Page 48 Complete Your CE Test Online - Click Here ● ● In the presence of an adrenal tumor, ACTH plasma levels are elevated. ACTH levels that are higher in the petrosal sinuses than in a vein in the forearm suggest the presence of a pituitary adenoma. ● ● Elevated salivary cortisol levels are considered significant. ● ● Elevated WBC count. ● ● CT scan, MRI, and/or ultrasound are used to detect the presence and location of a tumor in the pituitary or adrenal glands. High dose dexamethasone suppression test is used to determine if Cushing’s syndrome is due to pituitary dysfunction. If dexamethasone suppresses plasma cortisol levels, the test is considered positive. Failure to suppress plasma cortisol levels indicates the presence of an adrenal tumor or non-endocrine, corticotropin-secreting tumor. HCPs should be aware that this test can produce false positive results[4,11] . Treatment The goals of Cushing’s syndrome treatment are to[5,11] : ● ● Restore hormonal balance. ● ● Reverse Cushing’s syndrome. Treatment strategies may include surgery, radiation therapy, or drug therapy. Specific treatment depends on the underlying cause of the disease[6,11] . Surgical intervention Surgery is performed to remove adrenal or pituitary (hypophysectomy) tumors. Pituitary tumors may be removed via the transsphenoidal approach, during which the pituitary is removed through the nasal cavity, sphenoid sinus, and into the sella turcica[6] . Transsphenoidal hypophysectomy is extremely delicate surgery, and patients are usually referred to medical facilities that specialize in this type of surgery. The success rate when performed by a surgeon experienced in this procedure is more than 80%. If the surgery is not successful, or provides only a temporary cure, it can be repeated, often with good outcomes[28] . If a tumor has grown beyond the sella turcica a transfrontal craniotomy may need to be performed. If there is hyperplasia of both adrenal glands, bilateral adrenalectomy may be needed[6] . Nursing consideration: Before surgery, the patient must undergo treatment to control edema, diabetes, hypertension, and other cardiovascular effects caused by Cushing’s syndrome. Patients must be especially careful to avoid infection prior to and after surgery [5,11] . Immediately prior to surgery, the administration of glucocorticoids can help prevent acute adrenal hypofunction during the surgery itself. During and after surgery, cortisol therapy should be administered to help the patient deal with the physiologic stress caused by the removal of the pituitary or adrenal gland(s)[5, 11] . In the event that normal cortisol production resumes, steroid therapy may be gradually tapered and ultimately discontinued, usually within a period of 12 to 18 months[5,6,11] . However, if both adrenal glands have been removed (bilateral adrenalectomy), or if the entire pituitary has been removed (total hypophysectomy), life-long steroid replacement therapy is necessary[5,11] . Radiation therapy If surgical approaches fail, or if a patient is not a candidate for surgery, radiation therapy is a possible alternative treatment. Radiation treatment to the pituitary gland is generally administered over a 6-week period. Improvement is noted in 40% to 50% of adults and up to 85% of children[28] . Another option is stereotactic radiosurgery or gamma knife radiation. This allows for the delivery of radiation in a single high-dose treatment [28] . Radiation alert! It may take months or even years for patients to feel better after receiving radiation treatment alone. Radiation in conjunction with cortisol-inhibiting drugs can help speed up the recovery process[28] . Medications Patients with non-endocrine corticotropin-producing tumors require excision of the tumor followed by drug therapy. Drug therapy is also administered if the patient cannot undergo surgery. Medications prescribed include[5,6,11] : ● ● Mitotate: Mitotate (Lysodren) is toxic to the adrenal cortex. Its administration is referred to as medical adrenalectomy. Side effects of this drug include nausea, vomiting, diarrhea, somnolence, and depression. ● ● Meryrapone: Meryrapone (Metopirone) is given to control hypersecretion of steroids in those who fail to respond to mitotane. ● ● Aminoglutethimide: Aminoglutethimide (Cytadren) blocks cholesterol conversion to pregnenolone. This blocks cortisol production. Side effects include GI disturbances such as nausea, vomiting, and diarrhea, somnolence, and skin rashes. ● ● A combination of aminoglutethimide, cyproheptadine, and ketoconazole may be prescribed in an effort to decrease levels of cortisol. ● ● Aminoglutethimide may be given alone or along with metyrapone as part of the treatment for metastatic adrenal cancer. Nursing considerations: Nursing considerations of particular interest include [5,6]: ● ● Monitor patients carefully for signs of infection. Patients with Cushing’s syndrome are especially prone to infection. ● ● Facilitate physical and emotional rest. Cushing’s syndrome can trigger periods of emotional lability. Adequate rest is essential to help relieve some of this instability. ● ● Monitor weight, intake and output, electrolyte levels, hormone levels, and glucose levels. After bilateral adrenalectomy and/or pituitary surgery it is important that nurses[5,6] : ● ● Caution the patient to wear a medical identification bracelet. ● ● Instruct patients to inform their HCPs immediately if they develop infections, physical illness, and/or significant emotional stress, which may trigger the need for increased dosage of hormone therapy. Teach patients to take replacement steroid therapy with food or with antacids to reduce gastric irritation. It is often recommended that two-thirds of the dosage be taken in the morning and one-third in the early afternoon. This should mimic natural rates of adrenal secretion. Adrenogenital syndrome Donna is nearly 14 years old and has not yet begun to menstruate. She is starting to develop a faint mustache, which has made her the target of ridicule by her peers. Concerned, Donna’s mother, Shirley, decides to take her to be evaluated by a gynecologist. Physical examination shows excessive growth of axillary hair, failure to menstruate, and an enlarged clitoris. The physician orders a battery of diagnostic tests including serum electrolyte, aldosterone, renin, and cortisol levels. Test results in conjunction with history and physical findings indicate adrenogenital syndrome. Adrenogenital syndrome, perhaps more commonly known as congenital adrenal hyperplasia, is a syndrome caused by disorders of adrenocortical steroid biosynthesis. Most cases of the syndrome are due to the failure of the adrenal glands to produce enough cortisol[5,29] .