NOTE: Adrenal insufficiency is not one of those risks from surgery that may happen. In the hundreds of people I have watched have pituitary surgery in the past few years, nearly EVERYONE has gone to the hospital with adrenal insufficiency within the first six months after surgery. Many go multiple times. It is not only possible, it is probable.
For women, imagine the changes in your body during menstruation. Body aches, depression, cramping, irritability, fatigue, "drained." Well, that results from only a few hormones moving up and down, and they only affect you for a few days, maybe a week.
With Cushing's and the adrenal insufficiency we face after pituitary surgery, all of our hormones are going nutzo. Every single one of them. We suffer from the symptoms above, and those outlined below. Oh, and it doesn't last days. It lasts months and years.
Add to the mix this fun little fact: no one around you--not your local friends or family--have ever seen this happen to anyone.
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From the Pituitary Network Association website
Adrenal Insufficiency (Addison's Disease)
Categories: Secondary Adrenal Insufficiency or Secondary Addison's or Addison's Disease
Overview
Addisons disease is a rare endocrine, or hormonal disorder that affects about 1 in 100,000 people. It occurs in all age groups and afflicts men and women equally. The disease is characterized by weight loss, muscle weakness, fatigue, low blood pressure, and sometimes darkening of the skin in both exposed and non-exposed parts of the body.
Addisons disease occurs when the adrenal glands do not produce enough of the hormone cortisol and in some cases, the hormone aldosterone. For this reason, the disease is sometimes called chronic adrenal insufficiency, or hypocortisolism.
Cortisol is normally produced by the adrenal glands, located just above the kidneys. It belongs to a class of hormones called glucocorticoids, which affect almost every organ and tissue in the body. Scientists think that cortisol has possibly hundreds of effects in the body. Cortisol's most important job is to help the body respond to stress. Among its other vital tasks, cortisol:
* helps maintain blood pressure and cardiovascular function;
* helps slow the immune systems inflammatory response;
* helps balance the effects of insulin in breaking down sugar for energy; and
* helps regulate the metabolism of proteins, carbohydrates, and fats.
Because cortisol is so vital to health, the amount of cortisol produced by the adrenal's is precisely balanced. Like many other hormones, cortisol is regulated by the brains hypothalamus and the pituitary gland, a bean-sized organ at the base of the brain. First, the hypothalamus sends "releasing hormones" to the pituitary gland. The pituitary responds by secreting other hormones that regulate growth, thyroid and adrenal function, and sex hormones such as estrogen and testosterone. One of the pituitarys main functions is to secrete ACTH (adrenocorticotropin), a hormone that stimulates the adrenal glands. When the adrenals receive the pituitarys signal in the form of ACTH, they respond by producing cortisol. Completing the cycle, cortisol then signals the pituitary to lower secretion of ACTH.
Aldosterone belongs to a class of hormones called mineralocorticoids, also produced by the adrenal glands. It helps maintain blood pressure and water and salt balance in the body by helping the kidney retain sodium and excrete potassium. When aldosterone production falls too low, the kidneys are not able to regulate salt and water balance, causing blood volume and blood pressure to drop.
Causes
Failure to produce adequate levels of cortisol, or adrenal insufficiency, can occur for different reasons. The problem may be due to a disorder of the adrenal glands themselves (primary adrenal insufficiency) or to inadequate secretion of ACTH by the pituitary gland (secondary adrenal insufficiency).
Primary Adrenal Insufficiency
Most cases of Addisons disease are caused by the gradual destruction of the adrenal cortex, the outer layer of the adrenal glands, by the body's own immune system. About 70 percent of reported cases of Addisons disease are due to autoimmune disorders, in which the immune system makes antibodies that attack the body's own tissues or organs and slowly destroy them. Adrenal insufficiency occurs when at least 90 percent of the adrenal cortex has been destroyed. As a result, often both glucocorticoid and mineralocorticoid hormones are lacking. Sometimes only the adrenal gland is affected, as in idiopathic adrenal insufficiency; sometimes other glands also are affected, as in the polyendocrine deficiency syndrome.
The polyendocrine deficiency syndrome is classified into two separate forms, referred to as type I and type II. Type I occurs in children, and adrenal insufficiency may be accompanied by underactive parathyroid glands, slow sexual development, pernicious anemia, chronic candida infections, chronic active hepatitis, and, in very rare cases, hair loss. Type II, often called Schmidts syndrome, usually afflicts young adults. Features of type II may include an underactive thyroid gland, slow sexual development, and diabetes mellitus. About 10 percent of patients with type II have vitiligo, or loss of pigment, on areas of the skin. Scientists think that the polyendocrine deficiency syndrome is inherited because frequently more than one family member tends to have one or more endocrine deficiencies.
