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… ūüôā Also, for those of us who do not use MEPILEX, or prefer to use something else: in the past we have also used VISCOPASTE (this is gauze saturated with zinc oxide, and also MEPITEL. In this case, we cover the MEPITEL and/or VISCOPASTE with a sort of sponge called ALLEVYN, before we put on the 4 x 4’s. This absorbs the exudate. This is also a good method. The VISCOPASTE was especially effective. Zinc is good. There is another product called BREAKAWAY, that has a built in padding. It is also good. The diaper will also hold the bandage in place. Also, if it gets wet or dirty, just change the 4 x 4’s, that is of course if the MEPILEX is dirty also. Gee, I don’t mean to insult your intelligence.. Well I hope this helps. When Jenn was a baby and until she came with me in 1997, she had this problem. Now she has the scars to prove it… We do keep a nursing ointment (we call it BLUE GOO) on the scars if they look like they are starting to breakdown, and it works….

Jennifer’s Experience w/Anemia

Jennifer had been going to Chapel Hill, NC to the EB Registry as she was on the pilot study for Accutane. We go every three months via Angel Flights. They do thorough lab reports on her each visit, and she is seen by Madeline Weiner and Dr. Jo-David Fine. In June, 2001 we all noticed that Jenn’s RBC (red blood count) was really dropping. At that time her Hematocrit was about 26, which is low. Originally it had been at 32, which was do-able. Normally the Hematocrit should be between 35 – 45. However, in EBers trends are very important. For example, when Jennifer’s Hematocrit was at 32, she did very well, but the Hematologist wanted it higher as there is a “leak in her bucket”. Will explain that later.

We got in touch with her doctor here in Indialantic, Fl. Doctor suggested we have her kidneys checked. Kidneys produce the erythropoietin which stimulates the bone marrow to make the red cells. The kidney doctor did a thorough exam and found the kidneys are functioning properly but we needed to see if they were producing the Erythropoietine. He referred us to a Hematologist and we saw him sometime in July. When they drew blood the first time her Hematocrit was down to 23. Very bad. The Hematologist ordered a “mid-Line” and she was given intravenous iron (ferritin) every day for two weeks. A mid-line is actually a line that is introduced through the blood vessel and goes up the arm to the shoulder area. A pic-line is the same thing, except it goes past the shoulder area and down close to the heart. With a pic-line you also need an x-ray to be sure the line is not too close to the heart. This is all done on a out-patient basis. You should not have them draw any blood from the mid/pic-line as it will clog up the line and cause a blood clot. This is not a blood transfusion. However, a blood transfusion works for some EBer’s; in this case it was not what Jennifer needed. This was done at the I.V. Therapy Lab. At the end of two weeks they drew blood again, and the Red Blood Count (RBC) had gone up, slightly, but not enough. The Hematologist then prescribed Procrit Injections (Erythropoietin) to be given – I believe this was done once a week, with blood drawn every two weeks. Procrit is a pharmacological erythopoietin and is usually used in patients with depressed bone marrow function. This is not the usual cause of anemia in EB patients and a hematological work-up should be done prior to treatment with this drug. Other causes of anemia will not be corrected with Procrit and there are risks involved with this treatment. Jennifer’s RBC and Hematocrit was coming up and the doctor suggested we continue once a week with the Procrit Injection, with blood drawn once a month to keep checking the hematocrit and rbc. Jennifer’s hematocrit is now at 37.5, which is good. There is a pronounced difference in Jenn’s energy level and her skin is really looking good. Jennifer still gets Procrit every Friday, and we see the Hematologist and have blood drawn once a month. A great description of why Jennifer may need the Procreit for a long time – maybe not every week – but every two weeks or once a month – picture a large bucket full of liquid, with a tiny pin-hole leak in the bottom of the bucket. If you do not take measurements to keep the bucket full, eventually you will empty the bucket. EBers bleed constantly – as their wounds always have some seepage, being blood or some other liquid, usually blood. So it is very important to keep that “bucket full”. ūüôā

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‚Äôt 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

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. 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 РThis 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 РIt 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 РSome 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 РRemember 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 РBecause 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.

