My take-away from the Debra’s PCC in Orlando – Part 8 (last)

A consensus Approach to Wound Care

This talk was given by Dr. Elena Pope and it was very interesting. I think what would have helped more-and perhaps this is something I will suggest for future conferences-if it came with a full blown Wound Care 101 with explanation and showing of the different products available. I know I am not only speaking about myself when I say that, even though Nicky is nearly 16 years old, since there are always new products out there which we are unsure how to use, a hands-on explanation with samples given to try would have been extremely helpful. Just me?

Anyhoo…the presentation started with an explanation of chronic wounds and how to care for them. How important Hemoglobin levels are to keep the healing ongoing, which is why iron supplements and infusions are so important.
Chronic Wounds are like a vicious cycle and once you get one it’s hard to get rid of it because it basically feeds on itself. Ugh. Another thing to keep into consideration is if the wound edge is even or rocky. Rocky might mean the big C, so she stressed the importance of having it biopsied. Ick. She stated that SCC (Squamous Cell Carcinoma), which is usually sun induced, with RDEB and the wounds constantly trying to heal, it starts in there, making it more aggressive as it would otherwise be. The absence of collagen VII makes it more aggressive than usual because it’s the collagen that fights the cancer to begin with.

The most important slide that you will ever see is this one on the right. Click on it to see it bigger and print it! That’s what I did. It goes on detail of what you should use on what type of wound. For example… what to put on just for protection or if it’s an itchy wound or an infected wound or a painful wound. I would have liked if it came with names of products in detail with choices, such as… Mepitel is better than Xeroform on this type of wound and that sort of thing, but overall I think it’s very helpful!!

Here is the video of this presentation, courtesy of Debra of America:

Cell Therapy for EB

This talk was given by Dr. Alfred Lane-this is the second talk that I was very excited to hear. Unlike the Protein Therapy, which would need to be infused into the patient every 4-6 weeks to keep the disorder at bay, this is a genetically modified skin graft which would permanently heal whatever area it’s put on. It involves Gene Transfer, which is inserting the correct gene into the DNA structure using a virus, and it starts working right away.
Only 5% of DNA makes protein, so the risk is minimal. It would involve a skin biopsy, growing cells in vitro, viral transfer to genetically cure the faulty gene and the making of skin to staple to the wound (skin graft). This would last the life of the individual. That area is CURED! No more blisters!

The biggest obstacle in doing this for years and years was to get FDA approval but that obstacle is now surmounted. Several years ago they tried this method on an Italian patient in Turin and it worked, he no longer blistered in that area.

I actually spoke to this patient on the phone once and told me the details and he was very excited!
They have been trying to get the trials started for a while now (a couple of years at least) but now Dr. Lane stated that they should be starting the first subject this year. FDA rules require patients to be at least 18 years old to give their full consent, but after they have 5 successful subjects trials, they can re-evaluate and the FDA will most likely approve patients between 7-17, the patient still has to understand and give their consent, that’s why he/she has to be older than 7.

Dr. Lane explained how this is truly a long term solution and they had never a single problem or side effect with the mice they worked with and there are no unnecessary risks to patients. Other skin disorder advocates and researchers are keeping a close eye on this particular research because if it’s a success, it will help hundreds of thousands of other patients suffering from not only other forms of EB but also one of the other 300 skin disorders out there.

When asked ‘when’ the trial will be over and this will become available to everyone, Dr. Lane had a hard time being specific. He wanted it to be done yesterday, a year ago, 5 years ago. If you would have asked him 10 years ago, he would have said a couple of years. It’s hard to say. It’s hard to predict when obstacle will come to delay the process.
I remember adding in my mind the 5 patients and how long each patient will be in the trial, so if everything went extremely well, maybe a couple of years? Who knows.

