Addison’s Disease

Addison’s Disease

What is Addison’s disease?

Addison’s disease, which is also referred to as hypoadrenalism or primary adrenal insufficiency, is chronic and rare endocrine disorder that affects 40 to 60 people per million worldwide, although these numbers are higher in some developed countries. Addison’s disease affects the adrenal glands and results in these glands producing insufficient amounts of hormone, specifically cortisol and in some cases, aldosterone too.

Your body uses chemical messengers known as hormones to regulate a number of functions. Hormones are substances that affect the functioning and activity of other body parts, known as target site. Different hormones have different target sites. Your adrenal glands are located above the kidneys and produce aldosterone and cortisol, both of which are essential hormones for bodily health and function.

When the cortex (outer layer) of the adrenal gland responsible for the production of androgens, glucocorticoids (including cortisol) and mineralocorticoids (including aldosterone) is damaged, these hormones are not produced in sufficient amounts, and the result is Addison’s disease. One of the causes of the disease occurs as a result of the body’s immune system attacking the adrenal glands (specifically the adrenal cortices) as it mistakenly recognises these glands as a threat or foreign bodies and works to destroy them, making Addison’s an autoimmune disease.

**My Med Memo – The endocrine glands are a part of the endocrine system and secrete hormones directly into your blood, the adrenal glands form a part of this system.

The main function of the hormone cortisol, being the prominent stress hormone (released when under stress), is to aid the body in responding to stress.

Cortisol also helps the body to regulate the use of macronutrients, being carbohydrates, protein and fat and helps to maintain your cardiovascular function and blood pressure as well as control inflammation. It is, therefore, a vital hormone in the body and is needed for a variety of functions.

Aldosterone helps the kidneys to regulate water and salt intake, this aids in regulating blood volume and will assist in keeping blood pressure in check. If aldosterone levels are too low, then the kidneys will not be able to keep water and salt levels balanced, thus resulting in blood pressure dropping. We will look into these hormones further in the sections that follow.

A number of people with Addison’s disease are able to lead healthy and normal lives through treatment.

The following article unpacks and explains Addison’s disease as we cover everything from the symptoms to treatment and outlook.

Endocrine system explained

What is an (acute) adrenal crisis / acute adrenal insufficiency (AAI)?

It is vital that you do not confuse Addison’s disease with adrenal crisis, also known as adrenal insufficiency or Addisonian crisis. Addison’s disease was first described during the 19th century, by physician Thomas Addison as a long-term condition concerning adrenal insufficiency that gradually develops over a number of months and even years. The symptoms are typically those of weakness, weight loss, anorexia, fatigue and hyperpigmentation (darkening of skin).

On the other hand, an acute adrenal crisis may manifest through severe stress, abdominal pain, hypovolemic shock and vomiting. The condition, although rare, is potentially fatal.

Hypovolemic shock (also known as haemorrhagic shock) is an emergency situation wherein severe fluid or blood loss results in the heart being unable to pump a sufficient amount of blood to the body as a result of the adrenal glands not functioning and not producing enough cortisol.

An acute adrenal crisis can occur when your adrenal glands do not work properly in an extremely stressful situation.

If the adrenal glands are damaged in some way (as is the case with Addison’s disease) and do not produce the hormones needed for the body to function, this is able to trigger an adrenal crisis.

Those who suffer from Addison’s disease have an increased risk of experiencing an acute adrenal crisis, particularly if their condition has been left untreated. If you have Addison’s disease and experience a stressful situation such as injury, surgery or emotional stress, you may go into an Addisonian crisis.

What are the symptoms of Addison’s disease?

