Addison's disease

A small gland sits on top of each kidney: the adrenal gland. Its outer layer makes hormones without which the body cannot hold its blood pressure, loses salt and copes badly with any strain. In Addison's disease that layer is slowly destroyed. The condition is rare, it creeps in quietly, and for months it passes for burnout, a stomach problem or low mood. It is nevertheless very manageable: the missing hormones are given as tablets. What matters is recognising it in time and knowing what to do on the days when the body needs more hormone than usual.

What actually stops working

The adrenal cortex makes two hormones that matter most here. Cortisol keeps blood pressure and blood sugar up and carries the body through an infection, an injury or an operation — through any shock. Aldosterone holds on to sodium and clears potassium, which means it governs salt and the volume of fluid inside the blood vessels. In Addison's disease both run short: pressure drops, salt is lost in the urine, potassium builds up, strength drains away.

There is a second consequence. The pituitary gland, getting no answer, pushes the adrenals harder and harder with its command hormone, and that hormone has a side property: it makes skin produce pigment. Hence the darkening of the skin, which is often what finally sends a person to a doctor.

How the illness shows itself

The signs pile up over months, and no single one of them points at the adrenal glands. It is the combination that should raise a question:

• tiredness that rest does not fix, along with muscle weakness;
• weight loss without dieting, no appetite, nausea, vomiting, abdominal pain, loose stools;
• craving salt: salting everything, reaching for olives and pickles, sometimes eating salt straight;
• darker skin on the palm creases, elbows, scars, nipples, gums and the inside of the cheeks. It looks like a tan but shows up where the sun never reaches. On darker skin the palm creases and the mouth are the places to look;
• dizziness and greying vision on standing up, sometimes fainting;
• aching muscles and joints, cramps;
• irritability, low mood, trouble concentrating;
• thirst and passing urine often;
• in children, growth that stalls, delayed puberty and episodes of low blood sugar.

It is found most often between the ages of thirty and fifty, though it can begin at any age. Women sometimes notice underarm and pubic hair thinning out.

Why the adrenal glands fail

• Autoimmune destruction. In Europe this is where most cases begin: the immune system treats the adrenal cortex as foreign tissue. Thyroid disease, type 1 diabetes, vitiligo, coeliac disease or vitamin B12 deficiency often keep it company.
• Infection. Worldwide the leading one is tuberculosis, which can wreck both glands years after lung disease. Less often fungal infection or cytomegalovirus in people whose defences are down, including those living with HIV.
• Bleeding into both glands. Rare, and the most dangerous of all: severe blood poisoning, meningococcal above all, blood-thinning medicines, a tendency to clot. It develops within hours and announces itself straight away as a crisis.
• Tumours, secondary deposits and surgical removal of the glands.
• Causes present from birth — congenital adrenal hyperplasia and rare inherited disorders in which the cortex never forms properly or fails early.
• Medicines — those that block the cortex from making hormones, and cancer treatments that work through the immune system.

One look-alike deserves its own line. Somebody who has taken steroid tablets for a long time and then stops them abruptly is left without cortisol of their own, because the glands need time to switch back on. The picture is the same, only without the darkening of the skin. That is why such tablets are never stopped in one go.

Adrenal crisis: when to call an ambulance

A crisis is a collapse of hormone supply and it threatens life. It is set off by things a healthy person shrugs off: a stomach bug, flu, an injury, a tooth extraction, a bad shock, a missed dose. Sometimes the crisis is the first sign of an illness nobody suspected.

Call the emergency number for your country — 112 across Europe — if there is:

• sudden weakness with vomiting and diarrhoea that will not settle;
• severe pain in the abdomen, the lower back or the legs;
• fainting, confusion, drowsiness the person is hard to rouse from;
• very low blood pressure, a fast weak pulse, cold clammy skin;
• a seizure;
• fever together with weakness that is deepening fast.

If an emergency hydrocortisone injection kit is at hand, give it at once and still call for help: one injection is not enough, fluids and monitoring have to follow. Tell the crew plainly that this is adrenal insufficiency.

How the diagnosis is confirmed

Suspecting it is hard; proving it is not. The starting point is a blood test taken first thing in the morning: cortisol and the pituitary command hormone. Low cortisol alongside a high pituitary hormone is almost the answer on its own. Where doubt remains, a stimulation test is done: a synthetic version of that hormone is injected and the gland is watched to see whether it responds. Sodium, potassium and blood sugar in mmol/L are checked at the same time.

Then comes the cause: antibodies against an enzyme of the adrenal cortex and, if they are absent, a scan of the glands and a search for tuberculosis. The thyroid is checked as well. One safety point: if thyroid function is also low, treatment starts with the adrenal hormones, because thyroxine given first speeds up the metabolism and can tip a person into crisis.

The daily replacement treatment

The gland cannot be restored, but what it no longer does can be replaced accurately. Hydrocortisone stands in for cortisol, taken in several doses across the day to copy the natural rhythm of more in the morning and less towards evening. Fludrocortisone stands in for aldosterone; with it salt is not restricted, and in hot weather more is needed. Doses are set by an endocrinologist.

The rules that decide the outcome as much as the tablets do:

• carry a card or bracelet naming the diagnosis — if consciousness is lost it buys time nobody has;
• keep a supply of medicine and injection ampoules at home and when travelling, and know how to give the injection and how to teach a relative;
• never skip a dose and never stop the medicine on your own;
• if a tablet is vomited back within the hour, take it again;
• tell any doctor or dentist about the condition before treatment begins.

Sick days, surgery and pregnancy

A healthy gland answers stress by releasing extra cortisol by itself. Here the person makes that decision: the dose goes up according to a rule agreed with the doctor in advance, not improvised once things have already gone wrong.

• fever or infection — the dose is raised for as long as the illness lasts and for a day afterwards;
• vomiting or diarrhoea — the tablet is not absorbed; an injection and a doctor are needed, not a second tablet;
• surgery, childbirth or major injury — the hormone is given into a vein, so warn the team beforehand;
• heat, hard physical work or heavy sweating — more water and more salt;
• pregnancy — the requirement usually rises in the final months and the hormone is given into a vein during labour; obstetrics and endocrinology should follow the pregnancy together;
• long journeys — medicines in hand luggage, with a spare supply and a summary in a language understood where you are going.

Beyond that there is hardly a restriction: work, sport, vaccinations, dental care. With treatment properly adjusted, life expectancy comes close to anyone else's, and the real danger is not the disease itself but a crisis that goes unrecognised.

Online consultation: what it can do

A remote appointment suits the first step, while the picture is still vague: months of weakness, weight slipping away, skin turning darker, dizziness on standing. The doctor sorts through the complaints, looks at tests already done and advises what to request and in what order. Once the diagnosis exists, online contact carries the everyday questions: how to write down the sick-day rule, what to arrange before a trip or an operation, whether the regimen needs revisiting. Acute trouble is not handled this way — a suspected crisis needs an ambulance, not a message.

This material is for information only and does not replace medical advice.