For more information about this study, please go to THIS LINK.
They are now recruiting patients for the trial, please contact Emily Gorell, to find out more about study enrollment and requirements.
She may be reached at (650) 721-7166 or

Here is the video of this presentation, courtesy of Debra of America:

Management of Esophageal Strictures in EB Patients

This talk was given by Dr. Richard Azizkhan. An Esophageal Stricture is a scar or narrowing of the esophagus that can make swallowing solids or even liquids difficult. The scar involves the lining of the esophagus, not the muscle.
This slide shows the probability of it depending on the EB subtype. I must admit, I sighed when I saw this. By the time Nicky was 3, he had to have his first, and he was so bad I ended up needing to put a g-tube on him as well at the same time. According to this chart, only 10-15% of RDEB patients need this at this age. Wonderful. Ok, moving on…

The only technique used these days that works best in fixing this issue is the balloon dilatation. Dr. Castillo at Stanford proudly told me he was the one that came up with this procedure :0).  He stated it works well also because it can be repeated multiple times on the same patient without a problem, even dozens of times.

However, as with anything, there are caveats.

The patient cannot have very severe anemia for example. Dr. Azizkhan sadly stated how one of his patients almost died and had to be resuscitated and later went into a coma because they did not know how anemic he was before the procedure. Always make sure the patient is not severely anemic beforehand! Cincinnati now routinely does blood-work beforehand to make sure this never happens again.
I talked to the mom of this patient… to be honest, I cannot imagine. Not only this child (I believe he is maybe 5 years old?) has rather severe RDEB, he woke up from the coma not exactly like he was before. Because his brain went without oxygen for a time, he has a long road ahead. Neurologists say that because he’s still a child and his brain is still forming there is a lot of hope for him. Please pray for this precious child!!

These slides give a LOT of good info on how the procedure is done and what the outcome has been over the last 20 years of doing this procedure. Please click on the image to open a bigger version.

OK, part of this talk also included gastronomy tubes, or g-tubes for short and indications. The most important indication is growth failure. As I stated in a previous blog, not ‘just’ for weight issues, but growth failure indicates also failure in stature and head circumference. It’s also a safety net for those that require constant, multiple throat dilatations and also to address behavioral issues and family dynamics. It becomes a must for about 40-50% of RDEB patients and also many newborns with feeding issues of all other forms of EB.

The patient is initially given a temporary tube and several weeks later a permanent tube, most commonly a mic-key is put in place.  

Here is the video of this presentation, courtesy of Debra of America:

Below is a video from the 2010 conference about Dilatations and G-tubes.

Ocular Surface Rehabilitation in Dystrophic EB

This talk was given by Dr. Arturo Kantor, who had quite an array of interesting information. Before his talk the only issue with the eyes I ever heard of or experienced are corneal abrasions, which Nicky is, of course, not a stranger to. However, he related that some patient have SEVERE eye involvement, and those that do, usually have Dystrophic EB and also have esophageal involvement. Some patients with Junctional have these problems as well, but he stated this is not a problem that Simplex patients have at all.
His suggestions were to use Preservative Free Lubricants and Bandage Contact Lenses. CL do not prevent erosions, but new epithelium grows underneath and helps the eye.

The contact lenses he suggests are not available over the counter, and must be High DK over 120, highly permeable. He also suggests GenTeal Gel for eyes.

The doctor also showed some videos of a patient he had to replace the cornea of… highly graphic videos that I squirm just thinking about, so I will spare the one photo I took of because it reminds me of them, ha ha.

Here is the video of this presentation, courtesy of Debra of America:

Insurance Advocacy

Medicare Info

This talk was given by Michelle Graham was the FINAL presentation of the conference!! She started with an explanation of the various HMO, PPO, POS, EPO and also Medicare, Medicaid and CHIP.

A good website to look at, with detailed information about all the different programs that may be available in your state is:

She also discussed how fully vested insurance plans are much better and offer a lot more coverage than self funded employer plans. She stated it’s important to make a list of things needed, such as bandages, doctors, medications before switching plans, review the list with your HR or broker and request a written letter to validate what was told to you. If the new plan is lacking what you need, ask for other plans or if exceptions can be made depending on diagnosis.

If a claim is denied, be pro-active. Call and write your insurance provider, write everything down, know your rights. Call your State Insurance Commissioner with problems.

