A Disease Study of Cushing's Disease

8/8/2019 A Disease Study of Cushing's Disease

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A

DISEASE STUDY

IN

CUSHINGS SYNDROME

Submitted by:

Kristine Nelly M. Manuel, SN, UPHSI

BSN III A

Submitted to:

Mr. Jay-Ar C. Garcia, RN, RM, MSN

Clinical Instructor


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DEFINITION

CUSHINGS SYNDROME is a hormonal disorder caused by prolonged exposure of the

bodys tissues to high levels of the hormone cortisol. Sometimes called hypercortisolism ,Cushings 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 glucosealso calledblood sugarand high blood pressure, have an increased risk of developing the disorder.

CUSHINGS SYNDROME results from excessive secretion of one or all of the

adrenocortical hormones which includes glucocorticoid cortisol (predominant type), the

mineralocorticoid aldosterone, and the adrogenital corticoids.

CUSHING'S SYNDROME orHYPERCORTISOLISM is an endocrine disordercaused 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 (1869-

1939) and reported by him in 1932.

ETIOLOGY

CUSHINGS SYNDROME occurs when the bodys tissues are exposed to high levels ofcortisol for too long. Many people develop Cushings syndrome because they take

glucocorticoidssteroid hormones that are chemically similar to naturally produced cortisolsuch as prednisone for asthma, rheumatoid arthritis, lupus, and other inflammatory diseases.

Glucocorticoids are also used to suppress the immune system after transplantation to keep thebody from rejecting the new organ or tissue.

Other people develop Cushings syndrome because their bodies produce too much

cortisol. Normally, the production of cortisol follows a precise chain of events. First, thehypothalamus, a part of the brain about the size of a small sugar cube, sends corticotropin-

releasing hormone (CRH) to the pituitary gland. CRH causes the pituitary to secreteadrenocorticotropin hormone (ACTH), which stimulates the adrenal glands. When the adrenals,

which are located just above the kidneys, receive the ACTH, they respond by releasing cortisolinto the bloodstream.

Cortisol performs vital tasks in the body including:

y helping maintain blood pressure and cardiovascular functiony reducing the immune systems inflammatory responsey balancing the effects of insulin, which breaks down glucose for energyy regulating the metabolism of proteins, carbohydrates, and fats


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RISK FACTOR

CardiovascularHypertensionHypokalemiaImpaired glucose tolerance and diabetesObesity.HyperlipidemiaCoagulopathy

CLASSIFICATION

1.PITUITARY CUSHINGS SYNDROME (CUSHINGS DISEASE) is themost common cause of Cushings syndrome, and stems from hyperplasia of bothadrenal 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 CUSHINGS SYNDROME is associated with adenoma or

carcinoma of the adrenal cortex. The disease may recur after surgery.

3. ECTOPIC CUSHINGS SYNDROME results from autonomous ACTH

secretion by extrapituitary tumors (such as the lungs) producing excess ACTH.

4. IATROGENIC CUSHINGS SYNDROME is caused by exogenous

glucocorticoid administration.

SIGNS AND SYMPTOMS

The list of signs and symptoms mentioned in various sources for Cushing's syndromeincludes the 77 symptoms listed below:

y Specific childhood or adolescent Cushing's symptoms:oExtreme weight gainoGrowth retardationoMissed periods - in teenage girlsoExcess hair growthoBody hair growthoFacial hair growthoAcneoReddish-blue streaks on the skinoHigh blood pressure


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oTirednessoMuscle weaknessoVery early pubertyoVery late pubertyo

oAdultCushing's symptomsoExtreme weight gain

Upper body obesityRounded faceIncreased neck fat

oExcess hair growthoHigh blood pressureoSleep disturbancesoHigh blood sugaroDiabetes-like symptomsoHyperadrenocorticismoBruising easilyoSkin problems

Thin skinFragile skinPoor healingStretch marksAbdominal stretch marks

oMood changesIrritabilityAnxietyDepressionMoodinessFatigueMental disturbances

oWeak musclesBackachesThinning armsThinning legs

oWeak bonesRib fracturesSpinal column fractures

oSexual and fertility problemsIrregular menstrual periodsAbsent menstrual periodsReduced libidoReduced male fertilityVirilisationDeepening voiceBaldingLoss of scalp hair


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HirsutismIncreased facial hairIncreased body hair

oIncreased weightoObesity around trunkoSlender limbsoRed faceoSlow-healing skinoWeakened connective tissueoReddish-blue stretch marksoExcessive hair thickness in womenoMenstrual disordersoImpaired male fertilityoReduced male sex driveoAbsent male sex driveoHypertensionoBrittle bonesoFracturesoOsteoporosisoHyperglycemiaoSevere weaknessoPsychiatric disturbances

COMPLICATIONS

Metabolic syndromeOsteoporosisPsychologicaland cognitive alterationsAlterations of other endocrine systemsEffects 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 bloodstream.

One of cortisols most important jobsis to help the body respond to stress. For this

reason, women in their last 3 months ofpregnancy and highly trained athletes

normally have high levels of the hormone.People suffering from depression, alcoholism,


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malnutrition, or panic disorders also have increased cortisol levels.

