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Cortisol Deficiency and Steroid Replacement Therapy

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Cortisol Deficiency and Steroid Replacement Therapy 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.
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