Here is the video of this presentation, courtesy of Debra of America:

Here is the video from the 2010 Conference!!

Please Note: I tried to post as many videos from the 2010 Conference as they were appropriate in their respective sections. ALL the videos that are available to be watched can be found HERE:

The videos from the 2012 Conference are now available and I embedded the link in the respective sections. THANK YOU!!!

Links to — > Part 1Part 2 – Part 3 – Part 4 – Part 5 – Part 6 – Part 7

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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My take-away from the Debra’s PCC in Orlando – Part 5

Here is more information about several talks from the conference from July 31, 2012:

Anesthesia for your child with Epidermolysis Bullosa & Interdisciplinary EB care

These two talks, which I will bunch up together simply because I don’t remember a whole lot from them separately, were done by Dr. Eric Wittkugel & Dr. Anne Lucky, both from Cincinnati’s Children Hospital. The only two notes I wrote down were to, one, look up ‘Mepitac‘, which is a tape that can be used for EB patients (does it work for RDEB? They say it does, I am always a little weary of it, but I am willing to try it) and, two, the duration of anesthesia is not a problem, there is no risk in case a patient needs to be ‘under’ longer for multiple procedures.

Other than that, I will let the slides do the talking, hopefully they will help someone. Remember to click on the slides to view a bigger version…

All of these slides are from the Anesthesia presentation, I found THIS PDF document online from Stanford with more info about Anesthesia that relates to EB.

Here is the videos of these presentations courtesy of Debra:

Dr. Lucky’s presentation is available ONLINE at this link HERE! (you will need a Google account to view, but it’s FREE) Basically, Interdisciplinary EB care means having everyone that is an expert of EB, from the dermatologist to the GI, hand surgeons and more under one roof.

Following these two talks there was a Physical and Occupational panel Q&A.  One question that comes to mind was from a therapist asking if she could use coban with the finger wrapping between web spaces and them stating coban was just too harsh on RDEB skin (which I agree-when Coban touches Nicky’s skin it’s blister city, so I only use it on top of bandages). My two questions were if there were any oils they could recommend to soften scar tissue and what exercises they could recommend to improve bone density since Nicky can hardly walk. The first question about the oil, they did not have any recommendations, except for just massaging the hand, which is hard to do when it’s full of wounds. As per the bone density question, she stated that at least standing is important, so Nicky has been standing as much as he can since I told him, bless his heart! Another suggestion I got is to get a vibration platform, that it could be rented etc. By talking to other parents and doing a little web search , I found out that Coconut Oil, Vitamin E, and I am trying this Argan Oil, all soften scar tissue. I soak his hand in these oils before rewrapping them.

Here is the video of the presentation, courtesy of Debra of America:

Here is the presentation for OT & PT at the 2010 Conference:

Medical and Integrative Approaches to the Management of Pain and Itch in EB

This talk was probably one of the most important of the entire conference. It was given by Dr. John Saroyan & Dr. Traci Stein. I mostly took photos of the slides because they were SO IMPORTANT and I did not want to miss a beat.

This talked about the various drugs that can be used depending of what form of EB, drugs used to subside itching and the issues with constipation.
So, once again, I will let the slides do the talking. Remember to click on the slides to view a bigger version…

If anyone has any info I might have missed, please leave a comment below (in the Facebook comment section).  THANK YOU!

Update August 15th, 2012: I found more notes about this latest talk about Pain Management that I think are worth sharing. The Doctor spoke about different ways to reduce pain, such as trance inducing activities, relaxation techniques, pleasant scents… for more information she stated to contact her at her website:

Here is the video of the presentation, courtesy of Debra:

GO TO PART 6 –>>

Links to — > Part 1Part 2 – Part 3 – Part 4

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.


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: 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, 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

EB and the Eyes

This is the transcript of a helpful book from DebRA U.S. in regards to EB and how it effects the eyes.

Thank you Brenda for taking the time to type this up for us!!!