The symptoms of Addison’s disease will typically develop gradually over a number of months. The signs and symptoms of the condition may include:

  • Decreased appetite (anorexia)
  • Weight loss
  • Extreme fatigue
  • Hyperpigmentation (darkening of skin)
  • Craving salt
  • Hypotension (low blood pressure) – Possibly fainting
  • Hypoglycaemia (low blood sugar)
  • Joint and/or muscle pains
  • Depression
  • Irritability
  • Abdominal pain
  • Diarrhoea
  • Nausea
  • Vomiting
  • Hair loss (particularly body hair)
  • Sexual dysfunction (in women)

When to see a doctor

It is advised that you make an appointment to see your doctor is you have any of the above-listed signs that are associated with Addison’s disease. The most prominent signs include:

  • Hyperpigmentation
  • Weight loss that is unintentional
  • Severe fatigue
  • Gastrointestinal issues such as abdominal pain, vomiting and nausea
  • Fainting or lightheadedness
  • Joint or muscle pains
  • Salt cravings

Addisonian crisis (Acute adrenal failure / acute adrenal insufficiency)

Due to the symptoms of Addison’s disease progressing slowly, the signs often go unnoticed until a stressful event such as surgery, accident (i.e. a car accident) or other illness occurs, causing the symptoms to rapidly progress.

When this occurs, it is known as an acute adrenal crisis or Addisonian crisis (as previously mentioned). This is often the first sign of Addison’s disease in one in four patients with the condition and the first realisation that they have the disease.

An acute adrenal crisis can be a fatal condition and is therefore considered a medical emergency warranting immediate medical attention and care.

The symptoms and signs of an Addisonian crisis may include:

  • Dehydration from severe vomiting
  • Confusion leading to loss of consciousness or coma
  • Light-headedness or dizziness
  • Flank or abdominal pain
  • Headache
  • High fever
  • Fatigue accompanied by severe weakness
  • Low blood pressure
  • Loss of appetite
  • Rapid heart rate
  • Slow and sluggish movement
  • Excessive and unusual sweating on palms or face
  • Rapid breathing (increased respiratory rate)
  • Organ failure (including the kidneys if blood is not able to be restored quickly)
  • Hyponatremia (low sodium) or hyperkalaemia (high potassium)

What causes Addison’s disease?

What do hormones have to do with Addison’s disease?

When the adrenal glands are damaged in some way (Addison’s disease is the result of damage to the cortices (outer layers) of the adrenal glands), they will produce insufficient amounts of hormones. The main hormone concerned is cortisol, however, in some cases, there may also be lower levels of aldosterone present which can pose additional issues.

Your adrenal glands are located above the kidneys and form a part of your endocrine system, producing hormones that are chemical messengers for virtually all the organs and tissues in the body.

Adrenal glands

The adrenal glands consist of two different sections. The medulla, also known as the interior, is responsible for producing hormones like adrenaline. The cortex, which is the outer layer, produces hormones known as corticosteroids, these include the male sex hormones (androgens), as well as mineralocorticoids and glucocorticoids. Addison’s disease is a result of the adrenal cortex being damaged.

Some of the hormones produced by the cortex are essential to maintain life, specifically the mineralocorticoids and glucocorticoids. The aforementioned hormones are explained as follows:

  • Sex hormones (Dehydroepiandrosterone (DHEA)) – These include androgens, which are male sex hormones, as well as oestrogen, which is the female sex hormone. This group of hormones will affect sex drive and sexual development.
  • Mineralocorticoids – Aldosterone is included in this group of hormones which help to control the sodium and potassium levels in the body and regulate blood pressure.
  • Glucocorticoids – Cortisol is included in this group of hormones which aid in influencing the body to convert fuels from foods into energy, while maintaining and controlling the glucose (sugar) levels. These hormones also suppress the immune system inflammatory response and assist the body in responding to stress.

The causes of Addison’s disease

Addison’s disease has two classifications, these are:

  • Primary adrenal insufficiency
  • Secondary adrenal insufficiency

In order for treatment to be commenced, your doctor will first determine which type of the disease is causing your condition.

