Pemphigus Vulgaris: My road to remission

---

In April, 2012 I saw my dentist about a sore which didn't heal and was given antibiotics. When these made no difference, I went back to the dentist. She seemed unsure what to do and I pushed for an investigation, saying "Are you sure it's not cancer?" I did this was because a friend had recently been diagnosed with oral cancer afetr mnoths of having an unhealed sore. She fortunately made a complete recovery. My dentist panicked and started drilling away at a good tooth in search of decay, despite the x ray showing none. Having drilled it away almost to nothing, she then agreed to send me to the local hospital for a biopsy.

At the local hospital I was told I had desquamative gingivitis and made to feel I had caused the problem by poor oral hygiene. As I have always looked after my teeth and no dentist had ever commented on this kind of neglect, I was a bit shocked. It turned out that this was not the reasi=on for my problem. By July, 2012 I had a diagnosis - as the oral medicine doctor said "The good news was I didn't have cancer, but I did have pemphigus vulgaris". She gave a differntail diagnosis of pyostomatitis vegetans so even the diagnosis of pemphigus vulgaris felt uncertain at this point. She suggested that a few steroids and a mouthwash would sort it out, but gave no further information about the disease. I asked her to write it down for me, so she probably guessed I was going to google it. She showed my mouth to a dental student and said he'd probably never see it again so I knew it was rare. She offered me a choice of care either at the local hospital, where presumably nobody had ever seen it or at Guy's, a London teaching hospital. Luckily I opted for Guy's!

Even googling the most up to date websites about pemphigus when first diagnosed is fairly harrowing. The seriousness of the disease is undeniable and the fact that there is treaatment is some comfort but it's clear the treatment brings its own problems. This was a thunder bolt for me and triggered all sorts of fears.I think this was not an unusual reaction. Most people seem to be worried in the early stages of the disease.

My symptoms worsened over the summer and I was worried about going away in case I became very ill. So I contacted Guy's. By some miracle the person on the switchboard directed me to dermatology where I spoke to a specialist pemphigus nurse. She was fantastic, reassured me, told me what to do if my symptoms worsened etc. 

By September my GP was concerned at the deterioration of my mouth. I had more sores, couldn't eat many foods and had a red and sore throat. Following the specialist nurse's advice, she called the on call dermatologist and insisted I should be seen that day. By the end of that day I had seen Dr. Richard Groves, Lead clinician, who said with authority that it was definitely pemphigus vulgaris. I had a whole host of blood tests done and was given some topical treatment. From then onwards I was under the care of a very efficient department with great experience of treating pv. I had an ENT appointment which made it clear that there was no long term damage to my throat. Phew! 

Dr. Groves kept in touch with me by phone. I had an appointment in November and it was agreed that I should start systemic treament then. In the meantime it was taking me ages to eat the smallest amount of food. I couldn't eat anything spicy, textured or acidic. Bread of any kind was out of the question. It was miserable. The misery increased, as I developed really painful nasal sores which became infected. The infection knocked me for six but luckily responded well to antibiotics. I also had 4 sores on my scalp that were painful and unsightly and I was starting to get nasty sores on my body. Dr. Groves commented that he hadn't been expecting all this when he next saw me! Thye disease was escalating fast.

The disease was treated with azathioprine and prednisolone. My treatment plan tallied exactly with one proposed by the IPPF. My peer coach there was a great support. She knew Dr Groves from an IPPF conference. It was reassuring to know he is held in such high regard and respected as a world expert. Anyway, the treatment brought its own issues - terrible insomnia at first, stomach upsets, weight gain, depression etc etc etc. I took azathioprine for 15 months. This dug had an adverse effect of my lymphocytes and so I never made it to 3 monthly blood tests. I had to be tested weekly because there was some risk of more serious problems. I took the prednisolone for 18 months in what seemed like the world's longest taper. During that time I waited sometimes if the disease showed signs of flaring so we managed it well and nothing really major happened. I did have a nasty burst blister in my throat which was a bit scary (I coughed up blood and felt like I was choking) but again Dr Groves managed it well and held my taper for a few weeks. This helped me in other ways, as the taper made me unbelievably tired and it was good to slow it down sometmes, even though I was desperate to come off what I called "the toxic cocktail".

I had a clinical remission quite early on in the treatment. My autoantibodies went down to normal levels. This was very encouraging and so I was able to come off all meds. HURRAY! I have been drug free for 16 blissful months, but have had to go back on prednisolone recently, as I have a slightly increased level of activity and my autoantibody levels had been creeping up. Typically they were lower 2 weeks after I started treatment!!! So here I am again, but this time I am sure I will come off the meds much more quickly and I am confident I will be fine.

It has been a very challenging journey, but I have made it through to the other side. I realise I have been lucky in having a very early diagnosis and propmt treatment by a wonderful medical team, not to mention the IPPF, Pemphigus Vulgaris Network and Pem Friends. These groups have given me generous support and contact with others with pv. 

Please do contact me if you want to know more about what happened to me and how I coped with the physical and psychological challenges of the disease.