Saturday, June 6, 2009

TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP

I have not actually done the wean itself yet. It comes after pituitary surgery!!

I found this helpful in explaining the overall hormone replacement process, and why it is "common" for Cushies to end up in the ER after pituitary surgery. I cannot attest to the tapering schedule and amounts of hydrocortisone to take and when. Please consult with your doctor. ~Melissa

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TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP: From Oregon Health and Science University

The experience of cortisol withdrawal during the first several months after being cured of Cushing's can be very unpleasant. During this period, cortisol must be given back to the patient and then tapered off over time. Classic symptoms of this withdrawal process include fatigue, aching, and depression. Abdominal pain, nausea and vomiting, and dizziness are indicators that the cortisol withdrawal is occurring too quickly (adrenal insufficiency is occurring) and the hydrocortisone taper should be slowed.

There are two main phases that a cured patient will go through after surgery. The first is the cortisol withdrawal phase. The features of Cushing's disease are due to cortisol excess and the body reacts to having the cortisol withdrawn. To make the process tolerable (and safe), large doses of glucocorticoids are given to the postoperative patient and then tapered over a month or so as symptoms allow. This can be an awful experience for the patient marked by fatigue, depression, and body aches. Headache may be present but if severe (especially if accompanied by nausea and dizziness) may indicate that the taper is happening to quickly. After the initial withdrawal phase, the second phase is marked by the temporary need for continued glucocorticoid replacement (typically 20 mg hydrocortisone or 5 mg prednisone) until the hypothalamic/pituitary/adrenal (HPA) axis awakens. This phase may last many months and may last over a year. Until this axis awakens, the patient is adrenally insufficient and should be treated accordingly.

Assessment of Cure
Just as the initial diagnosis of Cushing's disease can be difficult to make, "proving" someone has been cured of Cushing's can be very challenging as well. One early indicator of biochemical cure is the measurement of very low (or undetectable) post-operative morning cortisol levels (typically measured 72 hours after surgery). In this post-operative setting, morning cortisol levels are typically low because normal ACTH producing cells in the pituitary have been suppressed by elevated serum cortisol levels. Therefore, the removal of the ACTH secreting tumor leaves no source of ACTH, the adrenal glands are no longer stimulated and cortisol levels plummet. It is important to note that as high as 30% of patients with long term cure of cushing's disease do not have a history of undetectable 72 hour post-operative serum cortisol levels. Therefore, if 72-hour post-operative cortisols are not below 5 ug/dl, further evidence should be obtained to confirm the presence or absence of cure.

After a patient has been tapered off replacement glucocorticoids post-operatively, it is important to demonstrate the normalization of the tests that were initially used to diagnose Cushing's disease. For example if a patient had a significantly elevated 24-hour UFC pre-operatively, it should normalize if a cure was obtained. Other evidence of cure is the dramatic resolution of the features of Cushing's disease. For example, many patients note a dramatic weight reduction in the first few months after surgery. By contrast, even in the context of a biochemical cure, features may resolve slowly. Even in the context of a biochemical cure, the symptoms of fatigue and depression can persist for many months since they are also the symptoms of cortisol withdrawal. It is important to note that even with an incomplete cure (i.e., not all the pituitary tumor was removed), many symptoms will begin to resolve at first but they usually return over time.

In general, when a cure is obtained, most of the features of Cushing's disease will reverse over time. Some of these changes can be dramatic. Some may take years. Of note, the need for medical treatment for high blood pressure and diabetes should be evaluated closely and will likely need to be tapered.

Finally, even in the hands of an excellent neurosurgeon, there is a recurrence rate of about 10% over time for Cushing's disease. Therefore, even in the context of a "biochemical cure", Cushing's patients should be monitored by history, physical, and biochemistry over time for possible recurrence of their disease. Work up and treatment are similar to those done at initial presentation.

Long-term Course and Potential Complications

Cured Cushing's are typically delighted with the resolution of most of their Cushing's symptoms. This can, however, take from months to even years to fully happen. As stated above, recurrence does occur and should be watched for by the patient and their clinician. Associated diseases such as diabetes mellitus, hypertension, and osteoporosis should be followed (and treated if necessary) although each may resolve fully over time as well.


