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: http://www.drtracistein.com/

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

GO TO PART 6 –>>

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

Silvia Corradin

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.

Nicky’s Wound Pics Part 2

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

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

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

Left Wrist has also significantly improved with the Polymem.

Right Knee and top

Left Knee

Right Knee

Right Hand and wrist

Right wrist

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

Silvia Corradin

How To Make Surgilast Shirts

By Maria Oliveira

Finding a way to keep dressings in place for the upper body can be quite a challenge. Some people do well with ready-made garments made to accomplish this, such as Tubifast garments made by Molnlycke.

However, every patient is different and in my daughter Sarah’s case, these garments were too snug, especially in the arms, and hardly comfortable for her. For years I have made similar retention tops out of Surgilast or Spandage elastic retainers. The beauty of these home-made garments is that you can customize them, making the top itself or the sleeves as long as you like and you can make the neck come up higher to secure dressings in the collar/neck area. You can also make the armholes larger to provide even more comfort. Because Sarah has a feeding tube, I cut a small hole in the front to pull the tubing out and it holds everything nicely in place. You can wash and reuse these tops many, many times. (Note: I find that the Spandage brand holds up to more washes than the Surgilast, but either works well).

Cut some Surgilast or Spandage for the torso and some for the sleeves as the pattern below shows (Figure 1).

Figure 1

The size depends on what is comfortable for the person. Sarah is 13 years old and weighs 100 lbs. I currently use a size 10 for the torso and an 8 for the arms. To assemble the shirt, I stretch the torso piece over a box so that the corners of the box are sticking out through the armholes (Figure 2).

Figure 2

This makes it very easy to attach the sleeves. To attach the sleeves I stretch them over the corners of the box and stitch them to the armholes using a strip of 1” gauze. I attach a safety pin at the end of the gauze to weave in and out of the Surgilast as if I were hand-sewing. Knot the 1″ gauze to finish off the underarms, and you are done (Figure 3)!

Figure 3

Here’s what the finished product looks like:

Figure 4

Maria Oliveira

Mom to Sarah Oliveira (13 years old, RDEB) and David Oliveira (20 years old, EB Free).
Lives in Elizabeth, New Jersey and works as administrative assistant at Merck & Company.

What is EB?

By Emily Spurrier
From her Blog: Dys-Located

This is the first in a blog series on Epidermolysis Bullosa (EB) as part of EB Awareness Week (October 25-31).

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Some would describe EB as horrific. Some use words like “Painful”, “devastating”, “horrible”, “dreadful” or “insurmountable”. In reality, for several who do not know what EB is, there may simply be no words to describe it. EB is not like some disorders that are noticed later in life and then becomes progressively worse. EB is not just something that can be fixed with surgery. EB starts inside of the womb with painful blisters. The pain cannot be eased. Nine times out of ten, the mother is completely unaware that this is happening to their child. It only gets worse from there.

To put it simply, EB is a rare genetic condition affecting an estimated 13,000 people in the United States. For the most part children are impacted, but the disorder is lifelong. In some forms, life expectancy is only a few months. In others, left expectancy is about 30. The less-known cases have a normal life expectancy. Though there are treatments for EB, there is no official cure for EB. By cure, I mean never having any more symptoms of the disorder and ensuring that future generations are never to be impacted by this disorder again.

In all cases, though, friction on the skin, whether it be pushing a grocery cart, walking or even a simple hug, can cause the skin to erupt in painful blisters. In some instances, the skin completely comes off. In almost all cases, EB is not detected until birth. Just delivery can remove the skin from a child with EB. Then, there’s the wiping of the child, diapering, the identification bracelets, handling, hugging, bundling… all these things that happen in the first five minutes of an infant’s life can cause serious damage to a child with EB.

Epidermolysis Bullosa is fatal. The skin must be bandaged constantly to prevent infection and to protect from further pain. Imagine the pain you would feel if you scraped a small section of skin off of your leg. Now, imagine how that would feel if that scrape were your entire leg. Now, imagine what that would be like if your leg just wouldn’t heal and were constantly scraped open over and over again. This is the life of a child with EB.

When the skin of a child with EB tries to heal, sometimes, the healing is ‘overdone’ and new skin forms between fingers, causing then to fuse together. Healing wounds on the mouth causes the mouth opening to become smaller. Sometimes, eyes grow shut and sealed. Often, the esophagus strictures.

In a perpetual state of healing and re-injuring, the body tends to become anemic and sometimes starts to shut down. Sometimes, the pain is just intolerable. EB then potentially becomes terminal.

EB takes not only an emotional toll on every family impacted, but also a financial one. Items necessary for lifetime survival are sometimes not covered by average health insurance. Expenses then have to come out of pocket or other non-profit charities.

I’ve lost count of how many people I have personally known who have lost their battle with EB. Many I’ve cried hours over. Some I just bow my head, knowing there is no more pain for them.

Each October, the last week of the month is known as International EB Awareness Week and became officially so in 2006. Over the next few days, I’ll give links about EB and I will also tell a deeply personal story about how EB entered into my own life and still impacts it today.

Stay tuned.

Emily Spurrier

Emily was born in Southwestern Louisiana and has moved over 20 times in her life through nine different states. Most of her life was spent in the Twin Cities of Minnesota, where she met her husband and had her only child. Both she and her husband are also only children.

Emily was born with EBS-DM (EB Simplex, Downling Meara), and was in a wheelchair as a result until she was 18. She started improving at around 15, and this is why Kathryn is an only child.

She graduated from Stillwater (MN) High School in 1992 and from the University of Wisconsin in 1997 with a BS in Journalism. Three years later, she met her husband, George, and they married in 2002. Their daughter, Kathryn, was born early in 2004.

She relocated with her family back to Arkansas in 2005 after being away for 30 years. She currently works for the Arkansas Democrat Gazette as a Web Clerk and lives in North Little Rock.

When not taking care of her daughter, cooking, working, cleaning house, sewing, gardening, knitting, hiking, traveling or spending time with her husband, she volunteers as a Girl Scout leader over approximately 25 girls ages 5-11 for the North Hills Service Unit.

Fecal Hoarding and Constipation

Written by an Anonymous RDEB Patient

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Nicky’s Wound Pics

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

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

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

NIcky's ankle

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

Nicky's knee

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

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

Silvia Corradin

EB Info World

Supporting families dealing with Epidermolysis Bullosa.

Category Archives: Information about EB

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

Nicky’s Wound Pics Part 2

How To Make Surgilast Shirts

What is EB?

Fecal Hoarding and Constipation

Nicky’s Wound Pics