ADDISON'S DISEASE: CAUSES, RISKS, DIAGNOSIS & TREATMENT

   ADDISON DISEASE                                    

DID YOU KNOW?

• Addison’s disease is named after Thomas Addison, a British physician who first described the condition in 1855.
• The condition was initially considered a form of anemia associated with adrenal glands because little was known at the time about the glands then called suprarenal capsules.
• The former US President J.F Kennedy was one of the best-known Addison’s disease sufferers and was first to survive major surgery. 

ADDISON DISEASE

• Addison’s disease also known as Adrenocortical insufficiency or Hypocortisolism is a disorder of the adrenal cortex which results in insufficient production of Glucocorticoids (cortisol) and Mineralocorticoids (aldosterone).
•  It occurs when adrenal cortex function is inadequate to meet the patient’s need for cortical hormones.

INCIDENCE

• The condition is usually seen between ages 30 to 50 years.
•  Women are slightly more commonly affected than men.
• The disease is seen in all ethnic groups and the incidence is estimated at 40 to 60 cases per one million population.
• In the areas where tuberculosis is prevalent, it is the most common case

AETIOLOGY

• Autoimmune or idiopathic atrophy of the adrenal glands is responsible for the majority of the cases.
• It may also be caused by adrenal dysgenesis, impaired steroidogenesis or adrenal destruction from the disease process.

OTHER CAUSES

• Surgical removal of both adrenal glands.
• Therapeutic use of corticosteroids.
• Infections such as tuberculosis, histoplasmosis
• Malignant pituitary hypofunction
• Metastatic disease of the breast
• Hemorrhage
• Ankyloidosis
• Bronchial cancer 

PATHOPHYSIOLOGY

• Autoimmunity is the most common cause of adrenal insufficiency.
• Lymphocytes infiltration of the adrenal cortex is the characteristic feature.
• Addison’s disease is frequently accompanied by other immune disorders.
• Cortical destruction leads to chronic adrenal insufficiency.
• Continued loss of cortical tissue accompanies a deficiency of mineralocorticoids as well as glucocorticoids.
• Adrenocortical hypofunction results in decreased levels of mineralocorticoids (aldosterone), glucocorticoids (cortisol) and androgens. 

CLINICAL MANIFESTATION

Symptoms develop insidiously, Hyper- pigmentation (bronze like) mostly seen on the palms, soles, knees, knuckles, buccal mucosa and nipples is often the first manifestation and may be associated with good health for sometime before other features appear. These include;

• Hypoglycemia and hyponatremia
• Mental confusion
• Depression
• Menstrual disturbances

• Fatigue
• Muscle weakness
• Irritability
• Weight loss
• Abdominal pain
• Low blood pressure due to loss of sodium and water.
• Emaciation
• Reduced mental activity
• Drowsiness
• Desire for salty foods
• Chronic dehydration due to disturbance of sodium and potassium metabolism

                

• headache
• Anorexia
• Nausea and vomiting
• Intermittent diarrhea
• Apathy
• Hypotension due to lack of mineralocorticoids
• Loss of libido
• Sparse distribution of hair.

                 

ADDISON CRISIS

• It’s a constellation of symptoms that indicates severe adrenal insufficiency.
• This is a medical emergency and a potential life –threatening situation requiring immediate emergency treatment.
• It may be the result of previously undiagnosed Addison's disease, a disease process suddenly affecting adrenal function (such as adrenal hemorrhage) or an intercurrent problem ( infection and trauma )in someone known to have Addison’s disease.
• It usually occurs in patients who do not respond to hormonal replacement therapy or abruptly stops hormonal therapy.
• With disease progression and acute hypotension, Addisonian’s crisis 

CLINICAL MANIFESTATION

• Cyanosis
• Fever
• Signs of shock such as pallor, rapid weak pulse, rapid respiration and low blood pressure.
• Headache
• Nausea
• Abdominal pain
• Confusion
• Restlessness
• Fatigue and weakness

MEDICAL MANAGEMENT FOR ADDISON CRISIS

• Immediate administration of dexamethasone 4mg together with IV normal saline in glucose.
• Chronic states require glucocorticoids and mineralocorticoids supplements.
• The main dose should be increased in stressful situations such as surgery, infection, trauma when the body requires increased glucose and minerals.

DIAGNOSTIC MEASURES

• Diagnosis is confirmed by low levels of the adrenocortical hormone in blood and urine and decreased serum cortisol levels below the normal which is 6.5mmol/L.
• Other laboratory investigations such as;
•  Blood glucose; decreased blood glucose (hypoglycemia).
•  Decreased sodium levels (hyponatremia)
• Increased serum potassium concentration (hyperkalemia)
• Increased white blood cell count.( leukocytosis)
• Adrenal calcification may be seen on radiography.
• CT scan or MRI.
•  History and clinical manifestations

MEDICAL MANAGEMENT

• Aim; management is directed towards combating circulatory shock: restoring blood circulation, restoring fluid balance.
• Hydrocortisone is administered intravenously followed by 5% dextrose in normal saline.
• Vasopressor amines may be required if hypotension persists.
• Antibiotics may be prescribed to treat any infection.
• Replacement of corticosteroids (to correct metabolic imbalance) and mineralocorticoids (to correct electrolyte imbalance and hypotension) is required.

