Epidermolysis bullosa (EB): management of the newborn infant with EB

Epidermolysis bullosa (EB) is a genetically determined skin fragility disorder. 

Care must be modified in order to reduce trauma and resulting damage to the skin and mucosae where possible (Denyer, 2009b). 

Infants with EB are nursed in their local neonatal unit (there is no need to transfer to specialised centres) and managed as outreach (Denyer, 2009b). The Great Ormond Street Hospital (GOSH) EB Clinical Nurse Specialist team delivers an outreach service.

These guidelines are intended to support practitioners looking after infants with EB and to improve the care and safety of this group of patients.

Background

There are four major types of EB, each with different clinical outcomes. The common factor is an extreme fragility of the skin and mucous membranes (Fine et al, 2014). The type of EB is determined by analysis of a skin biopsy. Regardless of type of EB, care of the affected infant will be the same. Severely affected infants often present with extensive wounds over their limbs resulting from inter uterine movements and compounded by trauma during delivery.

Sections

  1. Immediate care after birth
  2. General care
  3. Nappy area
  4. Blister care
  5. Wound care
  6. Feeding
  7. Clothing
  8. Cannulation

Immediate care after birth 

(Denyer et al 2017)

  • Remove the infant's cord clamp and replace with a ligature (Rationale 1).
  • Nurse the infant in cot/bassinette unless an incubator required for medical reasons such as prematurity (Rationale 2). 
  • Handle the infant with care – avoid all rubbing or friction (Rationale 3).
  • Do not use adhesive tapes or name bands. Use photographic identification (Rationale 4).
  • Try to avoid wearing gloves if possible to prevent causing any further damage (Rationale 34)
  • If local policy dictates wearing gloves or if the professional does not have intact skin themselves, then apply 50:50 ointment (equal parts of white soft and liquid paraffin) or Emollin® Spray to the fingertips before handling the infant (Rationale 5). 

General care

Handling 

  • Lift the infant on soft pad. Avoid sliding your hands under the baby. Use a roll and lift technique (Rationale 6).
  • Avoid bathing until inter-uterine and/or birth damage has been healed (Rationale 7) (Stevens & Denyer, 2010). 

For removal of tape without damaging skin

  • Use a Silicone Medical Adhesive Remover (SMAR) (Rationale 8) such as Appeel® (Clinimed), Niltac® (Trio healthcare) or Peeleasy® (m&a pharmachem ltd). 
  • If SMAR not available, cover with 50% liquid/50% white soft paraffin, which will dissolve the adhesive and enable safe removal (Denyer, 2011).
  • When removing the tape, roll the tape back on itself rather than lifting it from the skin (Rationale 9).
  • Do not attempt to remove tape whilst it is functional, eg securing IV cannula. If a new cannula is required secure with a soft silicone tape (Rationale 10). 

Nappy area care

  • Cleanse with 50% liquid paraffin, 50% white soft paraffin mix or Emollin Spray® and gauze (Rationale 11).
  • Line the nappy with soft line eg Conti Cloth Soft® (Rationale 12).
  • Apply an emoillent/barrier cream such as Prosheild Plus® or Bepanthen® (Rationale 13).
  • Cover open lesions with Intrasite Conformable® dressings and change these at every nappy change (Rationale 14) (Denyer, 2009a). 

Blister care

  • Blisters are not self-limiting and will enlarge if not lanced (Abercrombie et al, 2008).
  • Prepare equipment – hypodermic needle, gauze, dressing if required

  • Wash hands. If not wearing gloves, please ensure seven step technique using liquid soap (plain or antimicrobial) for at least 60 seconds is adhered to GOSH Hand Hygiene clinical guidelines (Rationale 35), in order to minimise the risk of cross infection (WHO, 2009).

  • Position and prepare patient

  • Use a piece of soft gauze to gently compress the blister from the side to increase tension.

  • Use an orange or blue hypodermic needle and pierce the blister at its lowest point (Rationale 15).
  • Slide the needle through the blister to create an entry and exit point (Rationale 16).
  • Withdraw the needle and gently press the blister with the gauze (Rationale 17).
  • There is no need to dress the blister site if the roof has remained on the blister (Rationale 18). 

Management of the newborn infant with epidermolysis bullosa

When using needles there is a risk of needle stick injury. Therefore avoid re-sheathing any used needles and dispose of any sharps in accordance with the hospital’s waste management policy.

Wash hands in line with the GOSH Hand Hygiene clinical guidelines

Advice on immediate care following an injury is available to GOSH staff internally on the GOSHweb intranet site.

