Pain management in EB

1
Pain management in EB

• Jacqueline Denyer Senior EB CNS
• (Paediatric)
• Great Ormond Street, London
• And DebRA UK

2
Pain Assessment

• Patient focused
• Individual
• Cultural/social
• Assess and formulate a baseline pain score
• Use a recognised tool
• Behavioural characteristics
• Physiologically induced changes

3
Sources of pain - acute

• Bullae/wounds
• Reflux
• Dental
• Corneal ulcers
• Anal fissures
• Procedural

Watterson G 2004
4
Sources of pain - chronic

• Inflammatory pain
• Neuropathic pain
• Osteoporosis
• Constipation
• Contractures

Watterson G 2004
5
Acute Pain from Bullae/Wounds

• Give prescribed analgesia
• Prepare dressings
• Prepare environment, close windows, turn off
  fans. Distraction.
• Lance blisters to prevent from enlarging. Use
  Cryogesic spray (Ethyl Chloride) if
  pain/anticipatory fear
• Select atraumatic dressing

6
Atraumatic Dressing

• Term to define products, which on removal do not
  cause trauma either to newly formed tissue or to
  the peri-wound skin. An example could include
  dressings coated with soft silicone.
• Thomas S (2003) Atraumatic Dressings.
  www.worldwidewounds.com March 2004

7
Blistering of oral mucosa

• Use of topical analgesia in the form of teething
  gels for infants, Difflam spray (NSAID) for older
  children.
• Specialised teats for infants (Haberman)

8
Gastro Intestinal Tract

• Gastro oesophageal reflux a feature in all
  types of EB. May contribute to stricture
  formation in DEB. Responds to a combination of
  medication including ranitidine, omeprazole and
  domperidone
• Peri-anal blistering. Stool softners and
  laxatives. Soluble fibre added to feeds. Movicol
  (osmotic laxative, polyethylene glycol)

9
Acute Pain - Corneal Ulceration

• Regular ophthalmology review
• Use of lubricants particularly when contractures
  limit closure of eyelid
• Treatment of blepharitis
• Regular analgesia for acute injury.
• Apply ointments/drops to lid margin if eye closed
• Avoid light

10
Osteoporosis

• Low bone mass and bone pain are common symptoms
  in those with severe EB. Occasionally vertebral
  crush fractures occur
• Appropriate medication can alleviate pain
  -calcium supplements, bisphosphonates
• Intra venous pamidronate.
• Ref. Fewtrell M.S. et al. Bone Mineralisation in
  Children with EB, Br J Dermatol 2005, in press

11
Pain Management

• Mild Pain combine simple analgesia with a non
  steroidal e.g. paracetamol with ibuprofen
• Severe pain Opioid analgesia and anxiolytic
  sedation e.g. midazolam
• Fentanyl lozenges. Entonox (occasional use)
• Chronic background pain may require long acting
  morphine (MST)
• Neuropathic pain may respond to low dose
  amitriptyline or gabapentin

12
Peripheral opioid use

• 10mg of morphine sulphate in 15mg Intrasite gel
  with dressing changes
• (0.2mg/kg of morphine)

• Peripheral opioids in inflammatory pain
• Archives of Disease in Childhood 200489679-681
• Watterson G, Howard R, Goldman A.

13
Non Pharmalogical Therapies

• Physiotherapy
• Hydrotherapy
• Visualisation
• Guided imagery

14
Life, Epidermolysis Bullosa and Chasing
Tornadoes.
Journal of Wound Care vol 13.no 10 Nov 2004