EPIDERMIS BULLOSA

What is Epidermolysis Bullosa and what does it mean?
Epidermolysis Bullosa, or EB for short, is the name given to a group of rare genetic blistering skin disorders.
EB causes the skin to be so fragile that even minor rubbing can cause blistering. In severe EB, daily bandage changing is required to treat the many open wounds and blisters. EB can also effect other areas such as the eyes and internally such as the mouth, esophagus, stomach, intestines, upper airway, bladder, and genitals.
How common is Epidermolysis Bullosa?
It is estimated that about 10,000 Americans have some form of EB. Of these, less than 300 have the same form of EB that I was born with. (Recessive Dystrophic - Hallopeau Siemens Epidermolysis Bullosa.) Only one in a million babies are born with the Recessive Dystrophic form of EB (RDEB). I prefer to say I'm one in a million :o) EB occurs in all racial and ethnic groups and effects males and females equally.
What causes Epidermolysis Bullosa?
Most people with EB have inherited the condition through genes they have received from one or both parents. The faulty genes cause structural abnormalities in the skin. Genes govern the formation of different types of protein in the skin, including collagen and keratin. When any of these proteins is bad, the skin becomes so fragile it can literally fall apart.
How is Epidermolysis Bullosa inherited?
Some forms of EB are dominant, some are recessive and some are spontaneous mutations (abnormal changes in a gene that occurred during the formation of the egg or sperm) In dominant forms of EB, the disease gene is inherited from only one parent who has the disease and there is a 50% chance with each pregnancy the baby will have EB. In the recessive forms, the disease gene is inherited from both parents. Neither parent shows signs of the disease, they are just carriers of the EB gene. There is a 25% chance with each pregnancy that the baby will have EB. The severity of EB has a wide range. One form goes away after one year of age, others cause death at birth, some only effect the hands and feet while others need lengthy daily bandage changes due to the constant blisters and large burn-like wounds.
RDEB is considered one of the most severe forms. The structural weakness in the skin is in a deeper layer resulting in deep sores and extreme scarring. The skin is so fragile it can blister or sluff off from slight friction resulting in large blisters and wounds all over the body. The blisters need to be cut and drained or else they continue to grow. The sores need antibiotics and constant bandaging. (Please refer to my EB Medical Care web page for more information on caring for an EB patient) Most RDEB patients are practically bandaged head to toe. Many need wheelchairs because walking is too painful and tiring. This is also true with many of the Simplex varieties of EB because they effect the feet so greatly.
When most people hear the word blister they think they know what it's like to have EB, however EB blisters are generally much much larger and often times much more painful. In severe cases the wounds can become extremely large. The scarring is similar to that of a burn victim. Blisters can arise from everyday actions such as scratching an itch, friction from clothing, wearing glasses, bumping into someone etc. There is often spontaneous blistering as well.
Other symptoms of RDEB include:
*Severe scarring causing the fingers to fuse to the palm in the shape of a fist. This is called "webbing". This also effects feet and toes. Webbing can even cause the feet and hands to fuse into a bent position and the inability to stretch the legs and arms completely. Scarring in the esophagus causes it to become very narrow. This is due to the fact that scar tissue does not grow like normal skin. It continues to build up and create stricturing.
*Permanent loss of finger and toe nails at a young age. Blistering under the nails causes them to fall off and scarring causes them to never grow back.
*Severe Itching. When sores heal they tend to itch more. They also itch more if they are infected. With so many sores constantly trying to heal it causes constant itches and tingles. Scratching of course causes a lot more damage to the skin and unfortunately there are not many medicines that reduce the itching without also causing you to be very drowsy.
*Malnutrition. This is caused because of anemia and because most of the nutrients taken into the body goes toward healing the many open wounds and disrupts the growth of organs. It is also believed most RDEB patients can not easily absorb nutrients. It is also hard to eat due to blistering in the esophagus and mouth. This means only soft foods can be eaten. RDEB patients
often "choke" because food gets easily caught in their throat. Although RDEB does not effect the airway, so I have never actually had trouble breathing while food is stuck in my throat.
*Severe problems with teeth. I have had to have all my teeth pulled due to severe tooth decay. Oral hygiene in severe forms of EB can be very difficult. Dentures are not an option due to the fragility of the gums.
*Blisters in the eyes and Corneal Abrasions can be extremely painful. I have not had any eye blisters since I was a baby, however my eyes do dry out and get corneal abrasions very easily. This causes scarring over the eye and may impair vision.
*Anemia. Often times RDEB patients have Anemia as a result of inflammation, malnutrition and iron deficiency.
*Squamous Cell Carcinoma, a skin cancer that is fairly common in RDEB patients over the age of 20. If caught early, this cancer can be removed fairly easily, however there are no big signs to warn you. Unfortunately this is what takes many of the lives of RDEB patients.
Can Epidermolysis Bullosa be cured or treated?
There is currently no cure for EB. At this time the best hope is gene therapy. In 1993 the gene that causes Dystrophic EB was located. That was the first major step. More advances are being made every year giving much hope for the future. Scientists are currently testing the delivery of modified cells to genetically altered mice that have EB traits. The goal is to eventually be able to take the cells from a person with EB, correct the faulty gene, grow the new "gene corrected" healthy skin in the lab, and graft it onto the original EB donor.
Is Epidermolysis Bullosa contagious?
EB is a genetic disorder and is not at all contagious.
Is Epidermolysis Bullosa lethal?
There are currently only two types of EB that are considered lethal. The first is Junctional Herlitz, which is often deadly to newborns. Junctional Herlitz effects internally, including the airway and other internal organs causing major complications. Many with this form pass away within a few weeks of birth.
The other form is RDEB-HS (the kind I have). Most with RDEB-HS do not live past their 30's due to severe malnutrition (which can cause heart failure), a skin cancer called Squamous Cell Carcinoma and severe infection. There are also other forms of EB that can be very severe at birth however tend to get better with age. RDEB is considered to get worse with age however, due to the constant breakdown of the skin and severe scarring.

