Emergency steroid management plan for patients on long term, high dose glucocorticoid treatment

Many patients in the trust are treated with high dose steroids to manage their condition. When these medications have been used on a long term basis the child or young person can develop adrenal suppression. This information sheet from Great Ormond Street Hospital (GOSH) explains about adrenal suppression and what action needs to be taken if adrenal suppression occurs. 
The adrenal glands rest on the tops of the kidneys. They are part of the endocrine system, which organises the release of hormones within the body. Hormones are chemical messengers that switch on and off processes in the body.
 
The adrenal glands produce cortisol which is the body’s natural steroid and has three main functions:
  • Helping to control the blood sugar level
  • Helping the body deal with stress
  • Helping to control blood pressure and blood circulation.
Adrenal suppression occurs when the adrenal glands stop producing cortisol because there are additional steroids in the body. Adrenal suppression means that when your child or young person is Ill, is having an operation or has a serious accident they may not be able to mount the usual ‘stress’ response in the body by increasing the amount of steroid hormones circulating in the blood. They will require additional fast acting steroids in the form of hydrocortisone tablets, or, in an emergency, as an injection. This effect can continue for up several months/years after stopping steroid medication. 
An impaired stress response can result in your child or young person becoming very unwell, very quickly. 

What to do if your child or young person becomes unwell

We have produced a flow sheet for managing any episodes of illness. Your child or young person may not necessarily need the extra doses of hydrocortisone, but it will do no harm. It is always better that they have the injection if you are concerned as more serious problems may occur if they do not get it when needed.

Unresponsive /hypoglycaemia

If for any reason you find your child or young person with symptoms of hypoglycaemia (low blood sugar) for instance, they are: 
  • pale
  • clammy
  • drowsy
  • confused
  • glazed
  • not responding as they would normally
You should give them the intramuscular injection of hydrocortisone and call an ambulance to take them to hospital immediately.
 
While you are waiting for the ambulance, if they are conscious you should give them a glucose gel. You give this by squirting the gel in their mouth between the gums and the inside of the cheek and then rub the cheek gently to help the gel become absorbed.
 
If your child or young person is unresponsive, never give them anything to eat or drink, including glucose gel.
 
 

Dental appointments and immunisations

If your child or young person requires any immunisations or dental work, with or without local anaesthetic, and they are on an ‘off day’ from their regular steroids please add in the extra hydrocortisone tablets four times a day. If they are on an ‘on’ day no additional hydrocortisone is needed unless symptomatic.

Planned or emergency operations (including dental work under general anaesthetic)

Your child or young person will need extra steroid medication intravenously to be given as they go under anaesthetic.
Always tell doctors that your child or young person is taking long term steroid medication and may be at risk of adrenal crisis.
If the decision is made to come off steroids in the future these are weaned slowly to give the adrenal glands a chance to ‘wake up’ and start producing cortisol again. Giving the emergency doses of hydrocortisone if needed can help to ensure this process is done safely.

School packs

We provide information packs for schools and are happy for schools to contact us for information. Please let your team know about all changes of school.

Emergency kits

Your specialist treatment centre will issue you with three emergency medication kits, one of which your child or young person should carry at all times. Others should be kept at home and nursery/ school or college. 
 
You should make these up as emergency kits in a box or a tin that closes securely.
Each kit should contain:
  • 1x vial of 100mg/1ml hydrocortisone sodium phosphate 

Or 

  • 1 x vial 100 mg hydrocortisone sodium succinate and 1x vial of water for injection

And

  • 1x 2ml syringe
  • 2x blue needles
  • 1x tube of glucose gel with instructions for use
  • 1x leaflet ‘How to give an emergency injection of hydrocortisone®
  • 1x steroid card 
  • Some spare oral tablets of hydrocortisone
You should check the expiry dates of hydrocortisone tablets, injection and glucose gel and order replacements from your family doctor (GP) before they pass their expiry date. 

Medical jewellery

We recommend that all patients with adrenal suppression wear a medical identity bracelet or necklace at all times. Further information about these can be obtained from the clinical nurse specialists.
 
This should state that the patient has “adrenal suppression, at risk of adrenal crisis”. 

Steroid cards

Your child or young person needs to carry a blue ‘Steroid card’ at all times.

App for your mobile phone

My Cortisol – There is a free app available for Android and Apple devices to help with emergency care of patients with adrenal suppression.
Compiled by: 
The Endocrinology Department in collaboration with the Child and Family Information Group
Last review date: 
March 2020
Ref: 
2019F2208

Disclaimer

Please read this information sheet from GOSH alongside the patient information leaflet (PIL) provided by the manufacturer. If you do not have a copy of the manufacturer’s patient information leaflet please talk to your pharmacist. A few products do not have a marketing authorisation (licence) as a medicine and therefore there is no PIL.

For children in particular, there may be conflicts of information between the manufacturer’s patient information leaflet (PIL) and guidance provided by GOSH and other healthcare providers. For example, some manufacturers may recommend, in the patient information leaflet, that a medicine is not given to children aged under 12 years. In most cases, this is because the manufacturer will recruit adults to clinical trials in the first instance and therefore the initial marketing authorisation (licence) only covers adults and older children. 

For new medicines, the manufacturer then has to recruit children and newborns into trials (unless the medicine is not going to be used in children and newborns) and subsequently amend the PIL with the approved information. Older medicines may have been used effectively for many years in children without problems but the manufacturer has not been required to collect data and amend the licence. This does not mean that it is unsafe for children and young people to be prescribed such a medicine ‘off-licence/off-label’. However, if you are concerned about any conflicts of information, please discuss with your doctor, nurse or pharmacist.