This guideline is intended for doctors providing telephone advice to parents of children taking regular oral corticosteroids at home during an intercurrent illness or trauma.
Children who are administered regular oral corticosteroids (hydrocortisone, prednisolone, dexamethasone or deflazacort) are at risk of adrenal insufficiency at times of illness or accident, as they are unable mount an endogenous cortisol response to stress. This is a medical emergency as it is potentially life-threatening.
In these situations, extra steroid is required in the form of hydrocortisone, given either orally, intramuscularly or intravenously depending on the severity of the clinical situation. Intramuscular (IM) preparations of hydrocortisone include hydrocortisone sodium succinate and hydrocortisone sodium phosphate.
Separate PIER guidelines are available for:
those children requiring IV corticosteroids for an adrenal crisis (insert link for PIER adrenal crisis guideline)
those children taking regular oral steroids and require IV steroids for a procedure or surgery(PIER guideline under development, nearly finalised).
children taking steroids for Duchenne Muscular dystrophy (PIER guideline under development, nearly finalised).
Scope
Paediatricians working in paediatric endocrinology network centres across Wessex.
Purpose
The aim of the guideline is to provide an agreed clinical approach to managing children at home who take regular oral corticosteroids and who have an inter-current illness or trauma, to enable prompt assessment in hospital where required, improve patient experience and clinical outcomes.
Key Points to Consider for Telephone Conversation
Important points to guide the history
Symptoms of adrenal crisis (1):
Abdominal pain, nausea or vomiting
Back pain
Severe weakness
Fatigue
Dizziness or light-headedness
Confusion or coma
Symptoms of intercurrent illness:
Fever
Oral intake
Vomiting or diarrhoea
Antibiotic therapy
History of trauma including nature of injury. A concussion or fracture are significant injuries, rather than a cut, graze or bump from which a child immediately recovers.
Normal dose, frequency and type of steroid (hydrocortisone, prednisolone, dexamethasone or deflazacort).
Access to emergency IM hydrocortisone.
Obtain the child’s most recent weight (estimate or use recent clinic letter), in order to calculate their body surface area using the table in the BNF for children
Sick Day Management Advice for Different Clinical Situations for Children Administered Regular Oral Hydrocortisone
For home management at times of illness of children taking regular hydrocortisone, the hydrocortisone dose should be increased to aim for 30 milligrams/m2/day in 4 divided doses.
In practice, the dose of the hydrocortisone tablets that the family will have at home may mean it will not be possible to administer this dose exactly. It is therefore reasonable for the dose to vary 30 - 50mg/m2/day hydrocortisone depending on the tablet dose available at home.
Minor injury, illness, everyday mental and emotional stress (2)
Continue normal doses of oral hydrocortisone.
Febrile illness or infection requiring antibiotics, tolerating oral intake
Oral hydrocortisone doses should be increased to 30 milligrams/ m2 /day given in four divided doses (06:00, 12:00, 18:00, 00:00) (see Appendix A for worked examples).
Continue until recovery (usually 2- 3 days)1.
Recommend increased consumption of electrolyte-containing fluids1.
Recommend parents check on their child overnight (3-4 am).
Severe febrile illness, trauma or symptoms of adrenal insufficiency
Advise immediate administration of IM hydrocortisone, using a dose of 50 milligrams/m2 or using estimated doses as follows (1):
Infants: 25 milligrams
School-age children: 50 milligrams
Adolescents: 100 milligrams
Dial 999 for emergency transfer to hospital.
If parents do not have an emergency prescription of IM hydrocortisone, advise dialling 999 for emergency assessment and transfer to hospital.
All children who have received IM hydrocortisone in the community must attend hospital for assessment and be observed for a minimum of 12 hours.
Gastroenteritis
Oral hydrocortisone doses should be increased to 30 milligrams/ m2/ day given in four divided doses (06:00, 12:00, 18:00, 00:00) in both vomiting and diarrhoeal illnesses.
Recommend increased consumption of electrolyte-containing fluids.
Recommend parents check on their child overnight (3-4 am).
If a child vomits within 30 minutes of taking a dose of oral hydrocortisone, advise immediately repeating this dose.
If vomiting continues, advise giving IM hydrocortisone as above1and dial 999 for emergency transfer to hospital.
If parents do not have an emergency prescription of IM hydrocortisone, advise dialling 999 for emergency assessment and transfer to hospital.
Sick Day Management for Children on Regular Dexamethasone, Prednisolone or Deflazacort (3)
If a child is unable to tolerate their usual corticosteroid by mouth or if there are symptoms of adrenal insufficiency, advise dialling 999 for emergency assessment and transfer to hospital.
Consider advising administration of intramuscular hydrocortisone if available at home.
Worked Examples
Example 1:
A 7-year-old boy, with a diagnosis of congenital adrenal hyperplasia takes regular oral hydrocortisone (2.5 milligrams at 07:30, 2.5 milligrams at 12:00, 2.5 milligrams at 16:00 and 2 milligrams at 20:00).
His mum calls asking what to do with his steroid doses, as he has a fever and is on antibiotics for a lower respiratory tract infection from his GP.
He weighs 20 kilograms, which equals a body surface area of 0.79 m2
‘Stress dose’ of hydrocortisone = 30 milligrams/ m2/ day in four divided doses.
30 x 0.79 = 23.7 milligrams = 6 milligrams per dose to be given at the following times 06:00, 12:00, 18:00, 24:00.
As the family have 2 milligram hydrocortisone tablets at home, advise 3 x 2 milligram tablets per dose
Example 2:
A 15-year-old girl, with secondary adrenal insufficiency takes regular oral hydrocortisone (5 milligrams at 07:30, 5 milligrams at 12:30, 2.5 milligrams at 16:00 and 2.5 milligrams at 21:00).
Her mum calls asking what to do with her steroid doses, as she has a diarrhoeal illness.
She weighs 55 kilograms, which equals a body surface area of 1.6 m2
‘Stress dose’ of hydrocortisone = 30 milligrams/ m2/ day in four divided doses.
30 x 1.6 = 48 milligrams = 12 milligrams per dose to be given at the following times 06:00, 12:00, 18:00, 24:00.
As the family only have 10mg hydrocortisone tablets at home (which can be quartered), advise 12.5 milligram hydrocortisone per dose (equates to 31.25 milligram /m2/day)
Implementation
The guideline will be displayed on the PIER website and can be accessed by all healthcare professionals working within Wessex. This guideline will be disseminated to network centre leads within the Wessex Paediatric Endocrine Network.
Process for Monitoring Effectiveness
Compliance with the guideline will be audited and results disseminated to the Wessex Paediatric Endocrine Network. A service evaluation of parental satisfaction of initial management will be undertaken.
References
Diagnosis and treatment of primary adrenal insufficiency: And Endocrine Society Clinical Practice Guideline. The Journal of Clinical Endocrinology and Metabolism. Volume 101, Issue 3. February 2016.
Congenital adrenal hyperplasia due to steroid 21-hydroxylase deficiency: An Endocrine Society Clinical Practice Guideline. The Journal of Clinical Endocrinology and Metabolism. Volume 103, Issue 11. November 2018.
The PJ Nicholoff Steroid Protocol for Duchenne and Becker Muscular Dystrophy and Adrenal Suppression. PLos Currents. June 2017.
Park J, Didi M, Blair J. The diagnosis and treatment of adrenal insufficiency during childhood and adolescence. Arch Dis Child. 2016 Sep;101(9):860-5. doi: 10.1136/archdischild-2015-308799. Epub 2016 Apr 15