Caring for a child with adrenal insufficiency
Management of adrenal insufficiency
Glucocorticoid dosing is an integral part of managing adrenal insufficiency, aiming to replicate what the body normally produces. In response to physiological stress, normal cortisol production can increase up to six fold (Cooper and Stewart 2003). This does not happen in children with adrenal insufficiency hence the importance of adhering to the high doses prescribed in a child’s individual pre-planned sick day regimen. For most children, treatment is usually lifelong. The most commonly used medication in childhood adrenal insufficiency is hydrocortisone. Prednisolone and dexamethasone are more potent glucocorticoids but are generally not recommended for use in children for glucocorticoid replacement therapy (Hindmarsh 2009).
It is important to find a balance in prescribed regimens to minimise side effects for the child. Lethargy and hypoglycaemia can occur with insufficient dosing, while overtreatment can predispose a child to diminished growth velocity and weight gain. In CAH, sufficient glucocorticoid replacement is required to suppress ACTH secretion, as oversecretion can stimulate overproduction of adrenal androgens. The rationale is to suppress adrenal androgen secretion to minimise the risk of accelerated growth and bone age advancement, yet careful monitoring is needed to ensure that dosing does not affect final adult height (Sarafoglou et al 2014).
A lack of consensus and much debate exists about the monitoring of glucocorticoid therapy and frequency of dosing (Howlett 1997). Children may be on a twice or thrice daily dosing regimen, four times daily dosing has been suggested in CAH (Hindmarsh 2014). Dose adjustment is based on the child’s body surface area, in addition to clinical consultation. Auxology (detailed growth measurements and plotting of centiles) is useful in monitoring growth velocity and effectiveness of therapy (Dauber et al 2010).
Annual bone age (X-ray of left wrist) assessment is useful in comparing chronological age to bone age to monitor the child’s growth pattern. Routine bloods and blood pressure monitoring are also helpful in assessing the child’s treatment regimen. Cortisol day curve monitoring (Howlett 1997), salivary sampling and capillary finger prick blood spots can also be used to monitor efficacy of treatment. New glucocorticoid preparations are under development to mirror normal physiological secretion (Falorni et al 2013, Grossman et al2013).
Prompt intervention is crucial in managing this condition, particularly during intercurrent illness when a child may be unable to mount and sustain a sufficient physiological cortisol response, predisposing them to the onset of an adrenal crisis. Normal glucocorticoid replacement is inadequate during these instances and dosing must be increased to mimic normal physiological responses. These doses are commonly referred to as stress doses.
At risk situations include gastroenteritis as doses may not be tolerated or absorbed due to vomiting or diarrhoea. Before arriving at hospital, parents and carers may be unable to administer rectal or intramuscular hydrocortisone in these instances, leaving the child vulnerable.
Children on long-term exogenous glucocorticoid therapy are at risk of acute adrenal insufficiency when doses are weaned to less than the body’s normal physiologic requirements. A tapering regimen is recommended. Parents, carers and children are made aware of the risks of adrenal crisis, symptoms of steroid withdrawal (Box 4) and the importance of applying sick day rules until adrenal recovery is demonstrated.
Box 4. Symptoms of steroid withdrawal
- Lethargy/weakness.
- Nausea.
- Headache.
- Weight loss/decreased appetite.
- Hypotension.
- Joint pain/arthralgia.
- Fever.
A morning cortisol level of 200-600nmol/L, usually taken between 8am and 9am, is appropriate. It has been documented, however, that the HPA axis may not respond to stress for up to six to 12 months post discontinuation of therapy (Miller et al 2008).
Mineralocorticoid replacement and salt supplementation (usually required in babies up to one year of age in CAH) may be indicated in primary adrenal insufficiency. Side effects of mineralocorticoid dosing include hypertension and fluid retention.
A morning cortisol level of 200-600nmol/L, usually taken between 8am and 9am, is appropriate. It has been documented, however, that the HPA axis may not respond to stress for up to six to 12 months post discontinuation of therapy (Miller et al 2008).