Hand Surgeries

By Grammy Shirley

December 23, 2002

This is information regarding Jennifer Deprizio’s many hand surgeries.

Before Jennifer came to live with me in 1997, she had been through many hand surgeries to open the digits and to keep her hands from mittening.

Jennifer’s hand before surgery

None of them worked and the reason for this is lack of care. Lack of constantly using the braces and/or splints that Dr. Dell and the therapist made for her, to keep the skin from growing together and the webbing and mittening.

At this time in Jenn’s life the braces/splints were rather crude and they were harder than now. Now they are made of “Odoform” and other softer substances.

Odoform is like putty, which is shaped to the hand during surgery, and remains soft, but firm.

When Jennifer came with me in 1997 both hands were webbed and mittened. She had “pinchers” so that did enable her to use her hands a bit, but not much. Jennifer had her first hand surgery while with me in June 1998. Dr. Dell at Shands Hospital in Gainesville, Fl. did this as an outpatient. She had gas anesthesia. Jennifer did not have to be intubated as she was always going to be lying on her back. They put an excessive amount of Vaseline on her face so the mask would not disrupt the skin. It worked very well.

Jennifer’s hand after surgery

We stayed in Gainesville in a motel for a week, as Dr. Dell needed to change her bandages every day and check to see if the brace was in place. We live about three hours from Shands. Jennifer was uncomfortable when I brought her back to our room, but was still under the effects of the anesthesia and pain medication, so slept until we had to go back the next day. The bandages did drain a lot and that is normal. That is why the bandage needs to be changed every day for a while, also to watch for infection. Jenn was on a strong antibiotic to guard against infection.

Dr. Dell and Ruth, the therapist, made a brace/splint to fit Jenn’s hand while she was under so as to keep the digits separated and to stretch the hand. They also put “pins” in her fingers with a top to circle the pins, so to keep her fingers apart and straight. It took about three weeks before the pins could come out. Dr. Dell did this in his office. It was a little painful, but they gave her a bit of sedative while doing this. Sometimes the pins want to come out by themselves, and this is good.

Then Jennifer began therapy to measure, stretch and work the fingers and hand. This took about two months. The total healing time was approximately three months. Once the pins were out it healed fast. Jennifer wore her brace made of Odoform day and night.

During therapy they would make a new brace to fit her healing ever changing hand. We wrapped the ABC wrap and put the brace over the wrap. It works wonders and we still do it.

Jennifer’s hand after surgery, wearing her class ring!

Some people do not want to use the ABC Wrap but we firmly believe in it. All EBers are different so it may not be for them. Jennifer had all of the ordinary complaints; i.e., it hurts, it’s uncomfortable, it isn’t working, I don’t like it, but I persisted. Now, she hasn’t worn the brace in the last six months, unless her hand is tired or hurts for some reason, but we still do the ABC wrap and Jennifer actually wraps her hands herself since we have had the right hand done.

The right hand was done in May 2002 at Shands with Dr. Dell. Instead of gas anesthesia this time they gave her a block of the arm and Jennifer was awake and watched most of the surgery. Wow!! Dr. Dell thought the fingers were atrophied into place. This would have meant extensive surgery with the ligaments, etc. When he de-gloved Jenn’s hand, the fingers just uncurled one by one and Jennifer was so excited she just shouted, my hand, my beautiful hand.

Dr. Dell followed the same procedures as the left hand, pins, brace, etc. and we stayed at the motel for approximately four days. Again Jenn slept through the most painful part, was on pain medicine and antibiotics. This surgery healed much faster as the hand was not as bad as the left hand. Jennifer only had to wear the brace on her right hand for about three weeks, but we did the ABC wrap about one week after the surgery, and then put the brace on. She only needed about two weeks of therapy, as she knew she had to exercise those fingers, and she did. It is all in helping yourself heal.

