Addison’s disease

Difficulties in reaching the diagnosis

The whole picture became clearer in retrospect. Interviews with the medical team began to link many different, non-specific symptoms: the dizziness, low blood pressure, weight loss of about 4 kg. My periods had stopped. I had developed a bald patch on the back of my head. My underarm hair was practically non-existent. So was my libido (for which I had been referred to a psychotherapist). I had strong salt cravings, pigeon-holed as a bad habit best kept under wraps; and constant tiredness was easily attributed to the demands of two preschool children. Pigmentation, in my case, looked more like a lingering tan.

Such vague and disparate symptoms make early intervention in the case of Addison’s unlikely. My own various accounts during the course of several visits to the doctor, were selective, based on what I thought was relevant and what was not (such as not mentioning the salt cravings). It is perhaps not surprising that many patients are referred to several different specialists for, say, suspected depression, so that the eventual accurate diagnosis is delayed, or even too late as an untreated addisonian crisis can be fatal.

In my case, I was fortunate to have avoided a full blown crisis before the penny dropped. In hospital, steroid replacement therapy began immediately, resulting in an unforgettable energy surge: I felt that suddenly I had been plugged in and switched on. The relief was tremendous. I was told I would need lifelong, continuous treatment with steroids and that in the event of extreme physical stress (such as trauma, illness, or injury) urgent, extra cover would be essential to prevent a potentially life threatening addisonian crisis, in which without hydrocortisone the blood pressure falls very severely.

I was reassured that my children were unlikely directly to inherit the disease, although they would be more prone to developing some kind of metabolic disorder. I too would be regularly monitored for any signs of other autoimmune conditions.

Life with Addison’s

Coming to terms with a chronic condition inevitably takes time. At first there was the initial shock at what seemed like the betrayal of my own body and its new dependence on prescription drugs. I was grateful for my treatment, of course, but the loss of a “healthy” self and the idea of taking tablets on a daily basis took some getting used to.

Gradually I began to feel more in control, and six months later we returned to England. The condition seemed to be stable and I became better able to judge the effects of over- and under-medication. Two key turning points stand out here. Firstly, a hydrocortisone day curve analysis suggested that taking the hydrocortisone three times a day (on waking, at 12 noon, and at 5 pm) would be better than just twice (on waking and at 5 pm). This made an important difference to my energy and concentration levels in the afternoon.

Secondly, contrary to the printed instructions, it proved unnecessary for the hydrocortisone at such small doses (10 mg, 5 mg, and 5 mg respectively) to be taken with meals. Tiny as this piece of knowledge may seem, it was extremely liberating. Being advised to “take these tablets three times a day” seems simple enough. But that means “every day, for the rest of your life, regardless of what you happen to be doing at the time.” Many practical difficulties can get in the way of this (a work meeting, a school event, motorway driving, for example). It’s much easier to make sure that water is to hand at the right moment, rather than food as well.

Both these improvements owe much to the support of my endocrinologist in the United Kingdom and the quality of care he provides. Another considerable source of help and information has come from the UK Addison’s Disease Self Help Group. The group’s set of guidelines, not just for managing the disease on a day to day basis but also in terms of crisis management, provides a key reference when I need it. It is difficult to overstate the importance—and authority—of these guidelines in a hospital setting, not only when admission is Addison’s related, but also when it is not, such as for a procedure or condition that could easily destabilise the Addison’s into a potential crisis.

A recent colonoscopy brought home the potential precariousness of this condition. I had to be admitted to hospital overnight for the preparation, intravenous fluids, and intramuscular steroid cover. The Addison’s Disease Self Help Group’s guidelines recommend 100 mg hydrocortisone by injection every six hours during the preparation and again just before the procedure. But the time it can take to respond to the laxatives (in my case, about six hours) can vary hugely, and it made little sense to start the extra cover beforehand. In addition, the timing of the medication had to match the wait between the preparation and the actual examination: difficult when the colonoscopy unit runs late and the schedule slips an hour or two. Options are not necessarily clear cut; flexibility and on the spot judgments are needed. Fortunately it all went smoothly, but it is inevitable that, over the course of several shifts, not all members of the medical staff are necessarily familiar with Addison’s, or have experience in treating an adrenal emergency.

Understanding the condition

The Addison’s Disease Self Help Group continues to promote further understanding of the disease, both for patients and clinicians. It has successfully lobbied for paramedics to administer a steroid injection when needed. In an emergency, speed is imperative. Anecdotal evidence within the self help group suggests just how difficult it can be for patients and their families when encountering doctors who are hesitant in providing critical steroid cover. A short term excess of steroids is much safer than the converse.

Today, 15 years on, I feel fortunate to have a quality of life that not all those with Addison’s disease enjoy. Many continue to struggle with fatigue and other debilitating symptoms. I have witnessed—and benefited from—some substantial fine tuning of treatment, such as adding a small amount of dehydroepiandrosterone (DHEA) and splitting the fludrocortisone dose. Keeping fit helps me significantly. I have a fairly good understanding of my body’s limits, and the clues my body gives me if I start running a deficit. Above all, I am grateful for having had so few real scares to date and, in this respect to have faced few challenges.