Primary adrenal insufficiency (primary chronic adrenocortical insufficiency)

Autoimmune disorders

As previously stated, Addison’s disease is the result of the cortex being damaged and producing insufficient amounts of hormones. Primal adrenal insufficiency is when the adrenal glands are so severely damaged they are no longer able to produce the vital hormones needed for bodily functions. The adrenal glands failure to produce the adrenocortical hormones (hormones produced by the cortex) is often the result of an autoimmune reaction as the immune system attacks the adrenal glands as it recognises the adrenal cortex as an invader, something which it must attack and eliminate. The reasons for the immune system doing this are still unknown. Autoimmune reactions cause 70% to 90% of the cases of Addison’s disease.

Your immune system is the body’s natural defence against disease and infection. When you are sick, your immune system will produce antibodies, these are a specialised kind of protein that will destroy the organisms responsible for the disease. However, should your immune system develop an issue, it may attack the healthy organs and tissues in your body. This is referred to as an autoimmune condition or disease.

When an autoimmune disorder has destroyed more than 90 percent of your adrenal cortex, the adrenal glands will no longer be able to make sufficient amounts of the hormones (steroids) aldosterone and cortisol. As soon as the levels start to decrease, you will begin to notice the symptoms and signs of Addison’s disease.

**My Med Memo – The main symptoms of Addison’s disease are:

  • Weakness
  • Anorexia (not wanting to eat or having no appetite)
  • Hyperpigmentation (darkening of the skin)
  • Fatigue
  • Weight loss

Genetics

There has been some research that shows some people to be more susceptible to autoimmune disorders developing due to certain genes that they possess.

It is not yet clear what role these genes have in the development of Addison’s disease, however, your risk of developing Addison’s may be increased should you or a close relative have an autoimmune disorder such as:

  • Type 1 diabetes – This is a chronic disorder that is a result of the glucose levels in the blood being high.
  • Hypothyroidism (underactive thyroid gland) – This is a condition where the thyroid gland does not produce enough of the thyroid hormones it is responsible for and this interferes with heart rate and the regulation of metabolism.
  • Vitiligo – This chronic condition results in white and pale patches developing on the skin.

Other causes of adrenal gland failure

  • TB (Tuberculosis) – This is the most common cause of Addison’s disease. TB is a severe condition that involves an infection (bacterial and not viral) that affects mostly the lungs, but it is also able to spread to a number of other parts of the body. Addison’s disease is a result of TB when the condition damages the adrenal glands.
  • Haemorrhage – This refers to heavy bleeding that affects the adrenal glands and can be associated with meningitis or other types of severe sepsis (bacterial infections of the blood).
  • Amyloidosis – This is a condition when amyloid, which is a protein that is produced by the bone marrow cells, accumulates in the adrenal glands and results in damage taking place.
  • Infections – These include conditions such as AIDS or fungal infections.
  • Cancer – Should cancer spread to your adrenal glands and cause damage to them, this can result in Addison’s disease.
  • ALD (Adrenoleukodystrophy) – This rare and chronic condition affects the adrenal glands as well as neurons of the brain.
  • Cushing’s syndrome – Certain medications and treatments required for Cushing’s syndrome may cause a number of symptoms, one of which is increased cortisol levels.
  • Adrenalectomy – This is the surgical removal of the adrenal glands. If you have a tumour on your glands then they may have to be removed.

What are the risk factors for cases of autoimmune disorders leading to Addison’s disease?

The below is a list of various autoimmune conditions in which patients with these may have a higher risk of Addison’s disease developing:

  • Inflammation (swelling) of the thyroid gland may often result in the reduced functioning of the thyroid, this is known as chronic thyroiditis. Initially, the inflammation will result in excess thyroid hormones being produced, this is known as hyperthyroidism. Over a period of time, the ongoing inflammation will prevent the thyroid from making adequate amounts of hormones, this is known as hypothyroidism. The thyroid and adrenal glands work together to produce a number of essential hormones, if the thyroid glands are damaged, then the functioning of the adrenal glands may take a subsequent knock. Another issue with the thyroid includes hypoparathyroidism. This is when the parathyroid glands do not make enough of their hormones.
  • An itchy rash accompanied by blisters and bumps known as dermatitis herpetiformis.
  • Hypopituitarism, this occurs when the pituitary gland does not make enough hormones. The pituitary gland stimulates the adrenal cortices to produce their hormones.
  • Myasthenia gravis (MG), which is a condition that results in the weakening of muscles that are controlled by the nerves.
  • Type 1 diabetes

Secondary adrenal insufficiency

It is also possible for adrenal insufficiency to occur when the pituitary gland is infected or diseased. Your pituitary gland produces a hormone known as ACTH (adrenocorticotropic hormone). This hormone stimulates the adrenal cortex to produce vital hormones.

If there is an inadequate amount of ACTH, this may lead to a deficiency in the hormones created by the adrenal glands, regardless of the adrenal glands still being at their optimal health. This condition is known as secondary adrenal insufficiency.

Secondary adrenal insufficiency occurs more commonly as a result of the sudden discontinued use of corticosteroids for certain chronic conditions, some of which include arthritis and asthma.   

Addisonian crisis

As previously mentioned, this condition is often the result of Addison’s disease that is left untreated and can be provoked by infection, illness or physical stress such as an accident or injury.

This condition is a combination of symptoms that indicate the presence of severe adrenal insufficiency as a result of the low levels of adrenal hormones, cortisol being the main one.

Some of these symptoms include:

  • Dehydration from severe vomiting
  • Confusion leading to loss of consciousness or coma
  • Light-headedness or dizziness
  • Flank or abdominal pain
  • Slow and sluggish movement
  • Excessive and unusual sweating on palms or face
  • Rapid breathing (respiratory rate)
  • Organ failure (including the kidneys if blood is not able to be restored quickly)
  • Hyponatremia (low sodium) or hyperkalaemia (high potassium)

What are the main adrenal hormones and why are they important?

Cortisol

Cortisol is the most vital glucocorticoid hormone (class of corticosteroids) that is produced by the adrenal glands. Practically every single tissue and organ makes use of this hormone. Cortisol also helps the body to respond to stress and maintains the blood and heart vessel functioning, in addition, it aids in controlling blood pressure. On top of all of this, cortisol will also regulate your metabolism with regard to how your body uses food, and slow down the inflammatory response of the immune system to protect you against viruses, bacteria and other harmful pathogens in your body.

 Cortisol explained

Ultimately, cortisol is governed by your pituitary and hypothalamus glands. The hypothalamus will send CRH (corticotropin-releasing hormone) to your pituitary gland. From here, the pituitary gland will send out ACTH (adrenocorticotropic hormone) to your adrenal glands which will stimulate the production of cortisol. When your cortisol levels have reached their required levels, a message will then be sent back to the pituitary and hypothalamus glands to decrease the production of hormones.

Your cortisol levels are at their highest early in the morning and after you have eaten, they are at their lowest in the evening and when you are sleeping. Low levels of cortisol results in fatigue, weakness, low blood pressure and a number of other health issues. If you are healthy, your body is able to balance and maintain its cortisol levels.

Aldosterone

Aldosterone belongs to the class of hormones known as mineralocorticoids, these hormones are a family of steroid hormones that aid in balancing water and salt intake by the body. Aldosterone is also made by the adrenal glands and aids in controlling blood pressure as well as potassium and sodium levels. If aldosterone levels are low, then your body may retain more potassium than is needed or lose a large amount of sodium.

Sodium that is found in the blood will affect blood pressure and blood volume. If there is too little present then this can result in a condition known as hyponatremia, this tends to cause symptoms of fatigue and confusion as well as muscle twitches.

If the levels of sodium are too high, this may cause a condition known as hyperkalaemia. This does not normally result in any symptoms, but some people with this condition may experience nausea, a weak pulse and an irregular heartbeat.

Dehydroepiandrosterone (DHEA)

DHEA is also produced by the adrenal glands. This kind of hormone makes the sex hormones for men and women, androgen being the male sex hormone and oestrogen being the female counterpart. The testes produced androgen in men and the ovaries produce oestrogen in women. When the adrenal glands are damaged and produce inadequate amounts of DHEA, then the sex hormones are affected.

How is Addison’s disease diagnosed?

It is likely that your doctor will first ask you about your medical history and then conduct a physical examination to look for any symptoms and signs. If it is suspected that you are at risk or have Addison’s disease, then your doctor will suggest that you undergo one or a few of the below tests:

  • Blood test – A blood test is conducted in order for your doctor to measure the levels of sodium, potassium, ACTH and cortisol in your blood. These results will give an indication as to whether your adrenal glands are working properly (to check for adrenal insufficiency) and if this issue is the cause of your symptoms. It is also possible for a blood test to measure antibodies that are associated with other autoimmune disorders and Addison’s disease.

**My Med Memo -  For a blood test to be conducted your doctor will take a sample of your blood, which is normally extracted from a vein in your arm using a needle, or via a simple finger prick. The blood test sample will then to be sent to a lab for testing where lab workers will analyse the blood. Your results will be available within 24 hours for a simple blood test, however, you may have to wait longer depending on what you are being tested for. Your doctor will phone you once he or she has obtained the results.

  • ACTH (adrenocorticotropic hormone) stimulation test / synacthen stimulation test – ACTH stimulates the production of cortisol. This test will determine how well the adrenal glands respond to ACTH in order for your doctor to measure the cortisol levels in your blood before injecting you with synthetic After you have been injected your cortisol levels will be measured again. If there is any damage present in the adrenal glands then the ACTH stimulation test will show that the output of cortisol levels as non-existent or limited as a result of the synthetic ACTH injection.
  • Insulin-induced hypoglycaemia test – Your doctor may suggest this kind of test if he or she thinks that pituitary disease is the possible cause of your adrenal insufficiency, this is known as secondary adrenal insufficiency. This kind of tests will involve a blood test to check for the presence of glucose (sugar) in the blood, as well as the cortisol levels at a number of different intervals after you have been injected with insulin. Insulin is a hormone that aids in regulating the glucose in your body. If you are healthy, your glucose levels will fall as the insulin comes into effect and your cortisol levels will increase.
  • Imaging tests – You may have to have a CT scan (computerized tomography) conducted. This form of imaging test uses a combination of X-ray images that are taken from a number of different angles (cross-sectional) which will allow for your doctor to get a view of your internal organs and detect any abnormalities. In the case of an Addison’s disease diagnosis, your doctor will conduct a CT scan of your abdominal area to look at the size of the adrenal glands and if they appear abnormal in any way. You may also have to undergo an MRI scan (magnetic resonance imaging). This test will scan your pituitary gland if your doctor suspects you have secondary adrenal insufficiency. An MRI scan uses radio waves to create images of your internal organs. Thyroid function test - This test will determine whether your thyroid is functioning correctly. The thyroid gland is located in the neck and produces a number of hormones that aid in controlling your metabolism and body growth. Those who suffer from Addison’s disease are often diagnosed with hypothyroidism, this is an underactive thyroid. Your hormone specialist, known as an endocrinologist, will test the levels of specific hormones using blood tests and determine whether or not your thyroid is the cause of any of your symptoms.

Diagnosis for an acute adrenal crisis

When Addison’s disease is not treated, it will eventually lead to an acute adrenal crisis, meaning that the symptoms of Addison’s disease occur rapidly, as well as severely.

During this crisis, there will not be any time for a synacthen stimulation test to be performed to confirm that Addison’s disease is the cause. If it is possible, then your doctor may take a sample of your blood in order for it to be tested for abnormalities. An ACTH stimulation test will only be performed when the crisis has been controlled through steroid injections, as well as the administration of fluids that contain glucose and salt.

How is Addison’s disease treated?

The treatment for Addison’s disease will involve substituting or replacing the hormones that are normally produced by your adrenal glands in order to address the deficiency.

Cortisol deficiency

Cortisol will typically be replaced orally through hydrocortisone tablets which are a synthetic form of glucocorticoid. This will be taken once or in some cases, twice daily.

If there is a deficiency of aldosterone, this hormone will be replaced through oral doses of a tablet form of mineralocorticoid named fludrocortisone acetate (Florinef), this will also be taken twice daily.

Aldosterone deficiency

If you are receiving treatment for the replacement of aldosterone, then you may be advised by your doctor to increase your intake of salt (sodium) as your body’s ability to reabsorb sodium will be affected as aldosterone stimulates the use of salt in the body. Should you have secondary adrenal insufficiency, then you will typically be able to maintain your production of aldosterone naturally and will not require replacement therapy.

The doses of the different kinds of medications will be adjusted according to your individual needs.

Acute adrenal crisis

During an acute adrenal crisis or Addisonian crisis, you may experience symptoms such as:

  • Low blood pressure (hypotension)
  • High potassium levels (hypoglycaemia)
  • Low blood sugar (glucose)

These symptoms, if left untreated, can be fatal and the standard form of treatment will be immediate (within the first hour of symptoms manifesting) and involve intravenous injections consisting of salt water (saline), hydrocortisone, as well as sugar (dextrose).

This form of treatment will typically result in the rapid improvement of symptoms. It is also possible to take these medications and fluids by mouth, and hydrocortisone will be slowly decreased until the medication reaches a maintenance dose.

If there is a deficiency of aldosterone, then the maintenance therapy will also include fludrocortisone acetate taken in oral doses.

**My Med Memo – Hydrocortisone is a form of steroid that reduces the body’s natural inflammatory reaction which results in inflammation, swelling and redness. Fludrocortisone is also a form of steroid and is used to prevent the release of certain substances in the body that result in inflammation and to control the amounts of fluids and sodium in the body. Fludrocortisone is also used to treat low levels of glucocorticoids which are needed in order for the body to function. It works by stimulating the kidneys to retain the sodium present in the body and also acts as a replacement for cortisol.

Both hydrocortisone and fludrocortisone are corticosteroids which mimic the effects of natural hormones found in the body produced by your adrenal glands. When corticosteroids are administered in doses that exceed the body’s natural levels, they are able to suppress inflammation.

Monitoring adrenal insufficiency stress

If you suffer from adrenal insufficiency, it is vital that you are monitored and pay attention to situations that cause an increase in your stress levels, particularly if you are pregnant, undergoing surgery or are suffering from an injury or illness.

Partaking in sports that are strenuous can also play a role in your stress levels, as can working late shifts at work. Stress affects your cortisol levels, because of this, if you are often in these sorts of situations then you may require additional treatment in order for the ‘stress’ dosages of cortisol and other corticosteroids to be recovered. This treatment can be administered orally or intravenously.

Surgery and adrenal insufficiency

If you suffer from chronic adrenal insufficiency, meaning you suffer from both a cortisol and aldosterone deficiency, and undergo general anaesthesia you will need to be treated intravenously with saline and hydrocortisone. These injections will begin the evening before your surgery and will continue until you are awake and able to take these orally. The dosage will be adjusted until the maintenance level is reached before your surgery.

Pregnancy and adrenal insufficiency

If you are pregnant and have primal adrenal insufficiency (when the adrenal glands are so severely damaged they are no longer able to produce the vital hormones needed for bodily functions), you will be treated with the standard method of hormone replacement therapy. If you experience nausea followed by vomiting in the early stages of your pregnancy, this may interfere with the oral hormone medication and therefore infections may be required.

During the delivery of your baby, the treatment will be similar to that of patients undergoing surgery. After the delivery, the dosage will be gradually decreased and the maintenance dosage of fludrocortisone and hydrocortisone will be reached through taking the medications orally at about ten days after the birth of your child.

**My Med Memo – A maintenance dose is a dosage of hormones that will replace the deficient hormones at the same rate that they are lost or not produced by the adrenal glands (dosage is equal to the rate of elimination). A maintenance dose is normally a fixed and small dose and it may take time to reach this point.

Diet and nutrition and adrenal insufficiency

If you have Addison’s disease and also suffer from decreased levels of aldosterone, then it may be beneficial to maintain a diet that contains a high amount of sodium (salt). Your doctor or dietician may be able to help you with guidelines and specific suggestions as to how much sodium is required.

Some foods that are naturally high in sodium include:

  • Beetroot
  • Celery
  • Carrots
  • Meat (chicken, beef, lamb, pork, etc.)
  • Chard
  • Spinach

The side effect of treatment with steroids is often osteoporosis, this is a disorder wherein your bones lose some of their density and can break easily. This condition can be prevented through the use of calcium supplements and consumption of calcium-rich foods, as well as the inclusion of vitamin D, this will aid in maintaining sound bone health.

Living with adrenal insufficiency (Addison’s disease)

A number of people who suffer from Addison’s disease may find that through taking their medications, they are able to continue to lead normal lives and maintain normal diets, as well as exercise programmes. Bear in mind, when taking medication regularly, you may still experience episodes of fatigue, as this is a common symptom of Addison’s disease. Therefore, it may take some time to adjust to these episodes and learn how to manage them.

You may also discover that having to stick to the dosage of medications is restrictive to your routine and takes a toll on your emotional health and daily life. Taking your medication too late or missing can also result in insomnia or exhaustion.

Should you develop another condition such as an underactive thyroid (hypothyroidism) or diabetes, or have an existing condition, then you will require additional management of these disorders and potentially need more extensive treatment.

You will normally have to see your endocrinologist (hormone specialist) every six to 12 months, this will allow for the endocrinologist to review your symptoms and progress and change the dosage of medication if need be. It is also possible for your family doctor (general practitioner) to provide further support, as well as repeat prescriptions between the visits with your endocrinologist.

If you fail to regularly take your treatment, this may lead to an acute adrenal crisis, it is therefore vital that you:

  • Collect your prescriptions (monthly or weekly)
  • Take your medications regularly at the same time every day
  • Pack additional medication if you are going away
  • Carry the medication in hand luggage if you are flying in case of an emergency (your doctor will be able to provide you with a note to explain why this is necessary and allow for you to do so)
  • Keep any spare medication where necessary
  • Inform colleagues, family and close friends of your condition and explain what the symptoms of an adrenal crisis are and what they will need to do should you have one.
  • Wear your medical bracelet as this will inform medical staff who are treating you in a medical emergency of your condition. This is a piece of jewellery that is engraved with the conditions you may have and other vital information, as well as a contact number for emergencies.

Adjusting medication

You may be required to adjust your medication in certain situations, it is best that you speak to your doctor or endocrinologist before doing so, however, he or she is likely to have already prepared you on how to do this.

If you experience any of the below then you may have to adjust your medication:

  • You are in a car crash or an accident of some kind
  • You have to have surgery performed or a medical procedure of some kind such as an endoscopy or a dental filling
  • You are partaking in strenuous physical exercise that is not normally a part of your exercise regime
  • You have an infection or illness where you develop a fever

Adjusting your medication will aid in assisting your body to cope with any of the additional stress caused by various scenarios. Your endocrinologist will advise you on how to adjust your medication specifically. After taking medication over a longer period of time, you will eventually adjust to it and be able to identify what triggers any symptoms of Addison’s disease.

Bear in mind that the treatment for Addison’s disease is lifelong.

What to do during an adrenal crisis

As previously mentioned, it is advised that you train your partner, loved ones or close friend on how to handle an adrenal crisis. Your endocrinologist may suggest that you both see him or her for an appointment on training.

During an adrenal crisis, it is likely that you will be in shock, therefore it is vital that your partner knows what to do in this emergency situation. If you are in shock or experience diarrhoea or vomiting, then you may need to have an injection (an endocrinologist will give you an emergency injection kit).