Jennifer with Dr. Dell

The EBer MUST do what is necessary or the procedure may not work, or it may not work as well. With the experiences of Jennifer’s hand surgeries, I am convinced the child should be old enough to follow directions, and be willing to go through the steps to help them heal and have a workable hand. As you can see by the pictures, Jennifer hand does not look like mine, or yours without EB, but it is very functional and she can do anything she wants with them.

Jennifer is a sophomore at Brevard Community College (BCC) and is majoring in WebDesign/Desktop Publishing. Her fingers fly on the keyboard and her speed is up to 60 words per minute and gaining. She is an accurate typist and does not look at her fingers while typing – well, most of the time :-). She wears her school ring on her finger on her right hand. Something she has wanted to do since she graduated from high school… She wears other rings also.

It has been since 1998 since the first surgery and her left and right hand look wonderful. Jennifer is a trouper.

Grammy Shirley

P.S. If you are going to use the ABC wrap after surgery, never put the 1″ gauze against the fingers without using lubricated Vaseline gauze. It will stick to the fingers and pull the skin away, causing more webbing. I do not mean the insult intelligence; just want to make sure you are aware of this…

Bandaging and Severe RDEB ~My Story~

by Silvia Corradin

My story on how I came to care for my son started, as with all RDEB babies, when he was born. Since he was c-section he came out perfect except for some problem with his mouth. The nurses sucked all his skin out of his mouth when they did the routine suctioning of the liquid in his mouth when he was born, but we did not know it at the time. It wasn’t until the next morning that the pediatrician came into my room to tell me that they had to put Nicky in NICU in an incubator because of the problem with his mouth. They thought it was either a “Staph Infection”, “Herpes” or some rare skin disorder called “Epidermolysis Bullosa”. By then, in fact, he had started blistering all over the place, not just his mouth. The Neonatologist had a dermatologist on his way from the Children’s Hospital in Phoenix where he always worked on Tuesday from his office in Tucson. When the dermatologist arrived it was around noon, he told me that he had seen Nicky and did some sort of test with the eraser from a pencil and he wrote down the name “Epidermolysis Bullosa”. He was fairly sure that is what he had but did not know what form.

Nicky, 17 months old

After he left, the nurse took me down to NICU in a wheelchair and then left me. I was stunned to see that my baby had been put in isolation. They had to put an IV on him because he could not eat since his mouth was in such a poor shape, and he was attached to a heart monitor and other things. He was in an incubator to keep him warm, had no clothes on him aside some bandages and the diaper. They were keeping the tubes attached to his tiny body with gauze. They could not use tape. The tape had already damaged his tummy, and the hospital bracelets that they had put on his ankles had done some serious damage as well. I held him for what I thought was an eternity, the blisters on his little body were horrible, and I had never seen anything like it. On the right side of his head there were a lot of little red marks where the skin had torn off. The nurse told me he got those spots when I was trying to push him out, he kept banging his head onto my bone. The heart monitor they had put onto his head when his heartbeat had fallen caused other spots on his head.

I went back to see him later on with my mom and my mother-in-law, and it was there that my husband called me in tears. Earlier he had come to see us, and I had given him the name of that strange skin disorder. Now he had had the time to research the Internet about it, and he was completely devastated. He told me that he would most likely die from this. The Neonatologist comforted us a lot, and told us that EB is something that could be lived with. Another nurse told us that he had seen a newborn with EB before that did not survive, but that was in much worse shape than Nicky and that, unlike him, this baby’s wounds never healed. We did not know what to think.