NURSING MANAGEMENT

REST AND SLEEP

• Ensure adequate rest in a noise free environment due to restlessness and irritability.
• Provide side rails prevent falls.
• Perform nursing activities in bulk to enhance rest.
• Assist patient with activities of daily living due to fatigue.
• Assist patient to assume a comfortable and suitable position thus recumbent position with legs elevated to enhance blood circulation.
• Restrict visitors to allow patient have enough rest.

OBSERVATION

• Check vital signs 4 hourly and record notifying increased temperature( due to infection), blood pressure and pulse alterations as patient moves from a lying, sitting and standing position to assess for inadequate fluid volume.
• Weigh patient daily and record to detect any changes in weight.
• Assess the skin for color due to skin pigmentation.
• Observe signs of dehydration such as loss of skin turgor.
• Observe patient’s behavioral changes such as reduced mental activity, confusion and drowsiness.
• Strictly monitor fluid intake and output, record and balance daily to correct fluid imbalances.
• Observe for signs of sodium and potassium imbalances such as fatigue, weakness.
• Assess patient’s level of pain.
• Observe patient’s eating pattern since there is the desire for extra salt intake
• Assess the activity tolerance level of the patient since there is fatigue.
• Monitor patient for signs indicative of Addisonian’s crisis such as pallor, hypotension, rapid respiratory rate.

DIET

• Serve patient with high sodium diet such as salted fish due to low levels of sodium.
• Small meals at frequent intervals.
• Serve high carbohydrate diet ( yam, maize foods) and low potassium diets (potatoes, beans, bananas).
• Ensure adequate hydration by serving enough fluids as the patient can tolerance.

PERSONAL HYGIENE

• Ensure proper hand washing to decrease the risk of infection.
• Assist patient with his or her personal hygiene needs such as bathing, mouth care.
• Ensure adequate skin care such as bathing patient twice daily, applying emollient such as Vaseline and oil base creams on the skin and lips to enhance hydration of the skin and prevent cracks.

NURSING MANAGEMENT 

RESTORING FLUID BALANCE

• Check patient’s weight daily and record to detect early changes in weight.
• Monitor the fluid intake and output of the patient.
• Ensure adequate hydration by administering oral fluids as the patient can tolerate and prescribed IV fluids.
• Observe for signs of dehydration such as loss of skin turgor.
• Encourage client to report increased thirst.
• Monitor patient’s blood pressure and record. A decrease in systolic pressure may indicate depletion of fluid volume.
•  Observe for signs of hypovolemic shock such as pallor.
• Administer prescribed antiemetics to stop vomiting which will lead to fluid and electrolyte imbalance.

IMPROVING  ACTIVITY INTOLERANCE

• Until the patient’s condition is stabilized, the nurse takes precautions to avoid unnecessary activity and stress.
• Explain the rationale for minimizing stress.
• Place patients items in his or her reach.
• Assist patient with activities of daily living.
• Ensure patient takes enough rest when tired. 

PAIN MANAGEMENT

• Assess patient’s level of pain by using the pain scale such as the 0-10 visual analog scale.
• Identify the site of pain and precipitating factors.
• Provide patient with divertional therapy.
• Put the patient in a comfortable position thus recumbent with legs elevated.
• Administer prescribed medication 

PATIENT TEACHING

• Give patient and family education on the condition; its causes, signs and symptoms, complications of the condition and to report promptly to the hospital when necessary.
• Instruct patient to modify diet and fluid intake to maintain fluid and electrolyte balance.
• Urge patient to wear a medic alert bracelet if he or she is on corticosteroids.
• Encourage patient and family on the need to comply with review dates.
• Patient and family are given both written and verbal instructions on the replacement therapy and proper dosage when there is the need for lifelong replacement therapy.
• Educate on stress reduction and management.
• Educate family to support patient psychologically because of mood swings. 

COMPLICATIONS

• Addisonian’s crisis
• Renal failure
• Arrhythmias
• Hypovolemic shock

REFERENCES

• JOHNSON, Y.J(2004) HANDBOOK FOR BRUNNER AND SUDDARTH’S TEXTBOOK OF MEDICAL-SURGICAL NURSING.(10^TH EDITION)

• BADOE, A, ARCHAMPONG,E.Q, ROCHA, J.T PRINCIPLES AND PRACTICE OF SURGERY INCLUDING PATHOLOGY  IN THE TROPICS. (4^TH EDITION)

• SMELTER,C.S,BARE,G.B,HINKLE,L.J,CHEEVER,H.K(2010) BRUNNER AND SUDDARTH’S TEXTBOOK OF MEDICAL AND SURGICAL NURSING. (12^TH EDITION)

• BLACK, M.J,HAWKS, H.J MEDICAL SURGICAL NURSING CLINICAL MANAGEMENT FOR POSITIVE OUTCOME. (11^TH EDITION)