Epidermolysis Bullosa Registry

                                          EBCare Registry

                                  ebcare.patientcrossroads.org

                                                    
https://ebcare.patientcrossroads.org/

EBCare Registry

ebcare.patientcrossroads.org

The Epidermolysis Bullosa Registry was originally created in September of 1986 through Rockefeller University in NYC, NY.  The goal was to develop a list of EB patients with various forms of both inherited and acquired forms of EB.  Also, to create a data bank of 1) clinical 2) historical and 3) genetic information pertaining to these patients and to gather donated tissue biopsies, which include selected cells and DNA from selected EB patients to establish a permanent tissue/cell bank, from which researchers can study and to further research of all types and sub types of epidermolysis bullosa.  Below is the link to the clinical trials website that gives the basic information for the original EB registry study, which has since been completed and closed.

http://clinicaltrials.gov/ct2/show/study/NCT0000476

Below are a few excerpts from the EBCare Registry (ebcare.patientcrossroads.org) which is the 'new' EB registry:

 "a single, online database that collects and stores information provided by participants and includes personal and clinical information provided by patients with all forms of epidermolysis bullosa (EB). This information will be kept confidential by EBCare, LLC. The Registry is a research project that collects and stores information, and makes this information available to qualified researchers and others interested in studying the disorder with a view to diagnosing, characterizing, and treating the disease."

"The Registry facilitates research by all qualified investigators who wish to study patient populations that are well characterized by accepted criteria. Once established, a Registry is a research resource that can support many diverse types of investigations, including clinical trials, performed by qualified researchers to help diagnose and treat disease."

"By sharing your information with the EBCare Registry, you are helping create this valuable resource that does not currently exist." - information provided by EBCare Registry

"By enrolling in the Registry, you are in no way committing yourself to participate in any Registry-related research project.  Your personal and confidential information will be  protected in accordance with applicable United States and international privacy and security regulations such as the Health Insurance Portability and Accountability Act of 1996 (HIPAA) and the European Union Data Directive as well as applicable state or national laws."

"Vision

The vision for the Registry is to establish a global resource that will ultimately serve as a repository of information gathered from people affected by all forms of EB."

"Goal

The goal of the Registry is to facilitate communication between affected individuals, clinicians involved in their treatment, members of the health care community, and scientists who strive to advance research on the diagnosis and treatment of all forms of EB."

"Objectives

The objectives of the Registry are to:

1.     Characterize and describe the experiences of individuals affected by all forms of EB.

2.     Identify EB treating physicians.

3.     Assist the development of clinical care guidelines.

4.     Facilitate in the planning of clinical trials.

5.     Facilitate clinical trials by informing the EB community in a timely fashion about new EB clinical trials and research projects."

"By achieving these objectives it is hoped that the Registry will accelerate the development of effective therapies for all forms of EB."

"Who Maintains and Funds the Registry?

EBCare, LLC maintains and coordinates the Registry and is guided by a Board of Managers that provides oversight and includes DEBRA International, DEBRA of America and Lotus Tissue Repair, Inc.  Funding for the Registry is provided by Lotus Tissue Repair, Inc."

"How Can I Participate in the Registry?

The EBCare Registry was launched in February 2012, initially in English. In the future, we plan to develop versions of the Registry in other languages to facilitate more international participation. The Registry is accessed through the Internet and to participate you must register online and complete an easy-to-use questionnaire, which will be accessible to you after you register."

All information in parenthesis was provided from EBCare registry

PLEASE, PLEASE, PLEASE take the time to add your information to the registry.  It is vitally important to have as many patients sign up as possible in order to properly catalog as many cases of EB that they can.  The reason YOUR participation is so important is that this is where the researchers who study EB go to find the information they need to continue to search for a treatment/cure for EB.  It takes about 20-30 minutes to complete and your information is completely private and protected by HIPAA laws.  If you want to help the doctors and researchers who are searching, desperately, for a treatment/cure for EB then please sign up with the EBCare registry.  Without your participation there is no registry and, therefore, less statistical information for the doctors and researchers to pull from when studying this devastating disorder. 

