ADDISON'S DISEASE

DEFINITION

• A condition of insufficient production of corticosteroid, aldosterone androgens by the adrenal cortex. 

INCIDENCE

• Rare 4:100,000 patients.
• All ages. Common under the age of 60 years.
• Happens in both sexes. 

ETIOLOGY

1. Primary adrenal insufficiency

• Idiopathic atrophy of the adrenal gland.
• Congenital hyperplasia 
• Neoplasm in the adrenal gland
• Destruction of the gland due to chemical substances or auto immune.
• Infection - fungal / HIV 

2. Secondary haemorrhage and infarction.

• From pituitary gland or hypothalamus.

FUNCTION OF ALDOSTERONE 

PATHOPHYSIOLOGY

• Adrenocorticol hypofunction results in decreased levels of mineralocorticoids ( aldosterone ) , glucocoryicoids (cortisol ) and androgens.
• Aldosterone deficiency causes numerous fluid and electrolyte imbalances. 

( Usually , aldosterone acts on the kidneys to increase sodium and fluid retention ) 

A deficiency of aldosterone causes : 

1. Water excretion increases.

2. Extracellular water becomes depleted (dehydration)

3. Hypotension develops.

4. decrease cardiac output.

5. Atrophy of the heart - as a result of diminished workload. 

- Eventually hypotension becomes severe leading to circulatory collapse, shock and death.

- Potassium levels of more than 7 mEq/L results in arrythmias and cardiac arrest. 

• Glucocorticoid deficiency causes widespread metabolic disturbances. 
• Glucocorticoids promotes gluconeogenesis ( synthesis of glucose from non carbohyfrate sources, such as amino acids and glycerol. Occurs primarily in the liver and kidneys whenever the supply of carbohydrates is insufficient to meet the body's energy needs ) and have an "anti insulin" effect. 
• Low glucocorticoid = Low gluconeogenesis 

- Liver glycogen deficiency.
- Hypoglycaemia develops.
- Failure of negetive feedback causes increase of ACTH ( controls the secretion of adrenal hormone, cortisol - released in response to stress and a low level of blood glucocorticoids. Its primary functions are to increase blood sugar through glycogenolysis ) 
- Patient becomes weak, exhausted, anorexia, weight loss, nausea, vomitting. 

•  Increased ACTH secretion leads to increased pigmentation of the skin and mucous membrane.
• Therefore, patients with Addison's disease have increased levels of ACTH, bronzed or tanned appearance. 

• Adrogen deficiency fails to produce symptoms in men because the testes supply adequate amount of sex hormone. 
• Women depend on the adrenal cortex foe adequate secretion of androgens (plays a role in pubic and axillary hair in females) 

CLINICAL MANIFESTATION YOUTUBE 

1. Weakness.
2. Fatigue.
3. Nausea and vomitting.
4. Anorexia.
5. Loss of weight.
6. Emotional disturbances.
7. Reduced coping mechanism. 

8. Appearance.
- Dark, shiny skin.
- Mucosal, mouth and conjuctiva pigmentation.
9. Dehydration.
10. In severe condition.
- Severe dehydration.
- hypotension.
- Shock.
11. Increased potassium absorption. 
- Hyperkalaemia
- If level > 7 mEq/L -> arrythmias -> cardiac arrest. 
12. Muscle atrophy due to reduced mobility because of deteriorating metabolism.
13. Hypoglycaemia.
- Low gluconeogenesis is due to Low glucocorticoid. 

INVESTIGATION

1. Serum cortisol
- Cortisol concentration < 20 ug/dl 
2. Plasma ACTH 
- To confirm primary and secondary adrenal insufficiency.
3. Serum electrolyte. 
- Sodium level low
- Potassium and calcium high 
- Glucose is decreased.
4. ECG - QT Interval prolong. 

MEDICAL MANAGEMANT

1. Glucocorticoid therapy.
- Cortisone acetate 25mg in the morning and 12.5mg every evening.
- Hydrocortisone 20mg every morning and 15mg every evening. 
- Prednisolone 5mg every morning and 2.5mg every evening. 

2. Mineralocorticoid.
- Fludrocortisone Acetate 100ug daily in primary adrenal insufficiency. 

MANAGEMENT OF CARE

1. Physical assessment.
a) Assess patient's emotional status.
b) Assess the 'hump' or bone deformity.
- To prevent pressure. 
2. Observation.
a) Vital sign.
- Pulse.
- Blood pressure.
- Temperature.
b) Monitor for infection.
- Report any signs of infection eg: sore throat.
c) Monitor signs of dehydration.
- Assess sodium and potassium imbalance.
3. Medication.
- If not treated early => death 

COMPLICATION

Acute adrenocortical insuffiency (Addisonian Crisis)
1. A life threatening emergency caused by sudden decrease of these hormones. 
2. Triggered by : 
- Stress.
- Sudden withdrawal of corticoidsteroid hormone replacement therapy. 
- After adrenal surgery.
- Sudden pituitary gland destruction. 
3. Manifestation.
- Hypotension (postural)
- Tachycardia.
- Dehydration - severe vomitting and diarrhea.
- Hyponatremia.
- Hyperkalemia.
- Hypoglycaemia.
- Fever, weakness and confusion.
- Shock. 

TREATMENT

• Replacement therapy - hydrocortisone.
• Large volumes of 0.9% saline and 5% dextrose are administered to reverse hypotension and electolycte imbalance. 

NURSING DIAGNOSIS

1. Activity intolerance related to postural hypotension.
Obj : Patient will be able to remain free from fatigue during performance of usual activities. 
Intervention
- Provide bed rest for the first 24 hours.
- Avoid any unnecessary activities such as bathing for the first 12 hours. 
- Explain to patient that the energy level will increase when the hormones stabilize.
- Gradually increase the activities as hormone levels return to normal. 

2. Deficient fluid volume related to inability to conserve fluid. 
Obj : Patient will be able to have normal fluid status.
Intervention
- Monitor intake and output hourly.
- Monitor blood pressure and heart rate hourly until normal. 
Administer IV fluids as prescribed (5% dextrose)
- Monitor electrolyte status.
- Administer cortisol as prescribed. 

3. Risk of injury related to weakness and hypoglycaemia.
Obj : Patient will remain free from injury. 
Intervention
- Keep bed in lowest position.
- Keep side rails up at all times unless patient refuses.
- Monitor blood glucose levels every 4 hours.
- Instruct patient to call foe assistance when getting into or out of bed. 

4. Knowledge deficit related to lack of previous experience with new problem.
Obj : Patient will verbalize understanding of disease and its treatment. 
Intervention
- Explain care to patient and family so that no unexpected events occur.
- Focus on immediate care and home care later.
- Repeat teaching frequently.
- Provide written material to enforce verbal teaching. 
- Provide information about community resources that may be useful to patient and family. 

SUMMARY ADDISON'S DISEASE YOUTUBE 

REFERENCES

• http://www.mayoclinic.com/health/addisons-disease/DS00361/DSECTION=symptoms
• http://www.medicinenet.com/addison_disease/article.htm
• http://emedicine.medscape.com/article/116467-clinical
• Brunner & Suddarth's (2010). Assessment and Management of Patients With Endocrine Disorders, Medical-Surgical Nursing Text Book. 42(8), 1279-1281