A publication at AP Sense outlines some important steps of the early identification and treatment of Addison’s disease, a rare condition brought about by the body’s underproduction of certain hormones.

We built from that list to give you an even more comprehensive understanding of the condition and its management.

What is Addison’s Disease?

Addison’s disease is a highly uncommon condition, affecting only about one in every 100,000 in the United States.

Also known as adrenal insufficiency, Addison’s can develop if the adrenal glands are damaged somehow. If the damage is serious enough, the adrenal glands may start to produce inadequate amounts of the hormones cortisol and, frequently, aldosterone.

Aldosterone plays an important role in regulating blood pressure, mainly by acting on the kidneys and colon to regulate sodium and potassium levels in the blood and urine. Cortisol plays a more diverse role, performing a number of important functions around the body. Cortisol is partially responsible for regulating blood sugar levels, acting as an anti-inflammatory, and even contributing to the chemical process of memory formation.

Though Addison’s disease is generally somewhat manageable through the administration of hormone supplements, younger patients (diagnosed before age 40) have been observed to experience higher mortality rates. If well-managed, people with Addison’s have about the same life expectancy as an otherwise healthy individual – that makes early identification and aggressive treatment of the disease particularly important factors in a patient’s prognosis.

If left untreated, symptoms of Addison’s disease can arise quickly and aggressively. This can give rise to a condition called addisonian crisis, which can be fatal. Addisonian crises are signaled by a steep decline in blood pressure and sodium levels, and an overabundance of potassium. Symptoms include severe abdominal pain, confusion or delirium, and severe weakness.

Symptoms

Because of the many functions played by the hormones characteristically deficient in Addison’s patients, symptoms are varied in their character. Patients may experience:

• Fatigue
• Loss of appetite
• Skin darkening (“hyperpigmentation”)
• Irritability
• Loss of body hair
• Nausea and vomiting
• Drop in blood pressure
• Low blood sugar
• Abdominal pain
• Muscle and/or joint pain
• Salt craving

These symptoms may present slowly, growing worse with time.

Diagnosis

There are two “forms” of Addison’s disease that have nearly identical symptoms but differing causes.

Primary adrenal insufficiency is caused by damage directly sustained by the adrenal glands. Cancer, tuberculosis, and autoimmune disease can all cause adrenal failure and primary Addison’s.

Secondary adrenal insufficiency is caused by insufficient hormone production in the pituitary gland. Normally, hormone production in the adrenal glands is at least partially controlled by hormones produced elsewhere in the body. The pituitary gland, found in the brain, normally releases a hormone called ACTH to tell the adrenal glands to produce hormones. If ACTH levels of the pituitary gland are deficient, the adrenal glands may produce insufficient hormone levels in turn. The result is symptoms almost identical to primary adrenal insufficiency, but without damage to the adrenal glands. However, it’s worth noting that secondary Addison’s patients have a higher risk of low blood sugar than primary adrenal insufficiency patients, and do not experience hyperpigmentation.

Doctors use a number of tests to confirm the presence of Addison’s disease. These tests are also helpful for determining whether a case of adrenal insufficiency is either primary or secondary. Options include:

• Thyroid test – The thyroid is another gland, like the pituitary, responsible for triggering hormone production elsewhere in the body.
• Blood tests – Levels of sodium and potassium in the bloodstream are important markers of Addison’s disease.
• CT / MRI scans of adrenal glands – The physical condition of the adrenal glands can differentiate between Addison’s patients and healthy individuals, and also primary and secondary cases of adrenal insufficiency.
• Synacthen stimulation tests – If the need arises, tests can be ordered that directly sample levels of ACTH, cortisol, and aldosterone to more definitively identify cases of Addison’s.
  

Treatment

The good news about all of this is that today, Addison’s is relatively easy to treat with routine maintenance. Deficient hormones are replaced through the administration of various prescribed steroids to encourage normal body function.

Treatment options will likely include a mix of the following:

• Hydrocortisone / prednisone / methylprednisolone – Supplements for deficient cortisol levels
• Fludrocortisone acetate – Supplement for deficient aldosterone levels
  
• Dietary control
  • Increased consumption of salt
  • Controlled intake of protein, zinc, and magnesium

Emergency Considerations

Because Addison’s is a chronic condition, regular management and diligent observation of symptoms are important factors for ensuring long-term health.

To minimize the risks of a potential addisonian crisis, Addison’s patients may consider a steroid emergency card, which can inform physicians of their patient’s condition immediately upon admittance to the hospital.

For some cases, doctors may prescribe a glucocorticoid injection kit. This is a kit containing steroids, syringe, and needle for the emergency administration of steroids in the event of addisonian crisis.

Do you or does someone you know live with Addison’s disease? How does it affect your daily life? Patient Worthy wants to hear from you!