When looking at endocrine disorders, it’s often helpful to look at them in pairs whenever possible. For example, a few weeks back we talked about Hashimoto’s and then followed that up with Grave’s disease. Next, we’ll be looking at two endocrine conditions related to the adrenal glands: Cushing’s Disease and Addison’s Disease. Want this on audio? Tune in to Episode 77 on the Straight A Nursing Podcast.

Addison’s Disease

We’ll start with Addison’s Disease, which is also known as adrenal insufficiency. In this disorder, the patient suffers from decreased cortisol levels and, often, decreased aldosterone levels as well. Remember that cortisol is a steroid hormone often referred to as the body’s “stress hormone.” Aldosterone is a steroid as well, and its main role in the body is to maintain salt and water balance as a component of blood pressure regulation. 

There are two types of adrenal insufficiency: Primary and Secondary

Primary adrenal insufficiency occurs when the cortex of the adrenal gland is damaged and, as a result, does not produce adequate hormones. This can be due to autoimmune disease (the most common reason), tuberculosis, trauma or bleeding into the adrenal glands, removal of the adrenal glands, cancer and infection. 

Secondary adrenal insufficiency occurs due to problems with the pituitary gland, which as you recall is responsible for making and secreting ACTH. The malfunction can occur due to a pituitary tumor, trauma caused by surgery or inflammation. It can also occur temporarily when patients who have been taking corticosteroids for a chronic condition suddenly stop taking them instead of tapering off them slowly. 

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So let’s look a patient with Addison’s Disease using the Straight A Nursing LATTE method.

L: How will the patient LOOK? 

Pt will be thin, complain of decreased appetite, nausea, vomiting and weight loss. She may complain of feeling extremely fatigued and depressed. You may notice darkened areas of the skin (with primary adrenal insufficiency) and loss of body hair. The patient may complain of joint pain and abdominal pain. She may be irritable and possibly complain of decreased libido. The patient may also have low blood sugar and low blood pressure. One of the hallmark symptoms is that the patient may state they crave salt.

A: How do you ASSESS the patient?

When assessing patients with Addison’s disease, you’ll want to essentially watch for all those things they’re at risk for. Measure their blood pressure, assess pain, ask about depression and level of fatigue. Because this patient is at risk for decreased appetite, you’ll want to make sure you weigh them and ask about their eating habits. When talking with the patient about their eating habits, be sure to ask if they consume or crave salty snacks.

T: What TESTS will be conducted?

  • Blood test to assess the level of ACTH as well as sodium, potassium, cortisol, and antibodies associated with autoimmune Addison’s disease.
  • ACTH Stimulation Test: In this test, the patient’s cortisol level is tested at baseline and then again after an injection of synthetic ACTH. ACTH stimulates the adrenal glands to produce cortisol. The normal response in a healthy individual is for the cortisol level to increase after the injection; if it does not, then it is a positive result for Addison’s.
  • Insulin Tolerance Test: In a healthy individual with healthy adrenal glands, cortisol is released in response to hypoglycemia. In this test, the patient is given insulin to purposefully lower the blood glucose. Cortisol levels are checked prior to the insulin and again after to see if the hypoglycemia lead to cortisol release. If it does not, then this is indicative of Addison’s disease. Because this is a high-risk and potentially dangerous test to conduct, it is typically only performed by endocrinologists. It is also avoided in people who have very low baseline cortisol as the hypoglycemia could trigger an Addisonian crisis.
  • Fasting blood glucose test to assess for hypoglycemia
  • CRH Stimulation Test: Corticotropin-releasing hormone (CRH) triggers the pituitary gland to secrete ACTH, which is the hormone that causes the pituitary to secrete cortisol. In this test, a cortisol level is measured and then the patient is injected with synthetic CRH. The cortisol level is then measured every 30 minutes to see if there has been an increase. This test is used to determine if the adrenal insufficiency is due to a pituitary problem or a problem with the adrenal glands themselves. When the problem is with the adrenal glands, this is called primary adrenal insufficiency, and when it’s the pituitary it’s referred to as secondary adrenal insufficiency. 
  • CT scan may be done to assess the size of the adrenal glands
  • MRI to assess the pituitary gland if secondary adrenal insufficiency is suspected

T: What Treatments Will Be Provided?