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TAPERING YOUR DOSE OF CORTISOL REPLACEMENT POST OP, Cushing's Research and Support Foundation

Question: I have been trying to taper my replacement dose of hydrocortisone following pituitary surgery, and I have ended up in the Emergency Room several times due to adrenal insufficiency. I have been told that for my adrenal gland to start producing cortisol and working properly again, I need to be this low. Is that true? What tapering protocol would you recommend and what tests should be performed during the tapering phase?

Answer: These are very good questions. Tapering of hydrocortisone following the removal of an ACTH-producing pituitary tumor, bilateral adrenalectomy or a single Cortisol secreting adrenal tumor results in profound hypothalamic pituitary adrenal suppression, especially when it is successful. The reawakening of this axis may take six to nine months, sometimes longer.

In the interim, patients need to be replaced with glucocorticoid therapy. There is an additional confounding problem and that is when you have Cushing's, your body gets used to higher doses of glucocorticoids; therefore, such high doses need to be continued above replacement doses immediately after surgery and then slowly tapered.

Right after surgery we replace with Hydrocortisone 60 to 80 mg a day for two weeks and start a taper, diminishing 10 to 20 mg every 10 to 14 days, until you are down to physiologic, which is in the neighborhood of 15 to 20 mg per day.

We like to do a Cortrosyn stimulation test every two months to plot the hypothalamic pituitary adrenal recovery. Morning plasma cortisol before taking oral cortisone may also be useful, but we prefer the cortrosyn test. Once the Cortrosyn stimulation test is in the normal range, then Hydrocortisone can be safely discontinued, or tapered to a lower dose, then discontinued.

We do not suggest longer acting steroids, such as Prednisone, which might further prolong pituitary adrenal suppression. The use of Hydrocortisone alone is what is suggested immediately after successful surgery. There is not only a need for Glucocorticoid replacement to avoid adrenal insufficiency, there is a withdrawal from the high endogenous levels. When you withdraw from steroids, even though you are in the normal range for replacement, you will have symptoms of steroid withdrawal which include fatigue, depression, and muscle and joint aching. This is why endocrinologists will tell you that you will feel worse from steroid withdrawal after successful surgery than you felt with the Cushing's syndrome. The steroid withdrawal symptoms can last up to two years and they are again, fatigue, depression, and aching.

There is no good evidence that taking less steroid, or no steroid, accelerates the hypothalamic pituitary adrenal axis recovery. Certainly you cannot use high doses without continuing to suppress the pituitary adrenal axis, but in general, completely stopping steroids is not suggested, since adrenal insufficiency would be predicted under those sets of circumstances and also aggravation of severe withdrawal symptomatology.

In summary, it is important to treat adrenal insufficiency symptoms of lightheadedness and nausea, and also distinguish that from steroid withdrawal symptoms, which are fatigue, depression, and achiness, even though you might be protected from adrenal insufficiency.

PREPARING FOR CUSHING'S PITUITARY SURGERY

I'm sharing a few links that may be helpful. I have collecting these websites for months while preparing for pituitary surgery. There are so many articles out there. Keyword searches are your friend. Mix it up. An abundance of information awaits you. Ex: pituitary surgery, transphenoidal, cortisol hormone replacement, post op pituitary surgery. Just use your imagination, and it will appear! Dedicate yourself to learning as much as you can about your condition. Knowledge is power.


PITUITARY SURGERY

Good transphenoidal pituitary surgery thingee to share with family

Answers From a Pituitary Surgeon

Frequently Asked Questions About Transsphenoidal Surgery For Pituitary Adenomas: A Patient Guide



WHAT TO BRING FOR A MORE COMFORTABLE HOSPITAL STAY

Kate's Packing Tip

Kate's Pituitary Surgery Observations



POST OP INSTRUCTIONS

**These links are included for you to get a sense of how different facilities manage their patients after pituitary surgery. The inclusion should not be substituted for medical advice. Please follow your doctors' care.

So you had your surgery-now what? Theodore C. Friedman, MD, PhD.

After Pituitary Surgery Instructions

Cushing's Disease after Successful Transsphenoidal Surgery - What to Expect and How to Manage

Learn more about hydrocortisone (brand name Cortef) and how this synthetic hormone will replace your new lower cortisol levels after surgery.



ADRENAL INSUFFICIENCY

Learn more about how pituitary surgery may likely lead to secondary adrenal insufficiency.

Learn more about Solu-Cortef, the emergency injection of hydrocortisone you should always carry with you!!

I HEART THE PITUITARY GLAND

The Pituitary Network Assocation (PNA) has done a fantastic job of putting together this list of frequently asked questions. I even learned something new while posting this! Thanks PNA, bok bok!

 

FAQs by Pituitary Network Association

– What is the pituitary?

The pituitary is a small, pea-sized gland located at the base of the brain that functions as "The Master Gland." From its lofty position above the rest of the body it sends signals to the thyroid gland, adrenal glands, ovaries and testes, directing them to produce thyroid hormone, cortisol, estrogen, testosterone, and many more. These hormones have dramatic effects on metabolism, blood pressure, sexuality, reproduction, and other vital body functions. In addition, the pituitary gland produces growth hormone for normal development of height and prolactin for milk production.

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– Is "The Master Gland" connected to the brain?

The pituitary hangs from the hypothalamus, a part of the brain located immediately above it, by a thread-like stalk that contains both blood vessels and nerves. Hormones produced in the hypothalamus carry signals or messages to the pituitary gland. The hypothalamus serves as a "switching station" to relay signals from many different parts of the brain to the pituitary gland, which in turn transmits its own signals to various parts of the body. The pituitary is divided into a larger anterior region (adenohypophysis) and a smaller posterior area (neurohypophysis). It sits in a small pocket of bone in the base of the skull called the pituitary fossa, also known as the sella turcica, or Turkish saddle, because of the resemblance. The sella turcica is located deep within the skull, which can be imagined by drawing an imaginary line from the top of the nose through the brain to the back of the head, and from ear to ear. The point at which they intersect is where the pituitary gland sits.

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– Why are pituitary disorders so hard to diagnose?

Pituitary disorders can cause a wide spectrum of symptoms, both hormonal and neurological, due to its location near the brain, the intracranial nerves and blood vessels and because of the vital hormonal control that the gland provides. Diagnosis of pituitary disease may be extremely difficult because it's often confused with other disorders. It is dependent on symptoms, signs on examination, blood tests and MRI findings. Direct access to the pituitary can only be reached at surgery.

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– Which hormones are secreted by the anterior pituitary?

Thyroid Stimulating Hormone (TSH) causes the thyroid gland to produce and release thyroid hormones. Thyroid hormone controls the basal metabolic rate and plays an important role in growth and maturation. Thyroid hormones affect almost every organ in the body.

Growth Hormone (GH) regulates growth and metabolism.
Adrenocorticotropic Hormone (ACTH) triggers the adrenals to release the hormone cortisol, which regulates carbohydrate, fat, and protein metabolism and blood pressure. The adrenal glands sit above the kidneys and are also responsible for the body's fight or flight response.

Luteinizing Hormone (LH) and Follicle Stimulating Hormone (FSH) control the production of sex hormones (estrogen and testosterone) and sperm and egg maturation and release.

Melanocyte-Stimulating Hormone (MSH) regulates the production of melanin, a dark pigment, by melanocytes in the skin. Increased melanin production produces pigmentation or tanning of the skin; in certain conditions excessive production of melanocyte-stimulating hormone can cause darkening of the skin.

Prolactin (PRL) stimulates production of breast milk and is necessary for normal milk production during breast feeding.

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– Which hormones are produced and/or controlled by the posterior pituitary or hypothalamus?

Oxytocin stimulates contractions of the uterus during labor and the ejection of milk during breast-feeding.

Antidiuretic Hormone (ADH) increases reabsorption of water into the blood by the kidneys and therefore decreases urine production. Also referred to as Vasopressin.