When the amount of cortisol in the blood is adequate, the hypothalamus and pituitaryrelease less CRH and ACTH. This process ensures the amount of cortisol released by the adrenal

glands is precisely balanced to meet the bodys daily needs. However, if something goes wrong

with the adrenals or the regulating switches in the pituitary gland or hypothalamus, cortisolproduction can go awry.

PATHOPHYSIOLOGY OF CUSHING'S DISEASE

When stimulated by ACTH, theadrenal gland secretes cortisol and other

steroid hormones. ACTH is produced by thepituitary gland and released into the petrosal

venous sinuses in response to stimulation bycorticotropin-releasing hormone (CRH) from

the hypothalamus (Figure 2). ACTH isreleased in a diurnal pattern that is

independent of circulating cortisol levels:peak release occurs just before awakening,

and ACTH levels then decline throughout theday. Control of CRH and ACTH release is

maintained through negative feedback bycortisol 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. Ectopicsources 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 theresponse to negative feedback between pituitary and ectopic sources of excessive ACTH.

Cushing's syndrome is categorized as ACTH dependent or ACTH independent (Table 2).This division is convenient for organizing the work-up of patients with suspected

hypercortisolism.

Depression, alcoholism, medications, eating disorders and other conditions can causemild 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 theprimary process is successfully treated.17-19


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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 dexamethasonesuppression test(administration of

dexamethasone and frequent determination of cortisol and ACTH levels) and 24-hour urinarymeasurementfor cortisolhave equal detection rates (Raff & Findling 2003). A novel approach

is sampling cortisol in saliva over 24 hours, which may be equally sensitive. Other pituitaryhormones 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 theoptic chiasm causing typical bitemporal hemianopia).

When these tests are positive,CTscanning of the adrenal glandandMRIof the

pituitary glandare performed. These should be performed when other tests are positive , to

decrease likelihood of incidentalomas (incidental discovery of harmless lesions in both organs).Scintigraphy of the adrenal gland with iodocholesterol scan is occasionally necessary. Veryrarely, 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 Cushings syndrome are the 24-hour urinary free cortisol test , measurement ofmidnight plasma cortisol or late-night salivary cortisol, and the low-dose dexamethasone

suppression test. Another test, the dexamethasone-corticotropin-releasing hormone test, may beneeded to distinguish Cushings syndrome from other causes of excess cortisol.

y 24-hour urinary free cortisol level. In this test, a persons urine is collected severaltimes over a 24-hour period and tested for cortisol. Levels higher than 50 to 100micrograms a day for an adult suggest Cushings syndrome. The normal upper limit

varies in different laboratories, depending on which measurement technique is used.yMidnight plasma cortisol and late-night salivary cortisol measurements. The

midnight plasma cortisol test measures cortisol concentrations in the blood. Cortisolproduction is normally suppressed at night, but in Cushings syndrome, this suppression

doesnt occur. If the cortisol level is more than 50 nanomoles per liter (nmol/L),Cushings syndrome is suspected. 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, thentested to determine the cortisol level. Diagnostic ranges vary, depending on themeasurement technique used.

y Low-dose dexamethasone suppression test (LDDST). In the LDDST, a person is givena low dose of dexamethasone, a synthetic glucocorticoid, by mouth every 6 hours for 2days. Urine is collected before dexamethasone is administered and several times on each

day of the test. A modified LDDST uses a onetime overnight dose.


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Cortisol and other glucocorticoids signal the pituitary to release less ACTH , sothe normal response after taking dexamethasone is a drop in blood and urine cortisol

levels. If cortisol levels do not drop, Cushings 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 Cushingssyndrome. On the other hand, drugs such as phenytoin and phenobarbital may causecortisol levels to drop, falsely indicating that Cushings is not present in people who

actually have the syndrome. For this reason, physicians usually advise their patients tostop taking these drugs at least 1 week before the test.

y Dexamethasone-corticotropin-releasing hormone (CRH) test. Some people have highcortisol levels but do not develop the progressive effects of Cushings syndrome, such asmuscle weakness, fractures, and thinning of the skin. These people may have pseudo-

Cushings syndrome, a condition sometimes found in people who have depression oranxiety disorders, drink excess alcohol, have poorly controlled diabetes, or are severely

obese. Pseudo-Cushings does not have the same long-term effects on health asCushings syndrome and does not require treatment directed at the endocrine glands.

The dexamethasone-CRH test rapidly distinguishes pseudo-Cushings from mildcases of Cushings. 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-Cushings. Elevations of cortisol during this test suggest Cushingssyndrome.

