Addison's disease

"What is Addison's disease ?"Addison's disease is a rare endocrine disorder characterized

by a severe deficiency of hormones produced in the adrenal cortex.

Addison's affects between 1 and 4 in 100,000 people, including both men and women within all age groups. The disease tends to become clinically apparent during periods of metabolic stress or trauma.

Major disruption and illness can occur if the adrenal glands do not function properly. Cortisol is a glucocorticoid hormone that plays a vital role in the body.It mobilizes nutrients, regulates the metabolism of proteins, fats and carbohydrates, stimulates the liver to raise blood sugar levels, acts as an anti- inflammatory agent and helps the body respond to stress.

Addison's Disease can have a severe effect on all of these bodily systems .

Addison's diseaseWhat Causes Addison’s Disease?Approximately 70% of cases of Addison’s

disease are as a result of an auto-immune process.

The body’s immune system produces antibodies against the cells of the adrenal cortex, and slowly destroys the healthy cells. This is a slow process evolving over a long period and can take months, even years, to become clinically apparent

Famous Addisonians

United States President John F. Kennedy was one of the best-known Addison's disease sufferers and her sister Eunice Kennedy Shriver.

Osama bin-Laden  as he manifests all the key symptoms.

Addison's diseaseETIOLOGY:

Primary adrenocortical insufficiency (Addison's disease) - destruction and subsequent hypofunction of the adrenal cortex, usually caused by autoimmune process

Secondary adrenocortical insufficiency - ACTH deficiency from pituitary disease or suppression of hypothalamic-pituitary axis by corticosteroid treatment for nonendocrine disorders causes atrophy of adrenal cortex

Inadequate aldosterone produces disturbances of sodium,potassium, and water metabolism

Cortisol deficiency produces abnormal fat, protein, and carbohydrate metabolism: no cortisol during a period of stress can precipitate addisonian crisis, an exaggerated state of adrenal cortical insufficiency, and can lead to death.

Addison's diseaseRISK FACTORS:autoimmune diseasebilateral adrenal tuberculosisadrenal fungal infectionsbilateral adrenal hemorrhages due to

meningococcal septicemia, post partum, patient on anticoagulant therapy

Addison's diseaseSIGNS AND SYMPTOMS:

water loss, dehydration and hypovolemiamuscular weakness, fatigue, weight lossGI Problems-- anorexia, nausea, vomiting,

diarrhea, constipation, abdominal pain.hypotension, hypoglycemia, low basal

metabolic rate, increased insulin sensitivity.Mental Changes-- depression, irritability,

anxiety, apprehension caused by hypoglycemia and hypovolemia.

hyperpigmentation (darkening of an area of skin or nails caused by increased melanin.)

Addison's disease PATHOGENESIS:

Addison’s disease occurs when more then 90% of adrenal gland tissue is destroyed (primary Addison’s disease). The destruction can occur due to various causes:

Most frequently, it is due to autoimmune process, when immune system attacks adrenal glands.

Tuberculosis as a cause of Addison’s disease is more common in developed countries.

Rarely, adrenal glands can be destroyed by infections, cancer metastasis, amyloidosis, or hemorrhage. When pituitary gland fails to produce enough ACTH, adrenal glands are not stimulated to produce Cortisol and a secondary Addison’s disease develops. 

The idiopathic Addison’s disease is an autoimmune disease

Addison's diseaseCAUSES:

•Causes of adrenal insufficiency can be grouped by the way they cause the adrenals to produce insufficient cortisol.

•These are adrenal dysgenesis (the gland has not formed adequately during development), impaired steroidogenesis (the gland is present but is biochemically unable to produce cortisol) or adrenal destruction (disease processes leading to the gland being damaged).

Addison's disease MEDICAL MANAGEMENT:

Restoration of normal fluid and electrolyte balance: high sodium, low-potassium diet and fluids.

Treatment of glucocorticoids deficiency with such agent as hydrocortisone (Cortef) or prednisone (Orasone). Patients with chronic obstructive pulmonary disease and heart failure may require preparations with low mineralocorticoid activity, such as methylprednisolone (Solu-Medrol), to prevent fluid retention.

Mineralocorticoid deficiency is treated with fludrocortisone (Florinef) Cardiovascular support if indicated. Imeediate treatment if addisonian (adrenal) crisis or circulatory collapse if

imminent:a. I.V. sodium chloride solution to replace sodium ions.b. Hydrocortisone (Cortef)c. Injection of circulatory stimulants, such as atropine sulfate

(Atropine), calcium chloride (Calcium), epinephrine (Adrenalin).

Diagnosis and treatment of underlying cause of adrenocortical insufficiency or addisonian crisis (eg, antibiotic therapy to treat infection if this is a factor in crisis)

Addison's diseaseTests and diagnosis

Blood test. ACTH stimulation test. Insulin-induced hypoglycemia test. Imaging tests. 

Addison's diseaseNursing DiagnosisDeficient fluid volume, related to

hypovolemia secondary to adrenal insufficiency

Ineffective tissue perfusion: Peripheral, related to fluid volume deficit

Anxiety, related to lack of knowledge about the effects and treatment of adrenal insufficiency

Addison's diseaseNURSING INTERVENTION:

Achieving Normal Fluid and Electolyte Imbalance

Assess fluid intake and output and serial daily weights

Monitor vital signs frequently: a drop in BP may suggest an impending crisis

Monitor results of serum sodium and potassiumAssess skin turgor and mucous membrane for

dehydration

Addison's diseaseProtecting well-being Minimize stressful situation Protect patient from infection

a. Control patient's contacts so that infectious organismsb. Protect patients from drafts, dampness, exposure to cold.c. Prevent overexertion.d. Use meticulous hand washing and asepsis

Assess comfort and emotional status of the patient a. Control the temperature of the room to avoid sharp

deviations in patients temperature. b. Maintain a quiet, peaceful environment, avoid loud

talking and noisy radios. Observe and report early signs of addisonians crisis (sudden drop in

BP, nausea and vomiting, fever)

Addison's diseaseIncreasing Activity toleranceAssist the patient with ADLs.Provide for periods of rest and activity to

avoid overexertionProvide for high-calorie, high-protien diet.