Cushing’s syndrome is defined as the clinical effects of increased glucocorticoid hormone. It can be characterized by an excess production of glucocorticoids (primarily cortisol) by the cortex of the adrenal gland, but it is most commonly due to therapy with glucocorticoid drugs. Cortisol is an essential hormone for many body functions, including maintaining normal electrical excitation of the heart, blood glucose level, nerve cell conduction, and adequate circulatory volume, and for metabolizing proteins, fats, and carbohydrates. Overproduction of glucocorticoids leads to a host of multisystem disorders in metabolism, water balance, wound healing, and response to infection. Complications affect almost every system of the body. Increased calcium resorption from bones may lead to osteoporosis and bone fractures. A blunted immune response causes a high risk for infection, as well as poor wound healing. Cushing’s syndrome may also mask even life-threatening infections. Gastrointestinal (GI) irritation may lead to peptic ulcers, and both insulin resistance and glucose intolerance can cause hyperglycemia.
The causes are divided into three categories: iatrogenic, primary, and secondary. Iatrogenic causes are a result of excessive cortisol levels from chronic therapy with glucocorticoids. Approximately 25% of cases have a primary cause of excessive cortisol production from adrenal neoplasms such as adenomas or carcinomas. The tumors usually affect only one adrenal gland, and about 50% are malignant. Even with appropriate treatment, most patients with adrenal carcinoma die within 3 years because of metastases to the liver and lung. Secondary Cushing’s syndrome is more common than primary, and bilateral adrenal hyperplasia is the most common secondary condition. It is caused by excessive production of adrenocorticotropic hormone (ACTH) from the anterior pituitary gland.
Nursing care plan assessment and physical examination
Ask if the patient has had recent changes in memory, attention span, or behavior. Discuss the patient’s sleep-wake pattern, and evaluate the patient for sleep disturbances. Family members may comment on the patient’s changed affect, short-term memory, emotional instability, and ability to concentrate. Other signs include weakness, fatigue, back pain, general discomfort, difficulty completing activities of daily living, and changes in the urinary output. Be sure to question the patient about weight gain and changes in body proportions between the shoulders. Patients may also notice changes in their appetite and thirst. Other changes in appearance include hirsutism, oily skin, acne, purple striae, and poor wound healing. Women may have noted changes in menstruation, and both men and women may note changes in libido and in their feelings about themselves.
Changes in fat metabolism lead to generalized obesity, a round (“moon”) face, a hump in the interscapular area, and truncal obesity. Hyperpigmentation of skin and mucous membranes may be present as a result of increases in ACTH. Because of alterations in protein metabolism, loss of collagen support in the skin leaves the skin more fragile and easily bruised. Both males and females experience changes in secondary sexual characteristics and body hair distribution, along with an increase in acne. Some patients have peripheral edema from water and sodium retention. Muscle wasting, especially in extremities, leads to difficulty in getting up and down from a sitting position, difficulty in climbing stairs, or generalized weakness and fatigue. Note if the extremities are thin with atrophied muscles. Auscultate the patient’s blood pressure; most patients are hypertensive because of increased circulating volume or increased sensitivity of the arterioles to circulating catecholamines. Neck vein distension may be present.
A diagnosis of Cushing’s syndrome can be devastating. Determine the patient’s response to the disease and the effect the disease has had on the patient’s sexuality, body image, and relationships with others.
Nursing care plan primary nursing diagnosis: Fluid volume excess related to abnormal retention of sodium and water.
Nursing care plan intervention and treatment
The main focus is to find the primary cause of the cortisol excess and remove it if possible. In the case of iatrogenic Cushing’s syndrome, care is focused on alleviating as many of the signs and symptoms as possible when the therapy cannot be discontinued. If the patient has primary Cushing’s syndrome from an adrenal tumor, the tumor is removed surgically. Even if the tumor is unilateral, the patient is treated for adrenal insufficiency after the surgery because the high levels of cortisol from the tumor may have caused the unaffected adrenal gland to atrophy. Patients with adrenal carcinoma are treated postoperatively with mitotane to treat metastases. Throughout the patient’s recovery, fluid, electrolyte, and nutritional assessment and balance are essential.
For secondary Cushing’s syndromee from a pituitary tumor, the preferred option is a transsphenoidal adenectomy, a procedure that explores the pituitary gland to find microadenomas. It is successful in 20% to 70% of patients. A second option is the transsphenoidal hypophysectomy, a procedure that removes the entire pituitary gland and leads to a cure in 100% of the patients. It is usually used for more invasive tumors and requires lifelong hormone replacement (glucocorticoids, thyroid hormone, gonadal steroids, and antidiuretic hormone [ADH]). A third alternative is bilateral total adrenalectomy, which cures the signs and symptoms of excess cortisol but does not decrease ACTH secretion. The patient requires lifelong replacement therapy with glucocorticoids and mineralocorticoids. If the patient has secondary Cushing’s syndrome because of ectopic production of ACTH from a nonendocrine tumor, the first concern is to remove the source of the ectopic secretion of ACTH. If this is not possible, mitotane decreases cortisol production in the adrenal gland but may cause damage to the gland and is used with caution. Radiation therapy is used when the patient either has no defined tumor or needs an adjunct to tumor removal.
Patients with pituitary surgery need careful management of airway, breathing, and circulation. In the first postoperative hours, serial neurological examinations are important to identify the risk for increased intracranial pressure from edema. The incision is generally performed through the upper gum line; ask the surgeon about the procedure for oral hygiene. Nasal packing is removed after 2 days in most patients. At that time, observe for rhinorrhea and ask the patient to report a “runny” nose. Teach the patient to avoid coughing, sneezing, or blowing the nose immediately after surgery.
An important goal is to limit the risk of infection for the patient. Note, document, and report any signs of skin or pulmonary infection. Restrict visitors with upper respiratory infections. Unless contraindicated after surgery, encourage the patient to cough and deep-breathe, turn in bed at least every 2 hours, and use good oral hygiene. Focus on helping the patient deal with changes in body image, sexuality, and self-esteem. Let the patient know that many of the body changes are reversible with treatment; this information allows the patient to focus on setting goals. Include the patient’s partner in all education. Patient and family teaching occurs throughout the patient’s hospitalization and after discharge. Provide information about patient care and activity restrictions. Explanation of all diagnostic tests and their findings, as well as the treatment plan, are important. The patient and family often require time to consider treatment options. As time progresses, the patient and family need information about the disease process and lifelong management with medication and diet changes.
Nursing care plan discharge and home health care 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.