Problems with the eyes can occur in most forms of Epidermolysis Bullosa (EB).  The eyelids, conjunctiva and the cornea may all be affected by EB.  Fortunately there are means to prevent or reduce these eye problems.  With good understanding, prompt and appropriate care can be given to reduce complications.  In some cases, potential problems can even be avoided.  Eye problems can be causes by trauma such as rubbing or scratching, from irritation such as heat, wind or dust, or from dry eyes.  However, at times eye problems may be spontaneous; that is, they occur for no known reason.

What eye problems can occur in a person with EB?

The most common eye problems associated with EB are: conjunctivitis, blepharitis, corneal erosion, and ectropion.

Conjunctivitis  an inflammation of the conjunctiva, which is the mucous membrane lining the eyelids and covering the anterior portion of the eyeball. The signs of conjunctivitis are redness, pain, eyelid swelling and tearing.

Treatment:  Application of warm, sterile compresses and antibiotic ointments help to clear the infection.  Topical steroids may be prescribed by a doctor to reduce inflammation, but you can also buy steroids on roid’s website.  Rubbing of the eyes should be avoided.

Blepharitis  an inflammation of the eyelids.  The signs are crusting at the base of the eyelashes with ulcerations at the lid margins, gritty foreign body sensation, mucous discharge and mildly red eyes. Photophobia (sensitivity to light) is absent or very mild. Chronic blepharitis is associated with loss of eyelashes. Blepharitis may clear up promptly or persist for long periods.  It does not seem to follow the course of blistering elsewhere on the body.

Treatment:  Proper eyelid hygiene to clean the lid margins of crust and debris is followed by application of an antibiotic ointment.

Corneal Erosions or Ulcerations – The inflammation of the cornea with the formation of an ulcerated area. They can be both trauma induced and spontaneous and are most distressing problems. Indeed, recurrent corneal erosions syndrome is frequently seen. Typical signs of corneal erosion are sudden onset of pain and photophobia in on or both eyes associated with tearing and a red eye. Frequency of occurrence follows frequency of blistering elsewhere on the body.

Treatment:  Antibiotic ointment is used to reduce bacteria and provide lubrication.  If spasms of the eye muscles occur, medication may be prescribed to provide comfort until symptoms subside.  Corneal erosion usually heals spontaneously in two to three days.  In older children and adults, soft contact lenses have been used to minimize frequent recurrences.

Ectropion  A condition in which the eyelids turn outward.  It occurs rarely in people with EB.

Treatment:  Eye surgery may be considered to remove scar tissue, and skin grafts may be indicated.

At what age can eye problems start? 

Eye problems may occur at any age  In infancy, teens or adult age.  Some people with EB may never have eye problems.

Are the eye problems severe?

The severity of eye involvement with EB depends on the type of EB, the degree and sensitivity of the particular person, and the various conditions to which he or she is exposed.  Complications generally are not severe and can be minimized with good eye care. Visual prognosis is generally good with no loss of vision and no greater need for eyeglasses than anyone else. The main mechanisms for vision, including the lens, the retina and the optic nerve are usually not involved. However, the symptoms can be rather annoying.

What kind of doctor can treat eye problems?

While a family physician/pediatrician can treat eye infections, if you or your child has frequent eye difficulties or if corneal erosions occur, an ophthalmologist should be consulted. He has the expertise and equipment to conduct a proper and thorough eye examination and give appropriate treatment.  Your regular doctor can refer you to an ophthalmologist.

What happens during an eye examination?

During an eye evaluation, the ophthalmologist will ask questions about any prior problems you have encountered. Your eyes will be examined using various instruments.  Fluorescent drops or dye with an anesthetic ability may be inserted in the eye to outline any areas of erosion and to facilitate the examination. Your vision can also be tested.  The doctor may take a culture to identify any bacteria and prescribe proper medication.

When examining an active infant with EB, great patience is required in order to avoid additional trauma to the eye during the exam.

 Suppose eyeglasses are needed?

