- Cushing’s Syndrome results from excessive secretion of one or all of the adrenocortical hormones which includes glucocorticoid cortisol (predominant type), the mineralocorticoid aldosterone, and the adrogenital corticoids.
Types of Cushing’s Syndrome:
- Pituitary Cushing’s syndrome (Cushing’s disease) is the most common cause of Cushing’s syndrome, and stems from hyperplasia of both adrenal glands caused by over stimulation by adrenocorticotropic hormone (ACTH), usually from pituitary adenoma. The syndrome mostly affects women between ages 20 and 40.
- Adrenal Cushing’s syndrome is associated with adenoma or carcinoma of the adrenal cortex. The disease may recur after surgery.
- Ectopic Cushing’s syndrome results from autonomous ACTH secretion by extrapituitary tumors (such as the lungs) producing excess ACTH.
- Iatrogenic Cushing’s syndrome is caused by exogenous glucocorticoid administration.
The most common cause of Cushing syndrome is side effects from taking anti-inflammatory steroid medications for conditions such as asthma and rheumatoid arthritis.
The second most common cause is Cushing’s disease, which occurs when the pituitary gland makes too much of the hormone ACTH. ACTH stimulates the adrenal glands to produce cortisol.
Cushing’s disease affects women more often than men.
Cushing syndrome can also be caused by the following:
- Tumor of the pituitary gland or adrenal gland
- Tumor elsewhere in the body (ectopic Cushing syndrome)
1. Signs and symptoms of excess glucocorticoid (cortisol) secretion:
- Weight gain or obesity.
- Heavy trunk; thin extremities.
- Fat pad (Buffalo Hump) in neck and supraclavicular area.
- Rounded face (moon face); plethoric, oily complexion.
- Skin – fragile and thin; striae and ecchymosis, acne.
- Musculoskeletal – muscle wasting causes by excessive catabolism, osteoporosis, characteristic kyphosis, back ache.
- Mental disturbances – mood changes, psychosis.
- Increased susceptibility to infections.
2. Manifestation of excess mineralocorticoid (aldosterone) secretion:
- Hypernatremia, hypokalemia
- Weight gain
- Expanded blood volume
3. Manifestation of excess androgens:
- Females experience virilism (masculinization) with hirsutism (excessive growth of hair on the face and midline of trunk); atrophied breast, enlarged clitoris, masculinized voice, loss of libido, hermaphroditism (if exposed in utero).
- Males – loss of libido.
- Excessive plasma cortisol levels and loss of diurnal variation.
- Increased blood glucose levels, decreased serum potassium level.
- Plasma ACTH elevated in patients with pituitary tumors, very low in patients with adrenal tumor.
- Eosinophils decreased on complete blood count.
- Elevated urinary 17-hydroxycorticoids and 17-ketogenic steroids.
- Overnight dexamethasone suppression test, possibly with cortisol urinary excretion measurement, to check for:
- Unsuppressed cortisol level in Cushing’s syndrome cause by adrenal tumors.
- Suppressed cortisol level in Cushing’s disease caused by pituitary tumor.
- Skull X-ray detects erosion of the sella turcica by a pituitary tumor; CT scan and ultrasonography locate tumor.
Primary Nursing Diagnosis
- Fluid volume excess related to abnormal retention of sodium and water
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 ( transphenoidal hypophysectomy) is the treatment of choce (90% 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
- Post operatively, temporary replacement therapy with hydrocortisone maybe necessary until the adrenal glands begin to respond normally ( maybe several moths)
- If bilateral adrenalectomy was performed lifetime replacement of adrenal cortex hormones is necessary
- Adrenal enzyme inhibitors ( E.g. metyrapone or mitotine) maybe used with ectopic ACTH- secreting tumors that cannot be totally removed; monitor closely for inadequate adrenal function and side effects
- If Cushing’s syndrome results from exogenous corticosteroids, taper the drug to the minimum level or use alternate-day therapy to treat the underlying disease
- Mitotane 2–16 g per day in divided doses PO an antineoplastic, inhibits activity of adrenal cortex; used to treat inoperable adrenocortical carcinomas and Cushing’s syndrome
- Cyproheptadine 4 mg 2–3 times a day PO an antihistamine agent ; serotonin antagonist, inhibits the release of ACTH from pituitary gland; drug is considered last resort and often causes no improvement
- Other Drugs: Aminoglutethimide inhibits cholesterol synthesis, and metyrapone partially inhibits adrenal cortex steroid synthesis. These drugs may be used in conjunction with surgery or radiation if the tumor is not completely resectable or if complete remission is not expected.
- Monitor intake and output, daily weights, and serum glucose and electrolytes.
- Monitor for signs of infection because risk is high with excess glucocorticoids.
- After hypophysectomy, monitor for diabetes insipidus, hypothyroidism, and other endocrine changes.
- Assess the skin frequently to detect reddened areas, skin breakdown or tearing, excoriation, infection or edema.
- Handle skin and extremity gently to prevent trauma; prevent falls by using siderails.
- Avoid using adhesive tape on the skin to reduce trauma on its removal.
- Encourage the patient to turn in bed frequently or ambulate to reduce pressure on bony prominences and areas of edema.
- Assist the patient with ambulation and hygiene when weak and fatigued. Use assistive devices during ambulation to prevent falls and fractures.
- Help the patient to schedule exercise and rest. Advise the patient how to recognize signs and symptoms of excessive exertion.
- Instruct the patient to correct body mechanics to avoid pain or injury during activities.
- Provides foods low in sodium to minimize edema and provide foods high in potassium (bananas, orange juice, tomatoes) and administer potassium supplements as prescribed to counteract weakness re;ated to hypokalemia.
- Report edema and signs of fluid retention.
- Encourage the patient to verbalize concerns about the illness, changes in appearance, and alters role function.
- Explain to female patient who has benign adenoma or hyperplasia that, with proper treatment, evidence of masculinization can be reversed.
- Physical response: Vital signs, neurological assessment, cardiopulmonary assessment, wound healing, signs of infection (fever, wound drainage, productive cough), important laboratory deviations (serious electrolyte imbalances, alterations in glucose levels)
- Nutrition: Daily weights, appetite, food tolerance, food preference, response to diet teaching, calorie count if indicated
- Emotional response: Concerns over body image, self-concept, mood, affect
Discharge and Home Healthcare Guidelines
Describe the pathophysiology of the disease. Identify factors that aggravate the disease (stress, changes in diet, injury), as well as the signs and symptoms. Explore complications of the disease, and ask whom to notify if they occur. Describe the treatment plan and expected effects, as well as possible complications. Describe all medications, including the name, dosage, action, side effects, route, and importance of lifelong dosing if indicated.
Nursing Care Plan
- Risk for injury and Risk for infection related to weakness and changes in protein metabolism and inflammatory response.
- Self-care Deficit: weakness, feeling of tiredness, muscle atrophy and changes in sleep patterns.
- Impaired skin integrity related to edema, impaired healing and the skin is thin and fragile.
- Disturbed Body Image related to changes in physical appearance, sexual dysfunction and decreased activity levels.
- Disturbed Thought Processes related to fluctuations in emotions, irritability and depression.