It is likely that hydrocortisone will need to be administered immediately, in this case, your endocrinologist should be called immediately afterwards.

Your partner should call the local emergency number when you are suffering from an adrenal crisis. If able to do so, he or she will need to inject you with hydrocortisone. The injection can be administered in the upper leg muscle or buttock.

The signs and symptoms of an acute adrenal crisis are:

  • Pale, clammy and cold skin
  • Severe dehydration
  • Shallow and rapid breathing
  • Sweating
  • Dizziness
  • Loss of consciousness
  • Severe diarrhoea and vomiting
  • Severe muscle weakening
  • Severe drowsiness
  • Headache

Once you are in hospital, a medical team will ensure you are given fluid intravenously (via a vein in your arm) in order for you to be rehydrated. This fluid will contain a combination of sugars (dextrose and glucose) and salts (sodium), in order to replace what you are lacking. A nurse or doctor will also inject you with hydrocortisone if this has not already been done on the scene of the initial adrenal crisis.

If there are any underlying injuries and infections that caused the acute adrenal crisis, these will then be treated.

What Is Expected in the Long Term?

If you suffer from Addison’s disease, your treatment will be lifelong and medication will need to be administered daily. This hormone therapy will allow for your symptoms to be managed. In following your medication and treatment according to your doctor’s instructions, you will find that you are able to live a normal and productive life.

It is advised that you ALWAYS take your medications as directed. If not, this could land you in a dangerous situation such as an adrenal crisis. It is likely that your treatment will be re-evaluated and adjusted as you learn to manage and deal with your condition. Because of this, it is vital that you see your doctor on a regular basis.

 Addison's disease explained

FAQ about Addison’s disease

Is Addison's disease fatal?

If you suffer from adrenal insufficiency, also known as Addison’s disease, then you may experience an adrenal crisis, this is a life-threatening situation where your adrenal glands are damaged from injury, infection or Addison’s disease. As a result of this damage, the adrenal glands are unable to produce vital hormones needed to function, this results in a number of symptoms as cortisol levels in the body drop significantly. If medical care is not immediately sought, this condition can be fatal.

What causes hyperpigmentation in Addison's disease?

Hyperpigmentation is commonly a harmless condition that causes patches of your skin to darken. This is a result of increased levels of the adrenocorticotropic hormone (ACTH) binding to a receptor on the skin’s surface known as melanocortin 1, which results in certain areas of the skin darkening in colour. This is commonly seen in autoimmune Addison’s disease.

Is there a cure for Addison's disease?

Due to Addison’s disease being a result of natural hormone levels being lowered as a result of decreased functioning of the adrenal glands, treatment involves the replacement of these hormones. This can control the symptoms, however, medication is lifelong and needs to be taken daily.

Why is ACTH increased in Addison's disease?

When suffering from primary adrenal insufficiency, your body will respond by producing high levels of adrenocorticotropic hormone (ACTH), but do not produce enough cortisol. The pituitary gland will send out ACTH (adrenocorticotropic hormone) to your adrenal glands which will stimulate the production of cortisol.

If you suffer from secondary adrenal insufficiency, then your body will not produce ACTH or will have a delayed response in producing the hormone as the pituitary gland is damaged or infected.

What kind of specialist treats Addison’s disease?

An endocrinologist is someone who specialises in conditions affecting the endocrine system, this is the system in your body that controls hormones and thus this is the best specialist to treat Addison’s disease.

Can stress cause an adrenal crisis?

Your adrenal glands produce cortisol if these glands are damaged or infected in some way their level of production of vital hormones such as cortisol will be impacted.

When the levels of cortisol are decreased, this can result in fatigue, weakness and hypotension (low blood pressure). If you have Addison’s disease or your adrenal glands are damaged (i.e. in a car accident) or infected as a result of severe stress, this can result in an adrenal crisis.

Some doctors believe that living a stressful life can result in your adrenal glands not functioning correctly, this is known as adrenal fatigue which causes symptoms of fatigue and weakness.  This is not a proven medical condition. 

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