Patients with adrenal insufficiency will need hormone replacement therapy. This includes Hydrocortisone, methylpredisolone, dexamethasone or prednisone as replacements for cortisol. Fludrocortisone acetate is taken to replace aldosterone. 

E: How will you educate the patient and family?

  • The patient will need to eat a fair amount of salt due to the aldosterone insufficiency, especially when it’s hot out, during exercise or if the patient has diarrhea. 
  • The patient should carry a medical alert card or bracelet.
  • The patient should be advised to always have plenty of medication on hand as running out could be harmful, even if just missing one day.
  • The patient should carry an emergency kit that contains a steroid injection.
  • The patient may need to increase their medication dosage when experiencing stress such as when ill, experiencing a crisis, suffering from an injury or undergoing intense physical exertion
  • The patient needs to understand the signs of adrenal crisis, also known as Addisonian Crisis. This occurs when the body’s demands for cortisol exceed its supply. This can occur during times of stress, injury, illness, infection and physical exertion. This is a life-threatening condition that results in hypotension, hypoglycemia and hyperkalemia. Some of the signs are nausea and vomiting, abdominal pain, confusion, chills, fever, hypotension, severe weakness, dizziness, headache, fainting and tachycardia. If they experience these symptoms, they are to seek emergent medical care immediately. If the patient carries emergency dexamethasone make sure they understand how to inject the medication.

Cushing’s Disease

Cushing’s Disease is essentially the opposite of Addison’s disease. Whereas Addison’s was about not having enough hormone, Cushing’s occurs when there’s an excess of cortisol hormone. For that reason it is also called hypercortisolism. It can be caused by long-term use of corticosteroids as well as physiological disorders such as: 

  • adrenal gland disease
  • pituitary tumors, causing excess ACTH release, which results in the adrenal glands producing and secreting excess cortisol
  • tumors that secrete ACTH; these are often found in other parts of the body such as the lungs, thyroid, thymus or pancreas (referred to as “ectopic ACTH syndrome”)
  • familial Cushing syndrome, which causes tumors to develop on the adrenal glands. 

Left untreated, Cushing’s may lead to chronic infections, type 2 diabetes, hypertension and bone loss. Let’s go through Cushing’s disease using the LATTE method so you can see how the two opposite conditions compare with one another: 

L: How will the patient LOOK? 

The patient will carry excess weight in the trunk, yet have thin arms and legs. These patients also have a rounded face (referred to as a “moon face”) and a fat pad at the base of the neck, often called a “buffalo hump.” Their cheeks may be red and they probably have thin skin that bruises easily and heals slowly. The patient could likely also have high blood pressure and type 2 diabetes mellitus. You may also notice stretch marks at the abdomen and breasts. Patients with Cushing’s Disease often state they are suffering from weakness/fatigue, depression, joint pain, headache and cognitive difficulties (“brain fog”). Women may have irregular periods and hirsutism, while men often complain of decreased libido and erectile dysfunction. 

A: How will you ASSESS the patient?

  • Blood pressure
  • Current corticosteroid regimen (if that’s the cause)
  • Bone scan to test for osteoporosis
  • Assess any wounds for signs of infection
  • Assess level of fatigue
  • Monitor patient for signs of depression

T: What TESTS will be ordered?

