Adrenal Insufficiency (Addison Disease)

General

DRG Category: 644

Mean LOS: 4.3 days

Description MEDICAL Endocrine Disorders With Complication or Comorbidity

Introduction

Adrenal insufficiency occurs when the adrenal glands do not produce adequate amounts of hormones to support the body’s function. The medulla, the inner portion of the adrenal gland, is responsible for the secretion of the catecholamines epinephrine and norepinephrine; the cortex, or outer portion, is responsible for the secretion of glucocorticoids, mineralocorticoids, and androgen. The principal glucocorticoid, cortisol, helps regulate blood pressure, metabolism, anti-inflammatory response, and emotional behavior. The principal mineralocorticoid, aldosterone, is important for regulating sodium levels. Adrenal insufficiency is characterized by the decreased production of cortisol, aldosterone, and androgen. Cortisol deficiency causes altered metabolism, decreased stress tolerance, and emotional lability. Aldosterone deficiency causes urinary loss of sodium, chloride, and water, resulting in dehydration and electrolyte imbalances. Androgen deficiency leads to the loss of secondary sex characteristics. The major cause of illness or death for people with adrenal insufficiency is a delay in making the diagnosis, or failure to begin appropriate therapy with glucocorticoids and/or mineralocorticoids. Complications include bone loss, hypothyroidism, ischemic heart disease, acute adrenal insufficiency, and shock.

Causes

Adrenal insufficiency can be primary or secondary. Addison disease, or primary adrenal insufficiency, occurs rarely, but when it occurs, there is loss of at least 90% of the adrenal cortex. Idiopathic adrenal atrophy is the most common cause of primary adrenal insufficiency. It is not known exactly why this occurs, but it is believed to be related to an autoimmune response that results in the slow destruction of adrenal tissue. Tuberculosis, histoplasmosis, AIDS, malignancies, and hemorrhage into the adrenal glands have all been associated with destruction of the adrenal glands. Secondary insufficiency occurs from disorders of the pituitary gland, which produces hormones to regulate the adrenal gland. It results from hypopituitarism due to hypothalamic-pituitary disease or suppression of the hypothalamic-pituitary axis by corticosteroid therapy. Secondary adrenal failure can also occur with adrenocorticotropic hormone (ACTH) deficiency caused by disorders of the pituitary. Other causes include physiological stress, including surgery, anesthesia, fluid volume loss, trauma, asthma, hypothermia, alcohol abuse, myocardial infarction, fever, hypoglycemia, pain, and depression. Approximately 8% of people with adrenal insufficiency require hospital treatment each year for adrenal crisis (see Adrenal Crisis, Acute).

Genetic Considerations

Addison disease has a strong genetic component, with a recent twin study estimating a heritability of 97%. The most well-established risk factors include variants in the human leukocyte antigen (HLA) genes, which follow a recessive pattern of inheritance. There is also a rare form of X-linked congenital adrenal insufficiency that is associated with mutations in the NR0B1 gene that produce the DAX1 protein. Congenital adrenal hyperplasia is characterized by autosomal recessive mutations in one of the enzymes required for cortisol synthesis, impairing cortisol production. Adrenal insufficiency is often seen in families in association with other autoimmune disorders, most commonly with autoimmune polyendocrine syndrome (APS).

Sex Life Span Considerations

Addison disease, particularly idiopathic autoimmune Addison disease, occurs most often in adults from ages 30 to 50 years and affects females more than males. Children may develop the condition if they have genetic susceptibility.

Health Disparities Sexual/Gender Minority Health

Ethnicity, race, and sexual/gender minority status have no known effect on the risk for Addison disease.

Global Health Considerations

In developed countries, the incidence in Western countries is 50 cases per 1 million persons. No data are available on Eastern countries or many developing countries.

Assessment

History

Determine if the patient has a history of recent infection, use of corticosteroids such as prednisone, or adrenal or pituitary surgery. Patients may describe vague symptoms such as weakness, fatigue, dizziness, and weight loss. They may experience hyperpigmentation of the skin (particularly on the knuckles, elbows, knees, palmar creases), nailbeds, and mucous membranes because of melanin overproduction. Hyperpigmentation may have lasted for months or even years. Establish a history of poor tolerance for stress, weakness, fatigue, and activity intolerance. Ask if the patient has experienced anorexia, nausea, vomiting, or diarrhea as a result of altered metabolism. Some patients have dizziness with orthostasis due to hypotension. Elicit a history of craving for salt or intolerance to cold. Determine presence of altered menses in females and impotence in males.

Physical Examination

Assess the patient for signs of dehydration such as tachycardia, altered level of consciousness, dry skin with poor turgor, dry mucous membranes, weight loss, and weak peripheral pulses. Check for postural hypotension—that is, a drop in systolic blood pressure greater than 15 mm Hg when the patient is moved from a lying position to a sitting or standing position.