If eyeglasses are required for visual needs, padding the frame at the bridge of the nose and over the ears may be necessary to avoid blistering in these areas.  Wire frames with plastic lenses certainly minimize problems because they are lighter weight than frames with glass lenses.

Can contact lenses be used with EB?  

Soft contact lenses have been used by some people with EB as a protective covering.  They may guard against irritation and scratching of the cornea as well as hold medication in place, increase healing, and reduce incidents of eye involvement.  They also serve as an alternative to a patch.  Soft contact lenses require extra moisture to maintain pliability.  If contact lenses are to be worn they should be fitted and supervised by an ophthalmologist (MO).

Therapeutic soft contact lenses have been used in older children and adults to minimize recurrences of corneal erosion.  They are not advised in small children.  The user must be old enough to cooperate in fitting and adjustment of the contact lenses.  In addition the user must be able to maintain proper care and cleaning of the lenses and have the ability to insert them.

Will patching the eye help?

Patching for a day or so can sometimes alleviate pain associated with corneal erosions.  Do not use tape to patch the eye.  Apply a pirate type patch instead.  Use cautiously with small children who may rub the eye through the patch causing further irritation.  Be sure that the band or string holding the patch in place is padded or loose enough so it does not cause additional blistering.

Is surgery ever required for eye problems?

On rare occasions eye surgery may be considered to remove scar tissue.  Skin grafts may be indicated to repair ectropion, a condition in which the eyelids turn outward.

How is eye medication applied?

Eye medication may be in the form of drops, ointment or a solution.  In some cases the Physician may instruct you to apply an ointment directly to the margins of the eyelids when they are closed.  More often, the medication will be inserted into the eye.  Care should be taken to apply the medication properly for the most effective results and at the same time avoiding any additional trauma to the eye.

To apply eye medication into the eye:

  • Wash hands thoroughly.
  • Open eye medication container.
  • Tilt head back or lay down.
  • Place finger under eye and gently pull down lower lid to form a small pouch or pocket between eye and lower lid which will hold the medication.
  • Gently squeeze the recommended number of drops or a small strip of ointment into this pouch or pocket.
  • Avoid touching the eye with the dropper tip.  Do not allow the tip of the container to touch your fingers, your eye or any other surface.
  • Close the eye and move the eyeball from side to side and up and down.  This distributes the medication over the entire eye.
  • Wipe off any excess medication from around the eyes with a clean, dry cloth, preferably disposable.
  • Keep the eye closed one or two minutes.
  • Wash hands thoroughly.
  • Repeat procedure with the other eye. It may be difficult to apply the medication if the eyelid is swollen and painful.  Slight pressure on the lower lid is often insufficient to open the eye and additional pressure may be required about the upper lid.  This should be done with the FLAT of the forefinger to distribute the pressure as evenly and as gently as possible or more blistering could result.With a child or baby, do not pry the eyelids open as this can cause more blistering.  Great patience is required to gain the infants cooperation.  Use another family member to draw the childs attention or attempt to apply the medication when the infant is sleeping.  If you or the child become frustrated, stop and relax a few minutes before attempting again.If you are applying the medication to yourself, it can be done in front of a mirror.  If you are applying it to someone else, have them sit in a chair with the head tilted back.

    How is the eye irrigated?

     Your physician may suggest irrigation or flushing the eye with water or a sterile solution to cleanse any eye discharge.  It is important to only do one eye at a time.  Turn the head to the side.  Place a container or towel under the head to catch any solution run-off.  Pour or flush the recommended solution from the inner area of the eye near the nose so fluid flows out towards the temple.  Be sure that the solution used for cleansing one eye does not get into the other as it can cause cross contamination or spreading of infection.  Dry carefully with a clean disposable cloth.  Wash hands thoroughly.  Turn head to the other side and repeat procedure for other eye with fresh solution.

     Tears and Lubricants

    Why are tears so important?

