ADDISON’S DISEASE

Definition:

• Hyposecretion of the adrenal cortex hormones

• 90% of the gland is destroyed• Originates within the adrenal gland and

is characterized by the decreased secretion of mineralocorticoids, glucocorticoids and androgens.

• Usually caused by an auto immune process

• Uncommon; At any age and in both sexes

Causes:

- Bilateral adrenelectomy

- Hemorrhage into adrenal glands

- TB, histoplasmosis, cytomegalovirus

- Family history

•"Addisonian crisis" or "adrenal crisis” - is a constellation of symptoms that indicate severe adrenal insufficiency.

•This may be the result of either previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal, or an intercurrent problem in someone known to have Addison's disease.

•It is a medical emergency and potentially life-threatening situation requiring immediate emergency treatment.

ADDISON’S CRISIS

Addison’s Crisis

CHARACTERISTIC SIGNS AND SYMPTOMS

hyperkalemia due to reduced aldosterone secretion.

BUN increase, as the GFR is reduced.

Hyponatremia is caused by reduced aldosterone

secretion.

Reduced cortisol secretion leads to impaired

glycogenesis and a reduction of glycogen in the liver and

muscle, causing hypoglycemia.

• Sudden penetrating pain in the legs, lower back or abdomen

• Severe vomiting and diarrhea

• dehydration

• Low blood pressure

• loss of consciousness and ability to stand

• Confusion, psychosis, slurred speech

• Severe lethargy

ADDISON’S DISEASE

PATHOPHYSIOLOGY

ETIOLOGY

(Autoimmune: e.g. TB, Fungal Infections, AIDS)

Destruction of own tissue

Affects functioning of Adrenal Cortex

Decrease production of Mineralocorticoid and glucocorticoids

• Cortisol deficiency causes altered metabolism, decreased stress tolerance, and emotional ability.

• 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.

ADDISON”S DISEASE

ETIOLOGY

(Defects, surgically removed)

Depressed Anterior Pituitary gland function

Decreased ACTH production

Depressed adrenal stimulation

Affects functioning of Adrenal Cortex

Decrease production of Mineralocorticoid and glucocorticoids

ADDISON”S DISEASE

Addison’s Disease/Crisis

DIAGNOSTIC TESTS

• This test involves measuring the level of cortisol in a patient’s blood before and after an injection of synthetic ACTH.

• If the adrenal glands are functional, cortisol levels will rise in response to the ACTH stimulation.

• If they are damaged or non-functional, then their response to ACTH will be minimal.

• Normal: double from a baseline of 20-30 ug/dl within 60 minutes

ACTH STIMULATION TEST

• Serum Sodium levels: “low” (hyponatremia)

• Serum Potassium levels: “high” (hyperkalemia)

• ABGs: metabolic acidosis

• FBS: “low” (hypoglycemia)

• X-rays: presence of granuloma (TB infection)

• Serum cortisol levels: “low”

• Serum ACTH levels: “high”

• Serum Aldosterone: “decreased”

Addison’s Disease/Crisis

MEDICAL MANAGEMENT

• Treatment of Addison's disease involves replacing, or substituting, the hormones that the adrenal glands are not making.

• Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day.

• If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid called fludrocortisone acetate (Florinef), which is taken once a day.

HORMONE REPLACEMENT THERAPY

• An ample intake of sodium is recommended, especially during heavy exercise, when the weather is hot, or if you have gastrointestinal upsets, such as diarrhea.

• Your doctor will also suggest a temporary increase in your dosage if you're facing a stressful situation, such as an operation, an infection or a minor illness.

Treatment must usually be continued for life.

• Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar).

• This treatment usually brings rapid improvement.

• When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved.

• If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.

ADDISON’S CRISIS

NURSING DIAGNOSIS

• Fluid volume deficit, related to hypovolemia and vomiting secondary to adrenal insufficiency

• Decreased cardiac output, related to hypovolemia

• Risk for imbalanced nutrition: less than body requirements

• Risk for injury related to weakness

NURSING DIAGNOSIS

• Disturbed body image related to change in skin pigmentation

• Activity intolerance related to weakness, fatigue, and/or muscle aches

• Disturbed thought process related to depression and irritability

NURSING INTERVENTIONS

• Monitor vital signs, especially BP

• Monitor intake and output closely.

• Take and record weight at the same time daily.

• Monitor electrolytes, and report abnormal results.

• Provide small, frequent feedings.

NURSING INTERVENTIONS

• Decrease stress in the environment.

• Prevent exposure to infection.

• Provide rest periods to prevent fatigue.

• Provide diet high in protein, Na and carbohydrates; low K

• Administer steroids after meals or with milk.

• Provide written and verbal instructions on administering steroids

NURSING INTERVENTIONS

• Monitor neurological status, noting irritability and confusion.

• Administer IV fluids (5% dextrose in saline, plasma) as ordered to treat vascular collapse

• Administer IV glucocorticoids as ordered