title: Pain care for patients with epidermolysis bullosa: best care practice guidelines.
abstract: BACKGROUND
  Inherited epidermolysis bullosa (EB) comprises a group of rare disorders that have multi-system effects and patients present with a number of both acute and chronic pain care needs. Effects on quality of life are substantial. Pain and itching are burdensome daily problems. Experience with, and knowledge of, the best pain and itch care for these patients is minimal. Evidence-based best care practice guidelines are needed to establish a base of knowledge and practice for practitioners of many disciplines to improve the quality of life for both adult and pediatric patients with EB.
  
  
  METHODS
  The process was begun at the request of Dystrophic Epidermolysis Bullosa Research Association International (DEBRA International), an organization dedicated to improvement of care, research and dissemination of knowledge for EB patients worldwide. An international panel of experts in pain and palliative care who have extensive experience caring for patients with EB was assembled. Literature was reviewed and systematically evaluated. For areas of care without direct evidence, clinically relevant literature was assessed, and rounds of consensus building were conducted. The process involved a face-to-face consensus meeting that involved a family representative and methodologist, as well as the panel of clinical experts. During development, EB family input was obtained and the document was reviewed by a wide variety of experts representing several disciplines related to the care of patients with EB.
  
  
  RESULTS
  The first evidence-based care guidelines for the care of pain in EB were produced. The guidelines are clinically relevant for care of patients of all subtypes and ages, and apply to practitioners of all disciplines involved in the care of patients with EB. When the evidence suggests that the diagnosis or treatment of painful conditions differs between adults and children, it will be so noted.
  
  
  CONCLUSIONS
  Evidence-based care guidelines are a means of standardizing optimal care for EB patients, whose disease is often times horrific in its effects on quality of life, and whose care is resource-intensive and difficult. The guideline development process also highlighted areas for research in order to improve further the evidence base for future care.
authors.affiliation: Pain Management Center, Department of Anesthesiology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Kenneth.goldschneider@cchmc.org.
authors.forename: Kenneth R
authors.initials: KR
authors.lastname: Goldschneider
authors.affiliation: Lucille Packard Children's Hospital, Department of Anesthesia (by courtesy, Pediatrics), Stanford University, Stanford, California, USA. julieg@stanford.edu.
authors.forename: Julie
authors.initials: J
authors.lastname: Good
authors.affiliation: Helen and Douglas Hospices, Oxford and John Radcliffe Hospital, Oxford, USA. emilyharrop@yahoo.com.
authors.forename: Emily
authors.initials: E
authors.lastname: Harrop
authors.affiliation: University of Southampton, Southampton, UK. Christina.Liossi@gosh.nhs.uk.|Great Ormond Street Hospital for Children NHS Trust, London, UK. Christina.Liossi@gosh.nhs.uk.
authors.forename: Christina
authors.initials: C
authors.lastname: Liossi
authors.affiliation: Pain Management Center and Division of Behavioral Medicine and Clinical Psychology, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Anne.Lynch-Jordan@cchmc.org.
authors.forename: Anne
authors.initials: A
authors.lastname: Lynch-Jordan
authors.affiliation: National Paediatric Epidermolysis Bullosa Centre, Great Ormond Street Hospital NHS Foundation Trust, London, UK. Anna.Martinez@gosh.nhs.uk.
authors.forename: Anna E
authors.initials: AE
authors.lastname: Martinez
authors.affiliation: Department of Anesthesiology and Critical Care, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. MAXWELL@email.chop.edu.
authors.forename: Lynne G
authors.initials: LG
authors.lastname: Maxwell
authors.affiliation: James M. Anderson Center for Health Systems Excellence, Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. Danette.Stanko@cchmc.org.
authors.forename: Danette
authors.initials: D
authors.lastname: Stanko-Lopp
mesh_terms: D000328:Adult; D002648:Child; D004820:Epidermolysis Bullosa / Q000150:complications / Q000523:psychology / Q000628:therapy*; D006801:Humans; D055048:Integrative Medicine / Q000379:methods / Q000592:standards; D008297:Male; D010146:Pain / Q000209:etiology; D059408:Pain Management / Q000379:methods / Q000592:standards*; D010166:Palliative Care / Q000379:methods / Q000592:standards*; D011613:Psychotherapy / Q000379:methods / Q000592:standards; D011788:Quality of Life