cell Carcinoma) for EB patients unless they have RDEB. RDEB patients have a 6% chance of getting SCC at 20 years of age, 21% chance at 25 years of age, nearly 40% at 30 and 53% at 35. Even at 60 years of age, Junctional patients have never been reported statistically as getting SCC at all, simplex patients only have a minute chance of getting SCC, barely over 1%, DDEB patients almost 4% at 60 years age, but, in contrast, a 76% chance for RDEB patients.
As far as Malignant Melanoma, this is actually fairly rare to get, barely 1-2% by 55 years of age with all forms of EB except for Junctional, with a 0% instance.
For Basal Cell Carcinoma, they state that by 40 years of age <1% of all patients with EB simplex and DDEB have experienced a BCC, higher with RDEB, with 4%

Q. What is the medical explanation for some people with EB living to be 72 and others maybe only 10?

A. One word: severity. The patient that dies at 72 age has a much milder form of the condition than the 10 year old. It is also true that perhaps the 72 year old was much better cared for than the 10 year old. Perhaps the parents of the 10 year old were living in a poor country or were not aware of how to properly care for their child. It is all up for debate, however, in most cases, it is a case of severity.

Q. Do all EB patients have to have a G-tube?

A. Again, this depends on the form and severity of their form. Simplex rarely has problems in the mouth, but IT DOES HAPPEN! Most if not all Recessive Dystrophic patients, however, can and will blister everywhere…lips, tongue, gums, mouth, throat esophagus. Some patients may be able to eat enough by mouth (which is then usually mushy or liquid foods) and others cannot, that is why those patients might end up with a Gtube.

Q. I believe mouth blisters might not be from having EB, but from the dental fluoride treatment or the toothpaste with fluoride, or the children’s multivitamin with fluoride, etc. Which the parent thinks is good for the child!

A. Fluoride could never cause the damage to EB children’s mouths that I have witnessed. If so, any child with fluoride treatments would have their gums webbed, tongue adhered to the bottom ofthe mouth, and scarring in the esophagus, which, as we know, does not happen. Nicky was not on fluoride treatments when his mouth blistering started (at birth) and is not now, although he should, and he still blisters badly.

Q. I think that if EB patients could get laser treatments to ‘burn the scars off’, healthy skin would grow underneath!

A. I myself do not even know if the above statement would even work on a healthy patient, but even if it was true, and, for conversation’s sake, let’s say it is true and the scars would ‘burn off’ and healthy skin would grow underneath on a healthy patient and it was tried on an EB patient, even if the treatment would work, the patient would still have EB after all is said and done. He/she would still get blisters and still scar. This is because EB is a genetic defect, and no laser treatment can cure EB unless it can magically alter the DNA of the EB patient.

Q. Instead of wrapping these children from head to toe, isn’t better to dry out the wounds?

A. While that is possibly the preferred method with most children with simplex, very mild dystrophic and even some junctional non-herlitz patients, simply because the wounds are superficial and don’t normally scar because they lie within the epidermis, or the wounds are few, letting the wounds dry with moderate to severe Dystrophic patients means they will scar and also cause deformities in the extremities. Wounds can also develop infections if air dried, and it has been observed that moderate to severe children with RD that are never wrapped have a much shorter life span because most of their little bodies are covered with scars. A scar is weak skin that can brake down much easier than an area that was properly healed, hence these children develop huge areas of weak skin that once breaks down will be open forever and have a high chance of developing nasty infections and even become cancerous in later years.
Bandages for RD patients serve many purposes: They allow for proper healing of wounds, they protect the healthy skin from braking down in the first place, and they allow the patient to do much more than without it. Most patients could not even walk without bandages on their feet. In areas where a bandage cannot be put, it is strongly recommended to keep the area moist by applying either zinc oxide 40% or Aquafor or any ointment twice a day to allow moisture to penetrate the wound and heal it properly.

Q. Is it common for those with RDEB to need diapers? If so may I ask the cause??

A. Many parents of severe RDEB children have many issues regarding toileting. Many children just can’t walk to the toilet and/or may not be able to take their underwear down if they tried due to wounds on their hands or deformities. Others can’t sit on the toilet due to many wounds on their bottom, others yet hold it in constantly because they are afraid of skin tearing in the anus and are extremely constipated. This is a psychological issue that many patients with this form of EB have, and it may not be until they are older that they can solve it, because they have to want to solve it themselves. No trying talks or bribes truly work. Children that get accustomed with holding poop in end up leaking poop all day long which makes it impossible for them to wear any underwear. The only way they’ll go is if enough laxatives are given so they have no choice but go because it’s basically liquid, which makes it even more impossible for them to wear underwear. The fact that RDEB children are anemic and we have to give them iron does not help either, since iron constipates. It’s a losing battle and for many severe RDEB children diapers are the only answer…

Q. Why do some RDEB patients require blood transfusions and why are family Drs suggesting to have this done? In what ways does it help??

