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Addison's disease

Addison’s disease (also known as primary adrenal insufficiency or hypoadrenalism) is a rare disorder of the adrenal glands.

Addison’s disease (also known as primary adrenal insufficiency or hypoadrenalism) is a rare disorder of the adrenal glands.

The adrenal glands are two small glands that sit on top of the kidneys. They produce two essential hormones: cortisol and aldosterone.

In Addison's disease, the adrenal gland is damaged, and not enough cortisol and aldosterone are produced.

About 8,400 people in the UK have Addison's disease. It can affect people of any age, although it's most common between the ages of 30 and 50. It's also more common in women than men.

Early-stage symptoms of Addison’s disease are similar to other more common health conditions, such as depression or flu. You may experience:

  • fatigue (lack of energy or motivation)
  • muscle weakness
  • low mood
  • loss of appetite and unintentional weight loss
  • increased thirst

Over time, these problems may become more severe and you may experience further symptoms, such as dizziness, faintingcramps and exhaustion. You may also develop small areas of darkened skin, or darkened lips or gums.

Although these symptoms aren’t always caused by Addison’s disease, you should see your GP, so they can be investigated.

Read more about the symptoms of Addison’s disease and diagnosing Addison’s disease.

Why it happens

The condition is usually the result of a problem with the immune system, which causes it to attack the outer layer of the adrenal gland (the adrenal cortex), disrupting the production of steroid hormones aldosterone and cortisol. It's not clear why this happens, but it's responsible for 70-90% of cases in the UK.

Other potential causes include conditions that can damage the adrenal glands, such as tuberculosis (TB), although this is uncommon in the UK.

Read more about the causes of Addison’s disease.

Treating Addison's disease

Addison’s disease is treated with medication to replace the missing hormones. You'll need to take the medication for the rest of your life.

With treatment, symptoms of Addison's disease can largely be controlled. Most people with the condition live a normal lifespan and are able to live an active life, with few limitations.

However, many people with Addison's disease also find they must learn to manage bouts of fatigue and there may be associated health conditions, such as diabetes or an underactive thyroid.

People with Addison's disease must be constantly aware of the risk of a sudden worsening of symptoms, called an adrenal crisis. This can happen when the levels of cortisol in your body fall significantly.

An adrenal crisis is a medical emergency. If left untreated, it can be fatal. If you or someone you know has Addison’s disease and is experiencing severe symptoms, dial 999 for an ambulance.

Read more about treating Addison's disease.

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Addison's disease

To help diagnose Addison’s disease, your GP will first ask about your symptoms and review your medical history. They're also likely to ask if anyone in your family has an autoimmune disorder

To help diagnose Addison’s disease, your GP will first ask about your symptoms and review your medical history.

They're also likely to ask if anyone in your family has an autoimmune disorder (a condition caused by a problem with their immune system).

Your GP will examine your skin for any evidence of brownish discolouration (hyperpigmentation), particularly in certain areas, such as:

  • where skin creases on your palm
  • in your elbow crease
  • on any scars
  • your lips and gums

However, hyperpigmentation doesn't occur in all cases of Addison’s disease.

You'll also be tested for low blood pressure (hypotension) while you're lying down and again shortly after you stand up. This is to see whether you have postural or orthostatic hypotension (low blood pressure when you change position).

Blood tests

If Addison’s disease is suspected, blood tests will be carried out to measure the levels of sodium, potassium and cortisol in your body. A low sodium, high potassium or low cortisol level may indicate Addison’s disease.

You may need to see a hospital hormone specialist (endocrinologist) for your blood to be tested for the following:

  • a low level of the hormone aldosterone
  • a high level of adrenocorticotrophic hormone (ACTH)
  • a low level of glucose (sugar used for energy)
  • positive adrenal antibodies (antibodies designed to attack the adrenal gland)

Any of the above could be a sign of Addison’s disease.