  • Tests to assess cortisol level via blood, urine and/or saliva. 
    • Blood tests are typically done twice in the same day, due to the levels of cortisol changing frequently throughout the day
    • A urine test is typically a 24-hour collection
    • The saliva test is done at night because cortisol is normally lowest between 11pm and midnight.  If the level is high, this is indicative of Cushing’s.
  • Blood test to assess glucose level
  • CT or MRI to assess adrenal glands and pituitary
  • Scintigraphy is used to identify ACTH-secreting tumors located outside the adrenals or pituitary gland. It involves an imaging study conducted after the injection of a radioactive substance.
  • Petrosal sinus test which measures levels of ACTH from the petrosal sinuses (which are veins that drain the pituitary gland) and compares that against blood taken from a vein in the arm. 
  • High-dose dexamethasone suppression test – The patient is injected with a high dose of dexamethasone, which usually suppresses ACTH production by pituitary tumors. If the levels do not fall, it is suspected that the root cause is a tumor not associated with the pituitary gland. 

T: What TREATMENTS will be given?

Because many patients have Cushing’s syndrome secondary to corticosteroid use, the first course of treatment may be to see if the regimen can be decreased or replaced with other medications. Note that patients who have been taking long-term steroids must NOT stop taking them abruptly. Doing so could put them into an Addisonian crisis. They will need to taper slowly so the body can adjust and get used to making cortisol on its own.

There are several medications that control cortisol production at the adrenal gland, and a combination drug for people with both Cushing’s and type 2 diabetes is also available (mifepristone). A new medication on the market is pasireotide (Signifor), which targets the pituitary tumor when surgery does not resolve the problem or is not an option.

In cases of ACTH-secreting tumors, surgery (with or without radiation) may be needed. After removal, the patient will need to take corticosteroids as the body adjusts and returns to normal adrenal function. 

If all else fails, the patient may need to undergo a bilateral adrenalectomy, which will cause the patient to be deficient in cortisol and dependent on replacement for the rest of his life. 

E: How will you EDUCATE the patient and the family?

  • Teach the patient that they may need to take medication for life
  • After treatment, symptoms will usually resolve slowly and may take up to 2-12 months
  • Cushing’s puts the patient at risk for bone loss, so the patient should be instructed to engage in weight-bearing exercise (water aerobics is excellent) and get adequate calcium. 
  • Options for hirsutism include creams, chemical hair removal, bleaching, waxing, shaving, electrolysis and laser hair removal. 
  • General education about the management of type 2 diabetes, if present.
  • Home remedies for the joint pain that often accompanies Cushing’s disease, such as gentle exercise and NSAIDs.

Compare and Contrast

So now that we’ve taken a look at both Addison’s and Cushing’s, let’s review briefly as we look at them side-by-side.

Addisons Cushing’s
Too little cortisol Too much cortisol
Thin Adipose tissue at trunk, upper back and face
Thin arms/legs
Loss of body hair Too much body hair
Hypoglycemia Hyperglycemia
Hypotension Hypertension

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References

Endocrine Web. (n.d.). Addison’s Disease Diagnosis. Retrieved from https://www.endocrineweb.com/conditions/addisons-disease/addison-disease-diagnosis

Mayo Clinic (n.d.). Addison’s Disease. Retrieved from https://www.mayoclinic.org/diseases-conditions/addisons-disease/symptoms-causes/syc-20350293

MedicineNet. (n.d.). Addison’s Disease: Get the Facts on Treatment. Retrieved from https://www.medicinenet.com/addison_disease/article.h

My Health Aberta. (n.d.). Cortisol in Urine. Retrieved from https://myhealth.alberta.ca/Health/Pages/conditions.aspx?hwid=tw12871&lang=en-ca

Niemann, L. K. (2019, April 12). Patient education: Cushing’s syndrome (beyond the basics). Retrieved from https://www.uptodate.com/contents/cushings-syndrome-beyond-the-basics#H3211988350

Society for Endocrinology. (n.d.). You and Your Hormones. Retrieved from https://www.yourhormones.info/hormones/cortisol/

WebMD. (2017, February 6). What Is a Cortisol Test? Retrieved from https://www.webmd.com/a-to-z-guides/cortisol-test#1