A. My Nicky’s pediatrician referred him to a hematologist because Nicky had no energy and he had been severely anemic for a very long time. Nicky’s hematologistis is Dr. Coates, which spoke at the conference about EBers problems related to anemia. He is not only an expert hematologist, he is an expert in hematology and how it relates to EB.

Nicky’s blood count had been dangerously low for along time, but at this time they were below borderline. Having a very low count means he’s extremely anemic, which means that Nicky’s life was in danger if nothing was done. A blood transfusion gives him some iron-rich bloodplus trasferrin (sp?) which he was also missing. Transferrin is something the blood needs to have to absorb iron in foods and supplements and that somehow many severe RDEB patients lose after several years of bleeding through the wounds. After a couple of blood transfusions he started iron-transfusions which work better since his blood now has the trasferrin which enables his blood to absorb the iron.

Most RDEBers have this problem and a few children have even died because of it…

Q. For those w/spontaneous mutations for the first time in the family, I am not totally convinced it isn’t something in the environment. I understand that the recessive types occur when two people carry the gene and if you have a dominant type and pass that on as well… but I still don’t understand how it could just occur out of the blue…?? Especially when there are other types out there you have to have/carry for your child to have it and it’s called the same thing? Are the other conditions that can occur spontaneously as well? 

A. There are alot of conditions out there that are ‘flukes’ at conception, so many things can go wrong during conception I often wonder the wonder of nature of how many people are actually born healthy to be honest! LOL. But… I digress…

A few years ago there was a group of Vietnam Vets that contacted me because many of them had children with EB Simplex, which as we know, it’s a dominant condition and a fluke at conception. They thought there was something to it. We went back and forth for a while, and we never came back to a conclusion. It is possible.

As per your question regarding how a condition that is a fluke at conception/dominant can be associated to a recessive condition that someone carries… the answer is actually quite simple. It’s because of the nature of the beast. Even though they are two different beasts, they are associated because the symptoms are similar:Blistering. You have to remember that the word EB is just an ‘umbrella’ for various blistering conditions, something Doctors associated more than a hundred years ago. We often speak how Simplex is vastly different from Dystrophic and how Dystrophic is vastly different from Junctional etcetera, so different that they are treated quite different and the same type of products do not work from one form to another. They should not be listed with the same name (EB), but they are because it was something done before they even knew of all the differences. The word EB per se means the blistering of the epidermis, and if you think about it, it doesn’t really fit the dystrophic forms because the blistering forms in the dermis, not in the epidermis! But, again at the time EB was named and all the forms associated, they weren’t even aware of any differences, and now it’s too late to change it!

Q. I saw a documentary about a man with EB and the narrator stated that he never went through puberty. Why is that? What causes it? Is this a common problem with EB patients?

A. It’s important to understand that going through puberty requires a lot of calories and nutrition. In general, people who do not have much food to eat or are unable to eat much will have a delayed puberty or will not go through puberty at all, and this is the main reason why RDEB patients sometimes do not go through this stage of life. Why is that? RDEB patients have a scarred and strictured esophagus, and eating is always a big problem in general due to the mouth and throat being prone to easy blistering and painful sores. This is not the case for Simplex or Junctional patients, as their mouth, throat and esophagus is not normally damaged nor CAN get damaged to the extent that they cannot eat enough to sustain them. However, it is a HUGE problem for RDEB patients. My son Nicky, who is 8.5 years old as I write this, most likely would not be alive today without his g-tube and his constant throat dilatations to enlarge his esophagus that has been so closed up, the passing was only 1mm large. Because of the g-tube feedings now he is as big as a normal 8.5 year old would be and will most likely go through puberty without any problems. G-tubes have only been around since the early 90s.

Q. I noticed that RDEB patients seem to have a discoloration of the skin. Why is that? 

A. The skin is not really discolored. Most RDEB patients are very anemic due to the heavy loss of blood through their wounds, and lack of iron in anyone’s blood will make the skin look very pale.

Q. Does EB effect the teeth, and if so, how? Does it affect the gums? I was looking at a picture of a child with RDEB (Nicky) and noticed that the teeth looked different.

