Hand deformity happens early in children with dystrophic epidermolysis bullosa
REPORTING FROM EB 2020
Effects of hand surgery do not last long
How long the surgery’s effect will last is “what everyone wants to know, and I don’t think anyone has found a really good answer. It is variable, but unfortunately it’s a lot shorter than we’d like,” said Ms. Smith.
Indeed, data in another poster presentation by Ms. Smith and colleagues showed that the situation can be ‘back to square one’ within just a couple of years. Of the seven patients who had surgery at a mean 7 years of age (range 6-10 years), “most had returned to their original total score by 2 years post surgery,” the team wrote. All children “were initially happy with both appearance and function after surgery” they added; however, “happiness gradually decreased with time as they lost function and their scores increased with recurrence of contracture.”
The team noted that “sometimes after surgery a different component of the hand contracture worsened but function was preserved.”
While the ACE tool used by the team has not yet been validated, they believe it to be “a systematic tool with a structured method of administration.” As such it can help with informed decision making, they believe, and it could be used with functional measures to see how hand contractures might be impacting hand function and quality of life.
The ACE tool can be downloaded for free from the GOSH website.
SOURCE: Jessop N et al. EB 2020. Posters 42 and 43; Smith G et al. Poster 63.