Tuberculosis (TB) accounts for about 20 percent of cases of primary adrenal insufficiency in developed countries. When adrenal insufficiency was first identified by Dr. Thomas Addison in 1849, TB was found at autopsy in 70 to 90 percent of cases. As the treatment for TB improved, however, the incidence of adrenal insufficiency due to TB of the adrenal glands has greatly decreased.
Less common causes of primary adrenal insufficiency are chronic infections, mainly fungal infections; cancer cells spreading from other parts of the body to the adrenal glands; amyloidosis; and surgical removal of the adrenal glands. Each of these causes is discussed in more detail below.
Secondary Adrenal Insufficiency
This form of Addisons disease can be traced to a lack of ACTH, which causes a drop in the adrenal glands production of cortisol but not aldosterone. A temporary form of secondary adrenal insufficiency may occur when a person who has been receiving a glucocorticoid hormone such as prednisone for a long time abruptly stops or interrupts taking the medication. Glucocorticoid hormones, which are often used to treat inflammatory illnesses like rheumatoid arthritis, asthma, or ulcerative colitis, block the release of both corticotropin-releasing hormone (CRH) and ACTH. Normally, CRH instructs the pituitary gland to release ACTH. If CRH levels drop, the pituitary is not stimulated to release ACTH, and the adrenals then fail to secrete sufficient levels of cortisol.
Another cause of secondary adrenal insufficiency is the surgical removal of benign, or noncancerous, ACTH-producing tumors of the pituitary gland (Cushings disease). In this case, the source of ACTH is suddenly removed, and replacement hormone must be taken until normal ACTH and cortisol production resumes. Less commonly, adrenal insufficiency occurs when the pituitary gland either decreases in size or stops producing ACTH. This can result from tumors or infections of the area, loss of blood flow to the pituitary, radiation for the treatment of pituitary tumors, or surgical removal of parts of the hypothalamus or the pituitary gland during neurosurgery of these areas.
SYMPTOMS
The symptoms of adrenal insufficiency usually begin gradually. Chronic, worsening fatigue and muscle weakness, loss of appetite, and weight loss are characteristic of the disease. Nausea, vomiting, and diarrhea occur in about 50 percent of cases. Blood pressure is low and falls further when standing, causing dizziness or fainting. Skin changes also are common in Addisons disease, with areas of hyperpigmentation, or dark tanning, covering exposed and nonexposed parts of the body. This darkening of the skin is most visible on scars; skin folds; pressure points such as the elbows, knees, knuckles, and toes; lips; and mucous membranes.
Addisons disease can cause irritability and depression. Because of salt loss, craving of salty foods also is common. Hypoglycemia, or low blood sugar, is more severe in children than in adults. In women, menstrual periods may become irregular or stop.
Because the symptoms progress slowly, they are usually ignored until a stressful event like an illness or an accident causes them to become worse. This is called an addisonian crisis, or acute adrenal insufficiency. In most patients, symptoms are severe enough to seek medical treatment before a crisis occurs. However, in about 25 percent of patients, symptoms first appear during an addisonian crisis.
Symptoms of an addisonian crisis include sudden penetrating pain in the lower back, abdomen, or legs; severe vomiting and diarrhea, followed by dehydration; low blood pressure; and loss of consciousness. Left untreated, an addisonian crisis can be fatal.
DIAGNOSIS
In its early stages, adrenal insufficiency can be difficult to diagnose. A review of a patients medical history based on the symptoms, especially the dark tanning of the skin, will lead a doctor to suspect Addisons disease.
A diagnosis of Addisons disease is made by biochemical laboratory tests. The aim of these tests is first to determine whether there are insufficient levels of cortisol and then to establish the cause. X-ray exams of the adrenal and pituitary glands also are useful in helping to establish the cause.
ACTH Stimulation Test
This is the most specific test for diagnosing Addisons disease. In this test, blood and/or urine cortisol levels are measured before and after a synthetic form of ACTH is given by injection. In the so called short, or rapid, ACTH test, cortisol measurement in blood is repeated 30 to 60 minutes after an intravenous ACTH injection. The normal response after an injection of ACTH is a rise in blood and urine cortisol levels. Patients with either form of adrenal insufficiency respond poorly or do not respond at all.
When the response to the short ACTH test is abnormal, a "long" ACTH stimulation test is required to determine the cause of adrenal insufficiency. In this test, synthetic ACTH is injected either intravenously or intramuscularly over a 48- to 72-hour period, and blood and/or urine cortisol are measured the day before and during the 2 to 3 days of the injection. Patients with primary adrenal insufficiency do not produce cortisol during the 48- to 72-hour period; however, patients with secondary adrenal insufficiency have adequate responses to the test on the second or third day.
