What Is Addison's Disease and How Is an Adrenal Crisis Treated?

President John F. Kennedy's birthday and the International Addison's Disease Day are the same—May 29. It's no coincidence. President Kennedy had Addison's Disease, a condition affecting his stress hormone or cortisol levels. His diagnosis came to light after his assassination.

A small group of other famous people with Addison's Disease includes his sister, Eunice Kennedy Shriver, Jane Austen, and Helen Reddy.

Let's look at what Addison's Disease is, its signs and symptoms, how it's diagnosed, how it's treated, and how you can save on prescriptions at a pharmacy nearby.

What is Addison's disease?

Addison's disease is named after Thomas Addison, who described the disease in the 1850s. Addison's disease is a condition in which the adrenal glands don't make enough cortisol and sometimes not enough aldosterone.

Let's break this down.

You have two adrenal glands, one nestled above each kidney. The adrenal glands' job is to produce two hormones:

• Cortisol: a steroid-type hormone that increases your blood sugar, helps you respond to stressful situations, and reduces inflammation
• Aldosterone: a steroid-type hormone that tells your kidneys to retain salt (sodium) and fluid, thereby raising your blood pressure

Addison's disease is also called adrenal insufficiency. This descriptive name sums up the process—your adrenal glands don't make sufficient cortisol and maybe not enough aldosterone.

Types of adrenal insufficiency

There are three types of adrenal insufficiency:

• Primary: your adrenal glands don't make enough hormones
• Secondary: your adrenal glands don't get a message from the pituitary gland to produce enough hormones
• Tertiary or Drug-Induced: communication breaks down at the beginning; your brain's hypothalamus doesn't send a message to the pituitary gland, which then doesn't signal the adrenal glands to make cortisol

Primary adrenal insufficiency

Addison's disease is the same as primary adrenal insufficiency. It results from direct damage to the adrenal glands, results in low cortisol levels, and makes it difficult for you to respond to physical stress.

Causes of primary adrenal insufficiency

With an autoimmune disorder, your body mistakes healthy adrenal cells for foreign cells and destroys them. Your body may attack the adrenal glands and prevent them from making cortisol and aldosterone. Up to 85% of people develop primary adrenal insufficiency in this way.

Besides the adrenal glands, your body can attack other glands and cells:

• The pancreas: causes Type I diabetes
• The parathyroid glands: causes low levels of blood calcium
• The thyroid: causes an enlarged thyroid and possibly abnormal thyroid hormone levels
• Hair follicles: causes hair loss

Although much less common, other causes of primary adrenal insufficiency include:

• Infections, including HIV/AIDS, fungal infections, blood infections, syphilis, tuberculosis
• Injury to adrenal glands from trauma, hemorrhage, or cancer
• Medications: certain antifungal and anesthesia medications
• Inherited disorders that affect how the adrenal glands develop or function

Secondary adrenal insufficiency

Secondary adrenal insufficiency occurs when the pituitary gland in your brain doesn't produce enough adrenocorticotropic hormone (ACTH). The hormone serves as a messenger, telling your adrenal glands to make cortisol. A low level of ACTH causes a low level of cortisol.

Secondary adrenal insufficiency has several causes, including:

• Autoimmune Disease
• A pituitary tumor
• Surgical removal of the pituitary gland
• Traumatic brain injury
• Bleeding into the pituitary gland
• Inherited disorders affecting the development or function of the pituitary gland

Tertiary or drug-induced adrenal insufficiency

Tertiary or drug-induced adrenal insufficiency occurs when your brain's hypothalamus doesn't produce enough corticotropin-releasing hormone (CRH). This condition affects the pituitary gland and, ultimately, the adrenal glands.

Although any disease or damage affecting the hypothalamus can cause tertiary adrenal insufficiency, most people develop it after prolonged steroid use.

Suppose you've taken steroid pills (prednisone or dexamethasone) for asthma or arthritis or have received multiple steroid injections for joint or back pain. In that case, you may develop tertiary or drug-induced adrenal insufficiency.

Here's how that happens:

1. Steroids reduce the amount of corticotropin-releasing hormone (CRH) made in your brain's hypothalamus.
2. With less CRH, the pituitary gland produces less ACTH.
3. With less ACTH, the adrenal glands produce less cortisol.

Who gets adrenal insufficiency?

An estimated 4 to 6 people in 100,000 are diagnosed with primary adrenal insufficiency in the United States each year. Most are diagnosed between 30 and 50 years of age. It affects men and women equally. The prevalence of adrenal insufficiency may be higher since some people are never diagnosed with the disease.

Secondary adrenal insufficiency is more common, affecting 15 to 28 people per 100,000. More women than men have secondary adrenal insufficiency. And the diagnosis is made later in life.

Some cases of autoimmune triggered adrenal insufficiency are inherited. Your risk of having Addison's Disease is higher if you or a family member have vitiligo, an underactive thyroid gland, or Type I Diabetes.

Signs and symptoms

The signs and symptoms of Addison's Disease overlap with many other medical conditions that are more common. You may develop symptoms so gradually that you barely notice the change in your health.

Here is a list of signs and symptoms you may have:

• Tiredness to extreme fatigue: the most common symptom
• Changes in your skin color: darkening of scars, darkening of knuckles or skin creases, white patches of skin (vitiligo), black freckles on your forehead, face, or shoulders
• Loss of appetite and weight loss
• Abdominal pain
• Nausea, vomiting, and diarrhea
• Low blood sugar
• Low blood pressure or fainting
• Craving salt
• Muscle and joint pain
• Depression, irritability, or difficulty concentrating
• In women, loss of body hair and a diminished sex drive

Adrenal crisis

Your first signs and symptoms of adrenal insufficiency may be an adrenal crisis. It occurs when you don't have enough cortisol to respond to a stressful event, such as an injury, severe infection, fever, other illness, or surgery.

