Adrenal insufficiency

Adrenal insufficiency is an endocrine, or hormonal, disorder that occurs when your adrenal glands don't produce enough of certain hormones. Your adrenal glands are located just above your kidneys. They produce important hormones including cortisol, aldosterone, adrenaline and androgens. 

Adrenal insufficiency can be primary (about 1 in 4 cases) or secondary (about 3 in 4 cases):

• Primary adrenal insufficiency is also known as Addison's disease, named after the doctor who described this condition. Addison’s disease is rare. It affects 110–144 of every 1 million people in developed countries. It occurs when your adrenal glands are damaged (from infection, injury or autoimmune problems) and can't produce enough of the adrenal hormones cortisol and sometimes aldosterone.
• Secondary adrenal insufficiency is more common and occurs when your pituitary gland (a pea-sized gland at the base of your brain) doesn't produce enough adrenocorticotropin (ACTH). This hormone stimulates your adrenal glands to produce the hormone cortisol. Without enough ACTH your adrenal glands can shrink over time. 

The symptoms of adrenal insufficiency are non-specific. They often come on gradually, first appearing during times of stress and then more often over weeks to months. Such symptoms include:

• abdominal pain
• fatigue
• muscle weakness
  
• low blood pressure that drops further you stand up, causing dizziness or fainting
• diarrhoea (runny poos) or constipation (hard to poo)
• nausea (feeling sick) and/or vomiting (being sick)
• weight loss
• joint and muscle aches
• headache
• hypoglycaemia (low blood sugar)
• sweating
• hyperpigmentation (only in Addison’s disease) – dark patches or skin discolouration, especially in the folds of your skin or around areas such as your nipples, lips and rectum (bottom)
  
• dehydration (only in Addison’s disease)
  
• salt craving (only in Addison’s disease)
• irritability and depression
• irregular or absent menstrual periods
• in women, loss of interest in sex.

Addison's disease (primary adrenal insufficiency)

Addison’s disease is usually caused by an autoimmune disorder (in 8 out of 10 cases). Autoimmune disorders occur when your immune system attacks your own cells and organs. In autoimmune Addison’s, your immune system gradually destroys the outer layer of your adrenal glands, known as the adrenal cortex. This is most common in middle-aged women.

Other causes include:

• infections – viral (cytomegalovirus), bacterial (tuberculosis, Neisseria meningitidis) and fungal 
• tumours
• medicines
• genetic defects
• amyloidosis.

Secondary adrenal insufficiency

Anything that affects your pituitary gland can result in fewer pituitary hormones reaching your adrenal glands to tell them to produce hormones. This can include pituitary tumours, surgical removal or damage to your pituitary gland, damage to the blood supply and certain medicines.

A temporary form of secondary adrenal insufficiency can occur when someone has been on corticosteroids, such as Prednisone, for weeks or months to treat inflammatory illnesses such as rheumatoid arthritis, asthma and ulcerative colitis.

Taking corticosteroids for a long time means your adrenal glands produce fewer hormones. When the corticosteroid medicines are stopped, your adrenal glands may be slow to restart. Prescription corticosteroid doses are reduced gradually over a period of weeks or months to prevent this happening.

It can be hard to diagnose adrenal insufficiency, especially when the disease is still in its early stages. Your doctor may suspect it after examining your symptoms and medical history.

Diagnosis is confirmed through urine (pee) and blood tests. The results of these tests to show whether your levels of cortisol are too low. Next, your doctor will want to work out whether you have primary or secondary adrenal insufficiency and what the underlying cause is. You may need a scan of your pituitary and adrenal glands. 

Read more about lab tests for adrenal insufficiency and Addison's disease(external link)(external link) (Lab Tests Online, Australia)

Adrenal insufficiency is treated using medicines that aim to replace the missing hormones. Depending on the cause, you may need to take these medicines for the rest of your life. With treatment, it is possible to largely control the symptoms of the disorder and live a full and active life. 

Taking too much or too little adrenal hormone replacement can lead to problems. The most dangerous problem an adrenal crisis (or an Addisonian crisis).

An adrenal crisis can occur when your body is not able to produce enough stress hormones to cope with a stressful event such as infection, injury or surgery. It can cause death if not treated.

Symptoms of an adrenal crisis include:

• sudden, severe pain in your lower back, abdomen or head
• vomiting and diarrhoea
• dehydration
• low blood pressure, dizziness
• fever
• confusion or loss of consciousness.

If not treated, an adrenal crisis can cause death. 

If you have been diagnosed with adrenal insufficiency, you will be given emergency medicine to take during stressful events. Intravenous corticosteroids are given during and after surgery. 

When to seek medical help

Contact your healthcare provider if: 

• your ankles start swelling
• you can’t keep your medication down as a result of vomiting
• you notice new symptoms
• your weight changes without reason
• you have stress like infection, trauma, injury or dehydration (see your doctor straight away).

The most important thing you can do is to make sure you understand what an adrenal crisis is and know what to do if you have one.

An adrenal crisis can happen when cortisol levels in your body fall significantly, resulting in sudden, severe symptoms. It is normally treated using adrenal hormones and you will be given corticosteroid tablets to have on hand at all times.

You also need to carry a corticosteroid injection with you at all times and ensure that family/whānau and workmates know how and when to give you this, if you are too sick to give it to yourself. 

You should also carry a card in your wallet and a MedicAlert bracelet or necklace stating you have adrenal insufficiency.

Read more about managing an adrenal crisis(external link)(external link) Adrenal Insufficiency NZ

Support

Adrenal Insufficiency NZ(external link)(external link) Information and support for people living with adrenal insufficiency