Helping Kids Cope

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Epidermolysis Bullosa, EB, is a very difficult condition to live with, even with the mildest forms.

These are things of outmost importance in helping your child coping with EB:

  •  Accept the EB and accept your child for who he is.
    This sounds simple, but it’s not. There is a certain degree of loss and mourning when a child is not born healthy, and it takes parents different amounts of time to learn to accept things how they are. This may take months or years, depending on the circumstances. The amount of time will depend on many factors… such as if this is your first child… or last, your age, and who you are and your upbringing. But, remember, the more accepting you are of your child’s EB, the less of a ‘big deal’ you make of things, the happier and comfortable your child will be.
  •  Remember, your child is #1, EB is to be treated separately and “secondly”.
    This is also easier said than done, especially when the children are small and there is so much care involved. But it’s important for the kids to grow up not thinking EB is all they are, because their little brains are working just fine, and they can do so much! Remember, most EB kids are straight-A students!! Skin care is important, as their health and life depends on it, but it is important for their mental well being, to help them be children, and you need to help them enjoy all the things in life a normal child enjoys, to play, to laugh, and to learn, with all the potential and need for joy as any other child.
  •  Don’t be afraid to consider your child ‘disabled’.
    This is one of those things no Dr will ever tell you, and parents are afraid to consider, but it’s the truth. Go ahead, get a wheelchair (it will save your child’s feet on long walks), get SSI (for financial help if needed), apply for help (Dept. Of Developmental Disabilities), get disabled plates (for extra wide spaces to get in and out of the car), it will make your child’s life much easier.
  •  Teach your child to explain EB
    There will come a time where you might be a breath away and not hear someone ask if your child got burned or has chicken pox. But your child heard and wants to answer. What is he going to say? This will happen often once he/she enters school, so, better be prepared. They can say something as simple as ‘I was born with a skin disorder’, or they can even hand out cards that explain what EB is (my favorite Option). Whatever you decide, make sure to instill in your child the notion that EB is not ‘who’ they are, just something they have.

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Coping with Baths/Bandage Changes

Bath and bandage change times are hardly fun times for either the patient nor the caregiver. The kids are scared of the ‘upcoming’ pain and they scream and holler in discontent. My son even hyperventilated-always did. He will do just about anything to get out of it, even take a nap (which he will always resist in taking otherwise).

Bandage change time is probably the only time EB kids get to release their frustrations, using it as an excuse to complain and cry about the whole situation, the powerless feeling of the condition, perhaps how they are treated by society etc. This, however, is not healthy. For kids with RDEB it’s a known fact that hollering and screaming hurts the throat-my son always throws up after a fit.
So, what do we, as parents, do to help the kids cope?

These are some suggestions that came forth from adult EBers.

  •  Diverting their attention AWAY from the wounds and the pain during the bath and/or bandage changes really work, such as watching a particular video, having someone else play with them or read them a story, or have them fiddle with a particular toy. Making a game out of bandage changes has worked with some kids too.
  •  IF your child loves a particular food, try to reserve it for bandage changes. My son *loves* M&Ms and Skittles, so I do not give them to him except during those times where he really needs the distraction. It does not always work, but it’s worth a try.
  •  Try to involve your child in his care as early as possible… the sooner the better. Whether that is letting them take the bandages off, having them hand you a needle or gauze, have them decide what limb to start with, etc. Basically, anything you can do to give them “a say” in what’s going on.
  •  Teach your child breathing techniques. Breathing helps control pain (and I know this one from having gone through 2 labors, lol), and once your child gets the hang of it, it will become second nature.
  •  Have your child pick a soft toy, they can beat, bite, punch, pull or whatever they need to do to channel their pain.
  •  Try saying things like, “I know it hurts.” or “this is going to hurt.” or, “Mommy does not like seeing you in pain but, we need to do this.” Be honest. And tell the child what you are doing as you are doing it. Instead of using the word “I” use the word “We” so, that the child hears he/she is part of what is going on and not just having this stuff done to them. Like, instead of saying “I need to pop this blister.”, say “We need to pop this blister.”. And so forth…

Helpful websites:

If you have any other helpful hints to share, please leave a comment below. Thank You!

Common Misconceptions about EB

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

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

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

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

Q. Can antibiotics CURE EB?

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

Q. Why does bleach in baths helps EB patients?

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

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

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

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

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

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

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

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

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

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

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

Q. Why do patients get EB?

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

Q. I bet there are no books about EB.

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

Q. Do patients recover from EB?

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

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

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

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

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

Q. Do all EB patients have Allergies?

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

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

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

Q. Why do patients die of EB?

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

More Questions and Answers that were sent to us.

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

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

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

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

Q&A about Recessive Dystrophic EB -Hallopeau-Siemens 

Will the skin get better with age?

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

How healthy are RD patients?

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

Does it get easier to handle emotionally?

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

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

Life with Andy

By Mary Jo Burgy

December 30, 1993 was the day we had been longing for. This was to be the birthday of our twin boys. We were as excited as we were scared. I had the “perfect” pregnancy. I gave up all the bad stuff and ate all the good stuff. All the tests indicated that they were healthy and ready to meet the world. We filled the nursery with two of everything. We talked of little league, climbing trees, riding bikes, high school sports, and all the girls hearts they were going to break. We were ready for anything, or so we thought.

At 8:34 and 8:35 am two beautiful boys came out into the world filling the room with their loud, healthy cries. We had done it. Good health and care over the past nine months proved successful. Moments later we would discover that all was not right with the world. There was something wrong with Twin 1 (Andrew). His nail beds were purple, he was missing skin on one of his fingers and he had blisters on his lips. They tried to assure me that all was okay and they would have a dermatologist called in to check him over. They brought him to me and laid him on my chest. He looked healthy to me. He was crying and crying. I talked to him and he stopped crying, he knew who had him. During the next hour or two my husband, myself and my mom spent time checking Alex and Andy over. Passing them around, taking pictures and examining them to see what parts of them looked the same. When up in our room they took Andy away and said that he needed to be in the NICU and needed to be isolated.

