Hypoadrenalism, (Primary) Adrenal Insufficiency
Age range at onset:
Specialists you may see:
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.
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
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
Muscle weakness and cramps
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. 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.
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).