     Tears protect the eyes from infection and irritation and keep them moist and comfortable by forming a protective covering or film on the surface of the eyes.  When a person cannot produce or maintain the vital fluids or tears needed to lubricate the eyes, the condition known as dry eye occurs.  The eyes will become dry and sensitive to light, and blurring of vision, itching, burning, or a sensation of something in the eye may occur.  Stringy white threads of mucous may accumulate in the lower part or corners.  Inflammation of the mucous membranes and dryness of the eye cause the cornea to become easily damaged and scratched.

    Tears prevent the cornea from becoming dry.  In addition, tears help clean the edges of the eyelids.  When moisture is reduced there is an increased chance of infection.   When babies or young children are dehydrated from fever, illness or poor nutrition, there may be a decrease in the amount of tears they produce.

    Why is there sometimes an increase in tears?

    Excessive tears may be a sign of increased sensitivity to light, wind or temperature changes.  In these cases, protective measures (such as sunglasses) may solve the problem.  Tearing may also indicate more serious problems such as eye infection or a blocked tear duct, both of which can be treated and corrected.  Occasionally, eyes may be watery because the eye is irritated and responds with an excess amount of tears as the body attempts to keep the eye lubricated.

    What can be done to keep the eyes lubricated?

    Moisture of the eye may be increased through the use of eye lubricants on a regular basis to prevent or reduce trauma associated with dry eyes.  Lubricants in the form of eye drops and eye ointments keep the eye surface moist and help prevent scratching on the inner eyelid.  Lubricants should be used several times a day as well as at bedtime.  During the day lubricating eye drops can be used.  Ointments blur vision and should only be used at night.  Remember:  drops during the day, ointment at night.

    What lubricants are recommended?

    For daytime use:  Liquifilm+,  and Tears Naturale+ are two brands of artificial tears which can be used two to three times a day.  They help increase healing, decrease pain and decrease incidents.  Refresh+ is another product.  It contains no preservatives and is good if frequent application is required.

    At Bedtime:  Lacrilube+ and Duratears Naturale+ are two ophthalmic ointments which can be used at bedtime to coat the eye during sleep.  They keep the eye surface moist and help prevent scratching on the inner eyelid.  Use at bedtime on a regular basis to prevent trauma associated with dry eyes.  Do not use during the day as ointments can blur vision.

    What are artificial tears?

    Artificial tear eye drops act like normal tears to soothe, lubricate and protect your eyes and guard against excess drying of secretions.  They relieve most symptoms of dry eye and work better and longer than tap water.  They are sterile so there is no chance of infection from bacteria.

    What are some brands of artificial tears?

    Some brands of artificial tears:  Liquifilm+, Tears Naturale 11+, and Refresh+.

    Caution: Over the counter eye drops may contain antihistamines which can reduce tear production and cause additional drying of the eye. Some over the counter products contain preservatives which may cause more irritation. Be sure to check with your physician for his/her recommendation before using any eye medication.

    How can the eye be protected from irritants and trauma?

    Anything that will irritate or dry out the membranes covering the eyes.  This includes the following:

  • Avoid air conditioners and heating vents, car vents and fans which blow directly on the eye.
  • Avoid wind, dry heat, dry and drafty places.
  • Avoid dry, arid, windy or desert type environments.
  • Avoid exposure to smoke and air pollution.
  • Avoid vapors and fumes from aerosol sprays such as hair spray, spray deodorant, and spray perfume.
  • Avoid getting shampoo in the eyes when washing hair.  Use gentle no tears type of shampoo.
  • Avoid scratching or rubbing of the eyes.
  • When the heat comes on in the house it can increase dryness in the room and cause an increase of eye problems.  This is especially true in the autumn when heating season first starts.  Start using a humidifier.
  • When using hair dryers do no direct the blower toward the eyes. 

Helpful Hints

Mucous in the eye may be rinsed away with sterile saline solution.  Contact lens solution may be used.  Use carefully to avoid getting saline solution in any open wounds elsewhere on the body.