Having just had a C-section I was heavily medicated and do not remember a lot about what happened next. My husband went with Andy and later I remember he said that things weren’t good and that the dermatologist was going to come to talk to us. I looked around the room and I saw my mother crying, now I was worried. The doctor told us that Andy had Epidermolysis Bullosa, a rare skin disorder. He stated another boy was born in the same hospital a few years ago and the family was on their way to the hospital to talk to us. Looking back on things now, I’m glad that I had medication go get me through this. I kept calling the NICU to get updates on Andy. I felt such a loss not being there for him and not being able to breast feed. I asked to go down there to do that for him, but they stated that he would not be able to due to blistering. I still did not have a firm grasp on what this disease was. I was finally able to go see him. There was my little boy in a glass box wrapped with gauze and boo-boos on him. When they handed him to be he was on a large piece of wool. I didn’t think that I would ever stop crying. How could this be happening? I did everything right. I immediately blamed myself. I’m the one who carried him the last nine months, I must have done something wrong.

Later they explained that it is genetic. It was nothing that I did. No one on either side of our families had anything like this. They explained that Andy’s body will be covered with blisters and missing skin. His hands and feet would web together. It would involve his esophagus and mouth. His nutrition would be compromised. This was all so overwhelming and frightening. Before we took Andy home we had to learn so much about him. We had to learn how to hold him, feed him, change him, what clothes he could and could not wear, etc. Most important, we had to learn how to take care of his skin. This meant learning how to wash him, pop his blisters, bandage him and protect him from infections. The hardest part was the day when I had to leave the hospital without one of my babies. I just wanted them to both be home with me. Many people say that they could never do what we do everyday. When you see your son needing help, attention and love, you would and could do anything for him. My husband and I were committed to making this work and to make our son better. We would soon find out that we could not make him better, but only care for him the best we could.

Finally with both the boys home we were ready to go on with the rest of our lives. We learned quickly how to bandage and were doing his dressings in record time. Mechanically things were going well, emotionally it was tough. We were basically alone other than visits here and there from family members that lived out of state. Having twins is difficult, but having one with difficulties almost seemed impossible. EB is a painful disease. Having to hear your child cry daily from dressing changes and baths, it breaks your heart and leaves you feeling helpless because you can’t do anything to take the pain away. There were many times after his birth that we wished his life would end. He could go to Heaven and be pain free. It was not fair for someone so small to have to endure this. There were many times when I would just break down. I would hold Andy and tell him over and over again how sorry I was. Through all this we still had to remember that we had another little boy who needed just as much love and attention. He had the instinct to be calm while Andy was being tended to and when that was done he would act up and we would care for his needs. There were many times when Alex would be calmly playing while we are tending to Andy and if we poked Andy with a needle Alex would cry out too.

Twins are amazing and wonderful. Andy is about to turn 6 as I write this. Life is easier and harder at the same time. He can tell us what hurts. He can tell us about his boo-boos and has taken on the responsibility to pop them on his own at times. He is more vocal about his disease. He will scream about how much he hates this disease.  He will cry and wonder why he can’t have skin like the rest of us. It seems is cries from pain are even louder now. I hear he and his brother planning quietly in their room about what fun things they will do when Andy gets a cure for his skin. I watch his brother run along with friends ahead of Andy and I comfort him when he cries because they left him behind. I see hear about his day in kindergarten. How excited he is everyday about what they do. Teachers tell me how hard he tries to not be different and how he fights being catered to. We find it amazing that gym is his favorite class. He is a survivor.

When you have a child with special needs you learn many things. Life is not to be taken for granted. Your life can change in an instant. We always thought we would have “perfect” kids. We took it for granted that we would. Why would anything go wrong, that happens to other people. You learn to appreciate many things. We appreciate the moments when Andy seems free and is running and jumping around. Most parents tell their kids to “stop jumping on the bed”, we join him. A smile, a kiss, and a warm hug mean so much more. When our boys want to play, this is what we try to do. The dishes, laundry and dirty floors can wait, our boys cannot. We appreciate many things that thankfully most parents do not. We enjoy days when Andy wakes up and isn’t stuck to his clothes or his bedding. A day without Andy crying in pain. Days without blood on his clothing and ours. We actually have become experts on getting blood and bacitracin stains out of clothing. When a bath is less than three hours long and his knees are the only thing we have to wrap are as close as we come to being “normal”. You just never know when life can turn. Today is a gift, that is why they call it “the present.” Many times I think he is stronger than his mom. Just when I think that I cannot handle one more cry, one more bandage, one more set of clothing filled with blood Andy offers me relief. After a painful episode he reaches for me, grabs my neck, looks at me like he is peering into my soul and without a word just hugs me as tight as can be as if silently saying, “It’s all right mom.”

True heroes are not in comic books or on the movie screen, but are actually real people right in front of you. He is our little “Hercules”. He is only 5 and he has been our teacher. He has an unrelenting spirit. He doesn’t let his limitations rule his life, he just tries his best everyday.

Andy became an angel on March 25, 2003.

Making Goals and Dreams Come True

By Bruce Gunn

As printed in the Summer 2000 issue of DebRA Currents

Bruce Gunn in DisneyWorld!

Bruce Gunn in February 2000 in DisneyWorld during the ‘Have a Heart for EB’ family event.  

During my teenage years I knew I was beating the odds given to me at birth. With every birthday I was achieving the impossible.
In high school I was treated fairly equal to everyone else, needing few adjustments to make getting around easier. Getting out of each class five minutes early to be on time for the next class and drinking soda toward the end of the day, kept me from being tired for the last class and improved my attention capabilities. Both were small changes in my schedule that made a big difference in my school success.
I knew that after graduation I would be able to work, but did not know what I would do. My special education teacher was a great source of encouragement for me, telling me I could drive and live independently. It was in her class that I first used a computer, giving me direction for work.
Graduation was an important milestone in my life. At the time of graduation, 1986, I was 19 years old but still looked like a little boy. I proceeded into Vocational Rehabilitation to help me find a job. I was placed with Janus Developmental Services, Inc., a workplace for mentally and physically handicapped adults. Janus brings in work from area businesses for the clients to work on for a paycheck. After hand surgery in the fall of 1986, I was able to begin work at Janus in the spring of 1987. I started at a workshop in Tipton, Indiana, a small workshop with about 20 clients working.
I knew I wanted a job with computers and was able to work making computer labels for Fisher’s Guide. The job consisted of receiving orders from Fisher’s Guide on when the labels needed to be run, description and part number of what they were. These labels consisted of all sorts of part numbers and descriptions. We had to make sure we had the right label format and I needed to just learn the ropes of something new, knowing the different labels and part numbers. We also had to tear the labels and package them. At first I would tear off one at a time and soon learned how to do more than one at a time and do quality work at the same time.
After working at Janus for about one year I began thinking of moving on from the workshop to a community job. This would necessitate learning how to drive. Janus vans had provided transportation to and from work for those who couldn’t drive, but now I would have to drive myself. I knew that my Dad wouldn’t be the best one to teach me after seeing my two older brothers and sister go through it. I knew I needed someone more patient for a driving instructor. Vocational Rehabilitation sent me to Crossroads Rehabilitation Center in Indianapolis where I first learned to drive. On the first day I went to to downtown Indianapolis, the Monument Circle. By my second day of driving I went on Interstate 465 and passed a Semi going 70 mph After my first driving class, my mom came home and said to my dad: “Put your thinking cap on and change it, because your son is going to learn how to drive”. My dad wasn’t sure my leg reflexes were good enough to drive a car, but I knew I could do it. My special education teacher always helped by saying I was capable and I had the confidence in myself.
My driving lessons were three days a week for 21 sessions and would be like 6-8 weeks of driving education. I only needed 13 driving lessons, by the end of my 13th session, I got my license and found a car. On July 7, 1989 I got my license and my car, a 1986 Oldsmobile Ciera. I knew this was important for me to be more independent. Nobody told me I had to get my driver’s license, Bruce Gunn in the wheelchair, surrounded by, on left, Jennifer DePrizio, her sister Jody, her dad, and Randy CameronI just wanted it and went for it. No one stepped me and it was my next major achievement in life. 

