Great Ormond Street Hospital for Children NHS Foundation Trust: Information for Families Cortisol deficiency and steroid replacement therapy

This leaflet explains about cortisol deficiency and how it is treated. It also contains information about how to deal with illnesses, accidents and other stressful events in children on cortisol replacement. Where are the The two most important ones are: adrenal glands and • Aldosterone – this helps regulate what do they do? the blood pressure by controlling how much salt is retained in the The adrenal glands rest on the tops body. If a person is unable to of the kidneys. They are part of the make aldosterone themselves, they endocrine system, which organises the will need to take a tablet called release of hormones within the body. ‘fludrocortisone’. Hormones are chemical messengers that switch on and off processes within the • Cortisol – this is the body’s natural body. steroid and has three main functions: The adrenal glands consist of two parts: - helping to control the blood „„the medulla (inner section) which sugar level makes the hormone ‘adrenaline’ which is part of the ‘fight or flight’ - helping the body deal with stress response a person has when stressed. - helping to control blood pressure „„the cortex (outer section) which and blood circulation. releases several hormones. If a person is unable to make cortisol themselves, they will need to take a tablet to replace it. Pituitary gland The most common form used is hydrocortisone, but other forms Parathyroid gland may be prescribed. Thyroid gland Medulla Cortex Adrenal Thymus gland Gland

Kidney Adrenal gland Pancreas

Sheet 1 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 What is In these circumstances, the amount cortisol deficiency? of hydrocortisone given needs to be increased quickly. This is done by, either: Cortisol deficiency occurs when the adrenal glands do not produce enough „„increasing the dose of oral cortisol. This can happen for four main hydrocortisone taken as tablets reasons: or by

„„When the pituitary gland is unable to „„giving an injection into the patient’s produce the chemicals needed to tell thigh (intramuscular hydrocortisone) the adrenal glands to ‘switch on’ their Increasing the dose of oral hydrocortisone cortisol production. The pituitary gland is usually done by giving a double or is the ‘master gland’ which controls treble dose, depending on how the child other glands in the body. responds. If you have any concerns call „„In a condition called congenital your specialist treatment centre. adrenal hyperplasia (CAH). CAH causes For instructions on giving intramuscular a blockage within the adrenal glands injections of hydrocortisone, please see so they do not allow cortisol to be our leaflet How to give an emergency produced. injection of Efcortesol®: Information „„If the adrenal gland itself fails (for for families. You must always call an example, in Addison’s Disease) or is ambulance to take the child to hospital if removed. they have had a hydrocortisone injection. „„The adrenal glands can stop producing cortisol because there are enough steroids in the body. The steroids Does the medication should be withdrawn slowly to give the have any side effects? adrenal glands a chance to ‘wake up’ The doses of hydrocortisone and and start producing cortisol again. fludrocortisone are calculated to mimic For more information about the child’s normal production levels and so are condition, please ask your doctor. unlikely to cause side effects. Side effects only tend to occur if someone takes Cortisol deficiency is easily controlled steroids above the level usually produced with replacement therapy as by the body for a long period of time. hydrocortisone tablets given several times a day. However, if a person with cortisol deficiency becomes very stressed or unwell, either emotionally or physically, they are unable to increase the production of cortisol in their system to help the body cope and this could be life threatening.

Sheet 2 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 When to increase the Vomiting child’s hydrocortisone „„It takes about an hour for oral dose hydrocortisone to be absorbed It can be difficult to know when a „„If the child vomits within an hour of child needs to increase their dose of taking the oral hydrocortisone, you hydrocortisone, but it may be necessary should repeat the dose if they are ill, have an accident or injury „„If the child is on milk feeds, it is or are going to have some medical important to try to give the medication treatments. The notes at the back of this one hour before the feed booklet should help you decide. If you „„If the child is only sick once, an have any questions at any stage, contact hour or longer after taking the oral your specialist treatment centre at Great hydrocortisone, you will not need to Ormond Street Hospital or University repeat it. However, you should think College Hospital (contact numbers on about why the vomiting has happened. the inside back cover) which provide a 24 It may be a one-off, or a warning of hours/7 days a week service. underlying illness An extra dose will not do any harm, so if „„Observe the child, and if they are sick in doubt it is better to give it than not, again, give them double or treble and then contact the specialist treatment the dose of oral hydrocortisone. centre for advice about what to do next. Seek medical advice from your GP, If the child is given a hydrocortisone local paediatric department, or your injection they must be admitted to specialist treatment centre. hospital afterwards to be monitored. You should insist that this is for a minimum „„If the child is unable to keep down of 12 hours. This is because the injection the increased dose and/or is getting lasts about 8 hours, and it is important more unwell, you will need to to check that the child is stable on their give an intramuscular injection of normal oral dose before going home. hydrocortisone and call an ambulance to take the child to hospital If your child is unwell, a further dose of immediately hydrocortisone should be given at 4am – set your alarm clock! This should be double the usual morning dose. Diarrhoea

It is important not to ignore diarrhoea „„If the child develops diarrhoea, there is and vomiting, especially if the a risk that the oral hydrocortisone will child is also taking tablets such as pass through the gut too quickly and fludrocortisone or DDAVP. These not be absorbed. You therefore need medicines can cause them to become to double or treble the usual dose of dehydrated with imbalanced salt levels. oral hydrocortisone until the diarrhoea stops.