Mineralocorticoid replacement and salt supplementation (usually required in babies up to one year of age in CAH) may be indicated in primary adrenal insufficiency. Side effects of mineralocorticoid dosing include hypertension and fluid retention.
- Glucocorticoid dosing is an integral part of managing adrenal insufficiency, aiming to replicate what the body normally produces.
- It is important to find a balance in prescribed regimens to minimise side effects for the child.
- Prompt intervention is crucial in managing this condition, particularly during intercurrent illness when a child may be unable to mount and sustain a sufficient physiological cortisol response, predisposing them to the onset of an adrenal crisis.
- Sick day rules advise doubling or trebling the child’s daily dose for a minimum of 24 hours after the illness, pain or temperature has ceased or to seek medical attention if stress doses are used at home for longer than three days.
- Children on long-term exogenous glucocorticoid therapy are at risk of acute adrenal insufficiency when doses are weaned to less than the body’s normal physiologic requirements.
- Symptoms of steroid withdrawal are lethargy and/or weakness, nausea, headache, weight loss or decreased appetite, hypotension, joint pain/arthralgia and fever.
Sick day rules
Sick day rules traditionally advise doubling or trebling the child’s daily dose for a minimum of 24 hours after the illness, pain or temperature has ceased or to seek medical attention if stress doses are used at home for longer than three days. Glucocorticoids may have to be administered parenterally if a child is unable to take medication orally.
The following guidelines are usually recommended:
- Double or triple the normal oral dose in the event of illness, pain or injury, including fever.
- This dose is usually continued for 24 hours until symptoms have settled.
- Stress dosing is not usually indicated in times of psychological stress such as school examinations, worry and sadness. However, if a child appears particularly anxious, this may need to be reviewed on an individual case basis.
- Rectal hydrocortisone may be given when a child is vomiting. Parents and carers need to be aware that a baby or young child may expel the suppository and are advised to check their nappy to ensure the medication has been absorbed. Debate still exists about the use and reliability of hydrocortisone suppositories at home (Ní Chróinín et al 2003) and not all centres advocate their use.
- If a child is not getting better following stress dosing or if parents or carers are concerned, they are advised to seek immediate medical attention.
- Intramuscular hydrocortisone is indicated when the child is vomiting and/or has diarrhoea. It is particularly important that parents seek medical attention, especially if the child is on desmopressin (an analogue used in the treatment of DI) or fludrocortisone as gastroenteritis can lead to dehydration and salt imbalances. Parents and carers are also advised to bring the child promptly to the local hospital if an injection has been administered.
- Some children may be more susceptible to the risk of hypoglycaemia when unwell – children with multiple pituitary hormone deficiencies (MPHD) and CAH. If a child is unwell, but not vomiting, parents and carers are advised to make sure that their child has regular food intake during the day and once or twice overnight, particularly infant formula/breast milk/sugary drinks. This may mean waking an older child up overnight.
- The use of glucogel may be indicated as a second-line treatment when a child becomes unwell and not anxious to feed. It is given into the mouth or squirted onto a finger and massaged onto the jaw to aid absorption (half a tube can be used in babies and young children) but should not be used if a child appears unusually sleepy or drowsy. It is important to remind parents that stress doses of hydrocortisone need to be given in the first instance via the appropriate route.
- Complete time out activity 4Referring to a current edition of the British National Formulary for Children, review the pharmacological actions and side effects of hydrocortisone and fludrocortisone.
Nursing care interventions in hospital
Hydrocortisone therapy
Children with adrenal insufficiency need to have hydrocortisone medication administered on time; the first dose is usually given in the morning, preferably on waking, and the last dose is given just before bedtime. Dosing and frequency may vary depending on the attending specialist centre. Hydrocortisone suspension tends to be unreliable. Tablets can be cut, crushed or dissolved for administration in younger children and infants. If a child is unwell, dosing should be double or triple their usual daily dose. If a child is nil by mouth, or unable to take oral medication, then alternative routes of hydrocortisone administration either intravenous or intramuscular must be considered.~
Nursing responsibilities
Scheduled dosing must never be discontinued or withheld. Blood glucose monitoring may also be indicated if hypoglycaemia is suspected. Sick day dosing must apply until the child demonstrates an appropriate recovery.