February 2000 – Again in DisneyWorld.
Bruce Gunn in the wheelchair, surrounded by, on left, Jennifer DePrizio, her sister Jody being hugged by Taylor Cameron, Richard (Jennifer and Jody’s dad) and Randy Cameron

After working for 2 years, I began to get irritated-I wasn’t going on job assignments and it was hard to find a job I could do in the office. I needed a chance to try and an employer to five me a chance. As work was slowed down, around Christmas, I took time off from the workshop. In this way I could get their attention because I wasn’t happy still being in the workshop and wanted a community job. At one of the annual meetings, our workshop director mentioned that I wasn’t getting the credit I deserved, that I had worked long and hard for three years at the workshop. After all the work I had done for the organization, I appreciated the director/manager standing up for me.
At the end of May 1990, after being there for 3 years, a position became available in the Janus front office starting a data information computer system. This meant putting client’s data information in the system and typing the annual and semi-annual client reports required by state law as well as learning other skills in the office. I had to drive 30 minutes to Noblesville and knew the job would mean traveling. The secretary had known about me in the workshop and wanted me for a position as a receptionist at one of the other workshops, but I couldn’t take it because there was no central air in the workshop at the time. When this job became available at the office, she had me in mind. My social coordinator thought about it thought and I wasn’t happy with the social coordinator anyway: three months later in October of 1990 I wasn hired and began my training.
It is hard to believe I’ve been with Janus for 10 years now and have undergone many changes through it all. When we went without a receptionist and needed someone dependable, I was the one to fill in. I’ve worn many hats at Janus, taking on various responsibilities at the office. I have 5 case coordinators under me, one handling the adult clients 18 years or older in the workshop as well as 4 case coordinators for children who are developmentally delayed in their fine and gross motor skills and speech/language development for children under 3 years of age. We have about 150 children in the program and 65 adults. I type letters to Doctors, parents and type case conferences notices to be sent out, as well as keeping the data information system updated, typing annual and semi-annual client progress reports throughout the year in addition to being the backup receptionist when she is sick, at lunch or on vacation. Whatever needs to be done, I’m the one to do it for staff support in the office.
I’ve learned much in my ten years as an administrative aide. Until five years ago I didn’t even need to use my medical insurance, when I needed to have my first hand surgery. I have been using my insurance since then and the benefits are great, not to mention the pay is getting better.
If you are EBer you must never lose the dreams or goals you want to achieve in life. My parents never told me ‘no’ and I did what I was capable of doing within reason. I realize parents are protective of EB children, but you need to let them explore what life has to offer and at least encourage them to find out for themselves what they are capable of doing. Only your child knows his or her limits and not every EBer is capable of doing what they set out to do. I want’s always sure of myself, but I knew there wasn’t a cure for EB and sitting around wasn’t good for me either. I was on disability when I first turned 18 and got off when I started working full time. I wanted more than what disability could give me, but that was my choice.
Having RDEB isn’t easy and working full time isn’t always easy either. There are many days I go in whether I am sick or not, when I need to push myself, but that’s me. Co-workers would know if I wasn’t feeling well, or walking really slow because I broke out in blisters on my legs and knees, but I never complained and moved on. Now and then I will complain, but there is nothing wrong with that. I have learned to pace myself and do what I’m capable of doing and not worry about what I can’t and move on.
Focus on what you can do, not what you can’t. Parents, don’t give up hope for your child and think positively about your child’s future with EB. One day we will have a cure, so don’t stop living life and don’t think there is no hope for your EB child, because there is. I’m just one example of and EBer who is making it and there are many more out there.

Leo

By Debbie Kay Hatley

In search of a new pair of eyeglasses, I entered a well known optical shop. It wasn’t very busy perhaps a handful of customers, one or two were being fitted with their new prescriptions.

Maria and Leo

Maria and Leo

I began browsing through the assortment of frames this shop had to offer. In the process of returning a set of frames, I heard a female voice from across a display rack say ” Excuse me, but can I ask you… what’s wrong with your hands?”

Suddenly all that emotion from deep inside me began to surface.
The years of those same dreaded questions asked in public places, making me feel different … set apart from others.
Some strangers would stare. Some of them thinking this condition might be contagious would make rude comments or gestures.
My feelings of hurt and embarrassment could make me reply with a hint of anger or sarcasm towards some. Most often however, it was easier to pretend I never heard their comments.
I felt somewhat relieved that on this day the store wasn’t overly crowded with customers whose prying eyes would make this more difficult for me to deal with.

Turning to reply to the female voice, my heart was immediately soften by the tender eyes which met my own. She must have noticed her question had caused me to feel uncomfortable, for the next few words from her were filled with unexpected kindness towards me.
“I’m sorry, I hope I didn’t embarrass you but I think you might have the same condition that my son was born with.”

If she didn’t before, she sure had my attention now, for most people (mistakenly) associated this condition with that of a burn.
“Leo (my son), was born with a rare skin disease called Epidermolysis Bullosa. It causes him to get huge blisters as well as much of his skin to tear away leaving large, raw areas of skin exposed.”