It was apparent from the very beginning that on spots where the nurses had bandaged his body he was not blistering anymore and he would not be able to rub his hands causing blisters on his hands and face if they were in mittens. Therefore I had my husband bring clothes and other things to put on him so the skin would not be so exposed. We had to learn this on our own because the Dermatologist told nobody how to properly care for our son, not even that the blisters needed to be punctured so they wouldn’t grow. Because he failed to tell us this, Nicky was in real bad shape. Both elbows were completely skinless, and so were his ankles, the top of one hand, several fingers, one of his knees, the big toe on his right foot, several spots on his legs, and his chest.

The dermatologist came back the following week, and because the blisters were not being popped Nicky was in absolute horrible shape; he talked to us for a while and managed to really depress us. Over and over again he did not want to give us any hope for his survival. He told us that we would be in week after week to treat infections, if not worse, and totally painted an extremely bleak picture for us. Afterwards he took us step by step on how to care for his wounds taking extreme care in doing so.

When we went back to see him at Phoenix Children’s Hospital a couple of weeks later, he brought in a lady, Heather, who had lost a son from Junctional Herlitz EB a few months earlier to help us and teach us proper wound care. When Heather saw our son, she immediately knew he could not possibly have HJEB, and that he was going to be OK. She showed us photos of her son and we could definitely see a difference. Heather was wonderful and told us how important wound care was; showing us exactly what she did to help her son. I never forgot it.

A month later we finally received the diagnose of RDEB and the Dermatologist emphasized how Nicky’s wounds, because they are deep, needed to be healed with moisture. A dry cell is a dead cell he explained, and could not heal back to “normal” and that is where scars come from.
In the beginning we had not bandaged all the wounds because they were not scarring and healed much faster, but as time went on it became apparent that moisture was indeed much needed, even though it slowed down the healing time considerably. One particular instance etched in my mind was when Nicky got a rather large wound on his tummy when he was 6 months old-it healed really badly because it was almost completely air dried, and it was absolutely awful. It looked so completely different than the normal skin surrounding it, it was an eye sore. Not only it looked bad, it was also much weaker than normal skin. It was only a matter of a few weeks that the complete area (and only that area, not the normal skin surrounding it) was a new wound. This time I made sure it healed with a ton of moisture and covered and protected, and although it took a while to heal, the skin looked a million times better, and, most of all, 13 years later, the wound has never re-opened!

As Nicky started crawling and moving more and more, it became very obvious that he needed more and more protection. At first it was just the hands, feet and elbows, and then his knees and more and more as time went on. It was apparent that the areas that had never gotten blisters in the first place were getting much tougher than the areas that had wounds before-those areas were much easier to get a wound on, so it was important to us to keep him as healthy and wound-free as possible. After we finally saw an EB specialist it became even more important to us to keep him bandaged because he stated that the #1 killer of RDEB children was infection, that many children are anemic due to the blood loss and, finally, the threat of cancer in non-healing areas was particularly upsetting to us, making us realize how important it really was to keep the baby as wound free as possible. One particular eye opener came a few months later. We were visiting Stanford for a routine check-up and we were able to meet a few other parents with RDEB children, and one in particular, the late and much loved Sheri Coil, decided to do an impromptu bandage “class” showing the parents how to wrap hands, armpits, torso, legs and feet. It was amazing. The things I learned that day I still use today. What was most impressive is how little damage little Brandi had on her body due to the heavy bandaging, and while at the time I thought she was “overwrapping” (a term I was referred to regarding my bandaging Nicky later on), at the same time I was amazed on how little wound mass this child had. I know some people feel that heavy bandaging means the child cannot move, but I beg to differ. It is because of the bandaging that Nicky is even able to walk and even run a little. It is because of the bandaging that Nicky is able to bump on things a little bit and be a little rougher without fear of getting hurt. It is because of the bandaging that he is not afraid of people touching him and hugging him. Sheri showed me how active her 3 RDEB children were despite the heavy bandaging and I became a believer.

