A DISEASE STUDY IN CUSHING¶S SYNDROME
Submitted by: Kristine Nelly M. Manuel, SN, UPHSI
BSN III ± A
Submitted to: Mr. Jay-Ar C. Garcia, RN, RM, MSN
Clinical Instructor
DEFINITION CUSHING¶S SYNDROME is a hormonal disorder caused by prolonged exposure of the body¶s tissues to high levels of the hormone cortisol. Sometimes called hypercortisolism , Cushing¶s syndrome is relatively rare and most commonly affects adults aged 20 to 50. People who are obese and have type 2 diabetes, along with poorly controlled blood glucose²also called blood sugar²and high blood pressure, have an increased risk of developing the disorder. CUSHING¶S SYNDROME results from excessive secretion of one or all of the adrenocortical hormones which includes glucocorticoid cortisol (predominant type), the mineralocorticoid aldosterone co rticoids. , and the adrogenital corticoids. CUSHING'S SYNDROME or HYPERCORTISOLISM is an endocrine disorder caused by excessive levels of the endogenous corticosteroid hormone cortisol. It may also be induced iatrogenically by treatment with exogenous corticosteroids for other medical conditions. It was discovered by American physician, surgeon and endocrinologist Harvey Cushing (18691939) and reported by him in 1932.
ETIOLOGY CUSHING¶S SYNDROME occurs when the body¶s tissues are exposed to high levels of cortisol for too long. Many people develop Cushing¶s syndrome because they take glucocorticoids²steroid hormones that are chemically similar to naturally produced cortisol² such as prednisone for asthma, rheumatoid arthritis, lupus, and other inflammatory diseases. Glucocorticoids are also used to suppress the immune system after transplantation to keep the body from rejecting the new organ or gan or tissue.
Other people develop Cushing¶s syndrome because their bodies produce too much cortisol. Normally, the production of cortisol follows a precise chain of events. First, the hypothalamus, a part of the brain about the size of a small sugar cube, sends corticotropinreleasing hormone (CRH) to the pituitary gland. CRH causes the pituitary to secrete adrenocorticotropin hormone (ACTH), which stimulates the adrenal glands. When the adrenals, which are located just above the kidneys, receive the ACTH, they respond by releasing cortisol into the bloodstream. Cortisol performs vital tasks in the body including: y y y y
helping maintain blood pressure and cardiovascular function reducing the immune system¶s inflammatory response balancing the effects of insulin, which breaks down glucose for energy regulating the metabolism of proteins, carbohydrates, and fats
RISK FACTOR
Cardiovascul ar
H ypertension ypertension
H ypok ypok alemi a
I mp mpaired
Obesity.
H yperlipidemi yperlipidemi a
oagulopathy C
glucose toler ance and di ab abetes
CLASSIFICATION 1. 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. 2. ADRENAL CUSHING¶S SYNDROME is associated with adenoma or carcinoma of the adrenal cortex. The disease may recur after surgery. 3. ECTOPIC CUSHING¶S SYNDROME results from autonomous ACTH secretion by extrapituitary tumors (such as the lungs) producing excess ACTH. 4. IATROGENIC CUSHING¶S SYNDROME is caused by exogenous glucocorticoid administration.
