Steroid replacement for adrenal insufficiency

Who is the leaflet for?

This information leaflet is intended to provide support with the management of steroid replacement for people diagnosed with adrenal insufficiency and taking prednisolone or hydrocortisone as steroid replacement.

What is its aim?

To empower our steroid-dependent patients to manage their medications independently, allowing patients to remain in control of their health and well-being.

What is adrenal insufficiency?

Adrenal insufficiency is a condition in which the adrenal glands (small hormone producing glands sitting above the kidneys) stop producing enough cortisol. Cortisol is a naturally occurring steroid hormone which regulates blood pressure, blood sugar and muscle strength. It is a hormone that is essential for health; severe deficiency can cause illness or even death.

The amount of cortisol produced by the adrenal glands is regulated by a hormone called adrenocorticotropic hormone (ACTH) which is produced by the pituitary gland (a small gland producing many hormones which sits under the brain). The adrenal glands also produce another hormone called mineralocorticoid. The production of mineralocorticoid is not regulated by ACTH.

Adrenal insufficiency can occur due to many reasons including:

• Addison’s Disease - Addison’s disease is a rare endocrine condition in which the adrenal glands cease to function, so that your body no longer produces enough essential hormones, known as steroid hormones.
• Hypopituitarism - This occurs when the pituitary gland does not produce enough of the hormone ACTH, in turn resulting in too little cortisol being produced by the adrenal glands. The adrenal glands produce enough mineralocorticoid in this condition.
• Hypothalamic Pituitary Adrenal Axis Suppression (HPA axis suppression) - Long term use of steroid medication such as prednisolone or dexamethasone can lead to suppression of the body's natural ACTH production, causing the adrenal glands to stop producing enough cortisol. There is sufficient production of mineralocorticoid in this condition. It is possible for the ACTH production from the pituitary to recover over time.
• Congenital adrenal hyperplasia - Congenital adrenal hyperplasia is an inherited condition caused by mutations in genes that code for enzymes involved in making steroid hormones in the adrenal glands. The most common enzyme defect, 21-hydroxylase deficiency, leads to excess amounts of male hormones being produced by the adrenal glands.

How is adrenal insufficiency due to HPA axis suppression treated?

Fortunately, cortisol can be replaced easily with daily steroid tablets. These steroid tablets are essential for your health and must never be stopped suddenly.

Your doctor will advise on your correct doses and also when or if, to reduce the doses. Steroid medication is called prednisolone or hydrocortisone.

• Prednisolone: This is a long acting steroid medication which is usually taken once a day in the morning on waking but occasionally a second dose will be required during the day.
• Hydrocortisone: This is a short acting steroid medication. It is usually given in two or three daily doses with the first dose always on waking. It is the medicine of choice used when attempting to 'wean down' steroid replacement if adrenal recovery is anticipated in HPA axis suppression. However, this should never be attempted without discussion with your endocrine doctor.

What do I need to know about managing my medications?

• Take your tablets every day at the right time of day. They are essential. Do not skip doses.
• You can take your steroid replacement medication on an empty stomach, unless you have pre-existing digestive problems.
• Always carry spare medication with you.
• Order your repeat prescription in plenty of time (ideally maintaining a month’s reserve supply) to ensure you do not run out of your essential medication.
• Take an extra supply of medication (i.e. double what you need) with you on holiday, plus your steroid injection kit.
• Carry your medication and a steroid injection kit in your hand luggage when travelling by plane, along with a doctor’s note explaining why you need to carry needles and syringes.
• If you are unwell, make sure that the person treating you knows you are at risk of adrenal crisis and show them your NHS Steroid Emergency Card.

What could go wrong if I don’t take enough medication?

In cases of vomiting or shock, people with adrenal insufficiency can experience a sudden drop in blood pressure. If you do not take sufficient extra medication, you may experience an adrenal crisis which is a medical emergency.

What is an adrenal crisis?

