Epidermolysis Bullosa (EB) Care of Neonates

• Do not nurse in an incubator unless required for other medical conditions, such as prematurity, as heat and humidity can exacerbate blistering.
• Secure the umbilical cord with a ligature rather than a cord clamp this is to prevent trauma to the umbilical area.
• If a cannula is required, secure with Mepitel Film or Mepitac tape.
• Use a Silicone Medical Adhesive Remover (SMAR) such as Peel-easy for removal of tapes or dressings without skin stripping enabling safe removal.
• Infant should be nursed on a neonatal mattress(such as Repose mattress)whilst in cot or incubator
• Limbs and vulnerable areas should be protected with suitable dressing material or clothing (if condition permits), a suggestion would be to dress in soft, front fastening baby grow; turn inside out to avoid damage from seams and label.  This will help reduce further skin loss from baby movements such as kicking and will protect skin when being handled for general care in cot/incubator and when being  lifted out of cot for feeding or contact with parents
• Line the inside of the napkin with a liner (e.g Conticloth )which is larger than new born size nappy ,placed inside nappy and resultingly overlaps at waistband and leg openings preventing friction and subsequent trauma from the edges of the napkin.
• Use a greasy emollient commonly known as 50/50 ointment(50% liquid paraffin/50% white soft paraffin) to cleanse napkin area in preference to water, this aids cleansing without further trauma and will reduce pain.
• Avoid bathing until inter-uterine and birth damage have healed and helps prevent damage from infant being handled naked.

• Use Latex Orthodontic feeding teat (commonly available in wards and cleft lip service) to avoid friction to oral mucosa.
• Lubricate feeding teat with teething gel that is suitable for use from birth(e.g Dentinox Infant Gum Gel).If mucosal blistering present Episil Oral Liquid can be used prior to feeds to alleviate mucosal pain and discomfort as this helps to alleviate pain from blistered mucosa.

• Analgesia prior to dressing change

Choices of analgesia should be chosen to reflect the likely degree of discomfort.  Choices will include Paracetamol or Morphine. Pleas refer to the WoS Neonatal Pain Guideline for details of neonatal dosages and pain assessment tools.

Recommended and First Choice dressings for neonates with EB - Skin

Dressing type:  Polymeric membrane         Brand:  Polymem
This is the first choice dressing for severe neonatal wounding/critical colonisation/infection.    Wear time is as determined by exudates level. 
Change when wet to avoid hypothermia.

Recommended and First Choice dressings for neonates with EB – Nappy Area

Dressing type:  Hydrogel impregnated gauze     Brand:  Intrasite Conformable or Hydrosorb
This is for wounds/blisters in nappy areas.  Can be used over nappy creams such as Bepanthen or barrier creams such as Proshield.  Change daily or when dry.  May need a primary contact layer dressing (Urgotul) if severe skin fragility. 
Small neonates may be at risk of hypothermia. 

Recommended and First Choice dressings for neonates with EB – Between Digits of fingers/toes

Dressing type:  Hydrofiber              Brand:  Aquacel
For very moist wounds where it is difficult to keep dressings in place.  Between digits where there is risk of fusion.  Change dressing every 3-4 days.
Should not be used if no wound exudates present. 

Cannulation - Use a site where skin is intact, hold limb firmly using soft gauze, compress the limb manually rather than using a tourniquet, to avoid shearing of the skin.  Wipe gently with an alcohol swab, do not rub.  Cannulae can be fixed using Mepitel Film or Mepitac ® or Siltape ® (the same thing on a roll), fix securely as it does become loose when moist.  Soft One ® (cohesive bandage) can also be used over tapes to secure them.
*Splints should be well padded.

Blood Pressure - Place Vaseline gauze with padding around the arm before application of blood pressure cuff.  Avoid taking blood pressure more often than necessary.

Pulse Oximetry - This should be done using sensors which can be placed by protecting digit with  clingfilm or Adaptic Touch.
If contact poor with using this base protective layer  then it may be necessary to  apply sensors directly to skin.

ECG Electrodes  Hydrogel Electrodes can be used but ensure that a Silicone Medical Adhesive Remover (SMAR) such as Peel-easy is used for safe  removal of electrodes without skin stripping

EB New Baby –Advisable items to be available