ADDISON’S DISEASE

ADDISON'S DISEASE. 

Addison’s Disease (Adrenocortical Insufficiency) 

Addison’s disease occurs when the adrenal cortex function is inadequate to meet the patient’s need for cortical hormones. Autoimmune or idiopathic atrophy of the adrenal glands is responsible for the vast majority of cases. Other causes include surgical removal of both adrenal glands or infection (tuberculosis or histoplasmosis) of the adrenal glands. Inadequate secretion of adrenocorticotropic hormone (ACTH) from the primary pituitary gland also results in adrenal insufficiency. Therapeutic use of corticosteroids is the most common cause of adrenocortical insufficiency. Symptoms may also result from sudden cessation of exogenous adrenocortical hormonal therapy, which interferes with normal feedback mechanisms. 

Clinical Manifestations 

Chief clinical manifestations 

include muscle weakness, anorexia, GI symptoms, fatigue, emaciation, dark pigmentation of the skin and mucous membranes, hypotension, low blood glucose, low serum sodium, and high serum potassium. The onset usually occurs with nonspecific symptoms. Mental changes (depression, emotional lability, apathy, and confusion) are present in 60% to 80% of patients. In severe cases, disturbance of sodium and potassium metabolism may be marked by depletion of sodium and water and severe, chronic dehydration. Addisonian Crisis 

This medical emergency develops as the disease progresses. 

Signs and symptoms include the following: 

• Cyanosis and classic signs of circulatory shock: pallor, apprehension, rapid and weak pulse, rapid respirations, and low blood pressure. •Headache, nausea, abdominal pain, diarrhea, confusion, and restlessness. •Slight overexertion, exposure to cold, acute infections, or a decrease in salt intake may lead to circulatory collapse, shock, and death.

 •Stress of surgery or dehydration from preparation for diagnostic tests or surgery may precipitate addisonian or hypotensive crisis.

Assessment and Diagnostic Findings 

Greatly increased plasma ACTH (more than 22.0 pmol/L); serum cortisol level lower than normal (less than 165 nmol/L) or in the low-normal range; decreased blood glucose (hypoglycemia) and sodium (hyponatremia) levels, increased serum potassium concentration (hyperkalemia), and increased WBC count (leukocytosis).

 Medical Management 

Immediate treatment is directed toward combating circulatory shock: 

• Restore blood circulation, administer fluids and corticosteroids, monitor vital signs, and place patient in a recumbent position with legs elevated. 

•Administer IV hydrocortisone, followed by 5% dextrose in normal saline. •Vasopressor amines may be required if hypotension persists. •Antibiotics may be administered if infection has precipitated adrenal crisis.

 •Oral intake may be initiated as soon as tolerated.

 •If adrenal gland does not regain function, lifelong replacement of corticosteroids and mineralocorticoids is required. 

•Dietary intake should be supplemented with salt during times of GI losses of fluids through vomiting and diarrhea. 

Nursing Management 

Assessing the Patient 

Assessment focuses on fluid imbalance and stress. 

• Monitor blood pressure and pulse rate as the patient moves from a lying, sitting, and standing position to assess for inadequate fluid volume.

 •Assess skin color and turgor. 

•Assess history of weight changes, muscle weakness, and fatigue. •Ask patient and family about onset of illness or increased stress that may have precipitated crisis. 

Monitoring and Managing Addisonian Crisis

 •Monitor for signs and symptoms indicative of addisonian crisis, which can include shock; hypotension; rapid, weak pulse; rapid respiratory rate; pallor; and extreme weakness.

•Advise patient to avoid physical and psychological stressors such as cold exposure, overexertion, infection, and emotional distress. •Immediately treat patient with addisonian crisis with IV administration of fluid, glucose, and electrolytes, especially sodium; replacement of missing steroid hormones; and vasopressors. •Anticipate and meet the patient’s needs to promote return to a precrisis state. Restoring Fluid Balance •Encourage the patient to consume foods and fluids that assist in 

Restoring and maintaining fluid and electrolyte balance. •Along with the dietitian, help the patient to select foods high in sodium during GI tract disturbances and in very hot weather. 

•Instruct the patient and family to administer hormone replacement as prescribed and to modify the dosage during illness and other stressful situations. 

•Provide written and verbal instructions about the administration of mineralocorticoid (Florinef) or corticosteroid (prednisone) as prescribed. Improving Activity Tolerance •Avoid unnecessary activities and stress that might precipitate a hypotensive episode. •Detect signs of infection or presence of stressors that may have triggered the crisis.

 •Explain rationale for minimizing stress during acute crisis. 

Promoting Home- and Community-Based Care 

Teaching Patients Self-Care 

•Give patient and family explicit verbal and written instructions about the rationale for replacement therapy and proper dosage. •Teach patient and family how to modify drug dosage and increase salt intake in times of illness, very hot weather, and stressful situations.

 •Instruct patient to modify diet and fluid intake to maintain f luid and electrolyte balance.

•Provide patient and family with preloaded, single-injection syringes of corticosteroid for use in emergencies and instruct when and how to use.

 •Advise patient to inform health care providers (eg, dentists) of steroid use. 

•Urge patient to wear a medical alert bracelet and to carry information at all times about the need for corticosteroids. •Teach patient and family signs of excessive or insufficient hormone replacement. 

Continuing Care

 •If patient cannot return to work and family responsibilities after hospital discharge, refer to home health care nurse to assess the patient’s recovery, monitor hormone replacement, and evaluate stress in the home. 

•Assess patient’s and family’s knowledge about medication therapy and dietary modifications. 

•Assess patient’s plans for follow-up visits to clinic or physician’s office. 

•Remind the patient and family about the importance of participating in health promotion activities and health screening.