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Cushing's Disease
Frequently Asked Questions (FAQ)
What is Cushing's Disease?

What are the symptoms of Cushing's Disease?


How is Cushing's Disease diagnosed?

MRI

Surgery

Following Pituitary Surgery

Diabetes Insipidus

Adrenal Insufficiency

Follow up

Radiation

How long does it take to recover from surgery?

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Growth Hormone Replacement

What is Cushing's Disease?
Cushing's disease is the name given to a condition caused by a pituitary tumor that secretes excessive amounts of adrenocorticotropic hormone (known as ACTH). This hormone stimulates the adrenal glands to produce excessive amounts of cortisol.

Other tumors or conditions also may lead to excess secretion of cortisol, such as tumors of the adrenal gland and lung. This is called Cushing's syndrome. If left untreated, Cushing's syndrome (as well as Cushing's disease) can seriously affect the quality and length of life.

Pituitary Disorders
  Prolactinoma
  Acromegaly
  Cushing's Disease

Symptoms

Upper body obesity

Round face

Iincreased fat around neck

Thinning arms and legs

Fragile and thin skin
stretch marks on abdomen, thighs, buttocks, arms, and breasts

Bone and muscle weakness

Severe fatigue

High blood pressure

High blood sugar

Irritability and anxiety

Excess hair growth in women

Irregular or stopped menstrual cycles in women

Reduced sex drive and fertility in men


Cushing's disease or syndrome may be confused with pseudo Cushing's. The symptoms of pseudo Cushing's may be caused by alcoholism, depression, or by taking steroid medications over long periods of time. Steroid medications may be used to treat conditions like rheumatoid arthritis, asthma, vasculitis, lupus, and a variety of other autoimmune disorders.

Cortisol is produced by the adrenal glands, which are located just above the kidneys. The adrenal glands are endocrine organs that secrete hormones into the blood stream. They are regulated by the pituitary gland to produce cortisol as needed. Higher amounts are needed in the morning and through out the early day and needed less in evening hours. This 24-hour rhythm is called a circadian rhythm. Extra cortisol is naturally produced during an illness or any type of physical or psychological stress. Cortisol belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Cortisol helps regulate blood pressure, the immune system, blood sugar, and helps regulate the metabolism of proteins, carbohydrates, and fats.

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Symptoms of Cushing's Disease
The symptoms caused by excessive cortisol secretion may vary from person to person. Many of the symptoms listed below can be attributed to numerous other diseases if viewed separately. However, several of these symptoms seen together could suggest testing for Cushing's disease.

Moon Face - The face becomes round and may look flushed.

High Blood Pressure- Almost all patients with Cushing's have high blood pressure because of complex actions of cortisol in the kidney and on blood vessels.

High Cholesterol - The high blood pressure interacts with high cholesterol levels that are often seen in patients with Cushing's to increase the risk of
atherosclerosis.

High Blood Sugar - Diabetes is common in patients with Cushing's. As many as 3% of uncontrolled diabetics may actually have Cushing's. The disorder is due to the effects of cortisol on insulin action and handling of glucose.

Truncal Obesity - An accumulation of fat around the abdomen, neck and collar bone.

Muscle Weakness - Legs and arms lose muscle mass and appear thin in comparison to trunk.

Backaches - Pain in neck shoulders and back are common in Cushing's.

Buffalo Hump - Fat can accumulate at the back of the neck and between the shoulder blades.

Skin Changes - Skin may become thin, fragile and easily bruised. Acne may develop in all ages.

Straie - Bluish-red stretch marks may form on abdomen, breast, thighs, upper arms and buttocks.

Weak Immune System - Poor wound healing and increased chances of infections.

Menstrual Periods - Periods may be irregular or cease.

Labedo - Lack of sexual desire for men & women. Men may have difficulty maintaining an erection.

Hirsutism - Abnormal hair growth on face.

Balding - Hair at temples and on the scalp may become thin.

Emotional Disturbances - Mood swings, depression, irritability, confusion, and poor memory.

Extreme Fatigue - Weakness and fatigue during the day and difficulty sleeping at night.

Osteoporosis - Fragile thinning bones. Bones loose their density and may fracture or break.

Kidney Stones - These are often the first manifestation of Cushing's syndrome. The calcium that comes from the bones leaks through the kidneys into the urine and can crystallize causing calcium stones.

Some patients have sustained high cortisol levels without the symptoms of Cushing’s syndrome or disease. These high cortisol levels may be compensating for the body’s resistance to cortisol’s effects. This rare syndrome of cortisol resistance is a genetic condition that causes hypertension and chronic androgen excess.