Debbie Kay and Leo's hands

Debbie Kay & Leon's hands

I felt a lump in my throat as I had just heard her give a very good description of my own skin disorder. Within minutes we were introduced, enjoying the company and the conversation of a rare topic.
Maria continued talking about her young son and she did so with much enthusiasm and pride for Leo.

Her eyes lit up when she spoke of Leo’s accomplishments, and tears fell as she shared with me the pain he went through. A short time later we had to end our conversation, but before I left we had exchanged phone numbers and a promise that we would get together very soon. Several weeks later My Mother accompanied me on a visit to Maria’s home. “Come here, mi hito,” Maria called to Leo as she tried to persuade him to come out of hiding and meet us but he appeared to be too shy. He would wander back to his room or hide behind the sofa wanting nothing to do with these strangers.

I sat relaxing on the sofa, listening to everyone talk.

“How old were you when you began to loose all your fingernails?” Maria asked, but before I could respond to her question I caught a glimpse of Leo. He had quietly come from behind the sofa where I was sitting and stopped very suddenly in front of me. He began glancing first at my hands, then his, back to mine again. Taking notice that his little hands looked very similar to that of mine. The lack of nails, a few blisters and the deep scarring were telltale signs that Leo and I had something in common.

Eric and Leo

Eric and Leo

Looking over at his mother and pointing one of his small fingers towards my hand Leo replied in a loud surprised tone.”yaya’s Mama, Yaya’s.” We all began laughing at the connection he’d made. Leo however, was still looking wide-eyed as though he’d just revealed a secret to everyone.
He became my little buddy that day when even in his young, childlike mind he knew we shared something very rare indeed.

A visit to her Doctor soon confirmed the fact that Maria was again pregnant. Her doctors advised her to terminate the pregnancy due to the fact that there was a 25% chance that this child might also have the same condition as Leo. Maria’s strong faith in God gave her courage to believe that a life though fragile and having countless limitations should be respected and cherished.
She soon gave birth to her second child (a son) who is EB free. She watched as her two sons’ grew.

Juggling her time between her job and the role of Mom. Maria gave great care to Leo, tending to his many wounds, draining blisters and the many bandage changes that had to be done. They even traveled to Germany seeking treatments for him. Always going forward, never quitting in the pursuit that eventually something could be found to benefit the health of her child.

On July 3, 1986, Maria and her small family were heading out on the highway to visit relatives for the up coming holiday celebration. In a head on car accident at the very young age of 22 Maria’s life was taken, leaving behind her husband and two young sons. Loosing her had left a huge void in so many lives.

It would be years before I saw Leo again, though on several occasions I would call him and his family to hear how he was doing. He had changed over the years (as I’m sure I have too). Still somewhat shy, very soft-spoken, he no longer was the little boy I remembered but a young man of 19.
His disease has progressed beyond my own. His feet are now encased due to the reoccurring scar tissue that has built up. Leo has had to have his esophagus removed and replaced with part of his colon. He is considering hand surgery that will release the digits of his hands, which are now fused.

Leo's family with Debbie kay

I realize now that the constant stares and comments from strangers had begun to effect me. I was developing a rather bitter and cynical attitude towards people. Some of who were rude, but most are just curious. Perhaps the pain we go through (if we allow it to) can create a heart that is tender and compassionate towards others. Helping those who might be going through similar circumstances, In fact, isn’t that most of what our journey is all about?

Pictured: Leo’s family with Debbie Kay

So, remember the next time you meet a stranger. You never know when you might create a lasting friendship or have an opportunity to converse with an angel unaware.
God bless you in your journey,

Debbie Kay Hatley
February 2, 2001

About Eddie Paul


By Helen Kling

We would like everyone who is affected by Recessive Dystrophic Epidermolysis Bullosa to know our son Eddie Paul.  This is very much a success story, and hope that by sharing this, it will give others inspiration as he gave to all who knew him.

We already had a little girl who was almost three, when we were excited to find out that we were expecting again.  On September 20, 1957 Eddie was born but things did not go as planned.  He was born without the skin on his legs and feet and one finger.  He blistered easily to the touch, and in 1957 no one knew what was wrong with him.  Finally two months later the doctors told us they thought it was EB.  They also said he probably wouldn’t live very long.

We brought him home and I learned how to bandage him.  I learned very fast that I had to pick him up by supporting his bottom and chest rather than under his arms.  It was hard raising Eddie because I had no information and it was all trial and error.  I wish I had known more because I could have avoided so much suffering for him to go through.

He couldn’t suck from a bottle because it blistered his tongue.  So he learned to drink out of a cup when he was seven months old.   It was hard taking him anywhere because we had to explain to the public about his condition, even though no one understood.  But we took him anyway because we felt everyone should know about EB.

Our doctor had prescribed penicillin for both children just to ward off infection.  We let them play and do almost everything that other children did including playing in the sandbox and dirt.

Ed with his daughter Melissa

We knew some damage might occur but they would be better off emotionally.  When Eddie asked to ride a bicycle, I felt bad but I had to say no because that would be too dangerous.  I told him that as soon as he’s old enough I would teach him how to drive a car.

As Eddie grew older he learned what he was able to do and what he couldnt.  If he couldnt accomplish things normally he would find a way that he could.  Actually he was a good inventor.  He was active in school and enjoyed impersonating famous people.  He liked to make people laugh and made a lot of friends.  Our favorite impersonation he did was Elvis.

Ed went to Kent Sate University and received a bachelors degree in Telecommunications.  He had various jobs including NBC, cable TV and Case Western Reserve University.  His last job was with Rainbow Babies and Children’s Hospital of Cleveland .  He was the producer of in-house programs on the Rainbow Channel.

He met his wife Sandy and married in 1991.  They had a daughter, Melissa, in 1995.

Ed was diagnosed with metastasis squamous cell cancer in the summer of 2001 and found out it was terminal on 9/11.   He suffered and told me its quite painful and I told him that I understood, because what hurts him hurts me also.  He fought a courageous battle but died October 4, 2001 at the age of 44.

A plaque now hangs at the hospital where he worked with the motto, Do just once what others say you cant do, and you will never pay attention to their limitations again.  This is the way Ed lived his life and why he is a success story.

Ed with mom and dad

We were very proud to be his parents.  We will miss him but will always have him in our prayers and in our hearts.