A. While some forms of EB (namely, Junctional) do effect the teeth (Junctional patients have very fragile teeth), the form of EB Nicky has (RDEB) does not effect his teeth per se. However, the problem arises in the fact that he cannot brush his teeth like normal people. Brushing his teeth like we do will cause the skin of his gums to fall off and would be excruciatingly painful. That is why many RDEB patients do lose all their teeth eventually, simply because of the inability to clean them properly. Mouthwashes and other methods have worked a little, but never fully.

More Questions and Answers that were sent to us.

Q. Is it the type of shoes you wear that causes the blisters?

A. No, I can get the blisters while goingbarefooted. I have the Localized Simplex Weber-cockayne disorder. I getthis question all of the time. ~Cindy

Q. I was scarred from burns all over my body. I went to a herbal doctor, and got on an herbal diet, and now my wounds are healed and I have little or no scarring. Can’t you do that for your son who has DDEB?

A. This question was asked to me by someone in a court house who happened to see my son’s DDEB from down the hall. Although herbal medicine, may help a little, it will not cure EB. Herbal medicine cannot change your genes. Since EB is a genetic defect, herbal medicine may help with healing, but will never CURE EB. ~Dawn

Q&A about Recessive Dystrophic EB -Hallopeau-Siemens 

Will the skin get better with age?

No. With RD, the more it has broken down, the easier it breaks down the next time. Andeventually, it does not heal at all. It is open to such a depth that it is extremely prone to serious infection. Strictures, mittening and syndactilly occur. Fifty percent of RD patients beyond the age of 10 develop Squamous Cell Carcinoma. There are RD patients who had multiple amputations before succumbing to this cancer. When the average person with RD passes away, the breakdown of skin is to the point that 75% of tissue lacks the ability to regenerate. The bottom line is, the skin does *not*get better in RDEB as it can with Simplex diagnosed individuals.

How healthy are RD patients?

Individuals with RDEB suffer from extreme malnutrition and are notoriously underweight. Some never even undergo puberty. The body puts all of its resources and energy into healing and fighting off infections, and growth and development come last. The 80-pound range seems to be a top figure for weight. Internal damage contributes. The mouth is constantly a mass of open sores. It strictures as it heals, causing macrostomia, tied tongues, and reduced ability to eat and drink. Esophageal dilatations are a way of life for many. G-tubes are common.

Does it get easier to handle emotionally?

Do you feel more upbeat as your child fades and is in more and more pain? Of course not. And even worse, you are the one that HAS to cause much of that pain, because if you don’t–the consequences are unthinkable. You have todo the zombie thing and poke and cut and bathe, smear medicine on areas too sore to even expose to the air without hurting them, and bandage thec hild. This is against everything that is in the handbook of lovingparenthood, of nurturing. Deliberately causing pain, even though it is theo nly choice, is not particularly high on the list. Do we get more calloused to this? Oh, dear God, I pray not. How could a nurturing parente ver lose compassion for their child’s pain. How could we ever accept their loss without extreme anguish, and accept it we must, without the future so-called “cure.” Until the medical community perfects a treatment plan, our challenge is to keep our children as whole as possible. To keep them alive. So many are dying around us! A number of children on theEB/NuSkin film (made with Steve Young) have passed away or are in the process. And yet we must go on, and encourage our children to live a life that is as normal and rich as we can make it. We must give them as much life as they can handle. We must not let our knowledge of what may lie in the future color their lives, and we must be upbeat with them.

Thanks to Sheri for writing this Q&A and helping me with the rest of this lengthy description!

The ABC Method of Wrapping

By Sheri Coil

A bit of History

Sheri Coil and her family

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, they offered us a free vein screening afterwards, but I was kind of scared. 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.

Medical Care for EB patients

Bathing

Bathing can be a good way to cleanse wounds because you can add different things to the bath water. Some patients bathe while others shower or do a combination of both. These are some of the things people use in their baths to help with infections:

Aveeno Oatmeal bath – Aveeno Daily Moisturizing Bath with natural colloidal oatmeal is a natural cleanser and helps with dry itchy skin. This product needs to touch the skin, it will not help if the patient takes a bath with the bandages on.

Domeboro – Domeboro astringent solution helps dry out oozing, infected wounds. If you don’t want to dry out all of your wounds you can make a compress only for certain wounds.

Bleach – Use one capful for a normal size tub.

Chlorine – Hot Tubs/Spas are wonderful also and used often because of the helpful effects of chlorine.

Vinegar – I am not sure of the suggested amount to add to a bath so please consult a physician first.

Special Bleach and Vinegar Combination – I am also not sure of the suggested amount, and you should be very careful with this method, however I’ve heard it works wonderfully for infections.