At the time, however, I was not able to start the heavy bandaging because the insurance was not covering any of my son’s supplies, so I had to wash and re-use bandages because they were so expensive and we simply could not afford them. Hence I tried my best to cover what I thought were his worse spots, but with time I had to do more and more because of the level of activity my son had.
I did not start wrapping the torso, for example, until one day when Nicky was 1.5 years old. He was wearing a white t-shirt that day and I will never forget how one moment the shirt was completely dry and after I turned around, the shirt was completely soaked. At first I was shocked, not knowing how he got all wet, until I took off his shirt for the biggest eye sore ever. He had a blister, the size of my hand, which went from the front of his chest, and covered the entire side and went all the way to the back. To date, that was the biggest blister he ever had, and the last he had on his chest, because I’ve been heavily wrapping it ever since.

Another bandaging area that I had to learn how important it was is the hands. When Nicky was a baby I was told his hands could “web” but I was never told about the “contracting”, or, in laymen’s terms, the curling up and webbing of the fingers into the palm. I was pretty consistent in keeping the palm or back of the hand bandaged if he got hurt, and made sure that if he got a wound in between fingers I kept the fingers moist and apart. But it wasn’t until the visit with the EB specialist that he pointed out to me how his index finger, at barely 8 months of age, was already curling up. I could not believe it; my 8 month old baby was already disfigured. The pediatrician sent us to a hand therapist and physical/occupational therapists to see if we could get a handle of hands and prevent further damage, and while the hand therapist made some splints to wear at night, the physical/occupational therapists basically showed me the importance of straightening those fingers every single day. Another mom sent me some gloves made of Lycra that were elastic, so that the tips of his fingers could be free. Since I still felt as if bandaging each finger was overkill, I thought this was the best course of action. Well, I was wrong. The splints were so incredibly hard to put on Nicky, we quickly gave up. There were times they were doing more harm than good. While the gloves really helped in protecting the hand, they did not help in keeping the fingers apart or straight because there simply was not enough tension. Lastly, the straightening of the fingers worked, but without a way to keep the fingers apart, the fingers were becoming useless anyway because they were webbing together. By the time Nicky was 2 years old his right hand (and he is right handed) was so badly webbed he would no longer use it. The fingers were webbed together and somewhat curled and Nicky was now doing everything with his left hand, which was in a little better shape than the right one. Something had to be done, and soon. After much research, talks to various Doctor and internal debates, we decided to go ahead and do the surgery, and that we would start aggressively doing the hand bandaging immediately after surgery. While the surgery and the recovery is something I rather forget because I had never seen my baby in so much pain, on the other hand, he came out with a hand from this procedure. I have been wrapping his fingers individually and the palm/back of his hands with Vaseline gauze first, to retain the moisture, then I cover with 1′ gauze. The surgery on that hand was now about 12 years ago, and while the right hand’s fingers are a little curled up at this point, he can still use them well, and there has been no loss to web space. The hand can probably hang in there for a few years to come without any surgeries, and even so, we’ll most likely opt for a “partial” surgery, just to straighten a couple of fingers.
The left hand benefited from having aggressive bandaging early on, but it was already is fairly poor shape, and a surgery became a need when Nicky was 3 years old. Unfortunately the recovery from this surgery did not go well and the fingers were not straight afterwards, I blame myself for it, I was working full time as a single mom and was not able to tend to this hand as much as I should have. Because this was, after all, his left hand, I pretty much “let it go” and it was unbelievable how fast the fingers webbed and curled into the palm of his hand. I was still keeping them apart with gauze, but he basically had no fingers to speak of. The only good thing that came out of that surgery was that the thumb was released and he could use it well now. I was always planning to have another surgery on that hand and tried to get the right Drs involved this time, which took time, but I did not seriously and aggressively look into it until after Nicky started kindergarten and he saw the other children’s hands. To see my son devastated because his hand was different than his peers and asking me if the doctors could straighten his hand was more than I could bear.

Just before his 6th birthday I was finally able to get him in to get a hand surgery with a Dr at Stanford and, by far, this was the best experience with a hand surgery yet, he wore a cast and took a lot of pain medicine and he did quite well. While Nicky himself told me he did not like the surgery or recovery, he is so happy he has a hand! Future surgeries, if any, I will leave up to him. It’s his hands, his pain to endure.

