Managing adrenal insufficiency and steroid replacement

This resource explains about adrenal insufficiency (cortisol deficiency) and how it is treated. It also contains information about how to manage steroid replacement with illnesses, accidents and other stressful events in children.

Steroid replacement is vital for your child to grow and develop normally. If the correct dose is used, there are no side effects. This is because the treatment is just replacing what the body should normally produce.

Normally, people produce more cortisol than usual in response to illness, injury and surgery. It is very important that children and young people receiving steroid replacement therapy increase their normal dose of steroids to copy this response.

What are the adrenal glands?

The adrenal glands are on top of the kidneys. They produce important hormones (chemical messages) to control how the body works. One of these hormones is called cortisol. Cortisol is the body’s natural steroid and has 3 main functions:

• helping to control the blood sugar level
• helping the body deal with physical stress
• helping to control blood pressure and blood circulation

If a someone cannot make cortisol (cortisol deficiency), they will need to take tablets to replace it. The most common medication used is hydrocortisone, but other forms may be prescribed.

Cortisol deficiency is easily managed with replacement hydrocortisone tablets given a few times a day.

However, if someone with cortisol deficiency becomes unwell or their body is stressed, they are unable to make more cortisol in their system to help the body cope and this could be life threatening.

If this happens, the amount of hydrocortisone given needs to be increased. This is done by either:

• increasing the dose of hydrocortisone taken as tablets
• giving an injection into your child’s thigh

This medication is vital and should not be missed. Please make sure:

• that your child wears a steroid medicalert bracelet (see below) or equivalent. This lets others know in an emergency that your child is on steroids. It can be life saving in some cases
• that you and your child know the dose of hydrocortisone and that it is given 3 times per day (refer to you treatment plan at the bottom of this resource)
• that you always have a spare supply of hydrocortisone available. Do not run out.
• As your child gets older they will need a bigger dose of hydrocortisone. Your doctor will tell you about this.
• If you are uncertain about any part of your child’s care and treatment, please ask your doctor or specialist nurse. It is important that you understand your child’s condition and why they are taking steroids.

Medical ID bracelet from The ID Band Company, with steel clasp and identification engraving.	Medical ID bracelet from Amazon, with Velcro fastening.
Available from The ID Band Company here.	Available from Amazon here.

Other medical bracelets are available online.

When do I increase my child’s hydrocortisone?

Your child is likely to need an increase in their dose of hydrocortisone when they are:

• unwell
• have an accident
• have an injury
• need medical investigation

An extra dose will not do any harm, so if in doubt it is better to give extra hydrocortisone and then contact the specialist endocrine team for advice about what to do next.

What if they need a hydrocortisone injection?

In the event that your child needs a hydrocortisone injection then they should be taken to the nearest emergency department (A&E) immediately by paramedic ambulance for further assessment.

If your child is less alert than usual you should call a paramedic ambulance. The paramedics do not carry hydrocortisone for injection so if you haven’t given the injection, please make sure you give the emergency kit to the paramedic.

Your child will be taken in to hospital for 12 hours. This is because the hydrocortisone injection takes about 8 hours to wear off and we need to make sure that it is safe for your child to go home.

What if my child starts being sick?

If your child is only sick 1 time, an hour or longer after taking the oral hydrocortisone, you will not need to repeat it. However, you should think about why they have been sick. It may be a one-off, or a warning of underlying illness.

However, if your child is being sick within 1 hour of taking their hydrocortisone tablet then give a 2nd dose of hydrocortisone.

If your child is sick again then give them 3rd the dose of oral hydrocortisone. Seek medical advice early to make sure your child does not become more unwell.

If they are being sick repeatedly or in large amounts, give them a hydrocortisone injection and bring your child to hospital in a paramedic ambulance. Your child may need to stay in hospital to receive intravenous (IV drip) hydrocortisone until they have stopped being sick.

What is my child has diarrhoea?

If your child has diarrhoea, there is a risk that the oral hydrocortisone will pass through their too quickly and will not be absorbed. You will need to double or treble the usual dose of oral hydrocortisone until the diarrhoea stops.

