by Siri Lowe, The PV Network, England

I imagine that everyone having an operation feels a bit nervous about the anesthetic, but if you’re also living with pemphigus there’s another whole set of questions and problems you worry about.

I hadn’t particularly thought about it until earlier this year when I had to have cancer surgery and suddenly realized there were no standard protocols for pemphigus patients and no-one was considering that maybe I would need different anesthesia procedures than the average patient. So I’m writing this article to share the solutions that were found for my particular situation, in case they may be useful for any other pemphigus patients facing surgery.

The initial problem, as usual, was that because pemphigus is rare, the surgeon and oncologists who were looking after me had never treated another pemphigus patient. Initially it seemed to me that they were completely unaware of how unstable pemphigus can be and, as is often the case, they were treating the disease (cancer), not the patient.

I’m sure that even if I hadn’t been pushing for people to consider the pemphigus question, my surgeon would have consulted medical sources to find out about it. However, I suspect that if a surgeon does a standard search the information would largely be about skin lesions – and my disease activity is internal, worst in throat, mouth and nose. Again, a standard search would say that most patients are on steroids and surgeon and anesthetist would be considering extra doses of steroids before the operation to protect patients from a flare up. But not absolutely everyone is on steroids. Some people are on immunosuppressives alone. I’m currently on Azathioprine and Cyclosporin, admittedly an unusual drug combination. So there isn’t an obvious safety measure to take with those drugs to protect against a pemphigus flare.

My particular worries were that medical trauma from the surgery might cause the pemphigus to flare. We never did find any protection for that, I just kept my fingers crossed and luckily nothing awful happened. My other big worry was that the actual tubes and equipment needed for the anesthetic would, by contact alone, cause the minor disease lesions in throat and mouth to go way out of control, possibly even during the operation itself – my worst nightmare being trauma to the vocal cords and loss of the ability to speak. (Not a crazy fear, it’s happened before but not through surgery.)

I was lucky that once I’d spoken to him about my fears, my dermatologist was very supportive and was happy to liaise with my surgeon. I think this liaison is crucial as I suspect my word alone would have been taken as a patient “flapping” unnecessarily. The problem, in the UK at least, is that it’s not always easy to arrange this kind of link-up between departments. I can only suggest that anyone in a similar situation doesn’t assume the links would happen automatically and does speak to their dermatologist personally and get them involved.
I also felt that I wanted to speak personally to whoever would be my anesthetist, and I wanted to do that at least a day before the operation in case they needed time to speak to other people or do any research on what is, after all, a very unusual medical condition. It did seem to me that I had to push unnecessarily hard to get this to happen, but it was well worth it. My anesthetist was absolutely terrific, very understanding and very happy to take my word on what pemphigus is like for me personally. So once I’d had a chance to speak to her I felt I could breathe a huge sigh of relief and just let the experts get on with the surgery. Again, I’d suggest that anyone who has any worries presses to have a proper talk with their anesthetist.

My lay person’s understanding of the problem was that modern anesthesia equipment often involves a face mask with tubes touching the mucous membranes in mouth and throat (excuse me everyone if I’ve got this wrong). Since even a small cut-up piece of food can prove impossible to swallow at times, I imagined an old-fashioned narrow tube would be least likely to cause me damage. Once I’d talked to my anesthetist I obviously left all the decisions in her hands and didn’t know exactly what she decided to do. So when I wanted to write this article I wrote asking if she could give me a run-down (in layman’s language) on the technique she used for me. She has very kindly replied and I’d like to quote her in the hopes that the information may be a useful starting point for any other doctors who have pemphigus patients facing an operation.

“As you mention there is little written in the medical press about the peri-operative anesthetic management of patients such as yourself. However, between ourselves we managed to formulate a plan. It involved:

1. Limiting the manipulation of the oro-pharanx (mouth and throat)
2. Avoiding the use of an oral airway if at all possible
3. Using a disposable oro-tracheal tube made from inert plastic
4. As always, using extreme care when inserting instruments into the mouth, trying to utilize as little force as possible

The airway needs of each anesthetic can be different depending on the particular operation and patient and as such there can be no absolute recipe…. I believe that adaptability will always be the key.”

Of course, apart from the problems for pemphigus patients about operations themselves, there is always the question of what happens when you’re back on the ward after the operation. How do you keep safe from infections? What’s the best way to make the nursing staff aware of the problems that go with pemphigus, like skin reactions to plasters etc.? But that’s another article in itself and I’m sure lots of us have already had to grapple with those situations during treatment for pemphigus. So I’ll end here and hope that this article proves useful.