Cushing's Disease

Question 1

Three Layers of the Adrenal Cortex

Answer 1

1. Mineralocorticoids
   > Aldosterone
2. Glucocorticoids
   > Cortisol
3. Androgen
   > Testosterone

Question 2

What is Cushing’s Disease?

Answer 2

1. Also known as HYPERCORTISOLISM; is the excess secretion of cortisol from the adrenal cortex causing many problems
2. Caused by a problem in the adrenal cortex itself, a problem in the anterior pituitary gland or a problem in the hypothalamus
3. MOST COMMON CAUSE is GLUCOCORTICOID THERAPY

Question 3

Causes of Cushing’s Disease

Answer 3

1. Pituitary Cushing Disease: when the anterior pituitary gland over-secretes ACTH, this hormone causes hyperplasia of the adrenal cortex in both adrenal glands and an excess of glucocorticoid production
2. Primary Cushing Disease: when excess glucocorticoids are caused by a problem in the actual cortex, usually a benign tumor (adrenal adenoma) and usually occurs in only one adrenal gland

Question 4

What Causes Cushing Syndrome?

Answer 4

1. Excess hormone production (CRH, ACTH, glucocorticoids), or excess exogenous corticosteroids
   > Small lung cell cancer

Question 5

Signs and Symptoms of Cushing Disease

Answer 5

1. General Appearance
   o Moon Face
   o Buffalo Hump
   o Truncal Obesity
   o Weight Gain
2. Cardiovascular:
   o Hypertension
   o Frequent dependent edema
   o Bruising
   o Petechiae
3. Immune System:
   o Increased risk for infection
   o Reduced immunity
   o Decreased inflammatory responses
   o Signs and symptoms of infection and inflammation possibly masked
4. Musculoskeletal:
   o Muscle Atrophy (most apparent in extremities)
   o Osteoporosis with
    Fragile Fractures
    Decreased height and vertebral collapse
    Aseptic necrosis of the femur head
    Slow or poor healing of the bone fractures
5. Skin:
   o Thinning skin
   o Increased facial and body hair
   o Striae and increased pigmentation

Question 6

History

Answer 6

1. Ask about the patient’s other health problems and drug therapies because glucocorticoid drug therapy is common
2. Reports of weight gain and increased appetite
3. Ask about changes in activity or sleep patterns, fatigue and muscle weakness
4. Ask about bone pain or history of fractures because osteoporosis results from hypercortisolism
5. Ask about history of frequent infections and easy bruising
6. Women often stop menstruating
7. GI problems include ulcer formation from increased hydrochloric acid secretion and decreased production of protective gastric mucus

Question 7

Physical Assessment

Answer 7

1. Changes in fat distribution may result in fat pads on the NECK, BACK, and shoulder (BUFFALO HUMP); an enlarged truck with thin arms and legs; and a round face (MOON FACE).
    Other changes include muscle wasting and weakness
    Assess for and document changes and use these finding to prioritize patient problems

Question 8

Physical Assessment: Skin Changes

Answer 8

1. Result from blood vessel fragility and include:
   
   • Bruises
   • Thin or translucent skin
   • Wounds that have not healed
   • Reddish-purple striae (stretch marks) occur on the abdomen, thins and upper arms because of the destructive effect of cortisol on collagen
   • Acne and fine coating of hair may occur over the face and body
   • In women: look for the presence of hirsutism, clitoral hypertrophy
   • In male: pattern balding related to androgen excess

Question 9

Physical Assessment: Cardiac Changes

Answer 9

1. Occurs as a result of disturbed fluid and electrolyte imbalance
2. Both sodium and water are retained, leading to hypervolemia and edema formation
3. BP elevated
4. Pulses are full and bounding

Question 10

Physical Assessment: Musculoskeletal Changes

Answer 10

1. Occurs as a result of nitrogen depletion and mineral loss
2. Muscle mass decrease (arms and legs)
3. Muscle weakness (increased risk for falls)
4. Bone is thinner, osteoporosis is common, increasing the risk for fractures

Question 11

Physical Assessment: Glucose Metabolism

Answer 11

1. Fasting glucose is high because the liver releases glucose and the insulin receptors are less sensitive; therefore, blood glucose does not move as easily into the tissues

Question 12

Physical Assessment: Immunity Change

Answer 12

1. Results in decreased immunity
2. Cortisol reduces the number of circulating lymphocytes, inhibits macrophage activity, reduces antibody synthesis and inhibits production of cytokines and inflammation chemical (histamine)
3. Infection risk is increased; the patient may not have fever, purulent exudate, or redness in the affected area when an infection is present

Question 13

Psychosocial Assessment

Answer 13

1. Hypercortisolism can result in emotional instability and patients often say they do not feel like themselves
2. Ask about mood swings, irritability, new-onset confusion or depression
3. Ask patient if they cry or laugh inappropriately or has difficulty concentrating
4. The patient may report sleep difficulties and fatigue