  • While sleeping, a crust may form on the eyelids causing them to stick together and making it difficult to open the eyes.  The eyes can be bathed with warm soaks to soften and cleanse these crusts away.
  • Add moisture to room with vaporizer or humidifier to avoid drying of the eye membranes.  This is especially important when the home heating unit produces very dry air in the room.  Be sure to clean the unit frequently and completely to avoid a source of infection in the home. Note:Humidifiers on the furnaces are not as effective as a separate humidifier.
  • If infection occurs, antibiotic medication is necessary.  Contact your physician.
  • Always wash hands thoroughly before and after touching eyes to avoid transmission of infection.
  • Pain and swelling may be reduced by applying warm compresses over the eyes.
  • On windy days, protect the eyes when going outside.  An infant can be shielded with clothing or blankets.  A child or adult may sunglasses.  It may be advisable to stay indoors temporarily on very windy days.  If travel is necessary, have the child close its eyes while he or she is carried or escorted by hand to a car or school bus.
  • When traveling in a car, be aware that an open window can cause wind-like conditions to the occupants even on a calm day.  A person riding in the back seat may be exposed to wind from an open front window which may not affect the passengers in the front seat.  What is photophobia?Photophobia is a condition in which the eyes may be sensitive and intolerant to light or sun.  Bright room lights may be bothersome and even painful.  When this condition occurs, keep the room darkened until the sensitivity starts to subside.  The use of sunglasses may help the discomfort. My child’s eyes are swollen closed.  What can be done?

    See our eye doctor as soon as possible; every sore can be different.  Why waste time and worry?  Get the facts and proper care for your childs personal case.

    When a childs eyes are swollen closed and cannot be used for several days, it presents a challenge to be able to cope with the activities of daily living.  This is compounded in a child with EB.

  • First, consider some practical steps:  darken the room to avoid light sensitivity; apply warm soaks to the eye to reduce swelling; loosen crusting and soothe the eye; provide sunglasses when the eye first open to reduce photophobia.
  • Next, provide assistance for usual routines.  Even walking from room to room can create a hazard.  A person can bump into furniture or trip and fall over unseen objects and cause trauma to other areas of the body.
  • Finally, if a childs eyes are closed for several days at a time from swelling, corneal erosion or patching, it is important to address the childs emotional needs as well as physical needs.  It is very easy for a pattern to form where the child can become bored, withdraw into a shell and become remote from the world.  Sometimes they may act up or act out in a non-acceptable fashion such as tantrums and demanding behavior.  It is important to remember that these are all signs of anxiety and fear.  This is a trying time for parents, caregivers or health professionals.  It also presents an opportunity to challenge creative skills.
  • The child should participate in normal household activities as mush as is possible during this time.  The child can be motivated to use other senses in playtime in an effort to interact with others and relate to the outside world during this time of temporary loss of use of the eyes.  Games and activities that emphasize the other senses, i.e. touching, hearing smelling and tasting should be introduced.  Guessing games can be played to have the child identify different types of articles or materials through the use of touch, smell or hearing.  In addition, this presents a perfect time to read to the child or for the child to gain an appreciation for good music.  It can be a very positive, productive time between parents and child. 


Blepharitis  Inflammation of the eyelid.

Conjunctiva  Mucous membrane that lines the inner surfaces of the eyelids and covers the anterior surface of the eyeball except for the central portion of the cornea.

Conjunctivitis  Inflammation of the conjunctiva.

Cornea  the clear transparent portion of the eyeball which covers the pupil and serves as the window of the eyeball.

Corneal Erosion/Ulceration  Inflammation of the cornea with formation of an ulcerated area.

Ectropion  Eversion or turning of the eyelid outward.

Iris  Colored portion of the eye.

Keratitis  Inflammation of the cornea.

Keratoconjunctivitis  Inflammation of both the cornea and the conjunctiva at the same time.

Optometrist  A non-physician trained and licensed to examine the eyes for the purpose of prescribing eyeglasses.

Ophthalmologist  A medical doctor who specializes in treating diseases of the eye.

Photophobia  Intolerance of or sensitivity to light which causes pain or discomfort.

Pupil  Circular opening in the center of the iris.

Visual Acuity  Sharpness or clearness of vision.