AANA Updates

Butterfly Children: Epidermolysis Bullosa and Anesthesia

  • Aug 8, 2019

For Immediate Release: August 8, 2019
For more information, contact: AANA Public Relations

Park Ridge, Ill. – Each year, approximately 200 children in the United States are born with Epidermolysis Bullosa (BP). Certified Registered Nurse Anesthetists (CRNAs) will have a unique opportunity to learn about anesthetic considerations and other best practices when providing anesthesia care for patients with this rare disease during the American Association of Nurse Anesthetists (AANA) Annual Congress, August 9-13, 2019, in Chicago.

Epidermolysis bullosa (EB) is a rare genetic skin disorder characterized by severe skin fragility. These individuals are often referred to as “butterfly children,” because their skin is as fragile as the wings of a butterfly. Patients with EB tend to present for diagnostic or therapeutic procedures in specialist centers but may present for an unrelated surgical condition in a local hospital, hence the anesthetic management of the condition should be well known to all. In all but the mildest forms of the condition, life expectancy is reduced.

“Currently, there is no cure for EB, so it is imperative that nurse anesthetists are familiar with this rare skin disease and feel empowered to properly take care of these fragile patients,” said AANA co-presenter Kristin Henderson, DNP, CRNA, CHSE.

It should be remembered that surgery for EB is often carried out in young children and all the usual pediatric anatomical, physiological, and pharmacological considerations apply. A thorough preoperative assessment inquiring about all co-morbidities should be completed and previous anesthetic records obtained.

Preoperative blood tests may be necessary as the majority of severe EB patients are malnourished and anemic. The decision as to whether to transfuse preoperatively will depend on the hemoglobin level, local protocols, and the intended surgery. Signs and symptoms of intercurrent infection should be sought, as recurrent infections due to impaired skin barrier function are commonplace. In addition, some patients may be taking long-term oral corticosteroids, and perioperative supplementation should be considered. Regardless of the surgery being undertaken, meticulous planning and involvement of multidisciplinary clinicians in paramount.

“Considerations for the care of these patients begin at the very basics of standard monitoring and lead up to anesthetic considerations regarding the pathophysiology of this disease that lie much deeper than the surface of the skin,” said AANA co-presenter, Jamie Hobson, MSN, CRNA.

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