Question 14

Diagnosis

Answer 14

1. Dexamethasone Suppression Testing
    Can take place overnight or a 3-day period
    Set dosages of dexamethasone are given
    A 24-hour urine collection follows drug administration
    When urinary 17-hydroxycorticosteriod excretion and cortisol levels are suppressed by dexamethasone, Cushing disease is NOT present
2. Serum Cortisol Levels
3. Electrolytes, Glucose
4. Urinary Free Cortisol >25 nmol/mmol
5. ACTH Levels

Question 15

Laboratory

Answer 15

1. Pituitary Cushing Disease: ACTH levels are elevated
2. Adrenal Cushing Disease of when Cushing Syndrome results from chronic steroid use: ACTH levels are low
3. Salivary Cortisol Levels: HIGH (normal level lower than 2.0 ng/mL)
4. Increased sodium
5. Increase blood glucose
6. Decreased lymphocytes (WBC)
7. Decreased calcium level

Question 16

Imaging Assessment

Answer 16

1. CT scan, MRI and arteriography
   o These images can identify lesions of the adrenal or pituitary glands, lung, GI tract or pancreas

Question 17

Intervention: Patient Safety

Answer 17

1. Patient Safety:
   
   1. Prevent fluid overload from becoming worse and lead to pulmonary edema
   2. Any age is at risk for these complications
   3. The older adult or one who has co-existing cardiac problems, kidney problems, pulmonary problems or liver problems are at greater risk
2. MONITOR for indicator of fluid overload (bound pulse, increasing neck vein distention, lung crackles, increasing peripheral edema, reduced urine output) at least EVERY 2 HOURS
3. Notify provider of any change that indicate fluid overload either not responding to therapy or is worse
4. Patient is AT RISK FOR SKIN BREAKDOWN
   
   • Use a pressure-reducing or pressure relieving overlay on the mattress
   • Assess skin pressure area (coccyx, elbows, hip, and heels) daily for redness or open areas
   • Those receiving oxygen, check skin around the mask, nares and ears under the elastic band
   • Help change the patient position every 2 hours

Question 18

Intervention: Drug Therapy

Answer 18

1. Steroidogenesis Inhibitors
   
   • Interfere with ACTH production or adrenal hormone synthesis for temporary relief
   • For patients with hypercortisolism resulting from increased ACTH production, CYPROHEPTADINE may be used to because it interferes with ACTH production
   • For adults with increased ACTH production who have type-2 diabetes and who not respond to other drug therapies, MIFEPRISTONE is a synthetic steroid that blocks glucocorticoid receptors
2. Monitor the patient for response to drug therapy, especially weight loss and increase urine output
3. Observe for symptoms of problems with fluid and electrolytes balance (especially ECG patterns)
4. Assess SODIUM and POTASSIUM levels when drawn

Question 19

Intervention: Nutrition Therapy

Answer 19

1. Restriction of FLUID and SODIUM intake to control fluid volume
2. Sodium restriction involves “no added salt” to ordinary table foods when fluid overload is MILD
3. More pronounce fluid overload, the patient may be restricted to anywhere from 2g/day to 4g/day
4. Teach family how to check food labels for sodium content and how to keep a daily record of sodium ingested
5. Explain the reason for any fluid restriction and the importance of adhering to the prescribed restriction

Question 20

Intervention: Monitoring Intake, Output and Weight to Assess Therapy Effectiveness

Answer 20

1. Weight must be accurate, not estimated
2. Schedule fluid offerings throughout the 24 hours
3. Check urine specific gravity (a specific gravity below 1.005 may indicate fluid overload)
4. If IV therapy is used, infuse only the amount prescribed
5. FLUID RETENTION MAY NOT BE VISIBLE. RAPID WEIGHT GAIN IS THE BEST INDICTATOR OF FLUID RETENTION AND OVERLOAD
6. Weigh patient at the same day each day, at the same time, wearing the same clothing type

Question 21

Intervention: Fragile Fractures

Answer 21

1. Happens from bone density loss and osteoporosis
2. Help patient to move in bed with a lift sheet
3. Remind patient to call for help when walking
4. Review the use of walkers or canes, if needed
5. Use gait belt when walking with patient who has bone density loss
6. Collaborate with a RDN to teach patient about nutrition therapy
   
   • A HIGH-CALORIE diet that includes increase amounts of calcium and vitamin D is needed
   • Milk, cheese, yogurt and green leafy vegetables add calcium to promote bone density
   • Advise patient to avoid caffeine and alcohol, which increases the risk for GI ulcers and reduce bone density

Question 22

Intervention: GI Bleeding

Answer 22

1. Common with hypercortisolism
2. Cortisol
   
   • Inhibits production of the thick gel-like mucus that protects the stomach lining
   • Decreased blood flow to the area
   • Triggers the release of excess hydrochloric acid
3. Interventions focus on drug therapy to reduce irritation, protect the GI mucosa and decrease secretions of hydrochloric acid
   