What Is A Computer?

Introduction
Described in its simplest form, a computer takes information (or inputs), processes it according to a set of instructions (a program), and gives back a result (or output). In this respect, it is very similar to a calculator, but obviously somewhat more complex.
The other key characteristic of a computer is that it is a multi-function platform. The same machine can perform many different tasks by using different programs. So, one program (e.g., a word processing application) allows letters or documents to be created, while another program (e.g., a graphics or drawing application) allows images to be created.
Computers in Education
Computers have many applications in education. They can act as a resource for students or teachers as they facilitate access to relevant information on the Internet and on CD-ROM/DVD/Memory Stick/mp3 players, Mobile Phones etc. They allow students and teachers to prepare presentations, documents, images and so on. Furthermore, there are specific educational programs, usually containing multimedia and animation, which are designed to teach or support learning in specific subject areas. In fact, the ways in which computers are integrated depends entirely on the imagination of both teachers and students.
Computer literacy is becoming a vital skill for all as we move further into this information age. Everyone involved in education has much to gain from the increased productivity that these machines can offer.
Key Components of a Computer
Although computers come in many different shapes and sizes, they are all made up of the same basic components. In fact, all computers are remarkably similar to each other.
Input Devices – telling the computer what to do
Computers have input devices to allow the user to control the machine. Essentially, an input device acts as a conduit for information from the user to the computer. It allows external information to be passed to the computer, which the computer then works with. Common examples of input devices include:
• Keyboard
• Mouse
• Microphone
Output Devices – the computer telling the user what it has done
Computers also have output devices which return the results of computer actions back to the user. Common examples of these are:
• Monitor
• Speakers
• Printer
© National Centre for Technology in Education June 2007
1
NCTE Advice Sheet – What is a Computer Advice Sheet 1
The Processor
The processor can be considered the ‘brain’ of the computer — it plays a pivotal role in almost all aspects of the computer’s functioning, including its performance and reliability. The processor, either directly or indirectly, controls all the work carried out in the computer. It is commonly known as the ‘chip’ or, more correctly, the central processing unit (CPU). The processor follows the instructions provided by both the user (via input devices) and the program that is running in order to perform a task.
Memory
In a similar way to the human brain, computers need some short-term memory (or working memory) to do the tasks they have been set. In a computer, this working memory is called random access memory (RAM). RAM is very important to the overall functioning of the computer as a system with a fast processor but only a small amount of RAM will not perform to its full potential. On the other hand, a slower processor with plenty of RAM will perform up to the limit of its ability.
Hard Drive
Computers need to be able to store both the programs that run on the system as well as the work that is created. The most common storage device is a fixed magnetic disk that sits inside the machine and this is called the hard drive. The hard drive is similar to long-term memory in human beings in that it can be a little slow. Consider the following analogy: if you were asked what you did on this day five years ago, you would have to think awhile (accessing the hard drive or storage area), but if you were asked what you were doing at this precise moment in time, you would have no difficulty providing an answer (accessing the working memory or RAM).
Extending the Computer’s Abilities
As mentioned earlier, a computer is a multi-function device. This means it is capable of carrying out a diverse set of functions. To actually do so, the computer must be set-up or adapted to take on whatever extra functions are most relevant to the user. The following list outlines some ways in which a computer’s abilities can be extended.
Software
An easy way to extend the computer’s capabilities is to install more programs. If users want to make posters and cards, for example, a suitable desktop publishing program can be purchased and installed. If users want to create or edit sound files, a sound editing program can be installed. There are programs (also termed applications) written to do everything imaginable.
Hardware
It is possible to increase the functionality of a computer by adding new or improved parts to it. For example, the working memory (RAM) can be increased and this will normally make the machine run faster. Similarly, a larger hard drive can be added which will allow more information to be stored.
Peripherals
Peripherals are devices that connect to a computer and perform specific tasks. They are usually related to the input or output of data, i.e., they are input or output devices. For example, a printer is a peripheral, as is a scanner and a digital camera, to name but a few. Other peripherals such as alternative keyboards, switches and touch-screens provide students with special needs with alternative methods of accessing and operating the computer (see Advice Sheet 28 for more information about special needs).
© National

ENDOCRINE SYSTEM
MAJOR ROLES: To relay chemical messages through the body. In conjunction with the nervous system, these chemical messages help control physiological processes such as nutrient absorption, growth, etc..
MAJOR ORGANS: Many glands exist in the body that secrete endocrine hormones. Among these organs are hypothalamus, pituitary

SKELETON SYSTEM
MAJOR ROLES: To protect organs; to provide support for our body to protect delicate internal organs; to provide attachment sites for the organs.
MAJOR ORGANS: Tendons, ligaments, bones, cartilage.

MUSCULAR SYSTEM
MAJOR ROLES: Muscles work in pairs to move limbs and provide the organism with mobility. Muscles also control the movement of materials through some organs, such as the stomach and intestine, the heart and the circulatory system .
MAJOR ORGANS: Cardiac muscle (heart), skeletal muscle.

NERVOUS SYSTEM
MAJOR ROLES: To relay electrical signals through the body. It directs behavior and movement and, along with the endocrine system, control psychological processes such as digestion, circulation, etc..
MAJOR ORGANS: Brain, spinal cord and peripheral nerves.

EXCRETORY SYSTEM
MAJOR ROLES: To filter out cellular wastes, toxins, and excess water or nutrients from the circulatory system.
MAJOR ORGANS: Kidneys, ureters, bladder and urethra, skin.