An adrenal crisis can happen if you suddenly stop taking steroid tablets, or if you are on steroid treatment and get another illness, such as an infection as there is insufficient circulating cortisol in the body. This can be life-threatening if not treated

Signs of crisis can include severe dizziness with serious vomiting and/or diarrhoea. People having an adrenal crisis may have sudden confusion, tiredness, headache, extreme weakness,chills or fever.

Are there any special precautions I will need to take?

Cortisol hormone is essential for life. Ordinarily, if we are sick or injured, our bodies immediately produce more cortisol to cope with this stress. Since your adrenal glands cannot do this reliably, you will need to take extra steroid medication. The general guidelines for extra steroid cover are:

• Illness with fever: If your temperature is raised, double your steroid dose for the duration of the illness. If you need to double the dose for more than seven days, you should consult your doctor. Once you feel better, you can gradually decrease the prednisolone or hydrocortisone back to your normal dose over a couple of days. However, if you are already on prednisolone 15 mg or more there is no need to take additional steroid medication.
• Vomiting or diarrhoea: If you vomit once, take an extra 5 mg of prednisolone OR 20 mg of hydrocortisone by mouth. If vomiting persists after you have taken the extra steroid dose, you must seek urgent medical attention: go to the Emergency Department, or call an ambulance via 999. Take your NHS Steroid Emergency Card with you and ensure that the team looking after you know that you are on steroid medication and that you are at risk of adrenal crisis and may need a steroid injection.
• Extremely unwell & serious injuries: Take an extra 20 mg of prednisolone OR 50 mg of hydrocortisone orally and seek medical advice.
• Minor Dental Surgery or minor surgical procedures - Take 5 mg of prednisolone OR 20 mg of hydrocortisone one hour prior to the procedure and take a double dose for 24 hours after the procedure, then return to your normal dose.
• Major Dental Surgery - You may need 100 mg of IM (intramuscular) or IV hydrocortisone before major dental work anaesthesia – discuss in advance with your dentist. Take a double dose for 24 hours after any dental procedure, then return to your normal dose.
• Surgery and invasive procedures - Ensure your surgical team is aware of your need for extra medication and that they have checked the surgical guidelines for the correct level of steroid cover. If in doubt, please ask your doctor for a letter for your surgeons, advising on the correct steroid dosages for you around the time of surgery. Steroid cover advice: 100 mg of hydrocortisone by IV or IM injection at the start of surgery (induction of anaesthesia) followed by 50 mg of hydrocortisone IV or IM every 6 hours. Alternatively some centres may give 100 mg hydrocortisone at the start of surgery followed by a continuous IV infusion of 200 mg hydrocortisone over 24 hours. Double usual oral dose when eating and drinking and reduce to usual dose over the next 1-2 weeks as you recover.
• Pregnancy – Please contact the endocrine team if you become pregnant to discuss any changes to your steroid replacement that may be required in pregnancy and to arrange for regular follow up during pregnancy. At onset of labour or start of a caesarean section, we suggest giving 100 mg IV (intravenous) hydrocortisone at the start of labour and then 50 mg every 6 hours until eating and drinking reliably, with double the usual oral dose to be continued for 48 hours after delivery. Alternatively, some centres may use a continuous IV infusion of 200mg hydrocortisone over 24 hours.

What about sport and exercise?

Gentle exercise such as recreational swimming or walking does not usually need extra medication. Challenging physical exercise such as competitive sport, may need extra medication. You may need to double your normal dose during the competition. For any sports with a risk of physical injury, you must ensure that a teammate has been trained to administer an emergency injection if needed.

You are entitled to receive your medication free of prescription charges. Your GP must certify your entitlement to free prescriptions on a Medical Exemption card.

References/ Sources of evidence

British Thoracic Society. (2021, July). NHS Steroid Emergency Card and National Patient Safety Alert. Retrieved from British Thoracic Society.

Society for Endocrinology . (2020). Steroid Sick Day Rules. Retrieved from Society for Endocrinology.

Society for Endocrinology . (2021). Society for Endocrinology. Retrieved from Society for Endocrinology.

You and your hormones. (2020). Congenital adrenal hyperplasia (opens in a new tab). Retrieved from You and your hormones.