Adrenal crisis is a life-threatening condition with these signs and symptoms:

• Low blood pressure and passing out
• Extreme weakness
• Confusion, delirium, or loss of consciousness
• Seizures
• Severe abdominal pain, vomiting, or diarrhea
• Lower back or leg pain

Diagnosing adrenal insufficiency

If you have signs and symptoms of Addison's Disease, you'll be given hormones by vein to pinpoint which body part isn't doing its job — the adrenal gland, pituitary gland, or hypothalamus:

• ACTH stimulation test: You'll be given ACTH, a hormone made in the pituitary gland. ACTH signals the adrenal glands to produce cortisol. If the blood cortisol level is low despite getting ACTH, you have primary adrenal insufficiency or a problem with your adrenal glands.
• Simultaneous cortisol and ACTH levels: low cortisol and high ACTH levels suggest primary adrenal insufficiency when you're not given any hormones for this test.
• Insulin tolerance test: insulin lowers your blood sugar. Your pituitary gland will send ACTH to the adrenal glands, telling them to produce cortisol and raise your blood sugar when it is too low. If you have secondary adrenal insufficiency, your blood sugar stays low in this test because the pituitary gland doesn't produce ACTH.
• CRH stimulation test: normally made in your brain's hypothalamus, CRH tells the pituitary gland to make ACTH. If the pituitary gland isn't working, the ACTH level remains low. If the ACTH level rises gradually, the problem stems from your hypothalamus, and your adrenal insufficiency is tertiary. This is secondary adrenal insufficiency.

Your healthcare provider may order an antibody test to see if your body is attacking the adrenal glands or pituitary gland (autoimmune disease).

Additional blood tests check for low sodium, high potassium, and low blood sugar.

And finally, you may also undergo imaging tests (x-ray, CT, or MRI) looking for causes of adrenal, pituitary, or hypothalamic damage.

Treating adrenal insufficiency

Cortisol replacement is the mainstay of treatment. Most commonly, your healthcare provider will prescribe hydrocortisone. Prednisone is prescribed less often.

If you also have low levels of aldosterone, you'll receive fludrocortisone.

You take these medications by mouth. You may need to increase the dose when you have a change in your health.

Treating adrenal crisis

Over the year, about 8% of people with adrenal insufficiency have a life-threatening adrenal crisis. This medical emergency may be caused by missing doses of oral steroids or added stress from infection, fever, an accident, or surgery.

The treatment is injected with hydrocortisone, called Solu-Cortef. Since speed is of the essence, some people carry their injections with them.

In addition to injected hydrocortisone, you'll be treated with:

• Intravenous fluids containing sodium
• Intravenous dextrose (sugar)

Ways to save on your prescription medications

To live your fullest and healthiest life, you'll need to take steroids every day. Finding the best price for your prescription at a pharmacy nearby helps your bottom line.

ScriptSave^® WellRx compares prices on prescription and over-the-counter medications, vitamins, and supplements within your neighborhood or city. You can find the lowest prescription prices quickly and easily online. Our 30-second video gives an excellent summary of how we help you save money.

WellRx also offers coupons for medications and prescription savings cards. Visit our website for more information about how prescription discount cards work and how to receive your free Rx savings card.

Nutrition and supplements

Bone loss is a side effect of the steroids you'll take to treat adrenal insufficiency. A diet rich in calcium and Vitamin D can help reduce bone loss:

• Fruits and vegetables: kale, broccoli, collard greens, soybeans, mushrooms, oranges
• Seafood: salmon, sardines, shrimp, tuna, trout
• Meat: beef liver, hamburger, chicken
• Dairy: milk, cheeses

If you're not getting enough calcium and Vitamin D (i.e., you can't just eat any more kale, sardines, or tofu), you can take calcium tablets with Vitamin D.

If your aldosterone levels are affected by adrenal insufficiency, you may need extra salt (sodium) in your diet because you lose too much sodium in your urine. Your healthcare provider or dietician will make recommendations based on the sodium level in your blood.

Living with adrenal insufficiency

Let your healthcare provider know if you need surgery, develop an infection, have an accident, can't tolerate oral steroids due to vomiting, or miss your steroid doses. You may temporarily need higher medication doses or injected steroids.

If you live in a remote place or are traveling without immediate access to a healthcare facility, ask your healthcare provider about a prescription for injectable hydrocortisone.

Be sure always to carry a card in your wallet and wear a medic alert bracelet stating you have adrenal insufficiency.

Teresa Otto, MD, is a freelance medical writer on a mission to inform readers about the positive impact of good nutrition and a healthy lifestyle. She is a retired anesthesiologist who practiced in Billings, Montana, for most of her career. She graduated from the University of Washington School of Medicine in Seattle and did her anesthesia residency and fellowship at New York University and Columbia-Presbyterian in New York.

References:

1. https://www.addisonsdisease.org.uk/famous-lives
2. https://www.ncbi.nlm.nih.gov/books/NBK441994/
3. https://www.healthdirect.gov.au/the-role-of-cortisol-in-the-body
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6. https://medlineplus.gov/genetics/condition/autoimmune-addison-disease/#causes
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