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Epidermolysis Bullosa (EB)

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Dear Marilette,

 

Please could you be so kind to describe the medical

background of Epidermolysis bullosa, and how to treat

persons, suffering from this

very severe genetic skin disease.

(My PH background: Certified Pranic Healing)

 

Thank you very much

Namaste

Gabriele

 

======================================================

 

 

Dear Gabrielle,

 

Namaste.

 

Thank you for your email.

 

Medical Background:

 

Alternative names - Junctional epidermolysis bullosa;

Dystrophic epidermolysis bullosa; Hemidesmosomal

epidermolysis bullosa; Weber-Cockayne syndrome

 

Epidermolysis bullosa (EB) is a group of inherited

bullous disorders characterized by blister formation

in response to mechanical trauma. Historically, EB

subtypes have been classified according to skin

morphology. Recent discoveries of the molecular basis

of EB have resulted in the development of new

diagnostic tools, including prenatal testing. Based on

a better understanding of the basement membrane zone

(BMZ) and the genes responsible for its components,

new treatments (eg, gene therapy) may provide

solutions to the skin fragility found in patients with

EB.

 

There are four main types of epidermolysis bullosa:

 

* Epidermolysis bullosa simplex

* Junctional epidermolysis bullosa

* Dystrophic epidermolysis bullosa

* Hemidesmosomal epidermolysis bullosa

 

Another rare type of epidermolysis bullosa, called

epidermolysis bullosa acquisita, is an autoimmune

disorder. This may be indistinguishable from another

autoimmune skin disorder called mucous membrane

pemphigoid.

 

Depending on the type present, epidermolysis bullosa

varies in severity from minor blistering of the skin

to a lethal form involving other organs. The condition

generally starts at birth or soon after that.

Epidermolysis bullosa aquisita, however, usually

appears in adults over age 50, although it has been

reported in children. Also, mild cases of

epidermolysis bullosa simplex may remain undiagnosed

until adulthood.

 

The classification of epidermolysis bullosa is

complicated. Even within the main types mentioned,

there are many different subtypes of this condition.

For example, Weber-Cockayne is the most common form of

epidermolysis bullosa simplex. This type involves

blistering of the palms and soles and may include

excessive sweating.

 

All of the different types of epidermolysis bullosa

are generally inherited. Therefore, having a family

history of the disease, especially an affected parent,

is a risk factor. The inheritance pattern may be

dominant or recessive. A dominant form means that an

offspring can inherit the gene or trait from one

affected parent. The recessive form means that both

parents must carry a gene and transmit that gene to

the offspring in order for the infant or child to be

affected. The recessive forms of epidermolysis bullosa

tend to be more severe.

 

The hallmark of these conditions is the formation of

large, fluid-filled blisters that develop in response

to minor trauma. Some infants may have large blisters

at birth. Others start shortly after birth. Chafing

(wearing away) of the skin, rubbing, or even increased

room temperature may cause blisters to form.

 

In the severe forms, scarring after blister formation

may cause deformities, fusion of the fingers and toes,

and contracture deformities (for example, at the

fingers, elbows and knees). If the mouth and esophagus

are involved, blistering and scarring lead to feeding

and swallowing difficulties.

 

Secondary infection is common.

 

Symptoms depend on the type of epidermolysis bullosa,

but can include:

 

* Blistering of the skin as a result of minor

trauma or temperature change

* Blistering present at birth

* Nail loss or deformed nails

* Blistering in or around the mouth and throat,

causing feeding difficulty or swallowing difficulty

* Blistering around the eyes and nose

* A hoarse cry, cough, or other respiratory

difficulties

* Dental abnormalities such as tooth decay

* Alopecia (hair loss)

* Milia (tiny white bumps or pimples)

 

For epidermolysis bullosa acquisita, there is an

association with Crohn's disease (an inflammatory

bowel disease) and, possibly, lupus. Symptoms of these

conditions may be present, therefore, in adults who

present with this type of epidermolysis bullosa.

 

The goal of medical treatment is to prevent the

formation of blisters and subsequent complications.

