Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

Greenblatt, Danielle; Pillay, Elizabeth; Snelson, Karen; Saad, Rebecca Anne; Pradilla, M. Torres; Widhiati, Suci; Diem, Anja; Knight, Caroline; Thompson, Kim M.; Azzopardi, Nazzareno; Werkentoft, Mia; Moore, Zena; Patton, Declan; Mayre-Chilton, K. M.; Murrell, Dédée F.; Mellerio, Jemima E.

doi:10.1111/bjd.20809

Cited by 8 publications

(7 citation statements)

References 50 publications

(382 reference statements)

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“… Good Practice Points (GPP) GPP—recommended best practice on the clinical experience of the guideline development group (Ratings table taken from Greenblatt et al [ 89 ] “Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus-based guideline”) …”

Section: Resultsmentioning

confidence: 99%

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Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Popenhagen,

Genovese,

Blishen

et al. 2023

Orphanet J Rare Dis

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Background Inherited epidermolysis bullosa (EB) is a cluster of rare, genetic skin and mucosal fragility disorders with multi-system and secondary effects, in which blistering and erosions occur in response to friction/mechanical trauma. Considering the incurable and potentially life-limiting nature of the condition and the challenges posed by its symptoms, a palliative approach to EB-related care is necessary. However, knowledge and experience related to the provision of EB palliative care is minimal. Evidence-based, best care guidelines are needed to establish a base of knowledge for practitioners to prevent or ease suffering while improving comfort at all stages of the illness, not just the end of life. Methods This consensus guideline (CG) was begun at the request of DEBRA International, an international organization dedicated to improvement of care, research, and dissemination of knowledge for EB patients, and represents the work of an international panel of medical experts in palliative care and EB, people living with EB, and people who provide care for individuals living with EB. Following a rigorous, evidence-based guideline development process, the author panel identified six clinical outcomes based on the results of a survey of people living with EB, carers, and medical experts in the field, as well as an exhaustive and systematic evaluation of literature. Recommendations for the best clinical provision of palliative care for people living with EB for each of the outcomes were reached through panel consensus of the available literature. Results This article presents evidence-based recommendations for the provision of palliative healthcare services that establishes a base of knowledge and practice for an interdisciplinary team approach to ease suffering and improve the quality of life for all people living with EB. Any specific differences in the provision of care between EB subtypes are noted. Conclusions Because there is yet no cure for EB, this evidence-based CG is a means of optimizing and standardizing the IDT care needed to reduce suffering while improving comfort and overall quality of life for people living with this rare and often devastating condition.

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Section: Resultsmentioning

confidence: 99%

“…(Ratings table taken from Greenblatt et al [ 89 ] “Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus-based guideline”)…”

Section: Resultsmentioning

confidence: 99%

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Popenhagen,

Genovese,

Blishen

et al. 2023

Orphanet J Rare Dis

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“…Pregnancy in affected individuals may be associated with malnutrition, severe anemia, and chronic infection and should be considered. (15) In 2022, experts from all over the world came together and based on previously published studies, they presented a consensus-based guideline for the management of pregnancy and delivery in patients with epidermolysis bullosa, which we used in patient management (20) .…”

Section: Discussionmentioning

confidence: 99%

Pregnancy and delivery in late diagnosed Kindler syndrome: A case report and review of the literature

Ghasemi,

Najafi,

Milani

et al. 2023

Preprint

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Epidermolysis bullosa is a collection of infrequent, diverse, and genetic disorders with four primary classifications, identified by the skin's vulnerability and, in some cases, mucosal fragility. The skin and mucosal layers separate when subjected to friction or mechanical stress, leading to blisters and erosions within these tissues. A woman aged 29, afflicted with Kindler syndrome, presented at our hospital experiencing labor contractions. Her skin condition remained unaltered throughout her pregnancy and post-childbirth period. A cesarean section was carried out at the 38-week gestation mark due to an unplanned membrane rupture and dense meconium presence. The healing of the surgical wound proceeded without complications. In this instance, the patient's pregnancy did not intensify the skin-related symptoms of Kindler syndrome. It is critical to manage perioperative care to safeguard susceptible skin and mucosa carefully. The patient's journey throughout pregnancy and the postnatal period proceeded without notable incidents. Individuals with EB giving birth often require substantial assistance due to their condition.

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“…Limited evidence from small case series suggests that women with epidermolysis bullosa (EB) can have successful pregnancies and deliveries. Consensus-based guidelines that provide recommendations for improving the preconception, pregnancy, childbirth, and postpartum management of women affected with EB have been recently published 4. They provide detailed guidance for the care of patients with EB who desire to plan a pregnancy.…”

Section: Dermatology — Other Dermatologymentioning

confidence: 99%

UpToDate®

2022

The Nurse Practitioner

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UpToDate ® and The Nurse Practitioner are collaborating to present select "What's New" content. UpToDate is an evidence-based, clinical support resource used worldwide by healthcare practitioners to make decisions at the point of care. For complete, current "What's New" content, or to become a subscriber for full content access, visit go.uptodate.com/np. "What's New" is free for all medical professionals.Disclaimer: This content is provided for reference purposes only and represents a portion of the UpToDate topic. Readers should not rely on the content or any information cited here as being applicable to specifi c patient circumstances. All topics are updated as new evidence becomes available and our peer review process is complete. Family Medicine -PreventionLow-dose aspirin for primary cardiovascular disease prevention (June 2022) Low-dose aspirin may prevent cardiovascular disease (CVD) in some patients but also increases the risk of bleeding. The 2022 United States Preventive Services Task Force (USPSTF) statement concluded that among people 40 to 59 years of age with a ≥10 percent risk of CVD over the next ten years, there is a small potential benefi t of low-dose aspirin. 1 The USPSTF did not recommend starting low-dose aspirin in persons over 60 years without heart disease. We believe the decision to prescribe aspirin for primary prevention should be made by the patient and their provider after discussing individual risks and benefi ts.

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Recommendations on pregnancy, childbirth and aftercare in epidermolysis bullosa: a consensus‐based guideline*

Abstract: Linked Comment: A.W. Lucky and E. Pope. Br J Dermatol 2022; 186:602–603. Plain language summary available online

Cited by 8 publications

References 50 publications

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Consensus-based guidelines for the provision of palliative and end-of-life care for people living with epidermolysis bullosa

Pregnancy and delivery in late diagnosed Kindler syndrome: A case report and review of the literature

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