If your child has severe diarrhoea and is also being sick, you should give them a hydorcortisone injection into their thigh and call an ambulance to go to hospital immediately.

What about infection and illness?

Coughs and colds

If your child has a cough and cold without a temperature then there is no need to increase their dose of steroids. However, if you are uncertain about what to do, ask your doctor.

High temperature and antibiotics

Normal body temperature is 36 degrees Celsius to 37.5 degrees Celsius.

If your child has a high temperature because of an infection or if they are on antibiotics to treat infection, then either double or treble their dose of steroids until 24 hours after the infection has cleared.

If they are moderately unwell (see table) then double their dose of steroids.

If they are very unwell (see table) then treble their dose of steroids.

If your child is on antibiotics for longer than 10 days, ask your doctor about how long to increase your child’s dose of steroids. Also give your child a double morning dose of hydrocortisone at 4am if they are very unwell. Always ask your doctor or specialist nurse if you are not sure.

Chicken Pox

Children on hydrocortisone replacement should treble their dose of steroids and also have an oral aciclovir (anti-viral medication). You can get Aciclovir from your GP.

What about if they need procedures?

Dental work

If your child is having a dental check up or teeth cleaning they do not need to increase their dose of hydrocortisone.

If your child needs a dental procedure that does not need a general anaesthetic, then double their dose of hydrocortisone for 24 hours after the procedure.

If they need a general anaesthetic, please see the information on general anaesthetics.

Immunisations

Children taking hydrocortisone should have all recommended childhood immunisations. If they are unwell or develop a temperature after their immunisation, then treble their dose of steroid until they improve.

General anaesthetics

If your child needs a general anaesthetic then they will need intravenous (IV) hydrocortisone which will be given before the operation or procedure. This includes MRI scans.

Please make sure that the doctors and nursing staff know that your child is on steroid replacement. If their operation is in the morning your child should have their normal dose of hydrocortisone the night before their operation.

If their operation is in the afternoon, your child should have their normal morning dose of hydrocortisone. The specialist team will let you know what to do with your child’s dose of steroids following their operation.

What about accidents and injuries?

Minor injuries

If your child has a minor injury such as a bump, cut or graze and they can recover straight away and resume activities, then you do not need to increase their dose of hydrocortisone.

Major injuries

If your child has a significant injury such as:

• fracture
• head injury with altered level of consciousness or being sick
• serious burn

Unconscious

If your child becomes unconscious, it is vital that you give them an injection of hydrocortisone into their thigh immediately and then call a paramedic ambulance to bring them to hospital.

Special considerations

If your child is taking desmopressin (DDAVP) and needs to increase their dose of hydrocortisone because they are unwell, do not give more desmopressin until you have spoken to the specialist team.

Let your child drink water or other soft drinks freely.

When your child is unwell, giving these medications together may mean their sodium level drops. Because of this, they will need to have their sodium level checked in hospital.

Giving the hydrocortisone injection

The correct dose of hydrocortisone should be given by injection in emergencies. Remember that no harm can come from giving an unnecessary hydrocortisone injection. The dose that should be given is:

• 25mg for babies
• 50mg for children aged 1 to 5 years old
• 100mg for older children

Use a syringe to prepare the hydrocortisone solution. You should give your child their injection given in the middle of the outer side of their thigh. This is because there are no important structures that can be damaged. The injection will go into the muscle.

Your child’s condition should improve quickly, but you should still take them to hospital if you have given the hydrocortisone injection.

Please use the pictures below as a guide to giving the hydrocortisone injection:

Step 1: Break open the ampoule

If at all possible wash your hands before starting.

Find the coloured dot on the side of the glass ampoule. Apply pressure to the dot and bend to break the ampoule. Use a tissue when breaking the ampoule to stop the glass from cutting your finger.

Step 2: Draw up the hydrocortisone

Open the syringe package. Make sure that the needle is firmly attached to the syringe. If the needle touches any surface including your hand it is no longer sterile and should be changed.