Inspect the skin for pigmentation changes caused by an altered regulation of melanin, noting if surgical scars, skin folds, and genitalia show a characteristic bronze color. Inspect the patient’s gums and oral mucous membranes to see if they are bluish-black. Take the patient’s temperature to see if it is subnormal. Note any loss of axillary and pubic hair that could be caused by decreased androgen levels.

Psychosocial

Because an acute adrenal crisis may be precipitated by emotional stress, periodic psychosocial assessments are necessary for patients with adrenal insufficiency. Patients with an adrenal insufficiency frequently complain of weakness and fatigue, which are also characteristic of an emotional problem. However, weakness and fatigue of an emotional origin seem to have a pattern of being worse in the morning and lessening throughout the day, whereas the weakness and fatigue of adrenal insufficiency seem to be precipitated by activity and lessen with rest. Patients with adrenal insufficiency may show signs of depression and irritability from decreased cortisol levels.

Diagnostic Highlights

General Comments: To determine if a cortisol deficit exists, a plasma cortisol level is drawn in the morning; less than 10 mcg/dL suggests adrenal insufficiency; further testing may be needed to determine if the adrenal glands have a primary deficiency or if the pituitary cannot produce enough ACTH (secondary adrenal failure).

Test	Normal Result	Abnormality With Condition	Explanation
Serum cortisol level (adult)	6-8 a.m. levels should be 5-25 mcg/dL	Decreased	Determines the ability of the adrenal gland to produce glucocorticoids
Serum electrolytes and chemistries (adult)	Sodium 135-145 mEq/L; potassium 3.5-5.3 mEq/L; blood urea nitrogen 8-21 mg/dL; glucose 70-100 mg/dL	Hyponatremia; hyperkalemia; azotemia; hypoglycemia	Values reflect sodium loss from a deficit in mineralocorticoids with loss of fluids and poor glucose control because of decreased gluconeogenesis; most consistent finding is elevated blood urea nitrogen due to hypovolemia, decreased glomerular filtration rate, and decreased renal plasma flow
ACTH stimulation test	15-30 minutes after infusion of ACTH, the adrenal cortex releases two to five times its normal plasma cortisol level; the cortisol value should be above 20 mcg/dL and have increased at least 7 mcg/dL above baseline readings	Level ≤ 20 mcg/dL in 30 or 60 min	Adrenal glands cannot respond to the stimulation from ACTH and demonstrate that they are not functioning normally

Other Tests: Computed tomography of the abdomen; chest x-ray to determine heart size or presence of tuberculosis or fungal infections; metyrapone suppression test; urine 17-hydroxycorticosteroids (17-OHCS) and 17-ketosteroids (17-KS); thyroid-stimulating hormone; serum calcium; and electrocardiogram (ECG)

Primary Nursing Diagnosis

Diagnosis: Imbalanced nutrition: less than body requirements related to insufficient dietary intake as evidenced by anorexia, nausea, vomiting, and/or diarrhea

Outcomes: Fluid balance; Hydration; Nutritional status: Food and fluid intake; Nutritional status: Energy; Electrolyte & acid-base balance; Hypoglycemia severity; Hyponatremia severity; Hyperkalemia severity

Interventions: Fluid/electrolyte management: Hyponatremia, hyperkalemia; Hypoglycemia management; Nutritional management; Nutritional counseling

Planning and Implementation

Collaborative

Collaborative treatment of adrenal insufficiency focuses on restoring fluid, electrolyte, and hormone balances. The fluid used for adrenal insufficiency will most likely be 5% dextrose in 0.9% sodium chloride to replace fluid volume, glucose, and serum sodium. Correct sodium, potassium, calcium, and glucose abnormalities. Patients with adrenal insufficiency will require lifelong replacement glucocorticoid and mineralocorticoid therapy, which needs careful monitoring for signs of inadequate replacement. Patients on an appropriate maintenance dose should not experience morning weakness, dizziness, and headaches. Patients with diabetes mellitus will require insulin adjustments for elevated serum glucose levels.

Pharmacologic Highlights:

General Comments: Fludrocortisone promotes kidney reabsorption of sodium and the excretion of potassium. Overtreatment can result in fluid retention and possibly congestive heart failure; therefore, monitor serum potassium and sodium levels frequently during fludrocortisone administration.