The ABC Method of Wrapping

By Sheri Coil

A bit of History

Sheri Coil and her family

Shortly after Christmas one year, we received a phone call asking us if we were interested in caring for two little boys with a very rare disease that affected their skin. We are caregivers to medically fragile children and like to care for those with ‘challenging’ conditions. The oldest, Corey was 14 months and the younger brother, Alex, was only five weeks old and not yet five pounds. They had Recessive Dystrophic Epidermolysis Bullosa: Hallopeau-Seimens subtype, a rare double recessive genetic disease that affected the integrity of the collagen VII area’s in the skin. This meant that any shear pressure on their skin would cause it to lift causing blisters to form or for the skin just to peel off at the lamina densa level or in other words at full depth of skin. The fibrils that hold the skin to the flesh are made of Collagen VII are greatly compromised if not non existent. When the blisters form in an area of damage, they fill with more and more liquid and keep growing larger and larger since the anchoring fibrils were not there to hold the surrounding tissue together.

We said that we would take them but had no idea of the ‘challenge’ we had accepted. They were both hospitalized in Packard Children’s Hospital at Stanford for ”wound management”. Both children were on morphine for pain and in very poor condition. Corey had an absence of skin on about 75% of his body, his toes were webbed over completely and his fingers were encroaching his palms they were so badly mittened and contracted. What everyone failed to tell us was that no one expected them to live long enough to make it back to the next clinic appointment. Which turned out to be a good thing in the long run. We got them and only saw future. We did not concentrate on death, but rather on life! It was a year before we were told this expectation. When their little sister, Brandi, was born the next year, she also joined the family.

First Care

Care instructions were few and supplies were hard to come by at first. We were told to soak Corey every day in a whirlpool bath that had bleach and salt added. We were told not to try to wrap too much. Not to try to wrap to protect them, that they would get blisters anyhow. That the fewer bandages would give them more room to move as the wrapping would get in their way.

For some reason, we just could not get connected with the insurance and suppliers. The insurance that covers them will and does pay for all their supplies, but putting the three parts together was a frustrating challenge. But we persevered and prevailed! In the meantime we learned to stretch a bandage many ways.

Alex was released from the hospital a few days before Corey as his wounds were not as infected and serious. We had to stop at another hospital for our daughter to have some tests before we went home. I was holding this tiny scrap of humanity and noticed he had what looked like a chunk of raw liver in his mouth. Alex had formed a huge blood blister in his mouth and when it broke, it was impressive. This was our first blister and it was a good one! This was not what babies are supposed to be all about.

On the way home from the hospital the day we picked up Corey, he threw up in the car. We thought that it was car-sickness. We should have been so lucky! He had a bad case of flu with all the things that are inherent with it. It was impossible to keep him clean on either end. It is bad enough to have any baby get the flu, but one who is wrapped up like a mummy is really impossible to keep clean. No just stripping off the soiled clothes and washing the baby here. All the bandages had to be changed too. A two-hour job. Only he was not lasting even that long between bouts of nausea and diarrhea. He finally dehydrated so badly that the hospital near us could not find a vein to put the IV in. And this was made worse by the fact that every nurse they called in was frightened by his EB, they offered us a free vein screening afterwards, but I was kind of scared. Finally, in desperation, the Dr. put it in his jugular and they taped it to his neck in spite of my protests. They then transported him down to Stanford for another weeks stay.

The same week, Alex, who was almost big enough to see came down with pneumonia. And of course the technicians in the radiology lab, who I warned against handling him wrong, took all the skin off his feet and a goodly part of his upper body. I was very upset and vowed that this would never happen again as I would be a very involved and vocal, even physical, guardian angel from then on. And I have been.

This was our first week’s introduction to EB. We were impressed by our experiences and worried about the boys. As challenges go, this was a first rate one. It would take everything we could give it. And then some!

Things we observed and the evolution of how we care for them:

The boys were in extreme pain during bath time. They constantly developed new blisters during the process. Especially on areas like the heels and bottom. Picking them up, Corey with his mass of open wounds and Alex, so tiny and easily damaged, was very, very difficult when they were completely naked and that warm, wet skin seemed to be even more fragile. So, rather than completely stripping them and doing a full bath daily, we began to wash and rewrap smaller areas like the upper body, using a small tub (one or two gallons) of water with bleach (Dakin solution) and salt added. Using pieces of Kerlix as a washcloth and running the water over the wounds that didn’t fit in the tub with a turkey baster or the gauze squeezed out over them. Also, we keep a spray bottle with a gentle spray full of bleach water to spray on small areas quickly. After we wash, medicate and rewrap the area, say the arms and torso, we then go on to the next, lower body and feet, and repeat the procedure.

We noticed that whenever an area started breaking down, it continued to break down. It was fragile and very susceptible to damage. Those fibrils were broken and did not reform well. As the children got older, the skin areas that had never broken down, were stronger and not near as easily damaged as those areas previously blistered. And that when a blister was growing, it would stop dead where ever there was a bandage. So the wheels started turning. If the bandages could stop blisters from growing bigger where they would stop them, could they prevent the blisters from forming at all. Could this preventative, or prophylactic, bandaging keep that fragile and oh so tender baby skin in tact for a while and maybe the skin and fibrils, what few there were, might become stronger with aging. Thus began our journey of discovery, to find every possible way to wrap all the body successfully to protect it from the fast lane of breakdown.

The OT’s and PT’s were determined to find splints and gloves that would prevent some of the contractures from happening in their hands. We used everything we could think of to help. Plastic splints that were heat formed. Foam ones. All sorts of them. They were less than worthless and some even caused more pain and damage than they would have prevented.

We were assured that the burn glove web-spacers would prevent any more web space lost. In theory it sounded great. Reality for us was they were a no- go! They were hard to fit properly on these tiny little hands. Remember that my kids were losing web space from birth. And if they didn’t fit right and keep those web spaces very snug, they couldn’t do any good. They were stiff and the Velcro closures were bigger than the gloves! The burn garments are made from a very stiff latex. In trying to put it on fragile EB skin, it was causing a lot of shear pressure that was doing damage. A true case of the cure was worse than the illness. Any glove type product was a problem as it would do damage if it was snug enough to do a good job. I finally decided to find a way to wrap the gloves on them instead of pulling them on. So the hand wrapping technique was developed.

When ‘designing’ our wrap-on glove, we had two areas of concern or criteria that had to be met. One was that it must protect the hand and fingers from damage as much a possible and the other was that it keep the web spaces intact. We needed about a one inch bandage to wrap around those tiny fingers. The suppliers we were using assured us that no-one made a one inch roller gauze bandage. So we cut 2″ soft-cling in half to make 1″. The next year we found 1″ Conform at the hospital where Brandi was taken after her birth and changed suppliers!

