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Twelve years ago, Siri Lowe developed pemphigus vulgaris, a potentially fatal autoimmune blistering disorder. As her story shows, it can have a devastating impact on patients' lives, as can the side effects of the necessary treatments
When my mouth and tongue suddenly became badly blistered in 1995, I never suspected there could be anything seriously wrong with me—this was surely just some crazy infection in my mouth. But it was pemphigus vulgaris, a rare, life threatening, autoimmune disease affecting skin and mucous membranes, and, of course, I'd never heard of it in my life.
I deteriorated quickly and in only five weeks moved from being strong and healthy to being unable to eat solid food, drinking through a straw, and being in agonising pain. I remember walking the London streets in a total daze thinking, “This can't be true, it's like the script of a bad television play.” I had no idea how living with a chronic disease would change my life.
There's no cure for pemphigus. No smart drug to take it all away. It is nearly always controllable, but that control comes at a heavy cost. At that time, my only options were high dose corticosteroids or heavy immunosuppressant treatment. Not only had I a life threatening disease but my future suddenly became ruled by serious prescription drugs.
During the first three years, the drugs themselves brought their own health problems, some serious and some just hard to live with. I was hugely grateful that my disease wasn't as bad as some people's—at least my skin hadn't literally blistered off my body—but it gradually began to affect nearly every area of mucous membrane: mouth, nose, throat, eyes, vagina, cervix.
Coping with the condition was extremely difficult. The slightest change in drug regimen could disturb the precarious balance, and instead of controlling the disease I'd be tumbling down into another crisis. Those years were a constant nightmare until my consultant dermatologist found a drug combination that I could tolerate and which worked (with only minor flares).
I secretly hoped my “journey” would be over once I got this far. Consequently, I found it very hard to accept that my health was badly damaged and I couldn't return to my old self. High dose corticosteroids and immunosuppressants had been necessary, and undoubtedly saved my life. But there were side effects, and I was now disabled with problems with walking and pain. Prescription analgesics and pain management techniques could do no more than modify the pain levels.
Six months after becoming ill, it became clear I couldn't return to work. I had to retire. Suddenly I was no longer part of a dynamic publishing company. I was also far too ill to continue with the voluntary work I'd trained to do, and the hobbies I'd enjoyed before were now either physically impossible or too exhausting. By the time my condition was more stable, I'd lost many of the things that defined who I was. I'd lost my financial viability and my identifiable role in the world. I was also a claimant, constantly worrying about the next set of forms to be filled in. All I had was the illness. I was an illness and nothing but an illness.
But that's the downside. I also had wonderful companions. My surviving family were elderly and frail and needed to be protected from some of the unpleasant facts, but I had the constant presence of many loving and supportive friends. With them, I was able to explore new ways of relating to people. I'd usually been the “helper.” Now I had to learn to accept help, and it wasn't an easy lesson. I started to realise that I could still have a sense of identity but that it would have to be a different one.
One of the ways this happened was as a direct result of my condition. In the early days one of the things I had found most difficult was getting any information about pemphigus and what it was like to live with. How many people survived? Did everyone feel as awful as I did taking the drugs? Then a friend discovered the International Pemphigus Foundation in the United States, and I was able to get those myriad questions answered. It was so much easier to cope once there was someone else with pemphigus to correspond with. I still remember that sense of relief. So, together with friends, I started the UK patient support group, which is still running today.
Of course, my constant companions are the doctors who look after me, primarily my consultant dermatologist, who has stayed consistently committed to my care. Because pemphigus has affected my body in so many different ways, I also see seven other consultants in four different hospitals. Miraculously, I've mostly been able to stay with the same specialists. This continuity makes such a difference. It's grim enough spending so much time waiting in hospital outpatient clinics, but it's really awful having to explain, yet again, to yet another doctor, the problems that are important to me. Hospital notes should do this, of course, but somehow they never do.
Other serious medical conditions have added to the difficulties. I've had breast cancer in both breasts—five operations, radiotherapy, etc. Suddenly I can find myself seeing doctors or nurses who don't believe a skin disease is serious. At times it seems impossible for people to see that my needs as a cancer patient can't be dealt with in isolation from my needs as a pemphigus patient. My calm dissolves in the insanity of trying to communicate with a health professional who doesn't believe patients can know what they're talking about: I just have to remember all the fantastic doctors and nurses who can see the wider picture. Luckily this problem doesn't occur often.
I've learnt to live in new ways: to celebrate the good days and try to use them. Equally, I have to accept the bad times—even if that means days or weeks when all I can be is the “sick” person. My condition can change rapidly, so I have to stay ready to adapt within days.
Emotionally it's hard to shift paradigms at the drop of a hat. There's no point being permanently optimistic, for then it feels catastrophically painful to slip down again. Conversely, there's no point being permanently pessimistic, for then there'd be no joy in life, no possibility of experiencing each moment for itself.
I would love to have been more upbeat describing this journey, but the truth is more complex. Of course, I hope that medicine will provide a real cure, though there's no scientific indication of that on the horizon. So I can only hope that pemphigus, the drugs, pain, and disability will leave me enough space to live an altered, but still worthwhile, life.
Pemphigus is a serious autoimmune blistering disorder caused by circulating autoantibodies to the epithelial adhesion proteins desmoglein 1 and 3. These antibodies result in a failure of epidermal cells to adhere correctly to each other. This causes flaccid blisters and subsequently painful erosions in the skin and mucous membranes.
Before the introduction of systemic glucocorticosteroids in the early 1950s, pemphigus was a universally fatal disorder with extensive involvement of the skin and mucous membrane leading to prostration, sepsis, and failure of multiple organs. The use of steroid sparing immunosuppressants such as azathioprine, cyclophosphamide, methotrexate, and ciclosporin has been the turning point in the management of the disorder. Recently, rituximab (an anti-CD20 antibody which targets B cell differentiation) administered together with intravenous immunoglobulin has been shown to be another advance. Pemphigus is therefore now eminently treatable.
Ms Lowe's account does show, however, just what an unpleasant disorder pemphigus vulgaris is, and what a tremendous toll it has taken on her personal life. She describes how completely devastated she was by the diagnosis and how the treatment, particularly systemic corticosteroids, interfered with her wellbeing. She also had ready access to information particularly via the internet, and this must have been quite frightening to read.
As the physician central to her case for the past 10 years, I have found my role to be largely guiding Ms Lowe through her illness and adapting the use of steroids, azathioprine, and ciclosporin to fit her personal needs. It was possible to induce a complete remission fairly early on, although she did have a relapse five years ago, and she still worries that she might relapse again. During this time, she has also developed breast cancer, and was clearly concerned what effects surgery and intubation during anaesthesia might have on the disease. The dermatologist working in the hospital was able to liaise with her other specialists, explain her condition, and orchestrate her care, so that she managed psychologically to survive the trauma of her second illness.
The lesson that was reinforced to me about caring for her and for most patients with serious skin disorders is how essential adequately resourced specialist dermatology services are.
Ms Lowe has fought her illness with great fortitude. A measure of this has been the determination with which, with the support of the British Association of Dermatologists, she set up the first patient support group for pemphigus, which has been of immense help to other people with the condition.
Anthony du Vivier, consultant dermatologist, King's College Hospital, London (moc.loa@reiviVudynohtnA)
This is one of a series of occasional articles by patients about their experience of traumatic medical events that offer lessons to doctors. The BMJ welcomes contributions to the series. Please contact Peter Lapsley (firstname.lastname@example.org) for guidance.
Competing interests: None declared.
Provenance and peer review: Not commissioned; not externally peer reviewed.