“A corneal abrasion is a scratch on the eye's cornea. The cornea is the clear, protective covering over the iris, which is the colored part of the eye, and the pupil, the black circle in the middle of the eye. It's important both for vision and for protecting the eye. If you poke your eye or if something gets trapped under your eyelid -- such as dirt or sand -- your cornea may become scratched. When it does, it can cause significant pain and discomfort.” - info from WebMD.com

Corneal eye abrasions are an issue for many of those with EB.  A few examples of what can cause a corneal abrasion are; dirt, dust in eye, rubbing the eyelid, dryness, direct heat exposure (such as from opening an oven door or lifting the lid off a steaming pot), scratching, eye lash or hair in the eye.  These things can cause scratches on the cornea or, which is more common with EB, a fluid filled blister on the cornea.   

The questions I posted to others with EB regarding this issue are as follows:

1) How they occur
2) What you do to help them heal
3) What you do to make yourself comfortable while they heal
4) What supplies you use to help them heal
5) How long it can take for the abrasions to heal
6) What you do to entertain or distract yourself while they are healing

 
Below are some helpful tips from other’s with EB (or their parents) for dealing with corneal abrasions if/when they occur.  I have not used names unless given permission to do so.


1)  “I used an OB pad and Mepitac to make an eye patch for him. He is NOT happy, but he hasn't gotten it off yet!!”

2)  “wetting some small pieces of fluff, and tossing them in the freezer; when ready I take one out and press on her eye, when warm I wash it, and put it back in the freezer.”

3)  “warm wash cloths mainly, because it helped better than cold if I couldn't pry my eye open…it was stuck together from oozing all night.”

4)  “I sit in a dark room, put a cold wet tissue on it. It normally lasts a couple of days, can last up to a week.  Keeping it shut can help it heal but as I'm stubborn and don't like asking for help I try to open my eye and keep it open but I think it would heal faster if I kept it shut. I have RDEB.”

5)  Contributed by Samantha Rose Smith, teen with RDEB. (Causes = 1) Typically when the air is dryer, less humid. And just by rubbing your eye when u wake up or during the night.”

“(How to heal = 2) If they don't seem infected I apply lacralube, or aqua fore. And sleep the whole time or stay in a dark room with the eye closed.

“(Stay comfy while healing = 3) Stay on top of pain by not waiting until the pain gets worse and take it (meds) every 4 or 5hrs.”

“(What you use = 4)  Lacralube or aquarfor, if it's swelling and not healed but 5days an antibiotic eye ointment, or if you don't have ointment and it's absolutely necessary, drops but their harder and more painful to use.”

“(How long to heal = 5)  It used to take 3 days average but now that I'm older it only takes 1 1/2 days.  I still get some really bad ones that take 4ish days to heal, during/after that time I still apply loads of ointments to not scratch it, as even though u think it's healed it's not completely healed.”

“(What you do while healing = 6)  I listen to TV or music, if I can open it (my eye) I'll watch TV, or listen to mom reading me homework if I'm more behind.  I cant do anything else because I'm to sleepy and in too much pain. I have rdeb, (other or possibly inversa, I'm not sure but it's been getting kind of worse with age) and I would like to be referenced in case someone needs to know more!”


6)  “If you have to be in the light, sunglasses work wonders the big ones that cover the side of the eye. Also dilation drops help keep the eye open when going out.  That way you don’t have to strain and forcefully hold it open.  There are also numbing drops depending on the pain. Cold cloth helps the swelling and helps eye not to water.”

7)  “I scratch my eyes all the time in the summer. Actually looking into getting tear duct plugs to keep moisture in my eye.”

8)  “Humidifiers help so much in reducing the possibility of getting an eye scratch.”

9)  John N. writing for son Casey “Casey gets frequent eye abrasions.  He is 4years old with rdeb and any thing that touches his eye causes damage.  He has gotten them pillow fighting, rubbing, and walking into doors. He immediately cries and holds both his eyes closed. He is, at this point, incapacitated for 2-3 days.”

“At this point he cries every couple hours we need to keep his eye as moist as possible. We use sustain eye ointment. It doesn't have preservative.  Our ophthalmologist recommended it as the best but refresh or lacralube are equivalent.“

“He lays in his dark room and will listen to music or listens to his favorite movies, or we read to him.  We were given a dilating drop but they burn. It only helps with light sensitivity.”

“This is one of Casey’s most challenging times it is very stressful on us and most of all on him. There isn’t a lot to help with the pain he feels. Narcotics don't work, Tylenol, Motrin do nothing.  He won't put anything like a cool soak on his eye but that helps when I had a scratch.”