Fludrocortisone is a mineralocorticoid preparation given to replace aldosterone in primary adrenal insufficiency and is taken once a day usually in the morning – this does not need to be increased during episodes of illness. Children may require twice daily dosing if doses are high. Encourage oral fluids where possible as the child may be susceptible to dehydration as a result of their mineralocorticoid deficiency. If a child’s condition with adrenal insufficiency starts to deteriorate, it is probable that they require additional hydrocortisone. Notify the doctor promptly of any concerns you might have.
Management of a child for elective surgery should be discussed in advance of a scheduled admission to ensure that an individualised care plan is created to minimise potential risk situations. A suggested checklist for elective surgical procedures is outlined in Box 5.
Box 5. Checklist for surgical procedures for a child with adrenal insufficiency
It is preferable for a child with adrenal insufficiency to be given a priority slot on surgical theatre lists to avoid delays in medication regimens and exposure to prolonged fasting times. Liaise with the anaesthetist for any specific instructions. For afternoon surgical lists, the morning dose of hydrocortisone can be taken orally.
Children may require intravenous (IV) fluids pre-operatively. A dose of IV hydrocortisone is given at induction to ensure sufficient glucocorticoid cover. The dose will need to be repeated if the surgery exceeds four hours.
Post-operatively, children should receive hydrocortisone stress dosing until they demonstrate a good recovery. It is important to monitor the child’s condition for signs of complications and deterioration and to notify the doctor immediately.
Complete time out activity 5
Reflect now on how you might work with a parent to help anticipate the care plan requirements of a child with adrenal insufficiency who is scheduled for surgery. What do you need to explain? What parental insight could you draw on? Remember, parents may be experts to a considerable degree, but they may also have many fears.
Role of the paediatric endocrine nurse specialist
- The PENS is often the first point of contact for families, playing a crucial role in facilitating the care of children in hospital and at home, maintaining a direct link for families with the hospital (Low and Gilbert 2001).
- A key role of the PENS is the education of children and their families about their disease processes, medication and treatment regimens. The importance of an open working relationship with families cannot be underestimated.
When parents and carers are first faced with the diagnosis of their child having a chronic illness, it is a daunting, challenging and worrying time. Feelings such as anger, loss, guilt and sadness are common. Financial worries may also be a significant cause of concern. Education, information and support enable families and children to adapt to living with a long-term medical condition. Additional support from peer support groups and introduction to other families may be beneficial. Promoting the physical and psychological wellbeing of parents to avoid exhaustion is important following diagnosis as they adjust to a new home routine while learning about specific care responsibilities for their affected child. Depending on the underlying diagnosis, a child may have a relatively normal home routine, incorporating medication management, or may have alternating episodes of sickness and remission requiring regular hospital admissions.
Learning Points
- It is important that children with adrenal insufficiency have their hydrocortisone medication administered on time.
- The PENS is often the first point of contact for families, playing a crucial role in facilitating the care of children in hospital and at home.
- Education, information and support enable families and children to adapt to living with a long-term medical condition. Promoting the physical and psychological wellbeing of parents to avoid exhaustion is important.
Discharge planning
Once the child’s condition has been stabilised and the diagnosis and underlying cause of adrenal insufficiency has been established, preparation for self-care and home management becomes the focus. Optimal control relies on the abilities of parents and carers to recognise circumstances that can precipitate adrenal crisis, particularly during intercurrent illness, and awareness of how to avoid preventable risk episodes.
The significance of early intervention is emphasised because it greatly influences safer outcomes for their child. Parents and carers need to know how to monitor their child’s condition, how and when to adjust medication as well as changing the method of administration if required, for example, rectal or intramuscular hydrocortisone, and importantly when to seek medical attention.
Older children should be enabled to self-care and understand how to take responsibility for their condition, in particular during school and extracurricular activities. Adolescents want to develop their independence by understanding and managing their condition, taking control of their daily routine in preparation for adulthood, including appropriate sick day management.