The rest of our bandaging techniques just grew with the kids. We shared ideas with other parents and played with different ideas and products. We are ever-changing as the kids grow and as the blistered areas of concern move around, but all our wrapping is basically as we show you in the Wrapping the ABC Way pages. We encourage you to be creative to suit your own ideas and needs. This is just what works for us and is somewhere to start.

The kids  after five+ years of being bandaged this way are doing well. Corey still has ten fingers with very little lost web space. They are a bit crooked and are going to be straightened fall of ’99, but are still doing well, in spite of the fact that they are very fragile and the skin in tissue paper thin. I have no doubts that they would have been completely webbed in again within months had they not been wrapped. The wrapping also gives him a lot of protection for that so fragile skin and enables him to do so much with them that he could not do if they were not wrapped. He has gone from being 75% with out skin to 15%to 20%. His feet, shins, hands and elbows are almost always broken down, but the area reduces every year.

Alex maintained well with almost no breakdown, once we got him healed up, for some time  until he got the itchies. Then, the bane of EB reared it’s ugly head and he scratched himself from maybe 5% of breakdown to over 75% of his body without skin. He was a mess. A regimen of special creams (first our own concoction and then the Alwyn cream), and oral drugs (Claritin in the am and Atarax in the pm) has cleared almost all of this up, but has taken a year and has left him scarred. We had to do some very heavy wrapping to try and keep those persistent little fingers from doing constant damage. He was far more tenacious than we were. I cannot imagine what he would have looked like if he wasn’t wrapped at all. Any area he could reach was constantly blistered. But he is doing well now. His hands are exactly where they were when we started wrapping them fully at about six months, slightly crooked with a very small amount of lost web space.

And “Baby Princess Brandi” (as she calls herself) is amazing. She was a mess when we got her home from the hospital. A friend, who happened to be the attending neonatal nurse at her birth, told me that one could just stand and watch the blisters coming out all over her. This nurse was the home care nurse that came to our home when we first got the boys and gave us so much support and help, so she had an excellent handle on EB. She was able to tell the doctors right away all about Brandi. They sent her to a children’s hospital for care that knew nothing about caring for an EB child. I have never understood why she was not transferred to Stanford, just a few miles further. We did not get the final word that Brandi was ours for two weeks and could not care for her until then. As soon as we got the ok, we went to the hospital and started wrapping her. None too soon, either. As soon as her pediatrician there watched us wrap her and tell all about EB, she released her to us. Thank goodness! We had her healed up in about two weeks and has been wrapped ever since. Her feet are fragile, as they keep getting stepped on and find their way under bike tires frequently. And she got the dreaded itchies on one arm, which is hard to heal. She has had blisters on her elbows after falls and small ones on her hands as she persists in pulling off the finger wraps. (She looks like she has about fifteen fingers all the time as the wrapping stays in the shape of a finger) She takes out her knees pretty often from falling. Other than that, she has had virtually no breakdown. And is a very active child. She is even taking ballet lessons. We credit all to her being wrapped from the start.

Medical Care for EB patients

Bathing

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

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

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

Bleach – Use one capful for a normal size tub.

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

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

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

Showering

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

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

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

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

Preparing for a bath/shower

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

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

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

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

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

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

When You’re Finished

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

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

*****

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

Blood Pressure and Temperature

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

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

Blood Draw and IV Placement

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

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

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

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

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

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

Before Surgery

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

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

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

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

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

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

Miscellaneous

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

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

After Surgery

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

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

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

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

Specific Procedures/Surgeries

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

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

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

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

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

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

Dealing with Insurance Companies

By Melanie (Logan) England
(this information formerly on the EBmommas website)

Ignorance is not bliss. 90% of Epidermolysis Bullosa related claim denials from insurance companies are due to ignorance of this rare disease. As if you did not have enough to do, it is now your job to educate them on EB. People who have never seen a child with EB simply cannot fathom why we need all these bandages. Here are some steps to take to get your insurance company to work for you:

-Ask your doctor to write a plan of treatment that details the types and amounts of bandages needed for each dressing change and how often dressing changes should be done. Send a copy to your insurance company. Often since mom or dad knows the family routine best the doctor will allow you to write this yourself then they will review and “sign off” on it.
-Insist that your insurance company assign you a case manager. Having one person who can become familiar with both EB and your individual case can be invaluable. Send the case manage pictures of your unbandaged child. Pictures speak a thousand words.

Getting your claims processed

-Keep a copy of EVERYTHING! It’s much better to deal with piles of papers that to find you are missing an important form or piece of documentation.
-At each doctor’s appointment or hospital stay ask for at least two itemized copies of your bill. One for you, one for the insurance company. Almost all insurance companies will require an itemized bill rather than a receipt.
-If you have to complete your own claim forms, ask your insurance company to send you a stock of forms so you don’t run out. Make a copy of each form you fill out for your personal records, recording on it the date you mailed it in.
-Deal with a pharmacy that uses a computer system that allows them to make you a printout of all your prescriptions.

What to do when a claim is denied

-Ask your insurance company for a claim rejection in writing specifying why the claim was denied.
-Immediately file an appeal. Ask that your claim be examined by the companies’ medical review board.
-Ask each and every doctor or therapist who follows your child’s care to write a letter to the company on your behalf. Ask that they detail why the treatment or prescribed item is medically necessary and the possible complications of your child not receiving them. Keep a copy of these letters for your records.
-The best way to get a timely response for your appeal is to politely state in your letter that you will expect a response within a set period of time (ie;30 or 60 days), or you will contact your state insurance commission.
-Keep a phone log of every phone call to and from your insurance company. Note the date, time, who you spoke to and a summary of the conversation.
-Provide the insurance company with as much information as possible that supports your claim. Photographs, medical records, medical articles, and names of insurance companies that do pay for the same services or supplies.