10)  “The healing time can vary from same day to 4-5 days, depending on the severity of it. The eyes are one of the quickest healing parts of our bodies fortunately. They occur as a result of dryness and usually happen in the middle of the night. The cornea gets dried out, and at whatever point you open your eye in the night, the top layer of the cornea gets pulled back by your eyelid, causing an erosion. Just like friction on our skin.”

“The best way to get it to heal up is lots of moisture and lots of rest. I use Moisture Eyes PM ointment and Refresh Liquigel.  If it starts getting infected at all, I see my ophthalmologist right away to get some Vigamox (antibiotic drops). I learned to get into see him QUICKLY the hard way. One time I had one turn into a corneal ulcer and was told that I stood a 40% chance of losing my cornea that time. There have also been a couple times where my doctor had to "scrape the fluff" off of it to get an even abrasion so that it would heal properly (with numbing drops of course).”

11)  ”For me, corneal abrasions are just about the worst part of EB. It is PAINFUL and scary. When I get a bad one, I am completely down and out. Couldn't go to work or school or do anything but keep it closed and keep the lights dim. Cold compresses always made mine feel better. I have had a ton of them, but was told that all of my scarring is miraculously around the part that affects my vision, and not covering it, so I have had very little visual loss from it all.”

12)  “I put lacrilube in every night or my eyes blister.  If they still blister then I use viscotears and put mepilex light to keep it closed.  The pain is awful and it feels like you have grit in your eyes for days.  I have rdeb, non hs…I have permanent scars on both eyes…the secret is to never let the eyes dry out and never sleep without ointment in them.  I use viscotears in the day and close my eyes for 1 whole minute an hour-consultant said to!”

13)  “Steps my husband uses for eye abrasions:”
“1) over the counter- multi solution rinse in both eyes.”
“2)over the counter- refresh tears 2 drops in both eyes.”
“3) over the counter- refresh liquid gel 2 drops in both eyes.“
“4) prescribed- Prednisolone Acetate Ophthalmic Suspension 1%, given once or twice daily. It really depends on the Dr. When my husband first saw his eye Dr. she told him to put two drops in both eyes, one time per day. She would check him regularly while taking this med. because she said it can cause pressure in the eyes. When she said his eyes weren’t having new growth and pretty much stable she started to wean him off slowly. He now takes one drop in both of eyes every other day.“
“5) Then the last one he does as to help with the dryness and keep eyes from sticking especially at night-time is a prescribed ointment called Erythromycin Ophthalmic. This one he applies while holding one eye lid at a time. Squeeze along the eyelid on top and on the bottom eyelids. Then another over the counter ointment called Refresh P.M.“
”This process we have followed every morning and at bedtime. He reminded me to mention to at least wait five minutes between every process. Hope this is helpful! I know it seems like a long process but the good news is that so far my husband does not show any changes and from what his Dr. said this is very good.”

14)  “I use warm wash clothes mainly, cuz it helped better than cold if I couldn't pry my eye open like it was stuck together from oozing all night…”

15)  “Eye abrasions are our most painful part of EB. My son is Junctional-nH and has gotten corneal erosion's since he was 6 months old. His first was from a fingernail scratch (we think,) but others have occurred from having dry eyes, the wind, and other types of scratches.“

”His erosion's usually last 10 days, but some have healed in 5 days and others take weeks. When he has an erosion, we give him alternating Tylenol and Motrin for pain. We keep him in a dark room and have the TV, music, or our voices as a distraction.“

”We follow his lead as to what he can or wants to do while he is healing. We put muro ointment in his eyes every 4 hours to keep it lubricated and to help reattach the cornea. If there is any sign of infection we alternate an antibiotic drop with the muro.  We have found that we can prevent most of these erosion's by using the muro ointment nightly.”

16)  “I use Refresh gel drops, then a strip of mepitac to seal the eye. I have too many young ones to get the rest I need, but the gel helps a lot. My eyes will get torn when I'm having an allergic reaction, grit in my eye, and sometimes just rubbing. Never from contacts though, oddly enough.”

Also, there are contacts that can be worn to help reduce the risk of corneal abrasions.  They can be used by most ages, even younger children (I have heard from one set of parents whose child is 2 and uses the contacts.  They said it has helped immensely in reducing corneal abrasions).  There are mixed opinions regarding the contacts.  Some doctors and patients think they are very helpful, while others feel they could possibly be hurtful.  The contacts act as a shield against trauma and/or friction.  I will be following this post with an update regarding the contacts once I hear back from the health professional I had asked for information about them : ) 


Please feel free to offer any tips, info or advice that may not be mentioned here!