Polycystic ovarian syndrome is another condition that may share some of the same symptoms as Cushing’s. Patients with polycystic ovarian syndrome have menstrual disturbances, facial hair, weight gain, high blood pressure and high cholesterol. Polycystic ovarian syndrome does not cause abnormally high cortisol levels.

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Diagnosis
The first step in diagnosis is to determine if the symptoms are due to excess cortisol production. Demonstrating cortisol excess is somewhat complicated because cortisol production in a normal individual varies from highest in the morning to lowest at night. Cortisol can also vary according to a person’s health, stress level, and activity level. Therefore, simply measuring the quantity of circulating cortisol and ACTH in a patient's bloodstream in one blood test is not enough information to diagnose Cushing's Disease.

Your endocrinologist may want to start with a few tests that will require your help and ability to follow directions precisely. One test is a 24-hour free cortisol urine collection. You will use a kit to collect urine for 24 hours. This urine is analyzed in a laboratory to determine the quantity of cortisol in the urine. The Urine Free Cortisol test has a sensitivity of about 95%, but has the disadvantage of relying on patient management for proper collection

The 24-hour urine free cortisol (UFC) collection can be used with the Dexamethasone Suppression Test. Dexamethasone is a steroid drug taken by mouth. It is very similar to cortisol that the adrenal glands produce. In a healthy patient who has taken Dexamethasone, the endocrine system will recognize that plenty of the steroid resembling cortisol is in the blood and will turn off cortisol production. The results in healthy individuals will be low urine cortisol levels. In a patient with Cushing’s the dexamethasone does not suppress excess cortisol production and the amount of cortisol in the urine will be high.

One variation of the Dexamethasone tests is done with CRH (cortisol releasing hormone). This variation of the test is useful to identify patients who have Cushing's from those who do not.

Another test that can be used with the 24 hour (UFC) is a salivary cortisol screening test. It is a diagnostic tool that can be performed by the patient to obtain late evening cortisol levels measured from saliva. It is convenient method to use when obtaining PM cortisol levels (before bedtime). The test is done in the convenience of one’s home with out causing stress which could alter PM cortisol levels. This test can be repeated several days and evenings in a row. Salivary cortisol screening test is also helpful in diagnosing intermittent Cushing’s.

The next step is to determine if the source of excess cortisol is from an ACTH producing pituitary tumor, adrenal tumor or an ectopic tumor. There are several variations of the Dexamethasone test used to gain additional information. Many of these variations use different doses of dexamethasone administered at night or repeatedly at various intervals. The serum cortisol and urine cortisol is measured during the test.
Imaging techniques

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Magnetic Resonance Imaging
An MRI is used to look at the pituitary and CT scans to look at adrenal glands after a firm diagnosis of elevated cortisol and ACTH. The availability of diagnostic and imaging techniques has improved detection of pituitary microadenoma’s (a tumor under 10mm in size). Pituitary tumors that produce ACTH are usually small and 30 % are so small that they are not easily detected by MRI.

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Treatment
For patients with Cushing’s disease caused by a pituitary tumor, surgery to remove the tumor is generally recommended. Surgery or radiotherapy may be used to treat pituitary adenomas. The aim of treatment is to cure the hypercortisolism and to eliminate any tumor that threatens the individual's health, while minimizing the chance of endocrine deficiency or long-term dependence on medications.

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Surgery
Pituitary surgery should be done by a neurosurgeon who has experience removing pituitary tumors using the transphenoidal approach (under the upper lip) or transnasal (through the nose) approach. The neurosurgeon you choose should routinely perform more than twenty pituitary surgeries’ per year. Because pituitary surgery can cause ACTH levels to drop too low, some patients will require short-term treatment with a cortisol-like medication after surgery. Patients who need adrenal surgery may also require steroid replacement. You should take these medications as prescribed by your physician without interruption. If the entire pituitary gland or adrenal gland has been removed, the patient will need to take all hormone replacements for the rest of his or her life.

Following Pituitary Surgery
The following symptoms may occur after surgery and should be discussed with doctor. Sinus Infection, Worsening headache, fever, chills, yellowish green nasal discharge, and neck stiffness may all signify an infectious process.

Hyponatremia (low blood sodium levels). Some patients develop disorders of salt and water metabolism following pituitary surgery. Headache, nausea, vomiting, confusion, impaired concentration, and muscle aches might be due to hyponatremia (low blood sodium levels). This disorder typically occurs 7 to 10 days after surgery and is more common in patients who have had surgery for Cushing’s disease. If you develop these symptoms, contact your Endocrinologist and Neurosurgeon immediately.

