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Oh my... look at those blisters!

Independent consultant Bethann Siviter helps a friend with an extreme case of epidermolysis bullosa

Independent consultant Bethann Siviter helps a friend with an extreme case of epidermolysis bullosa

epidermolysis bullos
Picture used with permission of Wendy Hilling

A friend with epidermolysis bullosa (EB) called me. 'I know blisters hurt, but…' Her blisters were so extreme I found it hard to fathom how she coped, especially since at 67 she is well beyond the life expectancy of someone with EB. I suggested they looked infected, and asked what she could take for pain. 'Nothing' she said. Nothing? Wow.

Can you imagine having widespread lesions akin to second degree burns and have no relief? She spends hours daily dressing wounds, draining blisters, and she does this on a liquid diet as throat strictures make it difficult to swallow and to breathe. She can’t take opiates or NSAIDs as her skin (both inner and outer epithelium is affected) cannot tolerate them. And, she has done this without the support of community nursing.

Why? Perhaps it’s because EB is a moderately rare and confusing disorder which few GPs and community nurses are well informed?

Paramedics who have responded when her throat closed did not believe she could not breathe, until hospital staff told them what a crisis was at hand. Doctors have been dismissive. Why? She has a specially trained dog who alerts her husband if she has trouble breathing; he (dog, not husband) also calls the ambulance. Yet, what I feel could be her best support – community nurses – are absent from her care, and she is left to her own devices.

In my personal practice, I encountered one child with a mild form of EB. No one in our team or surgery knew what to do, and our instincts about wound care are contrary to good EB practice.

So, do we leave this lady on her own (well, with wonderdog Ted and hubby) or do we offer help, advice and support? It’s up to you, isn’t it?  Can you learn more about EB?  Start here

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