Cushing Syndrome

Cushing Syndrome results from excessive, rather than deficient, adrenocortical activity. It is commonly caused by use of corticosteroid medications and is infrequently the result of excessive corticosteroid production secondary to hyperplasia of the adrenal cortex. It may be caused by several mechanisms, including a tumor of the pituitary gland or less commonly an ectopic malignancy that produces adrenocorticotropic hormone (ACTH). Regardless of the cause, the normal feedback mechanisms that control the function of the adrenal cortex become inefective resulting in over secretion of glucocorticoids androgens imposibly mineralocorticoid. Cushing syndrome occurs 5 times more often in women ages 20 to 40 years than in men. Clinical manifestations - Arrested growth, weight gain and obesity, musculoskeletal changes, and glucose intolerance. - Classic features: central-type obesity, with a fatty "buffalo hump" in the neck and supraclavicular areas, a heavy trunk, and relatively thin extremities; skin is thin, fragile, easily traumatized ecchymoses and striae, - Weakness and lassitude; sleeo is disturb because of altered diurnal secretion of cortisol. -excessive protien catabolism with muscle wasting and osteoporosis; kyphosis, back ache, and compression fractures of the vertebrae are possible. - Retention of sodume and water producing hypertension and heart failure. - "Moon-faced" appearance, oiliness of skin and acne. - Increased susceptibility to infection; slow healing of minor cuts and bruises. - Hyperglycemia or overt diabetes. - Virilization in females (due to excess androgens) with appearance of masculine traits and recession of feminine traits (e.g) excessive hair on face, breasts atrophy, menses cease, clitoris enlarges, and voice deepens); libido is lost in males and females. - Changes occur in mood and mental activity; psychosis may develop and distress and depression are common. -If Cushings syndrome is the result of a pituitary tumor, visual disturbances are possible because of pressure on the optic chiasm.

Assessment and Diagnostic Findings: - Overnight dexamethasone suppression test to measure plasma cortisol level (stress, obesity, depression, and medications may false elevate results). - Laboratory studies (eg, serum sodium, blood glucose, serum potassium, plasma, urinary); 24hour urinary free cortisol level. -CT, ultrasound, or MRI scan or ultrasound may localize adrenal tissue and detect adrenal tumors. Medical Management: Treatment is usually directed at the pituitary gland because most cases are due to pituitary tumors rather than tumors of the adrenal cortex. -Surgical removal of the tumor by transsphenoidal hypophysectomy is the treatment of choice (80% success rate). -Radiation of the pituitary gland is successful but takes several months for symptom control. - Adrenalectomy is performed in patients with primary adrenal hypertrophy. -Postoperatively, temporary replacement therapy with hydrocortisone may be necessary until the adrenal glands begin to respond normally (may be several months). -If bilateral adrenalectomy was performed, lifetime replacement of adrenal cortex hormones is necessary. -Adrenal enzyme inhibitors (eg, metyrapone, aminoglutethimide, mitotane, ketoconazole) may be used with ectopic ACTH-secreting turmos that cannot be totally removed; monitor closely for inadequate adrenal function and side effects. -If Cushing syndrome results from exogenous corticosterioids, taper the drug to the minimum level or use alternate-day therapy to treat the underlying disease.

Nursing Interventions:
Decreasing risk of injury -Provide a protective environment to prevent falls, faractures and other injuries to bones and soft tissues. -Assist the patient who is weak in ambulating to prevent falls or colliding into furniture -Recommend foods high in protein, calcium, and vitamin D to minimize muscle wasting and osteoporosis; refert to dietitian for assistance. Decreasing Risk of Infection

-Avoid unnecessary exposure to people with infections -Assess frequently for subtle signs of infections Preparing Patient for Surgery -Monitor blood glucose levels, and assess stools for blood because diabetes mellitus and peptic ulcer are common problems Encouraging Rest and Activity -Encourage moderate activity to prevent complications of immobility and promote self-esteem -Plan rest periods throughout the day and promote a relaxing, quiet environment for rest and sleep Promoting Skin Integrity -Use meticulous skin care to avoid traumatizing fragile skin. -Avoid adhesive tape, which can tear and irritate the skin. -Assess skin and bony prominences frequently. -Encourage and assist patient to change positions frequently. Improving Body Image -Discuss the impact that changes have had on patients self-concept and relationships with others. Major physical changes will disappear in time if the causes of Cushing syndrome can be treated -Weight gain and edema may be modified by a low-carbohydrate, low-sodium diet; a high-protein intake can reduce some bothersome symptoms. Improving Thought Processes -Explain to the patient and family the cause of emotional instability, and help them cope with mood swings, irritability and depression -Report any psychotic behavior -Encourage patient and family members to verbalize feelings and concerns Monitoring and Managing Complications -Adrenal hypofunction and addisoninan crisis: Monitor for hypotension; rapid, weak pulse; rapid respiratory rate; pallor; and extreme weakness. Note factors that may have led to crisis (eg, stress, trauma, surgery). -Administer IV fluids and electrolytes and corticosterioids before, during, and after surgery or treatment as indicated. -Monitor for circulatory collapse and shock present in addisonian crisis; treat promptly. -Assess fluid and electrolyte status by monitoring laboratory values and daily weight. -Monitor blood glucose level, and report elevations to physician. Teaching Patients Self-Care -Present information about Cushing syndrome verbally and in writing to patient and family. -If indicated, stress to patient and family that stopping corticosteroid use abruptly and without medical supervision can result in adrenal insufficiency and reappearance of symptoms. -Emphasize the need to keep an adequate supply of the corticosteroid to prevent running out or skipping a dose, because this could result in addisonian crisis. -Stress the need for dietary modifications to ensure adequate calcium intake without increasing risk for hypertension, hyperglycemia, and weight gain.

-Teach patient and family to monitor blood pressure, blood glucose levels, and weight. -Stress the importance of wearing a medical alert bracelet and notifying other health professionals that he or she has Cushing syndrome. -Refer for home care as indicated to ensure safe environment with minimal stress and risk for falls and other side effects. -Emphasize importance of regular medical follow-up and ensure patient is aware of side and toxic effects of medications.