Medication or Drug Class	Dosage	Description	Rationale
Glucocorticoids such as hydrocortisone, dexamethasone, and prednisone	Varies by drug	Corticosteroid	Replacement therapy in deficiency state
Fludrocortisone	0.1 mg PO daily	Mineralocorticoid	Replacement therapy in deficiency state

Independent

Because of the negative effect of physical and emotional stress on the patient with adrenal insufficiency, promote strategies that reduce stress. If the patient develops an infection or heavy cold, or has a procedure such as a minor surgery or tooth extraction, teach the patient to double or triple their corticosteroid dose or to check with the provider about increasing the dose. Teach the patient to rest between activities to conserve energy and to wear warm clothing to increase comfort and limit heat loss. To limit the risk of infection, encourage the patient to use good hand-washing techniques and to limit exposure to people with infections. To prevent complications, teach the patient to avoid using lotions that contain alcohol to prevent skin dryness and breakdown and to eat a nutritious diet that has adequate proteins, fats, and carbohydrates to maintain sodium and potassium balance.

Finally, the prospect of a chronic disease and the need to avoid stress may lead patients to impaired social interaction and ineffective coping. Discuss with the patient the presence of support systems and coping patterns. Provide emotional support by encouraging the patient to verbalize feelings about an altered body image and anxieties about the disease process. Incorporate the patient’s unique positive characteristics and strengths into the care plan. Encourage the patient to interact with family and significant others. Before discharge, refer patients who exhibit disabling behaviors to therapists, self-help groups, or crisis intervention centers.

Evidence Based Practice Health Policy

Margulies, S., Corrigan, K., Bathgate, S., & Macri, C. (2020). Addison’s disease in pregnancy: Case report, management, and review of literature. Journal of Neonatal-Prenatal Medicine, 13, 275-278.

While adrenal insufficiency is an unusual complication of pregnancy, pregnant patients are at risk for multiple complications. The authors reviewed symptoms that can occur and provided a case study.
Complications that occurred in a healthy, 34-year-old woman included lethargy, skin hyperpigmentation, frequent urination, and salt craving. Her laboratory results showed hyperkalemia, hyponatremia, elevated adrenocorticotropic hormone, and low cortisol.
She was treated with hydrocortisone and fludrocortisone, which resolved her symptoms. The authors suggested that during pregnancy, patients with adrenal insufficiency have their medications increased due to the stress of pregnancy.

Documentation Guidelines

Physical findings: Cardiovascular status, including blood pressure and heart rate; tissue perfusion status, including level of consciousness, skin temperature, peripheral pulses, and urine output; fluid volume status, including neck vein assessment, daily weights, and fluid input and output
Laboratory findings: Blood levels of potassium, sodium, hematocrit, and blood urea nitrogen (BUN)

Discharge and Home Healthcare Guidelines

PREVENTION To prevent acute adrenal crisis, teach patients how to avoid stress. Emphasize the need to take medications as prescribed and to contact the physician if the patient becomes stressed or unable to take medications. Make sure the patient knows to alert the surgeon about adrenal insufficiency prior to all surgical procedures. Parenteral corticosteroids will likely be prescribed during any major procedure or times of major stress or trauma.

MEDICATIONS Be sure the patient understands the reason for steroids that are prescribed. (See Box 1 for a full explanation.) Some patients will be taught to give themselves an IM injection of steroids to be administered if they are unable to take their medication orally.

RESOURCES Referrals may be necessary to identify potential physical and emotional problems. Notify the hospital’s social service department before patient discharge if you have identified obvious environmental stressors. Initiate home health nursing to ensure compliance with medical therapy and early detection of complications. If you identify emotional problems, refer the patient to therapists or self-help groups.

Box 1 Patient Teaching for Corticosteroids

Emphasize the lifetime nature of taking corticosteroids.
Provide name, dosage, and action of the prescribed medication.
Explain the common side effects of weight gain, swelling around the face and eyes, insomnia, bruising, gastric distress, gastric bleeding, and petechiae.
Advise the patient to take the medication with meals to avoid gastric irritation and to take the medication at the time of day prescribed, usually in the morning.
Suggest the patient weigh self daily, at the same time each day, and call the healthcare provider if weight changes by 5 pounds.
Emphasize that the patient should always take the medication. Not taking it can cause life-threatening complications. Tell the patient to call the healthcare provider if they are unable to take medication for more than 24 hours.
Explain that periods of stress require more medication. Tell the patient to call the healthcare provider for changes in dose if they experience extra physical or emotional stress. Illness and temperature extremes are considered stressors.
Explain preventative measures. Tell the patient that to prevent getting ill, they should avoid being in groups with people who are ill and environments where temperatures change from very hot to very cold.
Teach the patient to recognize signs of undermedication: weakness, fatigue, and dizziness. Emphasize the need to report underdosing to the healthcare provider.
Teach the patient to avoid dizziness by moving from a sitting to a standing position slowly.
Urge the patient to always wear a medical alert necklace or bracelet to inform healthcare professionals of the diagnosis.