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Addison's disease

Published by Bupa's health information team, May 2009.

This factsheet is for people who have Addison's disease, or who would like information about it.

Addison's disease is a rare condition in which your adrenal glands become damaged, stopping the production of hormones that usually help control your blood pressure, fight off infections and regulate your blood sugar level.

If you have Addison's disease you will need lifelong treatment with replacement hormones.

About Addison's disease
Symptoms of Addison's disease
Causes of Addison's disease
Diagnosis of Addison's disease
Treatment of Addison's disease
Complications of Addison's disease
Living with Addison's disease
Questions and answers
Further information
Sources

About Addison's disease

You have two adrenal glands, one on each side of your body, located just above your kidneys (see illustration). Each adrenal gland has an inner core (called the medulla) and an outer shell (the cortex).

In Addison's disease, the cortex of your adrenal glands becomes irreversibly damaged or destroyed. The cortex normally produces the following hormones:

cortisol, which helps to maintain energy, and regulate your blood sugar level and muscle strength
aldosterone, which regulates the salt and water levels in your body, helping to control blood pressure
adrenal androgens (or 'sex hormones'), including DHEA, which may influence stamina and sex drive (libido) in women

When your adrenal gland becomes damaged in Addison's disease, your body can't produce enough of these hormones.

Addison's disease is a rare condition, only affecting about one in every 20,000 people. You can get the condition at any age, but the most common age of onset is between 40 and 60 years. Many people may live with the disease unaware that they have it for a long time.

Symptoms of Addison's disease

If you have Addison's disease you may get any of the following symptoms:

extreme tiredness
weakness
loss of appetite and weight loss
abdominal pain
feeling sick and vomiting
diarrhoea
discolouration of your skin, particularly your face and hands and recent scars
pain in your muscles and joints
dizziness/fainting when you stand up (postural hypotension)
cravings for salt
loss of pubic hair and hair in your armpit, if you are a woman

These symptoms may be caused by problems other than Addison's disease. If you get these symptoms, you should visit your GP for advice.

Causes of Addison's disease

In the UK and other developed countries, Addison's disease is usually an autoimmune condition. This means it's caused by antibodies from the immune system attacking your body. In Addison's disease, your immune system attacks your adrenal glands so that they cannot produce hormones.

Many people with Addison's disease also have another autoimmune disorder, such as a thyroid disorder or diabetes - or are likely to develop one in the future.

The exact reasons why you may develop autoimmune Addison's disease are not known.

Other rarer causes of Addison's disease include:

tuberculosis (this is a common cause in developing countries)
other infections, including fungal infections
surgical removal of your adrenal glands
cancer of your adrenal glands
injury to your adrenal glands, eg following a car accident

Diagnosis of Addison's disease

Your GP will ask about your symptoms and examine you. He or she may also ask you about your medical history.

Your GP may refer you to an endocrinologist - a doctor specialising in conditions that affect your hormones.

You will need to have blood tests in hospital to confirm whether you have Addison's disease. The main blood test measures how much cortisol your body can produce. It's called a Synacthen (ACTH stimulation) test. Additional blood tests for plasma renin, sodium and potassium, measure your aldosterone function.

You may also have imaging studies of your adrenal gland - either a CT scan or an MRI scan.

Treatment of Addison's disease

You will need lifelong treatment with steroid replacement therapy, to replace the hormones that your body is no longer making. You will usually be prescribed the following medicines.

Hydrocortisone - this replaces the hormone cortisol. It's taken as a tablet, usually in two or three doses throughout the day.
Fludrocortisone - this is a tablet taken to replace aldosterone. You will usually just need to take one dose, every morning.
DHEA - some doctors may also prescribe this medicine for women with loss of sex drive or a low mood, although there is no proof at present that it can help all women with Addison's disease. It's usually taken as a single dose in the morning.
Hydrocortisone injections - your doctor should also prescribe an injectable form of hydrocortisone to use if you are feeling sick or have had an accident or other severe injury. Your doctor will supply a sterile syringe and needle. Your practice nurse will teach you, or a friend or relative, how to inject the hydrocortisone into a muscle in your thigh.

Doses of these hydrocortisone and fludrocortisone tablets differ from person to person. You may need to have a number of tests to find the right dose for you, including blood pressure checks and blood tests to check how long the hydrocortisone stays in your body (sometimes called a day curve analysis).

At certain times, you may need to increase your dose - for instance if you have a fever, or if you are having surgery or dental work done. Your doctor will give you advice on when you should do this, but for most illnesses treated at home, you will need to double your hydrocortisone dose.

Usually, your endocrinologist will want to see you every six to 12 months. Your GP will provide regular health care and support in between these visits, and can issue you with repeat medication.

Complications of Addison's disease

One of the most serious potential consequences of Addison's disease is called an Addisonian crisis (or adrenal crisis). This happens when your supply of hormones becomes dangerously low for your body's needs.

This can happen if your body is under intense stress, for example, through surgery, a major illness or injury, or from fighting off a serious infection. Normally when your body is under stress, your adrenal glands produce more hormones to help your body cope. In Addison's disease, your adrenal glands can't cope with the added stress. If you haven't taken enough additional medicine, you may go into shock. This is a medical emergency and you will need urgent medical attention.

You may also have an Addisonian crisis if you are vomiting or have diarrhoea, preventing your medicine from being absorbed properly.

Warning signs of an Addisonian crisis are similar to untreated Addisonian disease and include feeling very sick, headache, dizziness, extreme weakness and confusion. If you have these signs, you should take extra medication and see your GP. If you are vomiting, you should use your emergency injection and seek urgent medical attention if vomiting persists.

Living with Addison's disease

With the right balance of medicine you can expect to have a normal lifespan and to lead a full and productive life if you have Addison's disease.

However, the medicines you take are essential for life and it can be a big responsibility to get used to. Here are some tips for managing your condition.

Never stop taking your medicine suddenly.
Always carry spare medicine.
Renew your repeat prescription in plenty of time, and always keep a month's spare supply.
Take double the medicine you would normally need when you go on holiday, as well as your emergency injection supplies. Carry medicine in your hand luggage when flying, together with a note from your GP to explain why you need to carry the medication and needles.
Wear an ID bracelet or necklace that states you have Addison's disease, so that in an emergency, medical staff will be aware of your condition.
Tell any doctor treating you about your condition.
Let your doctor know if you notice any new symptoms, which might mean you need to adjust your medicine.