Sunday, January 22, 2012

Itching is a MAJOR issue for those with any form of EB.  The worse the itching, the worse the trauma to the skin.  Sometimes there will be wounds or blisters underneath healed, calloused skin which makes stopping the itch more difficult.  Often during sleep is when itching can be a real issue as scratching can occur while asleep.  Sleep scratching can cause more damage than scratching while awake as we are unaware we are itching, therefor unable to make the conscious decision to NOT scratch.  Our main sources of heat output are head, feet, hands, under arms and groin area.  Keeping these areas cool will help reduce over all itching.  Here are a few more tips that can help with this problem.

* Please consult a physician for healthy over all body/skin temperature levels when dealing with infants, young children and the elderly *

1)  Cool showers or baths which can be followed by partially drying with the cool setting on a hair dryer or standing in front of a fan.

2)   Cold socks (this can be helpful for those with itchy feet).  Store them in the freezer (regular, soft cotton socks).  Also, you can get thin socks, keep them in the freezer, and apply one pair then (in the second pair) put the ice-less ice cubes (you can find some brands that make them with a soft, pliable plastic coating instead of the hard plastic) in the second pain and then put on over the first pair.  This allows the bottom of the feet to remain cool.  This is only recommended for those that are not yet walking, are unable to walk OR are using them while sitting, resting or sleeping.  You don't want to walk on them as this could cause them to burst.

3)  Inserts for the top of pillows and mattress's that slide into the pillow case or under the bottom sheet.  They help keep the pillow cool, thereby helping the head, neck and shoulder area remain cool and the mattress pad helps keep the entire body cool.  You can find these items online.

4)  Ice packs.  They can be placed under the covers at bed and/or nap time to help keep them cool while sleeping.   Can also be placed in strollers, car seats and motorized chairs.  I recommend the harder plastic ones as they are not susceptible to leakage from accidental punctures.  They are very inexpensive and can be found in camping sections of larger stores or household/kitchen areas of smaller stores

5)  It's a good idea to keep several instant ice packs in the first aid kit that you keep in your car and your home.  If you are out and an itching attack occurs you can pop them and place on the itching until you can get to a cooler area or allow time for any medication you may be taking for itching.  My experience with the instant ice packs is that they generally stay cold for about 10-15 minutes and cool for another 10, depending on how warm or hot the area is where they are placed.  They can stay cold/cool longer if placed on an area that is, itself, not very warm or hot.

6)  There are prescription medications that can be prescribed by your or your child's physician that are supposed to help with itching.  I recommend asking your doctor for more information regarding those medications.

7)  Topical anti itch cream, sprays and gels can also be helpful.  Benedryl brand (generic version is as effective) anti itch cream, spray, gel, hydrocortisone creams, aloe vera gel and aloe vera cooling gel (can be found at most pharmacies or pharmacy areas in your local grocery store).

8)  Lotions and creams:  Alwyn Cream, Cetaphil Lotion, all Eucerin products (Aquafor), Calamine lotion (not sure if calamine stings, I suggest trying a small spot and see the reaction before trying on larger areas).  Aveeno products, Remedy (with Olivamine) skin repair cream (can be purchased through medical supply companies like National Rehab.  Their information can be found in the post prior to this one : ) 

9)  Bath products:  Oatmeal baths (either using regular oatmeal or oatmeal products like Aveeno).

 Please see the original blog post for a few other ideas that are under the heading 'Itching'

I hope these ideas are helpful!  I would love to hear any other suggestions that I may not have mentioned.  By sharing the information we have we can all help each other learn new ways to handle the effects of EB, thereby allowing us to live a happier, less stressful life : )

Thursday, January 12, 2012

Epidermolysis Bullosa (EB)

                               
                            
         Epidermolysis Bullosa (EB)
                    


Epidermolysis Bullosa (EB) is a rare genetic connective tissue disorder that generally presents at birth with missing and/or blistered skin. Mucous membranes are often affected and can, in some types, also present airway and other internal involvement.  EB affects tissue both internally and externally and presents itself in 1 in 50,000 live births.  This genetic skin disorder does not discriminate between ethnicity or gender. No form of EB is contagious.


This blog is for anyone with any form of Epidermolysis Bullosa as well as family, caretakers, friends of those with EB and all who wish to learn about EB and help spread awareness.  My goal is to offer information about EB, wound care products and other supplies, as well as helpful tips from myself and others with EB.   

There are 4 main types of EB and many subtypes within those main types, each of which are caused by a mutation in different proteins within the genetic structure.  Below are the 4 main types and a few of the more common subtypes.

1) Junctional: Considered the most severe, and rare, of all types of EB.  Below are the main sub types of JEB.
  
   -
Non Herlitz 
     - Herlitz
   - With Pyloric Atresia
   - Inversa

2) Dystrophic and Dominant Dystrophic: Considered to be milder forms of EB while Recessive Dystrophic, a sub type of Dystrophic, has many sub types which can be moderate to extremely severe in presentation.  Below are the sub types of Dystrophic along with the sub types of each form of Dystrophic.
   
   -
Recessive Dystrophic

       - Hallopeau Siemens
       - Non Hallopeau Siemens
       - Inversa 

    -Dominant Dystrophic
     

3) Simplex: Considered to be the most common form of EB it has many sub types that range from mild to extremely severe.  While EB will never 'go away' some sub types of Simplex can become milder in presentation as the patient grows older.  Below are the most common subtypes of EBS.
    
    -
Dowling Meara
    - With Pyloric Atresia
    - Weber Cockayne
    - Koebner's
    - With Muscular Dystrophy

4) Kindler Syndrome: A rare form of EB which is caused by a mutation in the Kindling -1 gene.

There is one form of EB that is not inherited but is an autoimmune disorder.  It is called Epidermolysis Bullosa Aquisita and generally appears later in life. EBA is not contagious.


I hope you find this information helpful and I appreciate any feedback or other information, products or tips that you think should be mentioned or you find helpful.  
I would like to thank everyone who volunteered information.  I greatly appreciate your contribution : )  I have not included names or personal information from contributors in an effort to protect everyone’s privacy.  If you would like recognition for your entry I am happy to do so, just let me know : )  Thanks for being a part of this project and for being such strong and beautiful people!

Some information regarding types/sub types were found at http://www.ebnurse.org