Chapter 18 | Endocrine Disorders 439
● Postoperative nursing care involves maintaining IV fluids, monitoring hydration status, providing pain control, and postoperative assessment and initiation of medications. ● The nurse must monitor serum electrolytes.
CUSHING’S DISEASE
Cushing’s disease is a metabolic disorder caused by an overpro- duction of cortisol by the adrenal glands. The most common causes of Cushing’s disease are cortisol-producing tumors, adre- nal hyperplasia, and benign adrenal tumors. Children who are prescribed chronic steroids may be at risk for Cushing’s disease where the source of excess steroids is exogenous. It can be very difficult to diagnose and may take up to 5 years to manifest. Cushing’s disease is unusual in infancy and childhood. Clinical manifestations tend to develop slowly. Psychologically, the child may experience mood changes such as depression, anxiety, ir- ritability, and euphoria. Growth delay may be found on physical examination. Excessive cortisol levels may lead to hyperglyce- mia, causing diabetes, high blood pressure, or arteriosclerosis. Assessment Assessment for Cushing’s disease includes the following measures. Clinical Presentation ● Clinical manifestations develop slowly ● Alkalosis related to hypokalemia and hypercalcemia ● Excessive urinary calcium excretion ● Other symptoms include weight gain, pendulous abdomen, fatigue, muscle wasting, weakness of the extremities, round “moon-shaped” face, facial flushing, fat pad located between the shoulder blades known as a buffalo hump, and pink or purple stretch marks on the abdomen, thighs, and arms ● Females may experience irregular or absent menstrual cycles Diagnostic Testing ● 24-hour urine collection for urinary free cortisol and 17- hydroxycorticosteriod ● Dexamethasone suppression test: a test used to assess the status of the hypothalamic–pituitary–adrenal axis for the dif- ferential diagnosis of adrenal overproduction ● Saliva swabs to test cortisol levels ● Serum blood levels of glucose, cortisol, and electrolytes ● Bone scan to rule out osteoporosis ● MRI of the pituitary gland ● CT scan of the adrenal glands Nursing Interventions ● Nursing care is dependent on the cause of the overproduction of cortisol. ● Thorough knowledge of medications used to inhibit the pro- duction of cortisol. ● If surgery is not possible, the nurse may have to educate the patient/family regarding the use of radiation therapy and medications used to inhibit the production of cortisol. ● Surgical intervention may be necessary to excise or remove a tumor. The nurse must provide preoperative and postopera- tive education.
SAFE AND EFFECTIVE NURSING CARE: Promoting Safety
Lifelong Cortisol Replacement Therapy Patient and caregiver education for Cushing’s disease should focus on the patient’s need for lifelong cortisol replacement ther- apy. Without cortisol replacement, the patient is at risk for life- threatening adrenal insufficiency. Patients and caregivers should be made aware that during times of stress or illness, the dose may need to be increased. Patients and their caregivers should be taught to give cortisol injections as a replacement when they are unable to take anything by mouth or are vomiting. Caregiver Education ● Emergency care involves educating the patient regarding life- long cortisol replacement therapy daily and in emergency situations. ● The patient must be taught stress dosing with hydrocortisone injections during times when the child is extremely ill, has a fever, is vomiting, experiences trauma, or is having surgery. ● The parents should be reassured that the child’s symptomatic appearance will improve over time with treatment. ● Offer nutritional guidance for healthy food selections that will help the child maintain a healthy weight. ● Instruct the parents on signs of adrenal insufficiency, such as increased irritability, headache, confusion, restlessness, nau- sea and vomiting, diarrhea, abdominal pain, dehydration, fever, loss of appetite, and lethargy. These symptoms can be life-threatening, and the parents should take the child to the emergency department as soon as symptoms are identified.
CONGENITAL ADRENAL HYPERPLASIA
Congenital adrenal hyperplasia (CAH) is the inability to produce cortisol in the adrenal glands, caused by excessive amounts of CRH and adrenocorticotropic hormone production (ACTH). Because of the surplus of CRH and ACTH, the adrenal gland begins to develop hyperplasia, leading to excessive androgen hor- mone production from the adrenal glands. Defective conversion of 17-hydroxyprogesterone (17-OHP) to 11-deoxycorticol ac- counts for more than 95% of the cases of CAH. The defective conversion is caused by mutations in the CYP21A2 gene, hence making 21-hydroxylase deficiency one of the more common in- herited disorders (Merke, 2015). CAH can present in two clinical syndromes: (1) classic or (2) nonclassic (Merke & Auchus, 2021).
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