What to do if none of the above works

-If you don’t receive a timely response or your appeal is denied, do contact your state insurance commission. Send copies of all the denied claims, all the documents you provided the company, a copy of your phone log and all of their written responses to you. State in your letter that you are filing a formal complaint about the company. The commission will document your complaint and ascertain if any further action can be taken against the insurance company. Below is a listing of the address and telephone number of all 50 states insurance commissions. We also recommend to check out information provided from the Insurance Partnership company, which is one of the best companies you could rely on now a days

Alabama Department of Insurance
135 South Union Street
Montgomery, AL 36130
Phone: (334) 241-4126…..Licensing Manager
Phone: (334) 269-3550

Alaska Division of Insurance
Anchorage Office:
3601 C Street, Suite 1324
Anchorage, AK 99503-5948
(907) 269-7900 (phone)
(907) 269-7910 (fax)
Juneau Office:
9th Floor State Office Bldg
333 Willoughby Ave (99801)
PO Box 110805
Juneau, AK 99811-0805
(907) 465-2515 (phone)
(907) 465-3422 (fax)

Arizona Department of Insurance
INSURANCE LICENSING SECTION
2910 North 44th Street, Suite 210
Phoenix, Arizona 85018-7256
Phone: (602) 912-8470
Fax: (602) 912-8453

Arkansas Insurance Department
1200 W. Third St.
Little Rock, AR. 72201
Phone: (501) 371-2600

California Department of Insurance
License Bureau
320 Capitol Mall
Sacramento, CA 95814
E-Mail: General Information
Phone: (916) 322-3555

Colorado Division of Insurance
1560 Broadway, Suite 850
Denver, CO 80202
Phone: (303) 894-7499

Connecticut State Department of Insurance
LICENSING DIVISION
P O Box 816
Hartford, CT 06142-0816
Phone: (860)297-3845
Fax: (860)297-3872
Director: Raymond Claytor

State of Delaware Insurance Commissioner’s Office
Wilmington Office:
1st Federal Plaza
710 North King Street
Wilmington, De. 19801
Phone: (302) 577-3119

Dover Office:
841 Silver Lake Blvd
Rodney Building
Dover, De. 19903
Phone: (302) 739-4251

Florida Department of Insurance
200 East Gaines Street
Tallahassee, Florida 32399-0300
Phone: (904) 922-3100

Georgia Insurance Department
716 West Tower
2 Martin Luther King Jr. Drive
Atlanta, GA 30334
Phone: (404) 656-2070
Phone: 1-800-656-2298

State Of Hawaii Insurance Division
250 S. King Street, 5th Floor
Honolulu, Hawaii 96813
Phone: (808) 586-2790

Idaho Department of Insurance
Licensing Division
700 West State Street
P.O. Box 83720
Boise, Idaho 83720-0043
Phone: (208) 334-4342
Fax: (208) 334-4398

Illinois Department of Insurance
320 West Washington Street
Springfield, Illinois 62767-0001
Phone: (217) 782-4515
FAX: (217) 782-5020
TDD: (217) 524-4872
or
Illinois Department of Insurance
100 West Randolph
Suite 15-100
Chicago, Illinois 60601
Phone: (312) 814-2427
FAX: (312) 814-5435
TDD: (312) 814-2603

Iowa Insurance Division
Lucas Building, 6th Floor
Des Moines, IA 50319
Receptionist: (515) 281-5705
E-mail: General Info.

Kansas Insurance Department
420 SW 9th St.
Topeka, Ks. 66604
Phone: 913-296-3071
Fax: 913-296-2283

Kentucky Department of Insurance
Agent Licensing Division
Post Office Box 517
Frankfort, KY 40602
Toll Free: (800) 595-6053
Phone: (502) 564-6004
Fax: (502) 564-6090

Louisiana Department of Insurance
Office of Licensing & Market Compliance
950 N. Fifth St.
Baton Rouge, LA 70804-9214
Phone: (800) 259-5300
or 5301 / (504) 342-5900

Maine Bureau of Insurance
Department of Professional and Financial Regulation
Bureau of Insurance #34 State House Station
Augusta, Maine 04333-0034
Phone: (207) 624-8475
Fax: (207) 624-8599

Maryland Insurance Administration
501 St. Paul Place
Baltimore, Maryland 21202-2272
Phone: (410) 333-6300
or 1-800-492-6116
Michigan Insurance Bureau
Mailing address
For US Postal Service mail, use this address:
Michigan Insurance Bureau
P.O. Box 30220
Lansing, MI 48909-7720
Delivery (street) address
For overnight services, UPS, or freight deliveries, use this address:
Michigan Insurance Bureau
611 W. Ottawa
Lansing, MI 48933
Receptionist: (517) 373-9273
General Information (Recorded Menu) (517) 373-0220
Licensing – Agents/Agencies (Recorded Menu) (517) 373-0234
Fax: (517) 335-4978

Minnesota Insurance Division of the Department of Commerce
133 East Seventh St.
St. Paul, MN 55101
Phone: (612)297-7161

Mississippi Insurance Department
P.O. Box 79
Jackson, MS 39205
Phone: (601) 359-3569
Fax – (601) 359-2474

Missouri Department of Insurance
P.O. Box 690
Jefferson City, MO 65102-0690
(573) 751-4126

Nebraska Department Of Insurance
Terminal Building
941 “O” Street, Suite 400
Lincoln, NE 68508-3690
Phone: (402) 471-2201
TDD: (800) 833-7352
Voice: (800) 833-0920

Nevada Department of Business & Industry Division of Insurance
Carson City/Reno
1665 Hot Springs Road, Suite 152
Carson City, Nevada 89706-0646
Phone: (702) 687-4270
Fax: (702) 687-3937
Las Vegas
2501 East Sahara Avenue, Suite 302
Las Vegas, Nevada 89158
Phone: (702) 486-4009
Fax: (702) 486-4007
E-Mail: Department Mailbox

New Hampshire Department of Insurance
Carol Beaudoin, License Supervisor
NH Insurance Department
169 Manchester Street, Concord, New Hampshire 03301
Phone: 271-2261 Fax: 271-7029

New Jersey Division of Insurance
20 West State Street
P.O. Box 325
Trenton, N.J. 08625-0325
Telephone: (609) 292-5360

New York State Insurance Department
New York City
25 Beaver Street
New York, NY 10004
Phone: (212) 480-6400

North Carolina Department of Insurance
P.O. Box 26387
Raleigh, NC 27611
Phone: (919) 733-7343
Fax (919) 733-6495

Ohio Department of Insurance
2100 Stella Ct.
Columbus, OH 43215-1067
Phone: 1-800 686-1526

Oklahoma Department of Insurance
3814 North Santa Fe
P.O. Box 53408
Oklahoma City, OK 73152
OKLAHOMA CITY AREA (405)-521-2828
In State Toll Free (800)-522-0071
Fax Phone Number (405)-521-6652