SIGNS AND SYMPTOMS The list of signs and symptoms mentioned in various sources for Cushing's syndrome includes the 77 symptoms listed below: y
Specific childhood or adolescent Cushing's symptoms: o o o o o o o o o
Extreme weight gain
Growth retardation Missed periods - in teenage girls Excess hair growth Body hair growth Facial hair growth Acne Reddish-blue streaks on the skin High blood pressure
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Tiredness Muscle weakness Very early puberty Very late puberty
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Adult ushing's ushing's C o
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symptoms Extreme weight gain Upper body obesity Rounded face Increased neck fat Excess hair growth High blood pressure Sleep disturbances High blood sugar iabetes-like symptoms Diabetes-like Hyperadrenocorticism Bruising easily Skin problems Thin skin Fragile skin Poor healing Stretch marks Abdominal stretch marks Mood changes Irritability Anxiety Depression Moodiness Fatigue Mental disturbances Weak muscles Backaches Thinning arms Thinning legs Weak bones Rib fractures Spinal column fractures Sexual and fertility fertility problems pro blems Irregular menstrual periods Absent menstrual periods Reduced libido Reduced male fertility Virilisation Deepening voice Balding Loss of scalp hair
o o o o o o o o o o o o o o o o o o
o
Hirsutism Increased facial hair Increased body hair Increased weight Obesity around trunk Slender limbs Red face Slow-healing skin Weakened connective connect ive tissue Reddish-blue stretch marks Excessive hair thickness in women Menstrual disorders Impaired male fertility Reduced male sex drive Absent male sex drive Hypertension Brittle bones Fractures Osteoporosis Hyperglycemia Severe weakness Psychiatric disturbances
COMPLICATIONS
M et et ab abolic
syndrome Osteoporosis P sychologic sychologic al and cognitive alter ations Alter ations of other endocrine systems E ffects ffects of subclinical hypercortisolism
NORMAL AND ABNORMAL The hypothalamus sends CRH to the pituitary, which responds by secreting ACTH. ACTH then causes the adrenals to release cortisol into the blood stream. stream. One of cortisol¶s most important jobs is to help the body respond to stress. For this reason, women in their last 3 months of pregnancy and highly trained athletes normally have high levels of the hormone. People suffering from depression, alcoholism,
malnutrition, or panic disorders also have increased cortisol levels. When the amount of cortisol in the blood is adequate, the hypothalamus and pituitary release less CRH and ACTH. This process ensures the amount of cortisol released by the adrenal glands is precisely balanced to meet the body¶s daily needs. However , if something goes wrong with the adrenals or the regulating switches in the pituitary gland or hypothalamus, cortisol production can go awry.
PATHOPHYSIOLOGY OF CUSHING'S DISEASE When stimulated by ACTH, the adrenal gland secretes cortisol and other steroid hormones. ACTH is produced by the pituitary gland and released into the petrosal venous sinuses in response to stimulation by corticotropin-releasing hormone (CRH) from the hypothalamus (Figure 2). ACTH is released in a diurnal pattern that is independent of circulating cortisol levels: peak release occurs just before awakening, and ACTH levels then decline throughout the day. Control of CRH and ACTH release is maintained through negative feedback by cortisol at the hypothalamic and pituitary levels. Neuronal input at the hypothalamic level can also stimulate CRH release. Although the adenomas of Cushing's disease secrete excessive amounts of ACTH, they generally retain some negative feedback responsiveness to high doses of glucocorticoids. Ectopic sources of ACTH, usually in the form of extracranial neoplasms, are generally not responsive to negative feedback with high doses of glucocorticoids. However , some overlap exists in the response to negative feedback between pituitary and ectopic ectop ic sources of excessive ACTH. e 2). syndrome is categorized as ACTH dependent or ACTH independent (Tabl This division is convenient for organizing the work-up of patients with suspected hypercortisolism.
ushing's ushing's C
Depression, alcoholism, medications, eating disorders and other conditions can cause mild clinical and laboratory findings, similar to those in Cushing's syndrome, termed "pseudo-
Cushing's syndrome." The laboratory and clinical findings of hypercortisolism disappear if the primary process is successfully treated.17-19
Dexamethasone, an exogenous glucocorticoid, is used to test for Cushing's syndrome.
This gluococorticoid does not interfere with cortisol assays but induces similar physiologic responses.
DIAGNOSTIC PROCESS When Cushing's is suspected, a dex amethasone suppression test (administration of dexamethasone and frequent determination of cortisol and ACTH levels) and 24-hour urinary have equal detection rates (Raff & Findling 2003). A novel approach measurement for cortisol is sampling cortisol in saliva over 24 hours, which may be equally sensitive. Other pituitary hormones may need to be determined and performing physical examination directed for any visual field defect may be necessary if a pituitary lesion is suspected (which may compress the optic chiasm causing typical bitemporal hemianopia). es est s are posit ive ive, CT scanning of the adrenal gl When these t MRI of the and and pituit are performed. These should be performed when other tests are positive, to ary gl and decrease likelihood of incidentalomas (incidental discovery of harmless lesions in both organs). Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Very rarely, determining the cortisol levels in various veins in the body by venous catheterisation working towards the pituitary (petrosal sinus sampling) is necessary.
No single lab test is perfect and usually several are needed. The three most common tests used to diagnose Cushing¶s syndrome are the 24-hour urinary free cortisol test, measurement of midnight plasma cortisol or late-night salivary cortisol, and the low-dose dexamethasone suppression test. Another test, the dexamethasone-corticotropin-releasing hormone test, may be needed to distinguish Cushing¶s syndrome from other causes o f excess cortisol. y
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urinary free cortisol level. In this test , a person¶s urine is collected several times over a 24-hour period and tested for cortisol. Levels higher than 50 to 100 micrograms a day for an adult suggest Cushing¶s syndrome. The normal upper limit varies in different laboratories, depending on which measurement technique tech nique is used. Midnight plasma cortisol and late-night salivary cortisol measurements. The midnight plasma cortisol test measures cortisol concentrations in the blood. Cortisol production is normally suppressed at night, but in Cushing¶s syndrome, this suppression doesn¶t occur. If the cortisol level is more than 50 nanomoles per liter (nmol/ L), Cushing¶s syndrome is suspected. The test generally requires a 48-hour hospital stay to avoid falsely elevated cortisol levels due to stress. 24-hour
However , a late-night or bedtime saliva sample can be obtained at home, then tested to determine the cortisol level. Diagnostic ranges vary, depending on the measurement technique used. y
Low-dose dexamethasone suppression test (LDDST). In the LDDST, a person is given a low dose of dexamethasone, a synthetic glucocorticoid, by mouth every 6 hours for 2 days. Urine is collected before dexamethasone is administered and several times on each day of the test. A modified LDDST uses a onetime overnight dose.
Cortisol and other glucocorticoids signal the pituitary to release less ACTH , so the normal response after taking dexamethasone is a drop in blood and urine cortisol levels. If cortisol levels do not drop, Cushing¶s syndrome is suspected. The LDDST may not show a drop in cortisol levels in people with depression, alcoholism, high estrogen levels, acute illness, or stress, falsely indicating Cushing¶s syndrome. On the other hand, drugs such as phenytoin and phenobarbital may cause cortisol levels to drop, falsely indicating that Cushing¶s is not present in people who actually have the syndrome. For this reason, physicians usually advise their patients to stop taking these drugs at least least 1 week before the test. y
Dexamethasone-corticotropin-releasing hormone (CRH) test. Some people have high cortisol levels but do not develop the progressive effects of Cushing¶s syndrome, such as muscle weakness, fractures, and thinning of the skin. These people may have pseudoCushing¶s syndrome, a condition sometimes found in people who have depression or anxiety disorders, drink excess alcohol, have poorly controlled diabetes, or are severely obese. Pseudo-Cushing¶s does not have the same long-term effects on health as Cushing¶s syndrome and does not require treatment directed at the endocrine glands.
The dexamethasone-CRH test rapidly distinguishes pseudo-Cushing¶s from mild cases of Cushing¶s. This test combines the LDDST and a CRH stimulation test. In the CRH stimulation test, an injection of CRH causes the pituitary to secrete ACTH. Pretreatment with dexamethasone prevents CRH from causing an increase in cortisol in people with pseudo-Cushing¶s. E levations of cortisol during this test suggest Cushing¶s syndrome.
MEDICAL MANAGEMENT y y y
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Monitor intake and output, daily weights, and serum glucose and electrolytes. Monitor for signs of infection because risk is high w ith 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 o n the skin to reduce t rauma 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 act ivities.
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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 o f 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.
SURGICAL MANAGEMENT y
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Pituitary surgery to treat pituitary pituitary Cushing¶s syndrome. syndro me. Transsphenoidal adenomectomy or hypophysectomy. o Transfrontal craniotomy may be necessary when a pituitary tumor has enlarged o beyond the sella turcica. Bilateral adrenalectomy is used to treat adrenal causes. Radiation therapy may also be used to treat pituitary or adrenal tumors. tu mors.
NURSING CARE MANAGEMENT Diagnosis:
Risk for infection
After 2 hours of nursing intervention the patient will be able to achieve timely wound healing.
Planning:
Interventions & Rationale:
I: Observe localized signs of infection at insertion sites of invasive lines, sutures, surgical incisions/ wounds. R: To determined the presence of infection.
a ll caregivers between therapies/clients. I: Stress proper hand hygiene by all therap ies/clients. /clients. R: A first-line defense against therapies I: Monitor client visitors/caregivers for respiratory illnesses. illnesses. ss-contamination. R: To limit exposures, thus reduce cro ss-contamination.
I: Provide for isolation, as indicated. R: Reduces risk of cross-contamination. I: Provide information/involve in appropriate community and national education programs. R: To increase awareness of/and prevention of o f communicable diseases.
Evaluation:
After 2 hours of nursing intervention the patient was able to achieve timely wound healing.
Impaired skin integrity related to slow sound healing
After 2 hours of nursing intervention the patient will be able to participate in prevention measures and treatment programs.
