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Addison’s Disease

Hypoadrenalism, (Primary) Adrenal Insufficiency

Inheritance:

X-linked Inheritance

Age range at onset:

Any

Specialists you may see:

Symptoms

The adrenal glands, found on top of the kidneys, produce hormones to maintain the functions of the body. These hormones are cortisol, aldosterone and DHEA. A person whose adrenal glands do not produce these hormones has primary adrenal insufficiency, or Addison’s Disease.

This rare condition is often autoimmune, meaning the body’s immune system makes a mistake and attacks the cells of the adrenal glands. If testing reveals that this is not the cause of a person’s Addison’s Disease, there is a small probability that it is a sign of adrenoleukodystrophy (ALD) in males.

Testing for ALD at this stage is very important and is actively encouraged by Alex TLC.

While many boys and men with ALD or its adult form, adrenomyeloneuropathy (AMN) also have Addison’s Disease, not all are affected by this.

ALD and AMN are rarely the cause of Addison’s Disease, but it is important to test for this if the cause is not known (idiopathic Addison’s Disease).

Cause

Addison’s Disease is a life‐long condition, which must be controlled with daily steroids to replace
hormones. If this medication is taken correctly and consistently, those with Addison’s can live full and
long lives. Those with Addison’s Disease should have regular medical support from those familiar with
the condition, particularly endocrinologists – specialists in hormone‐related conditions.

Typical signs and symptoms of Addison’s Disease are:

  •  Exhaustion and weakness
  • Weight loss
  • Deep skin pigmentation (sun tan even without going out in the sun)
  • Dizziness when standing
  • Low blood pressure
  • Poor appetite and salt cravings
  • Nausea or vomiting
  • Poor concentration
  • Muscle weakness and cramps
  • Headaches
  • Stomach pains

 

Medication Treatment Update (June 2022)

We have been informed that there will be no further manufacturing of injectable Hydrocortisone Sodium Phosphate by Advanz Pharma until 2023. Please be advised that you will need to order Solu-cortef instead if renewing supplies until early next year.

Addison’s Disease Self-Help Group have an advice page on drug shortages that you may find helpful: Drug Shortage Causes and Coping Strategies for people with Addison’s Disease | Addison’s Disease Self-Help Group (addisonsdisease.org.uk)

 

Condition Management Information

Emergency Injection Kit

Alongside regular medication, those with Addison’s Disease should carry an emergency injection kit in case of adrenal crisis. This sudden worsening of the condition, which can be brought on by stress or infection, can be life‐threatening. Emergency injections of hydrocortisone are required immediately to deal with this. This information is provided by kind permission of the Addison’s Disease Self Help Group.

 

Addison’s Self Help Group

For more information on managing Addison’s Disease, see the ADSHG free guidelines on Managing Your Addison’s and Adrenal Crisis Guidelines. To learn more about Addison’s Disease, see the ADSHG website and consider becoming a member.

 

Society for Endocrinology’s Adrenal Crisis Information

Patients with adrenal insufficiency require regular glucocorticoid replacement and the daily dose requires adjustments during periods of ill health, fever, surgery. If this is not done, patients can suffer an adrenal crisis.

For more information and for the NHS Steroid Emergency Card, please see follow the link: https://www.endocrinology.org/adrenal-crisis

 

Informational Videos

Below are the links to two short video infographics on Primary Adrenal Insufficiency. These ‘CoMICs’ have been developed by University of Birmingham in collaboration with the ADHSG: