Endocrine Disorder (Cushing's syndrome)

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1. MEDICAL SURGICAL NURSING-1 TOPIC: CUSHING SYNDROME PRESENTED BY SEIF SAID KHALFAN (BS c . N, student)

2. CUSHING’S SYDROME Cushing’s syndrome (hypercortisolism) is a hormonal disorder caused by prolonged exposure high levels of steroid hormones called glucocorticoids. It is commonly caused by use of corticosteroid medications and is infrequently the result of excessive corticosteroid production secondary to hyperplasia of the adrenal cortex.

3. Cont … It may also be caused by several mechanisms, including a tumor of the pituitary gland or less commonly an ectopic malignancy that produces adrenocorticotropic hormone (ACTH). Regardless of the cause, the normal feedback mechanisms that control the function of the adrenal cortex become ineffective, resulting in over secretion of glucocorticoids, androgens, and possibly mineralocorticoid.

4. Cont … Cushing syndrome occurs five times more often in women ages 20 to 40 years than in men.

5. Etiology of Cushing's syndrome Excessive activity of the adrenal cortex Corticosteroids / ACTH excess Adrenal cortex hyperplasia.

6. Pathophysiology Normally, the production and release of cortisol follows a precise chain of events. Corticotropin-releasing hormone (CRH) released from the hypothalamus causes the pituitary gland to secrete ACTH, which in turn results in the production of cortisol from the adrenal glands (located just above the kidneys).

7. Cont… In healthy people, cortisol begins to rise between 3 and 4 am, reaches a peak between 7 and 9 am, and then falls for the rest of the day to the lowest levels when the person is unstressed and asleep at midnight.  Cortisol controls its own production and release by switching off the hormones CRH and ACTH, a form of hormonal regulation known as “negative feedback”. 

8. Cont … ACTH and cortisol become elevated in response to stress, such as surgery, trauma, infection or other medical illnesses. i.e. Stress response.

9. Types of Cushing's syndrome Exogenous Cushing’s syndrome: caused by taking excessive amounts of medications e.g. prednisone, dexamethasone  for chronic asthma, rheumatoid arthritis, lupus, to suppress immune system after transplant to prevent rejection, other inflammatory diseases. Endogenous Cushing’s syndrome: excess cortisol produced by the adrenal glands. This is far rarer, but if left undiagnosed & untreated it can result in a shorter lifespan.

10. Signs and Symptoms  of  Cushing's  syndrome   Most people with Cushing syndrome will have: Upper body obesity (above the waist) and thin arms and legs Round, red, full face (moon face)

11. Cont.. Skin changes that are often seen: Acne or skin infections Purple/red marks (1/2 inch or more wide) called striae on the skin of the abdomen, thighs, and breasts Thin skin with easy bruising

12. Cont … Muscle and bone changes include: Backache, which occurs with routine activities Bone pain or tenderness Collection of fat between the shoulders (buffalo hump) Rib and spine fractures (caused by thinning of the bones) Weak muscles

13. Cont … Women with Cushing syndrome often have: Excess hair growth on the face, neck, chest, abdomen, and thighs Menstrual cycle that becomes irregular or stops Men may have : Decreased or no desire for sex Impotence

14. Cont … Other symptoms that may occur with this disease: Mental changes, such as depression, anxiety, or changes in behavior Fatigue Headache Increased thirst and urination

15. Diagnostic measure Diagnosis is based on a review of a person's medical history, a physical examination, and laboratory tests. X rays of the adrenal or pituitary glands can be useful in locating tumors . No single lab. test is perfect and usually several are needed. The most common tests used to diagnose Cushing's syndrome are :-

16. 1. 24-hour 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 is used.

17. 2.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.

18. Cont … The test generally requires a 48-hour hospital stay to avoid falsely elevated cortisol levels due to stress. 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.

19. 3.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.

20. Cont … 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.

21. 4.Dexamethasone-corticotropin-releasing hormone (CRH) t est . 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 pseudo-Cushing'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.