Showering

Some patients cannot shower because it may be too painful to have the water hitting open sores. Some things that are helpful when showering are:

Shower chairs – There are different kinds of shower chairs, some with backs, some without, some are padded, some are not etc. They are useful so you don’t have to worry about having to stand up the whole time. They’re easy to clean. You can put a soft towel on it for extra padding also.

Adjustable shower head – This is a nice thing to have because it allows you to adjust the pressure of the water and how much you want to come out. Some even have a setting that mists you. They can be expensive depending on the one you choose but worth it.

Loofah – This may be easier to use than a bar of soap or constantly squeezing out liquid soap. You can just lather it up and gently wash areas or just squeeze soapy water over sores.

Preparing for a bath/shower

It’s easiest in most cases to set out all of the bandages, ointments etc. that you will be using before you bathe so it is ready for you when you get out. If you have long hair it’s best to put it up to avoid it sticking to wounds. A shower cap works well if you’re not planning to wash your hair.

Bathing – If you’re bathing, prepare bath with any solutions if necessary. You may want to put a soft towel on the bottom of the tub to sit on. A loofah also comes in handy for gently washing healed areas and you can also use it to squeeze water over sores to rinse.

Removing bandages – Some prefer to remove all bandages before getting in the tub, while some prefer to soak first to allow their bandages to be removed easier. Some also bathe with bandages on and remove them and re-bandage one at a time afterwards to avoid any trauma to the skin.

Blister Popping – It is definitely important to cut and drain any blisters, however wait until after the shower/bath because the blister fills up with water while bathing and that can make it larger and cause pain.

Soaps – Soaps aren’t necessary, however you can use mild, non-drying soaps such as Dove.

Washing Hair – Some prefer to wash their hair separately, on a non-bath/shower day. If the patient is prone to a lot of FLAKES, a shampoo/conditioner such as Head and Shoulder works wonders.

When You’re Finished

Getting in and out – It is important to have hand rails or something or someone for support to grab onto when getting in and out of the tub. Non-slip rugs and mats are important also.

Drying off – Remember to pat dry, do not rub. The bigger and softer the towel the better. Make sure the towel you are using is free of hair and tiny fuzz balls etc. because they can get stuck in wounds. You can also use a hair dryer or small electric heater to help dry off. Some use an electric heater to keep warm during bandages.

*****

With Recessive Dystrophic EB, some type of surgery or procedure is inevitable, whether it be a G-tube placement, hand surgery to release fingers, esophageal dilatation, dental surgery, blood draws, transfusions, etc. It all can be scary especially when most doctors and nurses do not know the specific things you can and can not do when treating an EB patient. The following is a checklist to remember some things to tell the doctors, and suggestions on how to do things in an EB friendly way without causing trauma to the skin.

Blood Pressure and Temperature

Blood Pressure – A child sized blood pressure cuff may be needed. The blood pressure cuff should NOT be placed directly on the skin. Put something soft underneath such as your sleeve, a washcloth, webril or cast padding. Also make sure they do not put it on too tight.

Taking Temperature – Some patient take their temperature under the tongue however many with RDEB have fused tongues so it must be taken another way. If you do get yours taken under the tongue I would make sure to not let them place the thermometer themselves to avoid them poking you too hard. You can also have them use an ear thermometer, but remind them to be very gentle, sometimes they push down on the ear too hard. You can also get it done under the arm with a regular thermometer, although that can tend to rub or stick to the skin.

Blood Draw and IV Placement

Before Drawing Blood – First make sure the person drawing the blood knows that absolutely no adhesive can be used on the skin so this means no band-aid afterwards. Also tell them to be very gentle when touching the skin because the skin can literally come off. Don’t be afraid to tell them when they’re being to rough! Tell them to gently dab the alcohol on the skin, do not wipe.

Drawing Blood – A tourniquet can be used ONLY if a soft material such as your sleeve, a washcloth or webril is wrapped around the arm underneath it. Often times a family member may be asked to gently squeeze the arm instead of using a tourniquet. Ask for a butterfly needle if they aren’t already using one, it is the smallest they have.

Removing Needle – Have them use a small piece of gauze over the site while removing the needle, then you can apply some pressure with the gauze until any bleeding has stopped.

Before IV Placement – Again, make sure they know that absolutely no adhesives can be used on the skin. If possible, ask for a 24 gauge needle, I believe it is the smallest IV they have but they can’t always use that one. Get materials ready to secure IV once it is in. Tell them to gently dab the alcohol on the skin, do not wipe.

