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From the Chief Medical Officer for Wales

Distribution: As Appendix 1
From: Dr Ruth Hussey, Chief Medical Officer, Welsh
Government
Date: 2 May2013
Reference: CEM/CMO/2013/5a
Category: Non urgent (cascade within 48 hours)
Title: Adrenal Insufficiency (Addison’s disease) in
Children and Young People:Iinformation for
General Practitioners
What is this about: Information on the symptoms and signs and
diagnosis and treatment of Adrenal insufficiency
(Addison’s disease) in children and young people
Why has it been sent: For your information and to pass on to colleagues

To:

General Practitionersvia NHS Wales Shared Services Partnership

Out of Hours Providers via Chair of OOHs provider group

For Information:

Associate Medical Directors

Medical Directors

Directors for Primary Community Care & Mental Health

Consultant Paediatricians via Cardiff branch of the Royal College of Paediatrics and Child Health

Dear Colleagues
Adrenal Insufficiency (Addison’s disease) in Children and Young People:information for General Practitioners
I am sure you are aware of the death of 10 year old Robert Powell who died from Addison’s disease in 1990. Sadly this had not been diagnosed prior to his death and so consequently went untreated.

The First Minister commissioned an Independent Investigation into the circumstances of this tragic death to learn lessons for the NHS. The report was published in July 2012. The Minister for Health and Social Services published the Government’s response Learning for the future which identified a range of actions, which are currently being taken forward.

Clearly Addison’s disease is rare, but the outcome catastrophic if missed. I therefore thought it would be helpful to share the attached advice sheet as a reminder of the symptoms of Addison’s disease.

I would appreciate if you could ensure this letter and advice sheet is disseminated as widely as possible.

Yours sincerely

Dr Ruth Hussey OBE
Chief Medical Officer

ADVICE SHEET
Adrenal insufficiency (Addison’s disease) in children and young people:information for General Practitioners
Adrenal insufficiency (Addison’s disease) is rare in children but untreated can be fatal. Adrenal insufficiency can be primary or secondary. Primary adrenal insufficiency, also called Addison's disease, occurs when the adrenal glands are damaged (most commonly autoimmune) and cannot produce enough cortisol and often aldosterone. Secondary adrenal insufficiency occurs when the pituitary gland fails to produce enough adrenocorticotropin (ACTH), leading to a reduction in cortisol secretion.
Symptoms and signs
The symptoms of adrenal insufficiency usually begin gradually with the most common being:

  • chronic, worsening fatigue
  • muscle weakness
  • loss of appetite
  • weight loss
  • recurrent abdominal pain

Other symptoms that present as the disease progresses can include:

  • nausea
  • vomiting
  • diarrhoea
  • low blood pressure with postural hypotension causing dizziness or fainting
  • irritability and depression
  • a craving for salty foods due to salt loss
  • hypoglycemia
  • headache
  • sweating
  • in post menarchal girls, alteration or absent menstrual periods

Hyperpigmentation may occur in Addison's disease but not in secondary adrenal insufficiency. This is most visible on scars, skin folds, such as the elbows, knees, knuckles, and toes and the lips and mucous membranes.
Because the symptoms progress slowly, they are often ignored until a stressful event like an illness or accident causes them to worsen instigating an Addisonian crisis. Symptoms of an Addisonian or "adrenal" crisis include:

  • severe vomiting and diarrhoea
  • dehydration
  • dizziness and low blood pressure
  • loss of consciousness
  • pain in the lower back, abdomen, or legs

If not treated, an Addisonian crisis can be fatal.

Diagnosis and treatment
In its early stages, adrenal insufficiency can be difficult to diagnose as the symptoms may be vague. However, if Addison’s disease is suspected in a child then urgent investigation is required. This initially should include serum sodium, potassium, urea, creatinine, glucose, early morning cortisol and ACTH. Abnormal results or ongoing clinical concern about a diagnosis of adrenal insufficiency warrants urgent referral to a local paediatrician and/or discussion with a Paediatric Endocrinologist for further investigation including an ACTH stimulation test.
In children presenting with a suspected Addisonian crisis, treatment must begin urgently and the child should be sent to the closest paediatric admissions unit urgently.
All diagnoses of adrenal insufficiency should be discussed with a tertiary Paediatric Endocrinologist.

Appendix 1

To:NHS Wales Shared Services Partnership to forward to:

All General practitioners - please ensure this message is seen by allpractice nurses and non-principals working in your practice and retain a copy in your ‘locum information pack’

All Community Pharmacists

Deputising services

HB Chief Pharmacists

HB Prescribing Advisers

Independent/Private clinics and Hospitals and Hospices throughout Wales

To:Chief Executives of Health Boards

To:Medical Directors of Health Boards

To:Nurse Directors Health Boards

To:Directors of Public Health

To:Hospital Principals and Chief Pharmacists to action as per alert

Cc:Public Health Wales

Consultants in Pharmaceutical Public Health

Chief Executives, NHS Trusts

Principal Pharmacist Welsh Quality Control

Principal Pharmacist Continuing Care Services

Principal Pharmacist Welsh Medicines Information Centre

CSSIW

NHS Direct

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