Wound care

Use a validated neonatal pain assessment tool to ensure adequate analgesia (opioid analgesia is usually required) given prior to wound care (avoid the administration of rectal medication if possible as this can blister the anal margin). (Herod et al, 2002).

  • Prepare a clean trolley with clinical waste bag, hypodermic needles, all dressings (cut to shape using supplied template) and tape cut into short lengths (Rationale 19). See below for template.
  • Wash hands using 7 step technique using liquid soap (plain or antimicrobial) for at least 60 seconds (Rationale 35). Ensure lubricated gloves are worn if professionals skin is broken.

  • Carefully remove soiled dressings using the Silicone Medical Adhesive Removers or Emollin® spray if the dressing is stuck (Rationale 20). Disposal of all waste and sharps in accordance with the hospital’s waste management policy.
  • Lance any new blisters.
  • Raw wounds: Apply PolyMem® dressing as skin contact, secure by taping it to itself and not directly on to the skin (Rationale 21).
  • Secure PolyMem® with wrap around or tubular bandage. Change dressing when strike through observed (staining visible on the outside of the dressing) (Rationale 22).
  • Dress the infants fingers and toes individually if these are raw with one of the following recommended dressings: Urgotul®/Durafiber®l/Kytocel®/Mepitel One®. Take care that no tape comes into contact with the skin (Rationale 23)(Denyer & Murrell, 2010).

Wound care - Management of the newborn infant with EB

Figure 1.Template for foot and lower leg dressing

Feeding

  • Use Haberman feeder if mouth is sore (Rationale 24).
  • Protect the infants lips with Vaseline (Rationale 25).
  • Moisten teat with cooled boiled/sterile water prior to feeding or use teething gels if the mouth is very sore (Rationale 26).
  • Avoid using naso-gastric tube if possible (Rationale 27).
  • If naso-gastric feeding essential, use tube suitable for long-term feeding and secure with soft silicone tape (Rationale 28). 

Clothing

Dress the baby in soft, front fastening all-in-one baby grow; turned inside out (Rationale 29).

Cannulation

  • IV fluids/antibiotics should be administered to the infant only when necessary e.g. in the presence of sepsis or dehydration (Rationale 30).
  • Raised C Reactive Protein in an infant with EB is not necessarily an indication of infection in the presence of widespread inflammation (Mellerio, 2010).
  • Clean the cannulation area by dabbing the skin with antiseptic solution (such as Chloraprep®) rather than rubbing (Rationale 31).
  • Do not use a tourniquet (Rationale 32).
  • Protect skin with soft gauze when squeezing the limb (Rationale 33).
  • Secure the cannula with soft silicone rather than an adhesive tape. If soft silicone tape is not available use an adhesive tape but remove this following the guidelines above to reduce the risk of trauma.  

Rationale

Rationale 1: To avoid trauma to the surrounding skin.
Rationale 2: Heat and humidity can exacerbate blistering.
Rationale 3: Friction and shearing forces result in blistering and skin stripping.
Rationale 4: Name bands can rub causing trauma and blistering.
Rationale 5: Emollient spray applied to gloves reduces the risk of drag and subsequent trauma.
Rationale 6: Shearing forces result in skin stripping.
Rationale 7: It is not possible to prevent trauma from the infant moving in the bath and it is not possible to safely hold the infant without causing skin loss.
Rationale 8: Removal of adhesive products results in skin stripping.
Rationale 9: Reduces risk of epidermal stripping.
Rationale 10: Tape will not damage skin whilst in situ; it is the removal of the tape that has the potential for harm.
Rationale 11: Cleansing with water can cause pain from contact with open lesions. Using emollients to cleanse the skin reduces trauma.
Rationale 12: The edges of the nappy can rub leading to blisters and skin stripping.
Rationale 13: To protect wounds and blisters from faecal contamination.
Rationale 14: To protect any wounds and blisters and aid healing.
Rationale 15: Lancing at the lowest point allows maximum drainage of the blister.
Rationale 16: To allow the blister roof to lie flat after drainage.
Rationale 17: To ensure that all fluid is expelled.
Rationale 18: The underlying wound is protected by the roof of the blister.
Rationale 19: Advanced preparation of dressings reduces time required to undertake dressing change and reduces the trauma for the infant.
Rationale 20: To avoid the risk of skin stripping from the removal of adherent dressings.
Rationale 21: Taping dressings to the skin risks skin stripping on removal of the tape. Larger dressing sizes offer protection from trauma of limb movements.
Rationale 22: ‘Strike through’ indicates saturation of the dressing, loss of protection from microbes in the environment and a risk of maceration of the wound.
Rationale 23: Raw digits will fuse together creating pseudo syndactyly.
Rationale 24: The valve in the teat of a Haberman Feeder allows a weak suck (i.e. where the infant is experiencing pain from oral blistering) to generate a good volume of milk.
Rationale 25: To reduce the risk of trauma from the bottle teat.
Rationale 26: A dry teat can adhere to the oral mucosa and lead to skin stripping.
Rationale 27: Mucosal blistering can result both from passing the tube and when the tube is in situ.
Rationale 28: The long term tube is softer and will reduce the trauma for the infant.
Rationale 29: To reduce trauma from handling the infant whilst dressing the infant and to reduce friction from internal seams and labelling.
Rationale 30: The majority of infants with EB are able to feed orally. Prophylactic use of antibiotics is not recommended.
Rationale 31: Rubbing may result in skin stripping.
Rationale 32: Tourniquet use may cause blistering.
Rationale 33: Direct contact with the skin may cause blistering and skin stripping.