Securing IV – There are different ways to secure an IV without adhesive. It’s up to you. First have them put a small piece of gauze underneath the IV so it doesn’t get pushed down onto the skin. I usually bring my own Conform wrap and they wrap around semi-tightly and tape the gauze to itself to secure it. Remember to tell them tape can be used only if it does not touch the skin. Another thing you can use is Coban. It sticks to itself but not the skin and can be used to secure an IV. The IV can still come loose so avoid too much movement or bending of the arm (assuming that’s where the IV is)

Removing IV – Gently unwrap or cut away gauze and make sure someone is holding the IV in place so it doesn’t jiggle too much and cause discomfort. Once it’s all unwrapped they can place a piece of gauze over the site and pull out the IV while you apply some pressure with the gauze until any bleeding stops.

Before Surgery

Anesthesiologist – If at all possible, speak to the anesthesiologist before the surgery to go over the Dos and Don’ts.

Eyes – Since the eyes are very sensitive to begin with and anesthesia can cause them to dry out even more, it is important to remember to put a lubricant in the eyes before hand and ask them to reapply more a few times during the surgery. Usually any gel type lubricant works. Also obviously remind them that the eyes can not be taped shut! A soft, damp cloth or vaseline gauze can be placed over eyes instead. If “blow by” oxygen is used, avoid having it blow across the eyes.

Lips – You may also want to put some ointment on the lips and if they’re working in the mouth for dental surgery or dilatation you may want to remind the doctors to apply more throughout the surgery to avoid blistering.

Bedding/Moving – Move by lifting, NOT sliding onto OR bed. To make the hospital bed and operating table softer, egg crate or sheepskin can be used. Use them as a hammock to lift onto another bed.

Versed – A drug called Versed can be given before surgery to essentially make you forget everything that happens. This is used mostly for children who are very upset, scared and anxious about the surgery and getting put to sleep. I’ve had this once and it did make me forget ever getting put to sleep, however the effects last even after surgery and most people will wake and up go back to sleep continuously for several hours until it wears off. It also makes you a bit “loopy”. But it is a lifesaver in some cases to ease the fear and anxiety.

Anti-Nausia – Medication is usually given to help reduce nausea and the chance of throwing up after surgery, although it may still be comman to be nauseous or throw up afterward.

Miscellaneous

Instruments, Gloves and Face Masks – All instruments placed into the mouth should first be generously lubricated with a water based lubricant. Gloves should be lubricated with vaseline whenever possible. Face masks should also be lubricated.

Heart Monitors/Leads/Probes – If monitors are needed FIRST cut off the adhesive portion of ECG leads, probes, pulse ox monitors etc. The monitors can then be secured using webril, koban or any other type of gauze or tucked under netting. Pulse ox monitors can also be clipped onto thumb or toe.

After Surgery

Eye Abrasions – Even when precautions are taken, it can be comman to wake up from surgery with an eye abrasion. You may want to have some eye medication on hand and you may want to have a patch or cloth to lay on the eye or wrap gauze around the head to keep a patch on. Keep the room dark if possible.

Rebandaging – If any bandages were taken off during surgery and not put back on you may want to have some bandages ready to rebandage right after surgery.

Cleaning Up – With dental/mouth surgery there may be dried blood etc. around the mouth. A washcloth wet with warm water can be used to gently clean it off. Ointment may need to be applied to the lips also.

Throwing Up – Anti-nausea medicine can be given before surgery but it is very comman to still be nauseous and even still throw up. So having something nearby at all times to throw up into is important.

Specific Procedures/Surgeries

Barium Swallow – Be sure to pad the table with blankets or sheepskin etc. You can use a straw to drink the barium.

Dental Surgery – Because RDEB patients generally have very small mouth openings, dental surgeries can be difficult. Make sure all instruments going into the mouth are generously lubricated and the mouth and lips should be continually lubricated throughout the surgery. If a lot of teeth need to be pulled or worked on, it may be best to do it in stages (multiple surgeries).

Blood Transfusion – Blood transfusions are generally a simple procedure although they can take several hours depending on how much you are recieving. Read the IV Placement information above for more information.

Iron Transfusion – Premedicating with Benadryl may be important, they will do this at the hospital. Reactions of iching, hives or swelling is not too uncomman when getting an iron transfusion. Be sure to tell the doctor of these kind of reactions. Read the IV Placement portion above for more information.

Epo Shots – Epo shots can be given the same way as anyone else. Usually they are given on the thigh or stomach. Instead of wiping the alcohol onto the skin, gently dab.

EKG – Since the sticky monitors can not be plced directly on the skin, for an EKG you can have them first cut pieces of gauze, such as Conform, and get them wet. Apply them to the areas the monitors will be placed and put the monitors directly onto the wet gauze, making sure the sticky part does not touch the skin. Someone may need to hold each monitor in place. Dry gauze will not work, they need to be damp.