This educational package contains a variety of documents that can help support you and your patients with EB. The detailed guide contains topics relevant to the care and support of a patient with EB. Topics are listed in alphabetical order. Each document can be printed on an individual basis and can be shared with other health care providers, patients, and caregivers. Each patient with EB will have unique health care needs and the aim of this education package is to help support health care providers “pick and choose” educational and resource materials most relevant to their patient at that point in time. If questions arise that are not contained within this education package please contact SWRWCP@lhins.on.ca or visit www.debracanada.org for more information regarding EB.

See Bathing Recommendations

Considerations for Infants:
 

• Bath infant in infant bath tub or sponge bath
• Line tub with a receiving blanket
• Place infant on top of another receiving blanket and then take infant and blanket to the infant tub to the bath
• The blankets decrease risk of infant slipping around the tub and in your arm/hand
• Hold infant across the neck and back area with arm or hand and use the other hand to bathe

See Blister Management
 

• Wash hands thoroughly and all tools must be cleaned and sanitized
• The infant may need to be lying down during this process
• Use aseptic technique
• This process may be painful for the infant - analgesics may be required before lancing blisters (consult medical team)
• Demonstrate blister lancing to caregivers – ensure caregivers can demonstrate technique to health care provider

See Products for Daily Living
 

• Baby’s clothes can be turned inside out with seams facing out
• If this is difficult or if only certain seams or buttons cause blistering, use silicone dressing onto skin or on seams to prevent trauma
• Clothes should fit properly - Trauma can occur if clothes are too tight or too loose

See DEBRA - How we Support See Applying for DEBRA Canada Membership

DEBRA Canada is a registered charitable organization dedicated to providing support for people affected by Epidermolysis Bullosa (EB) and to increasing Canadians’ awareness of this challenging disease. DEBRA is the onlyregistered charity in Canada exclusively committed to the care and support of EB families and to improving their quality of life.

• Avoid lifting at the ankles
• Lift infant’s hip and buttocks area to change diapers OR turn infant on the side to change diapers
• Blisters:
• Remove diaper
• DO NOT rub dirty skin
• Apply KY Jelly, Muco Gel, or a hydrocolloid gel (example: intrasite gel) on to a piece of gauze
• Apply this gauze to the area
• During next diaper change, use already applied impregnated gauge to gently wipe the skin clean – replace impregnated gauze (or use intrasite conformable)
• Re-apply diaper

• Assess for constipation, anal fissures, and anal scarring
• Recurrent blisters may arise in the bowel tract – there may be blood in stools
• DO NOT use any form of suppositories
• If constipated – update MRP or EB team – oral laxatives may be prescribed

See Esophageal Strictures
 

See Eye problems in EB
 

• Corneal abrasions can occur
• May be treated conservatively with topical antibiotics, artificial tears, and pressure-patch dressings
• Often followed by Ophthalmology

See Infant Feeding
 

• Infants can get blisters around and inside the mouth and on the tongue
• DO NOT force infants to feed – can increase trauma and/or result in vomiting from excessive intake of liquid

See EB Genetics
 

• Itch is a very common symptom in the EB population
• Exact reason for itch is unknown
• Persistent skin inflammation, skin overheating due to dressings, skin sensitization from topical creams, and pain medications such as morphine and codeine may contribute
• Vigorous itch may lead to more skin blistering

Non-medical treatment options
 

• Change the topical routine (switching dressings or discontinuing topical antibiotics may be sufficient to bring the itching down to a manageable level)
• Avoid skin overheating (such as many layers of clothing)
• Use cooling compresses or blankets for temporary relief
• Moisturize after each bath or as often as possible to prevent dryness
• Apply on the skin only preparations that have a low risk for sensitization (e.g. no fragrances)
• Discuss with your doctor new skin preparations that you would like to use

See Mouth and Dental Care
 

• Microstomia (small mouth) and limited mouth opening, fixed and scarred tongue, limited neck movement due to contractures, poor dentition and oral blistering are all common features in some types of EB
• Dental caries and restorative dental work may be extensive

• Do not remove dystrophic or abnormal nails by force
• To soften dystrophic nails, apply a dot of Uremol 20% cream and rub into nail
• Only cut the nails if they become too long

See Nutrition in EB

See Pain in EB
 

• Prevent pain due to wounds by using suitable dressing
• Not all babies with EB cry because of pain
• Medical pain therapy is best in oral forms

See Psychosocial See Living with EB - Impact on Daily Life (DEBRA)

Infants and Children:
 

• Avoid lifting and holding from axilla (under the arms/armpit)
• Place infant on soft padding (receiving blanket, pillow, sheepskin)
• To lift: roll infant and padding to one side, place on hand beneath the hips and another beneath the nape of the neck and upper back – allow infant to roll back onto your hands
• Lift infant with underlying padding to avoid injuries
• To comfort: pat instead of rub
• Encourage caregivers to hold and cuddle the infant or child – offer support and assistance to caregivers

See Epidermolysis Bullosa: A guide for parents, schools, and playgroups (DEBRA)
See Recommendations for Children with EB in Schools – Occupational Therapy (NHS)

See Skin Cancer in EB

• Superficial and only suction what you can see
• May cause trauma – use only if necessary

• Avoid rectal thermometers
• Use oral or axilla thermometer
• Place and move thermometer probe carefully to avoid “yanking” probe – lift the probe carefully out of the mouth or axilla

See Wound Care
 

• DO NOT USE adhesive dressings or tapes
• If dressing sticks to the skin, reassess dressing and use Niltac to remove – follow manufacturer’s instructions
• Encourage the patient and family to carry emergency dressing supplies and Niltac with them

• EB Patient and Family Handbook
• Nursing Communication Form for EB
• Clinic Preparation For Patient/Family Form
• EB Traveling Treatment Card

• DEBRA Canada
• DEBRA International
• DEBRA UK