Oregon State Insurance Division
Phone: (503) 947-7984
E-Mail: Insurance Division

Pennsylvania Insurance Department
1326 Strawberry Square
Harrisburg, PA 17120
Phone: (717) 787-2317

Rhode Island Department of Business Regulation
233 Richmond Street
Providence, Rhode Island 02903
Phone: (401) 277-2246
FAX: (401) 277-6098
TDD: (401) 277-2223

South Carolina Department of Insurance
P.O. Box 100105
Columbia, South Carolina 29202-3105
Phone: (803) 737-6095
Fax: (803) 737-6232

South Dakota Division of Insurance
118 W Capitol
Pierre, SD 57501
Phone: (605) 773-3563
Fax: (605) 773-5369

Tennessee Insurance Division
500 James Robertson Parkway
Nashville, Tennessee 37243-0574
Phone: (615) 741-2693

Texas Department of Insurance
333 Guadalupe
Austin, Texas 78701
E-Mail: Elton Bomer
Phone: (512) 322-3503
Fax: (512) 322-4380

Utah Department of Insurance
3110 State Office Bldg.
PO Box 146901
Salt Lake City, Utah 84114-6901
Phone: (801) 538-3800
Fax: (801) 538-3829
TTY: (801) 538-3826

Vermont Division of Insurance
89 Main St (City Center)
Drawer 20
Montpelier VT 05620-3101
Phone: (802) 828-3303
Fax: (802) 828-3306

Washington State Insurance Commissioner
P.O. Box 40255
Olympia, WA 98504-0255
E-Mail: Kacy Brandeberry
Phone: (306) 407-0341
Fax: (306) 438-7629

West Virginia Board of Risk & Insurance Management
4501 MacCorkle Ave. SW
300 Ghannam Bldg.
South Charleston, WV 25309
Phone (304) 766-2646
Fax (304) 766-2653
toll free inside WV 1-800-345-4669

State of Wisconsin Office of the Commissioner of Insurance
121 East Wilson Street
Madison, Wisconsin 53702
Phone: (608) 266-3585
Madison: (800) 236-8517
Statewide: (800) 947-3529
(TDD) (ask for 608-266-3586)
Fax: (608) 266-9935

Wyoming Insurance Department
122 W. 25th Street
3rd Floor East
Cheyenne, WY
82002-0440
Phone: (307) 777-7401

Getting Ready for School

By Melanie (Logan) England

Dealing with Epidermolysis Bullosa in the Education System

Letting go can be two of the scariest words in the dictionary for the parent of a child with Epidermolysis Bullosa.

But eventually the time comes that our children are ready for a formal education and we have to consider the idea that they can begin to make it in the world without us. There are a wide variety of schooling options open today, and no single one will be the best situation for every child. Below are a few of those options, and some of the pros and cons of each of them.

Public School

Pros-Many laws in place to protect the rights of disabled children in the public school system.

Access to a wide variety of specialists and therapists to help your child in every way possible.

Variety of options such as special needs classrooms or mainstreaming into regular classes.

Cons-Can be a fight to get the school systems to pay for the services.

School overcrowding may leave teachers unable to meet your child’s needs.

You have to know the laws and know your rights-often the schools themselves don’t know them.

Private School

Pros-

Often classes are much smaller and can cater to your child’s needs.

Can be flexible in getting your child’s needs met as they don’t fall under the same laws public schools do.

Cons-Since they are not held accountable by the same laws as public schools, they may not have the services your child needs. They can also legally refuse to provide them.

Home schooling

Pros-

Individualized education tailored to your child’s needs.

You can “school” while your child feels well and leave off when your child is unwell.

Cons-Child’s need for socialization and peer interaction may not be met.

Parents more likely to suffer “caregiver burnout” from being parent, nurse and teacher.

Homebound Schooling

Pros-Public school system sends a teacher to your home to school your child if they are too ill to attend.

Child gets one on one schooling adapted to their needs.

Cons-

Child’s need for peers and socialization may not be met.

Child’s need to experience independence from parents may not be met.

After going over all these options with your child, pediatrician, and anyone else you might go to for advice chart our next step. If you choose a school setting then make a list and start visiting them in the spring of the school year BEFORE your child will begin. Meet with teachers, principal, special educators and therapists, and school nurses; anyone your child might interact with. Bring lots of literature on EB with you. Prepare a list of questions so you don’t forget anything.

Consider asking questions such as:

What therapies such as physical or occupational can the school provide?

Will the school prepare an Individualized Education Plan (IEP) for your child?

Will the school provide an aide to assist your child throughout the day? Will it be a one on one aide, and if not how many children will the aide be responsible for?

If your child is placed in a special needs class, will the school develop individual curriculums for each child in the class?

What are the facilities like? Is it flat or hilly, are there lots of stairs your child will have to climb?

Can/will the school provide adaptive equipment your child may need to get the most out of the classroom experience?

Does the school have a nurse?

Nicky in first grade After choosing a school invite the teacher, school aide, and school nurse to your home a couple of months before the school year ends. Let them see a bandage change and learn about the wound care involved and get to know your child and their individual needs. Take your child to tour the school and see where his/her classroom will be, where the bathrooms and cafeteria are, and meet all the staff they will be interacting with.  Once the school year starts, send a detailed care plan with your child as well as a well stocked first aid kit. Ask the teacher or school nurse to keep a daily log of any wound care that is done during the day or any problems your child has.

Get involved! Join the PTA, and volunteer to be a room mom. You will feel better being actively involved in your child’s education, and your child will be more comfortable having you around. If your child feels comfortable doing so, help them prepare a little presentation about EB to give to their class. The other students will probably be afraid of harming your child accidentally, and learning about the disease will help them all to be more comfortable. They will learn that while your child may not be able to play rough games at PE they are still a great friend to play with!

Lastly, as hard as it is, let go. Let your child take as much responsibility as he/she can for their care at school. Let them inform their teacher each day what owies hurt or might need attention, and what activities they feel they can or can’t participate in that day. This is your child’s first step into a big wide world where he will have to learn to be assertive about his needs and abilities-let him take as big of a step as he feels ready for. Just close your eyes, hold your breath, maybe grit your teeth, and pray :-).

Links:Discovery.com-list of education links

IEP information (some of this is specific to the state of California, but it has a good overview of what an IEP is and how it helps your child)

EB video for schools made by DebRA of America