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Pain care for patients with epidermolysis bullosa: best care practice guidelines

Pain care for patients with epidermolysis bullosa: best care practice guidelines
Pain care for patients with epidermolysis bullosa: best care practice guidelines
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
epidermolysis bullosa, pain, practice guidelines, RDEB, DEBRA, acute pain, chronic pain, recessive dystrophic epidermolysis bullosa, dystrophic epidermolysis bullosa research association international
1-23
Goldschneider, Kenneth R.
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Good, Julie
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Harrop, Emily
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Liossi, Christina
fd401ad6-581a-4a31-a60b-f8671ffd3558
Lynch-Jordan, Anne
85b084fb-978a-4a85-bbc0-e97bc26fcbea
Martinez, Anna E.
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Maxwell, Lynne G.
d930dc4a-47df-47d6-9ae8-d7a3238bd9fa
Stanko-Lopp, Danette
2b9467b0-0398-42a6-814d-71d327e412a1
Goldschneider, Kenneth R.
f4bf65a6-97ed-485d-b1da-e5cea3657ae9
Good, Julie
d290a106-2541-42ad-ad0e-53f203eba9f3
Harrop, Emily
01114a0a-f1c8-4d71-aaff-c50365561ddd
Liossi, Christina
fd401ad6-581a-4a31-a60b-f8671ffd3558
Lynch-Jordan, Anne
85b084fb-978a-4a85-bbc0-e97bc26fcbea
Martinez, Anna E.
7b3a0998-c62f-4928-ad42-506e30bca881
Maxwell, Lynne G.
d930dc4a-47df-47d6-9ae8-d7a3238bd9fa
Stanko-Lopp, Danette
2b9467b0-0398-42a6-814d-71d327e412a1

Goldschneider, Kenneth R., Good, Julie, Harrop, Emily, Liossi, Christina, Lynch-Jordan, Anne, Martinez, Anna E., Maxwell, Lynne G. and Stanko-Lopp, Danette (2014) Pain care for patients with epidermolysis bullosa: best care practice guidelines. BMC Medicine, 12, 1-23. (doi:10.1186/s12916-014-0178-2).

Record type: Article

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

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Published date: 9 October 2014
Keywords: epidermolysis bullosa, pain, practice guidelines, RDEB, DEBRA, acute pain, chronic pain, recessive dystrophic epidermolysis bullosa, dystrophic epidermolysis bullosa research association international
Organisations: Psychology

Identifiers

Local EPrints ID: 372283
URI: https://eprints.soton.ac.uk/id/eprint/372283
PURE UUID: f5056cbe-f23d-4f4d-9374-433cb95c940c
ORCID for Christina Liossi: ORCID iD orcid.org/0000-0003-0627-6377

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Date deposited: 04 Dec 2014 14:01
Last modified: 10 Sep 2019 00:43

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Contributors

Author: Kenneth R. Goldschneider
Author: Julie Good
Author: Emily Harrop
Author: Anne Lynch-Jordan
Author: Anna E. Martinez
Author: Lynne G. Maxwell
Author: Danette Stanko-Lopp

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