Diagnosis:
Planning:
Interventions and Rationale:
I: Evaluate client with impaired cognition, developmental delay, eed for/ use of restraints, long term immobility. R: To identify risk for injury/ safet y requirements. , texture and turgor. I: Note skin color R: To assess the skin for superinfection.
I: Periodically, remeasure/ photograph wound and o bserve for complications. R: To monitor progress of wound healing. I: Keep the area clean/dry, carefully dress wounds, support incisions. R: To assist body¶s natural process o f repair. I: Use appropriate barrier dressings, wound coverings, drainage appliances, wound vac, and skin protective agents for open/drainage wounds and stomas. R: To protect the wound and/or surrounding tissues. I: Limit/Avoid imit/Avoid use of plastic material. R: Moisture potentiates skin break down.
I: Encourage early ambulation/mobilization. R: Promotes circulation and reduces r isks associated with immobility.
Evaluation:
After 2 hours of nursing intervention the patient was able to participate in prevention measures and treatment programs.
Activity intolerance related to body weakness.
After 2 hours of nursing intervention, the patient will be able to participate willingly in necessary/desired act ivities. ivities.
Diagnosis:
Planning:
Interventions and Rationale:
I: Note presence of factor contributing to fatigue. R: fatigue affects both the clients actual and perceived ability to participate in activities. I: Note client report of weakness, fatigue, pain, difficulty accomplishing tasks, and/or insomnia. R: Symptoms may be result of/or contribute to intolerance intolerance of activi act ivity. ty. I: Ascertain ability to stand and move about and degree of assistance necessary/ used of equipment. R: To determine current status and needs associated with participation in needed/desires activities act ivities.. I: Adjust activi act ivities. ties. o verexertion. R: To prevent overexertion. I: Increase exercise/activity levels gradually; teach methods. R: To conserve energy.
I: Provide positive atmosphere, while acknowledging difficulty of the situation for the client. R: Helps to minimize frustration and rechannel energy. I: assist with activities and provide/ monitor clients use of assistive devices. R: To protect client from injury. I: Instruct client in monitoring response to activity and in recognizing signs and symptoms. R: it indicate need to alter activity activity level. I: Encourage client to maintain positive attitude, suggest use of relaxation techniques, such as visualization/guided imagery. R: To enhance sense of well being.
Evaluation:
After 2 hours of nursing intervention, the patient was able to participate willingly in necessary/desired activi act ivities. ties.
PROGNOSIS Iatrogenic Cushing's syndrome (caused by treat ment with corticosteroids) is the most common form of Cushing's syndrome. Endogenous (i.e. non-iatrogenic) Cushing's syndrome is relatively rare. Incidence of causes of o f Cushing's syndrome per million person-years: y y y
Cushing's disease: 10 Primary adrenal: 3 Ectopic ACTH: 1.4
of ushing's ushing's syndrome: Many cases can be cured. Results depend on the underlying C cause and severity of the t he problem. P rognosis rognosis
of ushing's ushing's syndrome: The prognosis for individuals with Cushing's Cushing 's syndrome C varies depending on the t he cause of overproduction of cortisol. With treatment, most individuals with Cushing's syndrome show significant improvement, while improvement for others may be complicated by various aspects of o f the causative illness. Some kinds of tumors may recur. Mo st cases of Cushing's can be cured. P rognosis rognosis
POINTS TO REMEMBER y
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Cushing¶s syndrome is a disorder caused by prolonged exposure of the body¶s tissues to high levels of the hor mone cortisol. Typical signs and symptoms of Cushing¶s syndrome include upper body obesity, a rounded face, skin that bruises easily and heals poorly, weakened bones, excess body hair growth and menstrual irregularities in women, and decreased fertility in men. Cushing¶s syndrome is caused by exposure to glucocorticoids, which are used to treat inflammatory diseases, or by the body¶s overproduction of cortisol, most often due to tumors of the pituitary gland or lung. Several tests are usually needed to diagnosis Cushing¶s syndrome, including urine, blood, and saliva tests. Other tests help find the cause of the syndrome. Treatment depends on the specific reason for excess cortisol and may include surgery, radiation, chemotherapy, or the use o f cortisol-inhibiting cortisol-inhibiting drugs.
REFERENCES http://endocrine.niddk.nih.gov/pubs/cushings/cushings.htm http://nursingcrib.com/nursing-notes-reviewer/cushings-syndrome/ http://www.aafp.org/afp/20000901/1119.html http://www.experiencefestival.com/a/Cushings_syndrome/id/2034692