22. Cont … 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.

23. Treatment/Management The management/treatment of Cushing's syndrome can be surgical, medical or radiation. Treatment is usually directed at the pituitary gland because most cases are due to pituitary tumors rather than tumors of the adrenal cortex . The following are the treatment/management of Cushing's syndrome. Surgical removal of the tumor by transsphenoidal hypophysectomy is the treatment of choice (80% success rate).

24. Cont … 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 may be necessary until the adrenal glands begin to respond normally (may be several months ). If bilateral adrenalectomy was performed, lifetime replacement of adrenal cortex hormones is necessary.

25. Cont … Adrenal enzyme inhibitors ( eg , metyrapone , aminoglutethimide , mitotane , ketoconazole) may be used with ectopic ACTH secreting tumors that cannot be totally removed; monitor closely for inadequate adrenal function and side effects . If Cushing syndrome results from exogenous corticosteroids, taper the drug to the minimum level or use alternate day therapy to treat the underlying disease.

26. GENERAL NURSING MANAGEMENT • Weigh each morning, using the same scale. • Maintain an accurate record of intake and output. • Ensure adequate lighting in the room, and wear glasses and shoes when getting out of bed. • Develop a written schedule of rest and activity periods. • If agreeable, provide a private room, and restrict visitors at this time.

27. Cont … • Use strict medical and surgical asepsis when providing care . • Provide time for discussion of the disease and treatment; encourage verbalization of feelings and identify successful coping mechanisms used in the past . •Encourage turning , coughing, and deep breathing and/or incentive spirometry every 2–4 hours

28. Complications If you don't receive prompt treatment for Cushing syndrome , complications may occur, such as: Bone loss (osteoporosis), which can result in unusual bone fractures, such as rib fractures and fractures of the bones in the feet High blood pressure (hypertension) Diabetes

29. Cont … Frequent or unusual infections Loss of muscle mass and strength When the cause of Cushing syndrome is a pituitary tumor (Cushing disease), it can sometimes lead to other problems, such as interfearing with the production of other hormones controlled by the pituitary.

30. NUSING CARE PLAN

31. DATE NURSING DIAGNOSIS NURSING GOAL/OUTCOME INTERVENTION EVALUATION Risk for injury related to weakness Decreased risk of injury to the patient. Provide a protective environment to prevent falls, fractures, and other injuries to patient. Assist the patient who is weak in ambulating to prevent falls or colliding into furniture. 3) Recommend foods high in protein, calcium, and vitamin D to minimize muscle wasting and osteoporosis; refer to dietitian for assistance. Patient has decreased risk of injury.

32. DATE NURSING DIAGNOSIS NURSING GOAL NURSING INTERVENTION EVALUATION Risk for infection related to altered protein metabolism and inflammatory response. Decreased risk of infection. Avoid unnecessary exposure to people with infections. 2) Assess frequently for subtle signs of infections (corticosteroids mask signs of inflammation and infection). Has decreased risk of infection

33. DATE NURSING DIAGNOSIS NURSING GOAL NURSING INTERVENTION EVALUATION Impaired skin integrity related to edema, impaired healing, and thin and fragile skin Improved skin integrity Use meticulous skin care to avoid traumatizing fragile skin. 2) Avoid adhesive tape, which can tear and irritate the skin. 3) Assess skin and bony prominences frequently. 4) Encourage and assist patient to change positions frequently. Attains or maintains skin integrity

34. DATE N.DIAGNOSIS NURSING GAOL NURSING INTERVENTION EVALUATION Disturbed body image related to altered appearance, impaired sexual functioning, and decreased activity level Improved body image 1) Discuss the impact that changes have had on patient’s self-concept and relationships with others. Major physical changes will disappear in time if the cause of Cushing syndrome can be treated. 2) Weight gain and edema may be modified by a low-carbohydrate, low-sodium diet; a high-protein intake can reduce some bother some symptoms. Achieves improved body image of the patient.

35. DATE N.DIAGNOSIS NURSING GOAL NURSING INTERVENTION EVALUATION Assess patient’s mental function, including mood, response to questions, depression, and awareness of environment. Improving Thought Processes Explain to patient and family the cause of emotional instability, and help them cope with mood swings, irritability, and depression. 2) Report any psychotic behavior. 3) Encourage patient and family members to verbalize feelings and concerns. Exhibits improved mental functioning Experiences no complications