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What is Addison's Disease?

A primary adrenocortical insufficiency in which all three classes of adrenal steroids-glucocorticoids, mineralcorticoids, and andgrogens-are reduced because of adrenal cortex hypofunction
- in secondary adrenocortical insufficiency (lack of pituitary adrenocorticotropic hormone (ACTH) secretion) corticosteroids and androgens are deficient, but mineralcorticoids rarely are


Up to 80% of Addison's disease cases in the US are caused by?

An autoimmune response
-autoimmune adrenalitis causes adrenal cortex to be destroyed by antibodies. This results in loss of glucocortiocid, mineralcorticoid, and adrenal androgen hormones. Addison's disease can be present along with other endocrine disorders. This condition is known as autoimmune polyglandular syndrome


Other causes of Addison's disease include?

Amyloidosis, fungal infections (histoplasmosis), AIDS, and metastatic cancer
-Iatrogenic Addison's disease may be due to adrenal hemorrhage, often related to anticoagulant therapy, chemotherapy, ketoconazole therapy for AIDS, or bilateral adrenalectomy


Clinical Manifestations of Addison's Disease

-Slow (insidious) onset
-Anorexia (loss of appetite)
-Progressive weakness
-Weight loss
-Skin hyperpigmentation seen in sun exposed areas of body; at pressure points; over joints; in creases (palmar creases)
-Abdominal pain
-Orthostatic hypotension
-Salt craving
-Joint pain


Patients with adrenocortical insufficiency are at risk for addisonian crisis (acute adrenal insufficiency) what is it?

A life-threatening emergency caused by critically low levels of adrenocortical hormones
-most dangerous manifestation is hypotension that may cause shock, especially during stress. Circulatory collapse is often unresponsive to usual Tx (vasopressors and fluid replacement)
-may be triggered by stress (from infection, trauma, surgery, or psychologic), sudden withdrawal of corticosteroid hormone therapy (often by patient lacking knowledge regarding replacement therapy), adrenal surgery, or sudden pituitary gland destruction


Addison's disease diagnostic studies

- Plasma cortisol levels are subnormal or fail to rise over basal levels with an ACTH stimulation test. A positive response to ACTH stimulation indicates a functioning adrenal gland and points to pituitary disease rather than adrenal disease
-Urine levels of free cortisol and aldosterone are low
-Serum electrolytes show hyperkalemia, hypochloremia, and hyponatremia
-CT and MRI used to localize other causes including tumors, fungal infections, and TB


Addison's Disease interprofessional care

-Tx focused on managing underlying cause. The mainstay of Tx is lifelong hormone therapy with glucocorticoids and mineralocorticoids. Pt's who take their meds consistently can anticipate normal life expectancy
-HYDROCORTISONE: hormone therapy, has both glucocorticoid and mineralocorticoid properties. Dosage increased in stressful situations to prevent addisonian crisis
-Mineralocorticoids replaced with fludrocortisone and increased dietary salt intake
-Women need androgen replacement with dehydroepiandrosterone (DHEA), because their only source of andgroen production is the adrenal glands


The patient in addisonian crisis requires?

Immediate aggressive shock management and high-dose hydrocortisone replacement. Large volumes of 0.9% saline solution and 5% dextrose are administered to reverse hypotension and electrolyte imbalances until BP returns to normal


Addison's disease nursing management:
When the patient is hospitalized, nursing management focuses on monitoring the patient while correcting fluid and electrolyte balance

1) Asses VS and signs of fluid volume deficit and electrolyte imbalance. Monitor trends in serum glucose, sodium, and potassium
2) Daily weights, protection against exposure to infection, and assistance with daily hygiene
3) Protect from noise, bright light, and environmental temp. extremes. Pt can't cope with stress because corticosteroids cannot be produced
4) If hospitalized for adrenal crisis, pt's usually respond by second day and can start oral corticosteroids replacement
5) Because discharge frequently occurs before usual maintenance dose of corticosteroids is reached, the pt should be instructed on the importance of keeping scheduled follow-up appts.


Patient education for Addison's Disease

1) Teach S/S of corticosteroid deficiency and excess and report these to HCP so dosage can be adjusted
2) Critical that pt wear medical ID bracelet and carry wallet card stating pt has Addison's disease, so that appropriate therapy can be initiated in emergencies
3) Pt's should carry emergency kit with 100mg of IM methylprednisolone (Solu-Medrol), syringes, and instructions for use when hormone therapy cannot be taken orally. Have pt verbalize instructions, practice IM injections w/normal saline, and carry written instructions on when the dosage should be changed
4) Teach conditions requiring increased medication (trauma, infection, surgery, emotional crisis)
5) Teach prevention of infection and need for prompt vigorous Tx of existing infections
6) Provide management instructions for pt's who have diabetes and those who have elevated blood glucose levels when taking corticosteroids