Rationale 34: Surgical gloves (latex or vinyl gloves) are not recommended for handling a neonate with EB. They tend to grab the skin and can cause further damage.

Rationale 35: To minimise the risk for the patient, staff must ensure that they thoroughly wash their hands as outlined in the GOSH Hand Hygiene Clinical Guideline (using 7-step technique for a minimum of 40-60 seconds) each time.

 

References

Abercrombie, E. Mather, C. Hon, J. Graham- King, P. and Pillay, L. (2008) (Tissue Viability Supplement) Practical management of patients with epidermolysis bullosa, care of the adult patient with recessive dystrophic epidermolysis bullosa, British Journal of Nursing. 17(6): (No Sup3), S6- S20. 

Denyer, J. (2009a) Management of the infant with epidermolysis bullosa. Infant. 5(6): 185-188.

Denyer, J. (2009b) Management of the infant with epidermolysis bullosa. Infant. 5(9): 186

Denyer, J. and Murrell, D.F. (2010) Wound management for children with epidermolysis bullosa: epidermolysis bullosa: part II – diagnosis and management. Dermatology Clinic. 28: 257-64

Denyer, J. (2011) Reducing pain during the removal of adhesive and adherent products. British Journal of Nursing. 20(15): (No Sup 8), S28,S30-5. 

Denyer J, Pillay E, and Clapham J. (2017) Skin and wound care in epidermolysis bullosa.  An International Consensus. Wounds International. Best practice guidelines. [Last accessed 05.06.17]

Fine, J-D. Bruckner-Tuderman, L. Eady, R. A. J. Bauer, E.A. Bauer, J. W. Has, C. Heagerty, A. Hintner, H. Hovnanian, A. Jonkman, M.F. Leigh, I. Marinkovich, M. P. Martinez, A. E. McGrath, J. A. Mellerio, J. E. Moss, C. Murrell, D. F. Shimizu, H. Uitto, J. Woodley, D. and Zambruno, G. (2014) Inherited epidermolysis bullosa: updated recommendations on diagnosis and classification.  American Academy of Dermatology. 70(6): 1103–1126. [Last accessed 05.06.17]

Herod, J. Denyer, J. Goldman, A. and Howard, R. (2002) Epidermolysis bullosa in children: pathophysiology, anaethesia and pain management. Paediatric Anaesthesia. 12 (5): 388-397.

Mellerio, J.E. (2010) Infection and colonisation in epidermolysis bullosa. Dermatology Clinic. 28(2): 267-9.

Stevens, L. and Denyer, J. (2010) Bathing in epidermolysis bullosa: benefit over trauma? Wounds UK. 6(2): 79- 84.

World Health Organization (WHO) (2009). WHO Guidelines on Hand Hygiene in Health Care: a Summary. WHO.

Appendices

Document control information

Lead Author(s)

Finola Sheehan, EB CNS, EB/Dermatology

Additional Author(s)

Katie Plevey, EB CNS, EB/Dermatology

Document owner(s)

Katie Plevey, EB CNS, EB/Dermatology

Approved by

Guideline Approval Group

Reviewing and Versioning

First introduced: 
25 June 2013
Date approved: 
05 June 2017
Review schedule: 
Three years
Next review: 
05 June 2020
Document version: 
3.0
Previous version: 
2.0