Now that Nicky is¬†14 years old, he himself understands how important it is to get him wrapped, and he even asks me for MORE bandages in certain areas. If for some mistake I miss an inch of his body it would be blistered up next time I unwrap him, so he’s become extremely aware of the benefits of bandages and how much they improve his life. There are many parts of his body, such as his back, that haven’t blistered at all in years and the skin there is absolutely perfect and normal, even has some little hair growing out. His chest is also looking fairly good considering some of the problems we had early on. The hardest and most wound prone areas are his entire legs, ankle and toes, his hips, his armpits, elbows and hands. Even so, he has very little scarring elsewhere, which is a miracle of the bandaging, since I know how severe he is and how bad he would be if I was not bandaging him. The fact that he’s also not so active surely makes a big difference, but he does scratch a lot, hence some of the heavier bandaging to prevent him from hurting himself aside the anti-itch medicine he receives everyday.

To parents of severe RDEB children, don’t let anybody tell you that you are “overwrapping” your child. There is no such thing. Every child is different and needs to be protected. Perhaps some children deal with it better being underwrapped, and if it works for you, good for you. Bandaging, in my son’s case, has improved his life dramatically. He can still do everything he wants to do and more, because he feels protected, and his wounds at times only account for maybe 20-25% of his body, and mostly because some take a while to heal. New wounds are now fairly rare, and are mostly accounted for by his itching that we are trying to get under control.

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 and Dental Care

By Melanie (Logan) England
(this information was previously posted on the EBmommas website)

The Dystrophic and Junctional forms of Epidermolysis Bullosa are notorious for causing deformities in the enamel and dentin of the teeth, but people with all types of EB are prone to dental decay due to oral hygiene complications. Blistering in the oral cavity, fused oral vestibules and smaller than normal mouth opening can all make it difficult, if not impossible to brush and floss properly.

Here are steps parents and patients can take to keep the teeth as healthy as possible.

Start a regular routine of brushing your childs teeth as soon as the first teeth appear. If the mouth is too sensitive and blistered for regular brushing, a Toothette sponge can be used (ask your pharmacy for these), or the teeth can be wiped clean with a gauze sponge wrapped around your finger.

See a pediatric dentist who specializes in disabled children as soon as the first few teeth appear.

Check with your local water board to find out if your drinking water if fluoridated, and ask what the concentration levels are. This will help your dentist decide if your child needs a fluoride supplement, and what dosage is appropriate.

Soften the bristle of the toothbrush but running them under hot tap water before brushing. Oral-B extra soft childrens toothbrush ages 1-3 years is one of the best on the market, and the smallest for reaching back teeth in children with small mouth openings.

Use a fluoride toothpaste for sensitive teeth such as Sensodyne or Colgate Sensitive. Plain baking soda can be used if a toothpaste cannot be found that does not irritate the mouth.

Chewing sugar free gum for 15 minutes after each meal helps loosen plaque and food debris from the teeth.

When your childs mouth is severely blistered or ulcerated brushing may be impossible without great pain. During this period the mouth can be rinsed with a solution of 1tbs hydrogen peroxide mixed with 4tbs water and < teaspoon of salt. This will soothe the mouth and loosen debris. If you choose a commercial mouth rinse, read label and make sure it does not contain alcohol.

What your dentist can do:

Prescribe Decadron elixir- a liquid steroid that is swished around the mouth for 2-3 minutes then spit out. For a child too young to follow instructions, it can be swabbed inside the mouth with a toothette or gauze sponge.

For extreme pain your dentist can prescribe 0.05% topical lidocaine gel to apply to gums and inside of mouth 3 times a day.

Magic Mouthwash is a pain reliever that combines lidocaine, benadryl and Maalox, and many EB patients report good success with it. Your pharmacist will mix it in the proper combination per your doctors prescription.

