Epidermolysis Bullosa (EB) Care of Neonates

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Objectives

This Guideline is applicable to all staff in neonatal units in the West of Scotland.  Staff should also refer to relevant drug monographs

The guideline covers the immediate care of neonates with Epidermolysis Bullosa (EB), or neonates provisionally diagnosed with EB.

Scope

Neonatal/Paediatrics/Dermatology

Epidermolysis Bullosa (EB) is the generic term for a genetic determined blistering skin condition.

The common factor is the fragility of the skin and mucous membranes and a tendency for the skin to blister when exposed to minimal everyday friction and trauma.  EB is commonly sub divided into 3 major groups - simplex, junctional and dystrophic, however some conditions such as Bullous Ichthyosiform Erythroderma (BIE) are often included within the 'EB' umbrella as this condition also features dry skin and blistering.

In severe forms of EB, blisters and wounds are usually present at delivery or result from handling immediately after birth. In milder forms of the EB condition, these will often appear during the neonatal period.  Secondary infection is a primary complication. 

Contact details for EB nurses / Dermatologist with specific interest
  • Sharon Fisher EB Clinical Nurse Specialist NHS Scotland
    Tel:  07930854944   email:  Sharon.Fisher@ggc.scot.nhs.uk
  • On Call Paediatric Dermatologist via RCH Switch board 01412010000
Treatment of Skin
  • 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.
Feeding
  • 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.
Pain
  • 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

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

Additional considerations

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

Product list in EB pack provided for known births

EB New Baby –Advisable items to be available

Skin Care

 

White Soft Paraffin/Liquid Paraffin BP 50:50 Ointment 500g 

Flaminal Hydro 50g                                                                 

Peel-Easy Adhesive Remover50ml spray 

Bepanthen Nappy Care Cream 30G                                              

nappy area cleansing

Wound Care

 

Polymem Non Adhesive Roll 10cmx61cm                     

or

Urgotul (10cmx10cm/15cmx15cm )  

Kliniderm Lite Foam Silicone without border (10cmx10cm/15cmx15cm) 

Comfifast red line                                                          

K-Lite Bandages 5cmx4.5m

HYDROSORB Gel Dressings 10cmx10cm

First choice of dressing in Neonates with EB

 

Blister Care

 

Sterile Orange Needles         

 

Editorial Information

Last reviewed: 08 August 2019

Next review: 01 August 2022

Author(s): Sharon Fisher – Paediatric Epidermolysis Bullosa Clincal Nurse Specialist RHC.

Co-Author(s): Other Professionals consulted: Dr Catherine Jury, Consultant Dermatologist – RHC Glasgow

Approved By: West of Scotland Managed Clinical Network for Neonatology