Step 3: Flick air out of the syringe

It is very important to remove bubbles in the syringe. To do this hold the syringe at 90 degrees with the needle pointing upwards and tap the side of the syringe until the air bubbles have moved to the top. The air can then be removed by pushing up the syringe plunger until the air has passed through the neck of the syringe.

Step 4: Give the injection

• Give the injection in the middle of the outer side of the thigh
• Stretch the skin between your thumb and forefinger
• Hold the syringe at a 90 degree angle (like a pencil)
• It is important to hold the syringe straight to make sure that you inject into the muscle
• Push the needle into the skin with a firm quick action and inject the hydrocortisone
• Make sure to praise and reassure your child throughout the whole process. Apart from helping them to not feel scared, it will also help to prevent tensing of the muscle and make the injection easier

Step 5: Call for an ambulance

Make sure that you ring for an ambulance or go straight to the nearest Accident & Emergency Department.

My Cortisol app

Great Ormond Street Hospital have recently developed a free training app for giving an emergency injection of hydrocortisone. The app is a real time video showing the technique.

There is a silent video, and also a tab with the step by step photographs of the procedure on it.

Parents and carers can input doses, contact numbers and what to do when an A&E doctor takes over the care of your child.

The My Cortisol app is available for both Apple (Apple Store) and Android (Google Play) devices.

What about traveling?

Europe

A time difference of 1 to 3 hours should not cause any problems. Do not change the time you give your child their hydrocortisone.

North, Central and South America

To:

• Morning dose as usual
• Usual 2nd dose on arrival
• Evening dose before going to bed
• Normal treatment timings the next day

From:

• Evening dose as usual
• Normal treatment timings the next day

Middle East and India

To:

• Evening dose as usual
• Half morning dose on arrival
• Normal treatment timings from the morning of arrival day

From:

• Evening dose as usual
• Repeat evening dose on boarding aircraft
• Usual morning dose on arrival in UK
• 2nd dose late afternoon
• Normal evening dose
• Normal treatment timings the next day

Far East, Australia and New Zealand

For these journeys it is better to switch to 8 hourly treatment programme.

To:

• Normal daily schedule up to departure
• Switch to 8 hourly regimen for flight duration

From:

• Continue 8 hourly until the morning after arrival back to usual treatment programme

Other things to remember before you go away

• Always carry extra tablets and do not put them all in the same place in case your luggage is lost or delayed
• Take and wear your Medic Alert Bracelet or equivalent
• Take your steroid or treatment card. Either the standard Department of Health Treatment Card, or the Hydrocortisone Replacement Therapy Card (Child Growth Foundation) or the Congenital Adrenal Hyperplasia Therapy Card (CAH Support Group). Make sure it is filled in and up to date.
• Your emergency supply of hydrocortisone is for emergency use. Make sure that it is still in date. If it is not, ask for a new supply.
• Check that you have plenty supply of medication for the holiday duration. Getting medications abroad can be difficult sometimes and the formulations are not always the same.
• Don’t forget your “Letter for Customs” as you will need this for traveling with needles and syringes. Our endocrine or late effects specialist nurses can give you this

Dealing with emergencies abroad

The same sick-day rules apply with hydrocortisone:

• If your child is generally unwell then either double or treble the dose of hydrocortisone.
• If you need to give the intramuscular injection of hydrocortisone your child   should be taken to the nearest hospital as soon as it is possible and a let the doctor know that emergency hydrocortisone has already been administered and that your child is on steroid replacement therapy.
• Your child should not be discharged until the staff are satisfied that blood glucose and salt levels are normal.
• Remember to take your steroid card and medical details with you as this will help the local doctors.

My treatment plan

Name:

Date of birth: //

My oral hydrocortisone replacement dose is:

Double oral steroids

Triple oral steroids

My hydrocortisone injection expires:

In emergencies, phone 999 and ask for a paramedic ambulance. Do not forget to tell the paramedics that your child is on steroid replacement.