MEDICAL MANAGEMENT

y Monitor intake and output, daily weights, and serum glucose and electrolytes.y Monitor for signs of infection because risk is high with excess glucocorticoids.y After hypophysectomy, monitor for diabetes insipidus, hypothyroidism, and other

endocrine changes.y Assess the skin frequently to detect reddened areas, skin breakdown or tearing,

excoriation, infection or edema.y Handle skin and extremity gently to prevent trauma; prevent falls by using siderails.y Avoid using adhesive tape on the skin to reduce trauma on its removal.y Encourage the patient to turn in bed frequently or ambulate to reduce pressure on bonyprominences and areas of edema.y Assist the patient with ambulation and hygiene when weak and fatigued. Use assistive

devices during ambulation to prevent falls and fractures.y Help the patient to schedule exercise and rest. Advise the patient how to recognize signs

and symptoms of excessive exertion.y Instruct the patient to correct body mechanics to avoid pain or injury during activities.


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y 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.y Report edema and signs of fluid retention.y Encourage the patient to verbalize concerns about the illness, changes in appearance, and

alters role function.y Explain to female patient who has benign adenoma or hyperplasia that , with proper

treatment, evidence of masculinization can be reversed.

SURGICAL MANAGEMENT

y Pituitary surgery to treat pituitary Cushings syndrome.oTranssphenoidal adenomectomy or hypophysectomy.oTransfrontal craniotomy may be necessary when a pituitary tumor has enlarged

beyond the sella turcica.y Bilateral adrenalectomy is used to treat adrenal causes.y Radiation therapy may also be used to treat pituitary or adrenal tumors.

NURSING CARE MANAGEMENT

Diagnosis:

Risk for infectionPlanning:

After 2 hours of nursing intervention the patient will be able to achieve timelywound healing.

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.

I: Stress proper hand hygiene by all caregivers between therapies/clients.R: A first-line defense against therapies/clients.

I: Monitor client visitors/caregivers for respiratory illnesses.R: To limit exposures, thus reduce cross-contamination.


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I: Provide for isolation, as indicated.R: Reduces risk of cross-contamination.

I: Provide information/involve in appropriate community and national educationprograms.

R: To increase awareness of/and prevention of communicable diseases.Evaluation:

After 2 hours of nursing intervention the patient was able to achieve timelywound healing.

Diagnosis:

Impaired skin integrity related to slow sound healingPlanning:

After 2 hours of nursing intervention the patient will be able to participate inprevention measures and treatment programs.

Interventions and Rationale:

I:Evaluate client with impaired cognition, developmental delay, eed for/ use ofrestraints, long term immobility.

R: To identify risk for injury/ safety requirements.

I: Note skin color, texture and turgor.R: To assess the skin for superinfection.

I: Periodically, remeasure/ photograph wound and observe for complications.R: To monitor progress of wound healing.

I: Keep the area clean/dry, carefully dress wounds, support incisions.R: To assist bodys natural process of 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 use of plastic material.R: Moisture potentiates skin break down.


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I:Encourage early ambulation/mobilization.R: Promotes circulation and reduces risks associated with immobility.

Evaluation:

After 2 hours of nursing intervention the patient was able to participate inprevention measures and treatment programs.

Diagnosis:

Activity intolerance related to body weakness.

Planning:

After 2 hours of nursing intervention, the patient will be able to participatewillingly in necessary/desired activities.

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 of activity.

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 inneeded/desires activities.

I: Adjust activities.R: To prevent overexertion.

I: Increase exercise/activity levels gradually; teach methods.R: To conserve energy.


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I: Provide positive atmosphere, while acknowledging difficulty of the situationfor 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 andsymptoms.

R: it indicate need to alter activity level.

I:Encourage client to maintain positive attitude, suggest use of relaxationtechniques, 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 willinglyin necessary/desired activities.

PROGNOSIS

Iatrogenic Cushing's syndrome (caused by treatment with corticosteroids) is the mostcommon form of Cushing's syndrome. Endogenous (i.e. non-iatrogenic) Cushing's syndrome isrelatively rare.

Incidence of causes of Cushing's syndrome per million person-years:

y Cushing's disease: 10y Primary adrenal: 3y Ectopic ACTH: 1.4

Prognosis ofCushing's syndrome: Many cases can be cured. Results depend on the underlying

cause and severity of the problem.

Prognosis ofCushing's syndrome: The prognosis for individuals with Cushing's syndromevaries depending on the cause of overproduction of cortisol. With treatment, most individuals

with Cushing's syndrome show significant improvement, while improvement for others may becomplicated by various aspects of the causative illness. Some kinds of tumors may recur. Most

cases of Cushing's can be cured.


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POINTS TO REMEMBER

y Cushings syndrome is a disorder caused by prolonged exposure of the bodys tissues tohigh levels of the hormone cortisol.

y Typical signs and symptoms of Cushings syndrome include upper body obesity, arounded face, skin that bruises easily and heals poorly, weakened bones, excess body hair

growth and menstrual irregularities in women, and decreased fertility in men.y Cushings syndrome is caused by exposure to glucocorticoids, which are used to treat

inflammatory diseases, or by the bodys overproduction of cortisol, most often due totumors of the pituitary gland or lung.

y Several tests are usually needed to diagnosis Cushings syndrome, including urine, blood,and saliva tests. Other tests help find the cause of the syndrome.

y Treatment depends on the specific reason for excess cortisol and may include surgery,radiation, chemotherapy, or the use of 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

Documents

A Disease Study of Cushing's Disease