Candidiasis, commonly known as thrush, is another commonly found oral problem in people with EB. Anemia and use of antibiotics can cause thrush, which looks like a white coating on the tongue, palate, or inside of the cheeks. It can also make the surrounding tissues puffy and tender. Your doctor may prescribe a Nystatin solution to rinse and swallow, or liquid Diflucan to be take orally for 7-10 days.

Oral Surgery

Despite diligent care EB teeth may still develop carries and require fillings, crowns, or sealants. Talk with your dentist about which option is best for you or your child. Simplex patients who still have good enamel can often opt for fillings, but patients with enamel hypoplasia will require crowns because the fillings have nothing to bond to.

Its usually far less traumatic for children to be sedated rather than awake for dental procedures, and much safe for EB children to have it done as same day surgery rather than in the doctors office. Discuss all your options with the doctor and decide which is safest and most comfortable for you or your child.

Have the doctor go over the steps that will be taken to minimize trauma to your childs skin during the procedure. Make sure they include the following:
Orient all staff involved in the procedure on basic EB care
Have an assistant to manually retract gums and tongue instead of using metal retractors.
Coat all instruments with Vaseline or Aquaphor, as well as the gas mask the anesthesiologist will use.
Pop blisters as the form during the procedure so they dont grow and cause the patient even more pain.
Avoid if possible allowing suction to touch the oral membranes so less blistering occurs.

To find a dentist in your area contact your local chapter of the American Academy of Dentistry.

Links:
American academy of Pediatric Dentistry

Basic 101 Care

This page was put together with the aid of a little booklet distributed by DEBRA UK called “Care and Management of Children with Dystrophic Epidermolysis Bullosa”.

Because of the rarity of EB, many parents have not been helped by the medical community, and hospitals have even injured these children by applying tape, bracelets, sucking the liquid from the mouth… (only proceeding in taking all the skin off also in the process) on these fragile newborns. The following are BASIC 101 care needs and knowledge for an EB child… something that, unfortunately should go without saying, but every parent wishes they would have known (and wishes the doctors would have known as well) when their child was born…

Blisters

POP THE BLISTERS!!!!!!! Yes, you can’t get anymore basic than this. These blisters have a tendency of increasing in size incredibly fast, and since they leave behind quite a remarkable wound, the smaller the blister, the smaller the wound. Leave the roof on the blister because it will protect it. Do not just put a hole on the blister, make sure to tear it so there is no chance for it to re-fill. Sterile needles can be used or scissors.

Lifting the child

Before handling any child with EB, remember that friction will cause blisters and skin damage, but direct pressure will not (unless this is a Simplex DM child). Children of any age who suffer from Epidermolysis Bullosa must NEVER be lifted from underneath the arms, as painful blistering will always result and this area is notoriously difficult to dress and to heal.

Bathing

Naked children are very vulnerable, for this reason most babies are sponge bathed once a day until they can sit well on their own. At that time the child can be immersed in water on a piece of foam or soft tub, which prevents damage from the child kicking the tub and such. To pick up this child use arms instead of hands, as the children get older they should be encouraged to climb in and out of the bath themselves in order to avoid skin damage caused by lifting them. There is no need to give these fragile babies/children a bath every day, water dries the skin too which is bad news for any EB patient.

Wound Care

Gauze should NEVER be put over a raw wound… NEVER! When the gauze is taken off it will be stuck to the wound and it will be not only painful getting it off, it will also take off the healing taking place. Use either a non-stick pad such as Telfa, Mepital or Vaseline gauze (put extra Vaseline as needed). Tape can be used to secure the wound as long as it does not have any chance to come in contact with the skin, however, using tube gauze is far preferable to tape. It is a good idea to pad these children’s entire body to PREVENT new blisters. Remember, is far less painful to sweat than to have open wounds.