In patients suspected of having an addisonian crisis, the doctor must begin treatment with injections of salt, fluids, and glucocorticoid hormones immediately. Although a reliable diagnosis is not possible while the patient is being treated, measurement of blood ACTH and cortisol during the crisis and before glucocorticoids are given is sufficient to make the diagnosis. Once the crisis is controlled and medication has been stopped, the doctor will delay further testing for up to 1 month to obtain an accurate diagnosis.
Insulin-Induced Hypoglycemia Test
A reliable test to determine how the hypothalamus and pituitary and adrenal glands respond to stress is the insulin-induced hypoglycemia test. In this test, blood is drawn to measure the blood glucose and cortisol levels, followed by an injection of fast-acting insulin. Blood glucose and cortisol levels are measured again at 30, 45, and 90 minutes after the insulin injection. The normal response is for blood glucose levels to fall and cortisol levels to rise.
Other Tests
Once a diagnosis of primary adrenal insufficiency has been made, x-ray exams of the abdomen may be taken to see if the adrenals have any signs of calcium deposits. Calcium deposits may indicate TB. A tuberculin skin test also may be used.
If secondary adrenal insufficiency is the cause, doctors may use different imaging tools to reveal the size and shape of the pituitary gland. The most common is the CT scan, which produces a series of x-ray pictures giving a cross-sectional image of a body part. The function of the pituitary and its ability to produce other hormones also are tested.
TREATMENT
Treatment of Addisons disease involves replacing, or substituting, the hormones that the adrenal glands are not making. Cortisol is replaced orally with hydrocortisone tablets, a synthetic glucocorticoid, taken once or twice a day. If aldosterone is also deficient, it is replaced with oral doses of a mineralocorticoid, called fludrocortisone acetate (Florinef)), which is taken once a day. Patients receiving aldosterone replacement therapy are usually advised by a doctor to increase their salt intake. Because patients with secondary adrenal insufficiency normally maintain aldosterone production, they do not require aldosterone replacement therapy. The doses of each of these medications are adjusted to meet the needs of individual patients.
During an addisonian crisis, low blood pressure, low blood sugar, and high levels of potassium can be life threatening. Standard therapy involves intravenous injections of hydrocortisone, saline (salt water), and dextrose (sugar). This treatment usually brings rapid improvement. When the patient can take fluids and medications by mouth, the amount of hydrocortisone is decreased until a maintenance dose is achieved. If aldosterone is deficient, maintenance therapy also includes oral doses of fludrocortisone acetate.
SPECIAL PROBLEMS
Surgery
Patients with chronic adrenal insufficiency who need surgery with general anesthesia are treated with injections of hydrocortisone and saline. Injections begin on the evening before surgery and continue until the patient is fully awake and able to take medication by mouth. The dosage is adjusted until the maintenance dosage given before surgery is reached.
Pregnancy
Women with primary adrenal insufficiency who become pregnant are treated with standard replacement therapy. If nausea and vomiting in early pregnancy interfere with oral medication, injections of the hormone may be necessary. During delivery, treatment is similar to that of patients needing surgery; following delivery, the dose is gradually tapered and the usual maintenance doses of hydrocortisone and fludrocortisone acetate by mouth are not reached until about 10 days after childbirth.
Patient Education
A person who has adrenal insufficiency should always carry identification stating his or her condition in case of an emergency. The card should alert emergency personnel about the need to inject 100 mg of cortisol if its bearer is found severely injured or unable to answer questions. The card should also include the doctors name and telephone number and the name and telephone number of the nearest relative to be notified. When traveling, it is important to have a needle, syringe, and an injectable form of cortisol for emergencies. A person with Addisons disease also should know how to increase medication during periods of stress or mild upper respiratory infections. Immediate medical attention is needed when severe infections or vomiting or diarrhea occur. These conditions can precipitate an addisonian crisis. A patient who is vomiting may require injections of hydrocortisone.
It is very helpful for persons with medical problems to wear a descriptive warning bracelet or neck chain to alert emergency personnel. Bracelets and neck chains can be obtained from:
Medic Alert Foundation International
2323 Colorado
Turlock, California 95381
(209) 668-3333
Suggested Reading
The following materials can be found in medical libraries, many college and university libraries, and through interlibrary loan in most public libraries.
Wingert, Terence D. and Mulrow, Patrick J., "Chronic Adrenal Insufficiency," in Current Diagnosis, edited by Rex B. Conn. Philadelphia, MI.B. Saunders Company, 1985, pp 860-863.
Bravo, Emmanuel L., "Adrenocortical Insufficiency," in Conns Current Therapy, edited by Robert E. Rakel. Philadelphia, W.B. Saunders Company, 1987, pp 493-495.