The intensity of care depends upon the severity of the

disease. Recommendations often include measures to

avoid skin trauma and to avoid high environmental

temperatures.

 

To prevent infection, excellent skin care is required,

especially if any blistered areas become crusted or

denuded (exposed or raw). Follow the instructions of

your health care provider closely. Recommendations

might include regular whirlpool therapy and

application of topical antibiotics to these wound-like

areas. Your health care provider will let you know if

a bandage or dressing is required, and what type.

 

For difficulty in swallowing and/or if candida is

present in the mouth or esophagus, medication for that

secondary infection would be prescribed.

 

Good dental hygiene is very important, including

regular dental visits. It is best to be followed by a

dentist with experience treating those with

epidermolysis bullosa.

 

Proper nutrition is also important. When skin injury

is extensive, increased calorie and protein intake may

be necessary to help recovery. Work closely with a

nutritionist. When blisters or complications are

present in the mouth or esophagus, hard or brittle

foods (such as pretzles, nuts, and chips) should be

avoided. Eating soft foods can help prevent worsening

of these lesions.

 

Working with a physical therapist can help maintain

the range of motion of involved joint areas and

minimize contractures.

 

Skin grafting for denuded or ulcerated areas of the

skin may be necessary. Other surgical procedures for

complications of epidermolysis bullosa might be

recommended as well. Such surgeries include dilation

of the esophagus if a stricture (narrowing) is

present, repair of hand deformities, and removal of

squamous cell carcinoma lesions if they develop.

 

Other treatments under investigation for epidermolysis

bullosa include protein and gene therapy.

 

Epidermolysis bullosa acquisita may be treated with

oral steroids and medication that suppresses the

immune system. These may, however, increase the risk

of secondary infection, particularly the latter drug

category. Studies using interferon are also underway.

 

The outcome depends on the severity of the illness.

Mild forms of epidermolysis bullosa improve with age.

Scarring from several types of epidermolysis bullosa

can restrict mobility significantly and, therefore,

impair daily activities. Lethal forms of the

epidermolysis bullosa have a very high mortality rate.

 

 

Complications

 

* Infection, including sepsis

* Esophageal stricture

* Periodontal disease

* Loss of function of hands and feet

* Eye disorders, even blindness

* Muscular dystrophy

* Severe malnutrition secondary to feeding

difficulty, leading to failure to thrive

* Anemia

* Squamous cell skin cancer -- if someone with

epidermolysis bullosa survives childhood, metastatic

squamous cell cancer of the skin is the most common

cause of death. This skin cancer occurs in those with

recessively inherited dystrophic epidermolysis

bullosa. Those with epidermolysis bullosa often

develop this skin cancer between the ages of 15 and

35. In addition, the skin cancer can occur anywhere on

the skin. In other words, unlike the general public,

it is not more likely to happen in sun-exposed areas.

* Death -- death rate is as high as 87% in the

first year of life for infants with the lethal form of

junctional epidermolysis bullosa. On the other hand,

dominantly inherited simplex and dystrophic

epidermolysis bullosa, and mild forms of junctional

epidermolysis bullosa may not lessen life expectancy

at all.

 

If your infant exhibits any blistering shortly after

birth call your health care provider. If you have a

family history of epidermolysis bullosa, further

interaction with your health care provider or a

genetic counselor may be appropriate.

 

Prevention

 

Genetic counseling is recommended for prospective

parents with a family history of any form of

epidermolysis bullosa.

 

During pregnancy, chorionic villus sampling to test

the fetus is available. For couples at high risk of

having an offspring with epidermolysis bullosa, the

test can be done as early as week 8-10 of the

pregnancy. Speak with your obstetrician and genetic

counselor.

 

To prevent skin trauma and blistering, it may help to

wear padding around trauma-prone areas like elbows,

knees, ankles, and buttocks. Contact sports should be

avoided.

 

Sources - : M Peter Marinkovich, MD, Jacqueline A.

Hart, M.D., Senior Medical Editor, A.D.A.M., Inc.