Diapers

This one will depend on the child, but most kids do fine with Huggies Supremes or Ultratrims, use a size bigger if necessary to make sure they are not too tight. Cloth diapers are the only alternative. You may need to put Vaseline around the waist and leg areas to decrease friction, or cut the elastic around the diaper.

Nutrition

Children with EB have an increased requirement for nutrients as they are attempting to grow without diverting some of the nutrition into wound healing. Unfortunately, there are many factors (including blisters in the mouth) which impair eating in these children and heroic efforts must be made to supply the correct balance. For babies, use the powder formulas and increase the dosage of powder to make a more caloric meal-this tip came straight from Stanford. If your child cannot use powder formulas for whatever reason, try using extremely HOT water for mixing first, if not, use liquid concentrated formula, but check with the pediatrician first for dosage. For children over 1 year old, PediaSure seems to be the best bet.

Crawling and Walking

Children with EB often crawl and walk later than other children. This is because they tend to be cautious because they are hurting and/or are afraid of falling. However, once mobile, they quickly gain confidence. Each child develops different, so as one EB child might walk early, one other may walk very late, as normal children do too.

Eating Difficulties

Often babies with RDEB have a sore mouth, because the skin inside the mouth can blister the same way as the rest of the skin. Most children with Recessive Dystrophic develop “microstomia”, a small mouth opening, causing problems with putting food into the mouth. Fusion of the tongue to the floor of the mouth, makes it difficult to move the food to the back of the mouth for swallowing. Although teeth “can” be (not for all kids unfortunately) structurally normal, they are prone to decay in view of the necessity for a high calorie diet, poor access for teeth cleaning, and often a reluctance on the part of the child as the mouth is sore. The “labial sulcus”, the space between the inside of the lips and the lateral surface of the gums, is often lost as a result of scarring, this leads to a reduction in the circulation of saliva and adds to the problems experienced when chewing and swallowing.

Swallowing difficulties

Some children experience recurring blistering of the esophagus. Unfortunately, these blisters tend to heal with scarring in the same way as blisters on the external skin. This can cause strictures, which are narrowed areas of the esophagus. These strictures can be severe, and sometimes a semi-liquid or liquid-only diet can be taken. Many children will choke on a blister to pop it, but be reassured that it is the esophagus and not the trachea that is blistered, hence breathing is NOT impaired.
Side note from a patient with RDEB: some patients have had problems with breathing later in life due to scarring close to the esophageal flap.

Webbing and Contracting

Most children with Recessive Dystrophic EB, particularly the ones with the Hallopeau-Siemens subtype have a tendency of webbing and contracting on the fingers and toes. Webbing means that the fingers can become fused together, contracting meaning they will contract toward the palm. Despite meticulous care, some children require plastic surgery to divide the fingers and to restore function of the hand. This happens because of too much scar tissue prevents the growth of the hand.

Constipation

This has been described as being the worse complication of all. It may start somewhere around the child’s first birthday and it takes just one hard stool to blister the anal margin, and the cycle of holding back will begin. Even a young baby will soon learn to avoid opening his bowels and so avoid the pain. A combination of laxatives, increased fiber in the diet will help, but the dosage will need to be regularly reviewed and adjusted.

Anemia

Regular blood loss is the main cause of Anemia in children with RDEB. Iron supplements and a diet rich in iron, fiber but *especially* protein can help.

Care of the Eyes

In many children with RDEB the surface of the eye can blister in the same way as the external skin. Blisters on the eye are often caused by the infant or child rubbing the eye or by a foreign body traumatizing the conjunctiva. Child should immediately be taken by an optimologist and given a local antibiotic. The eye must never be forced open for medical examination as further damage will be done. Apply the ointment to the corner of the closed eye and it will melt and run in. Scarring may develop in the eye which can impair vision. ūüôĀ

Cancer

A complication of Dystrophic EB seen in adults is the appearance of skin tumors called “Squamous Cell Carcinoma”. If these are identified quickly they can be removed before metastatic spread occurs.