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Diabetes Insipidus
Excessive urination, thirst, and the need to ingest large quantities of fluids might be related to the onset of diabetes insipidus or diabetes mellitus. These disorders put you at risk for dehydration. The symptoms require urgent evaluation and determination of the underlying cause so that appropriate treatment may be given. If these symptoms develop, contact your physicians immediately.

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Adrenal Insufficiency
Patients who are dependent on cortisol replacement medications should consult their doctor immediately when experiencing flu like symptom. Cortisol replacement dosages may need to be increased during a fever, illness, or trauma.

If a person has to take steroid replacement (hydrocortisone, prednisone, dexamethasone), he or she should wear a Medic Alert bracelet or necklace, which identifies the need for steroid treatment in case of emergency. The medical ID should indicate deficiency.

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Follow up
In general, the first postoperative follow-up visit will be scheduled a few weeks after surgery. If problems develop prior to your appointment, you will be asked to return to your neurosurgeon as soon as possible. Your return appointments will be scheduled according to your needs. Lifelong follow-up is necessary. You should ensure that you receive appropriate follow-up by physicians knowledgeable in diagnosing and managing pituitary disorders.


Medical Therapy
For patients who continue to have excess pituitary production of ACTH following surgery, medications can be helpful along with other therapies. Although there is no medication to control Cushing’s disease, a few drugs can be used in reducing the levels of cortisol. Drugs such as ketoconazole, metyrapone, and mitotane, trilostane, and aminoglutethimide have been used with varying success. These drugs may be given after surgery (sometimes along with radiation treatments).

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Radiation Therapy
Radiation to the pituitary is not the first line of treatment for most pituitary tumors. Radiation may be used for patients who cannot undergo surgery, or when there is tumor remaining after surgery. Radiation does not produce an immediate effect to lower excessive hormone production or shrink the tumor. There are different methods of delivering radiation to the pituitary gland. The decision as to which type of radiation to administer must be made only after a careful review of the MRI scan to assess the size and location of the residual tumor.

Conventional Radiation - Pituitary radiation such as conventional (fractionated) radiation may take several years to be effective. Conventional (fractionated) radiation refers to delivery of a small amount of radiation daily for 4 to 5 weeks.

Stereotactic Radiation - Stereotactic radiation refers to delivery of a precisely focused beam of radiation, usually as one treatment. A large tumor near the optic chiasm (eye nerves) is not suitable for stereotactic radiation because of the intensity of the single treatment and risk of damage to vision. In general, stereotactic radiation is reserved for a small residual tumor, which is not near the optic chiasm.

Side Effects - The most common side effect of radiation is loss of pituitary function. This may occur within a year or many years after treatment. One study reported that 50% of patients treated with conventional radiation developed deficiency of one or more pituitary hormones within 2 years of treatment. Although development of a pituitary hormone deficiency is not desirable, hormone replacement therapy is available. An uncommon side effect is damage to vision. These risks must be weighed against the risk of tumor re-growth.

Gamma Knife - Gamma Knife radiosurgery is a method for delivering focused radiation therapy to pituitary tumors. Using Gamma Knife for a pituitary tumor is most often used as secondary therapy after surgery. The size and location of the residual tumor are the limiting factors in selecting a patient for this treatment. It can be used in cases where the entire tumor was not successfully removed. Gamma Knife radiation is focused at the residual tumor. If the tumor is not seen on the MRI scan the entire pituitary gland is usually targeted for treatment. Gamma Knife treatment may also be appropriate for patients who have an initial remission after surgery and then develop a recurrence of excessive hormone secretion. Gamma Knife radiation treatment is usually administered as a single treatment which requires most of one day. If the tumor remaining after surgery is too close to the optic chiasm (eye nerves) or is too large, Gamma Knife is not advisable because of the risk of damage to vision.

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Recovery
Cushing's affects every system of the body. It may take a long time for the body to reverse the effects of excess cortisol. With Cushing's muscles become thin and weak. It may take several months after surgery for the body to rebuild muscle and reduce excess weight. Usually about 9 to 12 months after surgery a patient will see markable improvements. Some improvements can be seen soon after surgery. Because of the long recovery time, a patient may want to keep a journal and note all of the small improvements. Taking one day at a time and looking for the good things in each day can make this a time of personal growth. When the recovery process seems to stand still it is helpful to read the journal and remember how much improvement you have made. Patience, perseverance, and positive attitude will improve recovery.




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