Sheet 3 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 „„You also need to seek medical advice to Dentists see if the child has picked up a stomach „„If the child has a dental appointment bug, which may need antibiotic for a check up or cleaning, they should treatment not need any extra hydrocortisone „„If the child has severe diarrhoea „„If the child has an appointment for and is also vomiting, they will need fillings or other treatments which hydrocortisone without delay. You require an injection, you should give should give the intramuscular injection them double or treble the dose of oral and call an ambulance to take the child hydrocortisone for the 24 hour period to hospital immediately around the appointment

„„If the child unexpectedly needs an Coughs and colds injection for fillings or other treatment, „„If the child has a minor head cold with give them double or treble the dose of snuffles, runny nose and a cough but is oral hydrocortisone as soon as possible, otherwise well, there is usually no need and continue for the next 24 hours to increase the oral hydrocortisone „„Major dental work, like having teeth removed, should only be carried out in hospital. Please call your specialist Temperatures treatment centre for advice before the „„If the child has a temperature, this appointment. shows that they have some sort of infection and will need double or treble the dose of oral hydrocortisone. This General anaesthetics will usually be for two to three days „„If the child needs a general anaesthetic until the temperature is back to normal. for any reason, they will need extra hydrocortisone given intravenously „„If your child is unwell, a further dose of (IV) as they go to sleep. The endocrine hydrocortisone should be given at 4am registrars are available to advise your – set your alarm clock! This should be local team on the dose needed and double the usual morning dose. can be contacted at your specialist treatment centre. Twenty-four hour Antibiotics advice is available for emergency admission. „„If the child has been prescribed antibiotics, you will need to give them double or treble the dose of oral hydrocortisone until the course of antibiotics is completed

Sheet 4 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 Immunisations for „„However, if they have a serious injury, childhood illnesses for example, bump their head and become unconscious, break a limb „„It is important that children who are or get burnt, you will need to give on hydrocortisone replacement therapy them an intramuscular injection of have all their immunisations. If the hydrocortisone and call an ambulance child is taking medications other than to take them to hospital immediately. hydrocortisone, you should ask your The child may not necessarily need the specialist treatment centre for advice extra dose of hydrocortisone, but it „„You should give double or treble the will do no harm. It is always better that dose of oral hydrocortisone for the 24 they have the injection as more serious hour period around the injection, if problems may occur if they do not get you have any worries, please ring your it when needed specialist treatment centre for advice before the child is due to have the immunisation. Unresponsive child/ hypoglycaemia „„Children may develop a high temperature and/or a rash several days „„If for any reason you find the child with after some immunisations, particularly symptoms of hypoglycaemia (low blood the MMR vaccine. You should keep sugar) for instance, they are: a close eye on the child after the • pale immunisation and follow the advice • clammy under ‘Temperatures’ if they become • drowsy unwell • confused • glazed Accidents and injuries • not responding as they would „„If the child has a fall, bump or bruise, normally but immediately recovers and carries you should give them the intramuscular on what they were doing before, they injection of hydrocortisone and call an may not need any extra hydrocortisone, ambulance to take the child to hospital but do contact your specialist treatment immediately centre if you have any worries „„While you are waiting for the ambulance, you should give the child a sugary gel called Glucogel®. You give this by squirting the gel in the child’s mouth between the gums and the inside of the cheek and then rub the cheek gently to help the gel become absorbed. You should never give an unresponsive child anything to eat or drink

Sheet 5 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 Emergency kits Your specialist treatment centre will issue you with emergency medication for your kit, one of which the child should carry at all times. Another should be kept at the child’s nursery, school or college.

Each emergency kit should contain:

„„1x vial of hydrocortisone as Efcortesol® My Cortisol „„1x 2ml syringe „„2x blue needles „„1x tube of Glucogel® gel with instructions for use Does your child carry an „„1x leaflet ‘How to give efcortesol emergency pack? an emergency injection Download our free “My Cortisol” app of Efcortesol®’

„„1x steroid card filled Available on the Apple App Store in with child’s details and Google Play Store which you should keep in a specially labelled container. You should check the expiry dates of Efcortesol® and Glucogel® and order replacements from your

GP before they pass their expiry date.

Sheet 6 of 7 Ref: 2014F0715 © GOSH NHS Foundation Trust March 2015 If you have any questions or queries, please call the following numbers:

Office hours Outside these hours (Monday to Friday 9am to 5pm) Clinical Nurse Specialist (GOSH) Call 020 7405 9200 and ask to speak (GOSH) 020 7813 8214 (answerphone) to the ‘On call Endocrinology Registrar’ or email [email protected] Clinical Nurse Specialist (UCLH) Call 0845 155 5000 and ask to (University College Hospital London) speak to the ‘On-call Registrar for 0845 155 5000 ext 79204 Paediatric Endocrinology’

Further information We recommend that all children on It is also important that they carry steroid replacement therapy wear a a ‘Medical card for patients on medical identity bracelet or necklace at hydrocortisone replacement’ or a ‘CAH all times. Further information about these steroid replacement’ card at all times. can be obtained from the clinical nurse You can get these cards from the clinical specialists at either site, or the Health nurse specialists at either site or from the Information Centre at GOSH. support organisations.

Special note for children with diabetes insipidus who are taking DDAVP If the child needs: Extra oral hydrocortisone for vomiting and/or diarrhoea, you should: n Give them double or treble the dose of oral hydrocortisone n Do not give them any more DDAVP n Allow them to drink if thirsty n Take them to hospital for a blood test to check their plasma electrolytes Intramuscular hydrocortisone for any reason, you should: n Give them an intramuscular injection of hydrocortisone n Do not give them any more DDAVP n Call an ambulance to take them to hospital immediately

Compiled by the Pharmacy & Endocrinology Departments in collaboration with the Child and Family Information Group

Great Ormond Street Hospital for Children NHS Foundation Trust Great Ormond Street London WC1N 3JH

www.gosh.nhs.uk

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