Working with young people ensures they are aware of how to manage their condition effectively and the consequences of non-compliance and impact on quality of life. A suggested checklist for home management is outlined in Box 6.Box 6. Checklist for home management
- Prescription for medication, including oral, rectal (if applicable) and intramuscular preparations of hydrocortisone. Instruction and written material must include dosing regimen and how to administer oral, rectal and intramuscular medication. An emergency injection pack is usually supplied by the hospital before discharge and advice about checking expiry dates at regular intervals and how to get replacement packs.
- Advice on safe storage of medication and the importance of having an adequate supply of hydrocortisone, particularly over bank holiday weekends and holidays abroad, in the event that stress dosing is warranted.
- Parents and carers need to be well informed of the condition and provided with verbal instruction and written information on adrenal insufficiency and underlying cause.
- Parents and carers need to be able to identify early warning signs and how to respond to these situations, understanding the significance of early intervention.
- Clear instructions must be given on when the child should present to either the GP or local emergency department for review.
- Provide a contact number for the paediatric endocrine nurse specialist and the endocrine department.
- A steroid therapy card should be carried on the child’s person. This provides a quick compact reference relating to their condition, doses of medication and stress dosing information. It should also include next of kin information and contact numbers for the attending hospital.
- Advice on stress dosing about vaccinations and routine dental work.
- Encourage young children and adolescents to wear a medi-alert bracelet. While medi-alert bracelets are not recommended in children under three years, it is recommended that older children wear one – when a child starts preschool is usually a good time to start.
- Education on signs and symptoms of hypoglycaemia and management at home including use of glucometer and indications for use of glucose gel where necessary.
- Encourage older children to enter next of kin contact details under ICE (In Case of Emergency) on their mobile telephone.
- Teachers and carers at school and nursery need to be aware of the child’s underlying diagnosis and how to respond if the child becomes unwell. An agreed healthcare plan between parents and teachers/carers should be created and emergency pack/instructions provided to ensure the safety of the child.
- Advice on travel emphasising the need to carry medication and emergency pack in hand luggage. Parents and carers need to be advised that they will require a letter for airport customs which they can obtain from their child’s attending hospital. General information on handwashing, food hygiene, insect bites and how to avoid gastroenteritis may also be helpful. Parents and carers may need a refresher session on sick day management and emergency intramuscular hydrocortisone administration before travel and to contact the paediatric endocrine nurse specialist to arrange this.
- Ensure that the child has a scheduled outpatient follow-up appointment date.
- Finally, the ‘take home message’ is that it is better to err on the side of caution. If in doubt, it is better to give a stress dose than not to give it.
- Once the child’s condition has been stabilised and the diagnosis and underlying cause of adrenal insufficiency has been established, preparation for self-care and home management becomes the focus.
- Parents and carers need to know how to monitor their child’s condition, how and when to adjust medication as well as changing the method of administration.
- Young children and adolescents should be encouraged to wear a medi-alert bracelet. Young children should start wearing them when they start preschool.
Conclusions
- Children’s nurses should know the implications of adrenal insufficiency.
- Adrenal insufficiency is a life-threatening condition with significant associated risks.
- The causes of adrenal insufficiency are multiple and prognosis relates to the underlying cause.
- Glucocorticoid dosing is integral to this condition and optimal management relies on understanding the principles of prompt action and sick day rules to prevent adrenal crisis.
Acronyms
17-OHP: 17-hydroxyprogesterone
ACTH: adrenocorticotropic hormone
ADH: antidiuretic hormone
ALD: adrenoleukodystrophy
APECED: autoimmune polyendocrinopathy-candidiasis-ectodermal dystrophy
CAH: congenital adrenal hyperplasia
CRF: corticotropin releasing factor
DI: diabetes insipidus
GST: glucagon stimulation test
HPA: hypothalamic-pituitary-adrenal (axis)
MPHD: multiple pituitary hormone deficiencies
MSH: melanocyte-stimulating hormone
PENS: paediatric endocrine nurse specialist
Points
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