Synacthen stimulation test

If cortisol in your blood is low, or your symptoms strongly suggest Addison’s disease, you'll need to have a synacthen stimulation test to confirm the diagnosis.

Your GP may refer you to an endocrinology unit (a unit that specialises in the study of hormones) for the test. How urgently you're referred depends on how severe your symptoms are.

Synacthen is a man-made (synthetic) copy of the adrenocorticotrophic hormone (ACTH). ACTH is naturally produced by the pituitary gland (a pea-sized gland below the brain) to encourage the adrenal glands to release the hormones cortisol and aldosterone.

When synacthen is administered, the adrenal glands should respond in the same way as they would to ACTH, and release cortisol and other steroid hormones into the blood.

A blood sample will be taken and tested for cortisol, before an injection of synacthen is given into your arm. After 30 and 60 minutes, a further blood sample will be taken for cortisol measurement.

If the ACTH level is high, but the cortisol and aldosterone levels are low, it's usually confirmation of Addison’s disease.

Thyroid function test

As well as a synacthen stimulation test, your thyroid gland may also be tested to see if it's working properly.

Your thyroid gland is found in your neck. It produces hormones that control your body's growth and metabolism.

People with Addison’s disease often have an underactive thyroid gland (hypothyroidism), where the thyroid gland does not produce enough hormones. By testing the levels of certain hormones in your blood, your endocrinologist (a specialist in hormone conditions) can determine whether you have hypothyroidism.

Scans

In some cases, your specialist may refer you for a scan of your adrenal glands  this could be a computerised tomography (CT) scan or a magnetic resonance imaging (MRI) scan.

Diagnosis during an adrenal crisis

If Addison’s disease is left untreated, it eventually leads to an adrenal crisis. This is where the symptoms of Addison’s disease appear quickly and severely.

During an adrenal crisis, there's not enough time to perform a synacthen stimulation test to confirm Addison’s disease.

If possible, blood will be taken and tested for any of the abnormalities listed above. While you're waiting for the results, treatment may be started with steroid injections, and fluids containing salt and glucose.

Read more about treating Addison’s disease.

Driving

If you're diagnosed with Addison's disease and have a bus, coach or lorry licence, it's your legal obligation to inform the Driver and Vehicle Licensing Agency (DVLA).

Read about driving and Addison's disease on the GOV.UK website.

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Addison's disease

If you have Addison’s disease, you'll need to take daily medication to replace the lost hormones. This should help you to live an active life, although many people find they still need to manage their fatigue.

If you have Addison’s disease, you'll need to take daily medication to replace the lost hormones. This should help you to live an active life, although many people find they still need to manage their fatigue.

In some cases, the underlying causes of Addison’s disease can be treated. For example, tuberculosis (TB) is treated with a course of antibiotics over a period of at least six months.

However, most cases are caused by a problem with the immune system that can't be cured.

Medication for Addison’s disease

Treatment usually involves corticosteroid (steroid) replacement therapy for life. Corticosteroid medication is used to replace the hormones cortisol and aldosterone that your body no longer produces. It's usually taken in tablet form two or three times a day.

In most cases, a medication called hydrocortisone is used to replace the cortisol. Other possible medicines are prednisolone or dexamethasone, though these are less commonly used. 

Aldosterone is replaced with a medication called fludrocortisone. Your GP may also ask you to add extra salt to your daily diet, although if you're taking enough fludrocortisone medicine this may not be necessary. Unlike most people, if you feel the urge to eat something salty, then you should eat it.

In general, the medications used for Addison's disease don't have side effects, unless your dose is too high. If you take a higher dose than necessary for a long time, there's a risk of problems such as weakened bones (osteoporosis), mood swings and difficulty sleeping (insomnia).

Living with Addison’s disease

Many people with Addison’s disease find that taking their medication enables them to continue with their normal diet and exercise routines. However, bouts of fatigue are also common, and it can take some time to learn how to manage these periods of low energy.