Previously reviewed by Jeffrey Drayer, M.D.

 

Pranic Healing:

 

Sources: Advanced Pranic Healing, Pranic

Psychotherapy both by Master Choa Kok Sui.

 

This is for patients 3 years old to 65 years old. If

the patient is below 3 years old or over 65 years old,

just use white prana.

 

1. Invoke and scan before, during and after

 

*Teach patient how to do proper pranic breathing. Ask

patient to do 12 cycles before start of treatment and

to continue pranic breathing during treatment.

 

2. General sweeping several times with LWG.

 

3. Localized thorough sweeping and energizing on the

entire head, the ajna chakra, crown chakra, forehead

chakra and throat chakra using LEV or EV.

 

4. Localized thorough sweeping on the front and back

heart chakra alternately with LWG and ordinary LWV.

Energize through the back heart chakra with LWG then

with more of ordinary LWV.

 

5. Localized thorough sweeping on the affected areas

alternately with LWG and LWO.

 

If the affected areas are near the heart, or on the

head do not use O; just use LWG and ordinary LWV

alternately for cleansing.

 

If the area in not on the head or near the heart or

spleen - Energize the affected part(s) with LWG then

LWB to soothe pain then with LWR then gold.

 

OR If the affected area in on the head, near the heart

or spleen - Energize the affected part(s) with LWG

then LWB then ordinary LWV then with gold.

 

6. Localized thorough sweeping on the front, sides

and back lungs. Energize through the back of the

lungs with LWG then LWO then LWR. Point your fingers

away from the patient's head when energizing with O.

 

7. Localized thorough sweeping on the basic chakra

alternately with LWG and LWO. Energize with LWR.

 

8. Localized thorough sweeping on the arms and legs

with emphasis on their minor chakras alternately with

LWG and LWO. Energize the minor chakras of the arms

and legs with LWR

 

9. Localized thorough sweeping and energizing on the

front and back solar plexus chakra. Energize the

solar plexus chakra with LWG then LWO. Apply more

localized sweeping.

 

10. Localized thorough sweeping on the front and back

spleen chakra using LWG. Energize the spleen chakra

with LWG then with ordinary LWV. This has to be done

with caution.

 

11. Localized thorough sweeping on the kidneys

alternately with LWG and LWO. Energize them with LWR.

 

12. Localized thorough sweeping on the meng mein

chakra.

 

13. Localized thorough sweeping on the sex chakra,

navel chakra and the lower abdominal area. Energize

the navel and sex chakras with LWR.

 

14. Stabilize and release projected pranic energy.

 

15. Repeat treatment 3 times per week.

 

For patient:

 

1. Provided there are no other health conditions that

will prevent the proper practice for the meditation,

practice of the Meditation on Twin Hearts with both

feet in a basin with water and salt regularly

especially after each pranic healing treatment. The

practice promotes the assimilation of healing energy,

refines and raises the energy level, promoting good

health. This meditation generates tremendous divine

energy needed to treat ailments with genetic

components.

 

If the patient is unable to practice the meditation

due to age or health, the patient's family and friends

may form a group to practice the Meditation as a group

regularly and include the patient's healing in the

MTH's blessing.

 

2. Practice proper pranic breathing (6-3-6-3) for 12

cycles upon waking every day and during the day

especially when experiencing stress.

 

3. Tithe regularly for healing.

 

4. Follow doctor's advice.

 

Love,

 

Marilette

 

 

 

Pranic Healing is not intended to replace orthodox medicine, but rather to

complement it. If symptoms persist or the ailment is severe, please consult

immediately a medical doctor and a Certified Pranic Healer . ~ Master Choa Kok

Sui

 

Miracles do not happen in contradiction to nature, but only to that which is

known to us in nature. ~ St. Augustine

 

Ask or read the uptodate pranic healing protocols by joining the group through

http://health./

 

For the latest International Information regarding GMCKS Pranic Healing, visit

http://www.pranichealing.org.

 

 

 

 

Mail - PC Magazine Editors' Choice 2005

 

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