Bondy, Philip K., "Disorders of the Adrenal Cortex," in Williams Textbook of Endocrinology, seventh edition, edited by Jean D. Wilson and Daniel W. Foster. Philadelphia, R.B. Saunders Company, 1985, pp 844-858.
Loriaux, D. Lynn and Cutler, Gordon B., "Diseases of the Adrenal Glands," in Clinical Endocrinology, edited by Peter O. Kohler. New York, lohn Wiley & Sons, 1986, pp 208-215.
Williams, Gordon H. and Dluhy, Robert G., "Diseases of the Adrenal Cortex," in Harnsons Principles of Internal Medicine, 11th edition, edited by Eugene Braunwald, Kurt J. Isselbacher, Robert G. Petersdorf, Jean D. Wilson, Joseph B. Martin, and Anthony S. Fauci. New York, McGraw-Hill Book Company, 1987, pp 1769-1772.
Baxter, John D. and Tyrrell, 1. Blake, "The Adrenal Cortex," in Endocrinolegy and Metabolism, second edition, edited by Philip Felig,, John D. Baxter, Arthur E. Broadus, and Lawrence A. Frohman. New York, McGraw-Hill Book Company, 1987, pp 581-599.
Other Resources
National Adrenal Disease Foundation
505 Northern Boulevard, Suite 200
Great Neck, New York 11021
(516) 487-4992
This epub was written by Eileen K. Corrigan of NIDDKs Office of Health Research Reports. The draft was reviewed by Dr. George P Chrousos, National Institute of Child Health and Human Development, Dr. Judith Fradkin, National Institute of Diabetes and Digestive and Kidney Diseases, and by Dr. Richard Horton, University of Southern California Medical Center.
This epub is not copyrighted. Readers are encouraged to duplicate and distribute as many copies as needed. Printed single copies may be obtained from the Office of Health Research Reports, NIDDK, Building 31, Room 9A04, Bethesda, Maryland 20892.
NIH Publication No. 90-3054
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This is the Adrenal Crisis publication distributed with the letter from Cushing's Research and Support Foundation to family and friends.
Adrenal Insufficiency & Cortisol Withdrawal
After pituitary and adrenal surgery, adrenal insufficiency can occur as replacement steroid hormones are tapered and adrenal hormone production slowly improves toward normal. If hormone production does not return to normal then replacement hormones will be needed on a permanent basis.
If you have pituitary or adrenal surgery it is recommended that you wear an ID bracelet, or necklace that will identify you as being adrenal insufficient. There are many places to purchase medical ID's. Your local jewelry store or pharmacy will probably have them. There's also a vast supply of merchants that sell medical ID's on the Internet. (I purchased mine through
With Cushing's the body is producing too much cortisol. Over a period of time the body gets accustomed to the high levels of cortisol.
After surgery for Cushing's, ideally the cortisol level will decline. As the decline in cortisol level continues, it is common to experience withdrawal. The pain from withdrawal can be horrible but it's a positive sign that you likely got a cure from the Cushing's through surgery. It is important that you follow your doctor's advice as he will adjust your replacement dosage accordingly.
It's important to KNOW the difference between what is considered to be common cortisol withdrawal symptoms and Adrenal Insufficiency. Death can occur from Adrenal Insufficiency due to overwhelming shock if early treatment is not provided. Always follow your doctor's advice and never adjust your replacement medications on your own.
Adrenal Insufficiency is an abrupt, life-threatening state caused by insufficient cortisol, a hormone produced and released by the adrenal gland.
Those who are at risk for Adrenal Insufficiency should be taught to recognize signs of potential stress that may precipitate an acute adrenal crisis. An Adrenal Crisis can occur suddenly and unexpectedly. Some people are taught to give themselves an emergency injection of hydrocortisone in times of stress. It is important for the individual to always carry a medical identification card that states the type of medication and the proper dose needed in case of an emergency. Never omit replacement medication. If unable to retain medication due to vomiting, notify the health care provider as you may need injectable replacement.
Some of the initial symptoms of Adrenal Insufficiency may include but not limited to some of the following symptoms headache, weakness, fatigue, nausea and/or vomiting, abdominal cramps, diarrhea, confusion, rapid heart rate, joint pain, chills, fever, low blood pressure, etc.
Later symptoms, as the Adrenal Insufficiency progresses, may include but not limited to dehydration, darkening of the skin, weight loss, appetite loss, rash, and coma.
Treatment:
Intravenous or intramuscular injection of hydrocortisone (injectable corticosteroid) must be given immediately. Treatment of low blood pressure is usually necessary and may be treated with intravenous fluids. Hospitalization is often required for adequate treatment and monitoring.
It's very important for someone who has the potential to have Adrenal Insufficiency to let their family members and friends know what the symptoms are and what actions should be taken in an emergency situation.