Some people find that needing to take regular doses of medication is restrictive and affects their daily life or emotional health. Missing a dose of medication, or taking it late, can also lead to exhaustion or insomnia.

Some people can develop associated health conditions, such as diabetes or an underactive thyroid, which require extra treatment and management.

You'll usually need to have appointments with an endocrinologist every 6-12 months, so they can review your progress and adjust your medication dose, if necessary. Your GP can provide support and repeat prescriptions in between these visits.

Failing to take your medication could lead to a serious condition called an adrenal crisis, so you must:

  • remember to collect your repeat prescriptions
  • keep spare medication as necessary  for example, in the car or at work, and always carry some spare medication with you
  • take your medication every day at the right time
  • pack extra medication if you are going away  usually double what you would normally need, plus your injection kit (see below)
  • carry your medication in your hand luggage if you are travelling by plane, with a note from your doctor explaining why it is necessary

You could also inform close friends or colleagues of your condition. Tell them about the signs of adrenal crisis and what they should do if you experience one.

Medical alert bracelets

It's also a good idea to wear a medical alert bracelet or necklace that informs people you have Addison’s disease.

After a serious accident, such as a car crash, a healthy person produces more cortisol. This helps you cope with the stressful situation and additional strain on your body that results from serious injury. As your body cannot produce cortisol, you'll need a hydrocortisone injection to replace it and prevent an adrenal crisis.

Wearing a medical alert bracelet will inform any medical staff treating you about your condition, and what medication you require.

Medical alert bracelets or necklaces are pieces of jewellery engraved with your medical condition and an emergency contact number. They are available from a number of retailers. Ask your GP if there's one they recommend, or go to the MedicAlert website. 

If you need to stay in hospital, the healthcare professionals responsible for your care will also need to know you need steroid replacement medication throughout your stay. It's important to note that this advice still applies if you're not supposed to eat or drink (nil by mouth) for any reason.

Adjusting your medication

At certain times, your medication may need to be adjusted to take into account any additional strain on your body. For example, you may need to increase the dosage of your medication if you experience any of the following:

  • an illness or infection  particularly if you have a high temperature of 37.5C (99.5F)
  • an accident, such as a car crash
  • an operation, dental or medical procedure (such as a tooth filling or endoscopy)
  • you're taking part in strenuous exercise not usually part of your daily life

This will help your body cope with the additional stress. Your endocrinologist will monitor your dosage and advise about any changes. Over time, as you get used to the condition and learn what can trigger your symptoms, you may learn how to adjust your medication yourself. However, always consult your GP or specialist if you're unsure.

Emergency treatment

You and a partner or family member may be trained to administer an injection of hydrocortisone in an emergency.

This could be necessary if you go into shock after an injury, or if you experience vomiting or diarrhoea and are unable to keep down oral medication. This may occur if you're pregnant and have morning sickness. Your endocrinologist will discuss with you when an injection might be necessary.

If you need to administer emergency hydrocortisone, always call your GP immediately afterwards. Check what out-of-hours services are available in your local area, in case the emergency is outside normal working hours.

You can also register yourself with your local ambulance service, so they have a record of your requirement for a steroid injection or tablets, if you need their assistance.

Treating adrenal crisis

Adrenal crisis, or Addisonian crisis, needs urgent medical attention. Dial 999 for an ambulance if you or someone you know are experiencing adrenal crisis.

Signs of an adrenal crisis include:

  • severe dehydration
  • pale, cold, clammy skin
  • sweating
  • rapid, shallow breathing
  • dizziness
  • severe vomiting and diarrhoea
  • severe muscle weakness
  • headache
  • severe drowsiness or loss of consciousness

In hospital, you'll be given lots of fluid through a vein in your arm to rehydrate you. This will contain a mixture of salts and sugars (sodium, glucose and dextrose) to replace those that your body is lacking. You'll also be injected with hydrocortisone to replace the missing cortisol hormone.

Any underlying causes of the adrenal crisis, such as an infection, will also be treated.

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