   • Antacids buffer stomach acid and protect the GI mucosa
     o Teach patient that these drugs should be taken on a regular basis
     o Omeprazole inhibits the gastric proton pump and prevent the formation of hydrochloric acid
4. Instruct the patient to reduce alcohol or caffeine consumption, smoking and fasting because these actions cause gastric irritation
5. NSAIDS and drugs that contain aspirin can cause gastritis and intensify GI bleeding. (THESE SHOULD BE AVOID OR LIMITED)

Question 23

Intervention: Preventing Infection

Answer 23

1. Protect the patient with reduced immunity from infection
   
   • Handwashing is IMPORTANT
   • Those with an upper respiratory infection must wear a mask upon entering the room
   • Observe strict aseptic technique when performing dressing changes or any invasive procedure
2. Continually assess the patient for possible infections
   
   • Symptoms may not be obvious because excess cortisol suppresses infection indicators caused by inflammation
3. Monitor the patient’s daily CBC (especially WBC neutrophils)
   
   • Inspect the mouth for lesion and mucosa breakdown
   • Assess the lungs q8 hours for crackles, wheezes or reduced breath sounds
     o Perform pulmonary hygiene q2-4 hours
     o Urge patient to deep breathe or use an incentive spirometer

Question 24

Intervention: Surgical Management

Answer 24

1. If hypercortisolism is caused by an adrenal tumor, an ADRENALECTOMY (removal of the adrenal gland) is needed
2. Preoperative Care:
   
   • Starts with correcting disturbances of fluid and electrolytes
   • Continue to monitor POTASSIUM, SODIUM and CHLORIDE levels
     o Dysrhythmias from potassium imbalance may occur and cardiac monitoring is needed
   • Hyperglycemia is controlled before surgery
   • At risk for complications of infection and fractures
     o Prevent infection with handwashing and aseptic technique
     o Decrease the risk for falls by raising top side rails and encouraging the patient to ask for assistance when getting out of bed
     o A HIGH-CALORIE, HIGH PROTEIN DIET is prescribed before surgery
     o GLUCOCORTICOID preparations are given before surgery
      The patient receives glucocorticoid during surgery to prevent adrenal crisis because the removal of the tumor results in a sudden drop in cortisol levels
      Discuss the need for long-term hormone replacement therapy
3. POSTOPERATIVE CARE:
   
   • Monitored in an ICU
   • Immediately after surgery, assess the patient every 15 minutes for shock (hypotension, a rapid weak pulse, and decreased urine output) resulting from insufficient glucocorticoid replacement
   • Monitor vital signs, central nervous pressure, pulmonary wedge pressure, intake and output, daily weights and serum electrolyte levels
   • After a bilateral adrenalectomy, patients require lifelong glucocorticoid and mineralocorticoid HRT, starting immediately after surgery
   • In unilateral adrenalectomy, HRT continues until the remaining adrenal gland increases hormone production.
   • Therapy needed for up to 2 years after surgery

Question 25

Home Care Management:

Answer 25

1. Patient usually has muscle weakness and fatigue for some weeks after surgery and remains at risk for falls and other injury
2. These problems may necessitate one-floor living for a short time and

Question 26

Self-Management Education

Answer 26

1. Patient taking glucocorticoid at home is at risk for fluid and electrolyte balance (fluid volume excess)
   o Teach patient and family to monitor and record the patient’s weight daily
   o Call provider of a weight gain more than 3 lbs in a week or 1-2 lbs in 24 hours
2. After bilateral adrenalectomy, lifelong HRT is needed to prevent adrenal insufficiency. Without the adrenal gland, the patient is completely dependent on the drug. If the drug is stopped for a day or two, no other glands produce the glucocorticoids, and the patient develops acute adrenal insufficiency
   o Teach the patient and family about adherence to the drug regimen and its side effects
   > Cortisol Replacement Therapy:
   * Take your medication in divided doses (first dose in morning, second dose between 4 and 6 pm)
   * Take medication with a meal or snack to prevent stomach irritation
   * Weigh yourself daily and keep record
   * Increase dosage as directed by primary provider for increased physical stress or severe emotion stress
   * Never skip a dose of medication. If persistent vomiting or severe diarrhea and cannot take your medication by mouth for 24-36 hours, call primary provider or go to the ER (you may need an injection to take place of your usual oral medication)
   * Always wear your medical alert bracelet or necklace
   * Make regular visits for health care follow-up
   * Learn (and have family members learn) how to give yourself an intramuscular injection of hydrocortisone in case you cannot take your oral drug)
3. Protecting the patient with reduced immunity from infection is important
    Use proper hygiene and social distancing and to avoid crowds or others with infection
    Encourage patient and family members for yearly influenza vaccinations
    Notify provider immediately if you develop a fever or any other sign of infection