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– What happens if the pituitary produces either too much or too little of these hormones?

An imbalance occurs, leading to more than a dozen disorders of the endocrine system. Deficiency of thyroid hormone, adrenal cortical hormone (cortisol) or antidiuretic hormone (vasopressin) is rapidly life-threatening. In patients with abnormalities of the other hormones, quality of life is significantly compromised.

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– What causes the pituitary to malfunction?

Tumors (overwhelmingly benign), inflammation, infections and injury can cause the gland to malfunction, as well as metastasis or spread of other tumors to the pituitary (rare). Radiation therapy to the brain can also cause normal pituitary cells to malfunction.

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– What is a pituitary tumor?

A pituitary tumor is an abnormal growth of pituitary cells. Pituitary tumors can either be nonfunctional (that is they do not secrete hormones) or produce specific hormones, such as prolactin (causing infertility, decreased libido, and osteoporosis), growth hormone (causing acromegaly), ACTH (causing Cushing's), TSH (causing hypothyroidism), or be nonfunctional (that is they do not produce hormones). These tumors behave according to their cell of origin and are named for the specific cell type affected. For example, if a tumor originates in a prolactin producing cell, the patient develops a prolactinoma-a prolactin secreting pituitary tumor that is common and usually treatable. High prolactin levels suppress production of the pituitary hormones (luteinizing hormone and follicle stimulating hormone) that stimulate production of estrogen or testosterone. Men with these tumors have low testosterone levels and lose their sex drive and eventually their masculine characteristics-hair, muscle, erections, and ability to produce sperm. Women with prolactin producing tumors often do not ovulate, experience low estrogen levels, and cease having menstrual periods. In both cases, patients with low sex hormones develop osteoporosis. It is important to remember that most pituitary tumors are benign and cancer is very rare. They have variable patterns of growth and affect different people in vastly different ways. Some are small and incidental, while others are small but cause hormone excess. Others may be rapidly growing mass lesions.

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– Are pituitary tumors deadly?

If diagnosed early enough, the prognosis is typically excellent. If not, some tumors can grow into macroadenomas that press on the optic nerves (causing loss of peripheral vision and, in some cases, blindness), the brain (impacting function) and the pituitary (limiting or eliminating hormone production). Large tumors can also invade the cavernous sinuses, which house the carotid arteries, the veins to drain the eyes, and nerves involved in eye movement. Surgery around the cavernous sinuses is difficult and should be performed by an experienced pituitary surgeon. The tumors can also secrete too much of a hormone, causing hypertension, diabetes, mood disorders, sexual dysfunction, infertility, osteoporosis, arthritis, accelerated heart disease and death.

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– Why is early diagnosis such a problem?

The confusing constellation of symptoms that can be produced by pituitary tumors and the difficult to visualize location make diagnosis very tricky. It is not uncommon for patients to have symptoms of either hormonal deficiency (caused by compression of the pituitary or its "stalk") or hormone excess (caused by unregulated production of hormones by the pituitary tumor). In a significant minority of patients diagnosis is not made until the individual has developed debilitating or life-threatening symptoms of heart disease or adrenal (uncommon), gonadal and/or thyroid insufficiency. Even in the 21st century death from a large pituitary tumor or hormonal deficiency still occurs, albeit rarely. Early diagnosis is usually a reflection of a high index of suspicion on the part of a physician. Unfortunately, many doctors have been taught that pituitary disease is rare, so it is not at the forefront of their list of possible diagnoses.

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– How prevalent are pituitary tumors/disease?

Autopsy reports and radiologic and MRI evidence from around the globe indicate that one out of every five people worldwide has a pituitary tumor. The earliest study took place in 1936, when Dr. R.T. Costello of the Mayo Foundation conducted a cadaver study and found pituitary tumors in 22.4 % of the population (Costello R.T. Subclinical adenoma of the pituitary gland. Am. J. Pathol. 1936; 12:205-214). Statistics have not changed much ever since. The clinical significance of these findings are critical to determine.

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– Why are these tumors so common?

We don't know because funding for benign brain tumor research is virtually nonexistent. That's about to change. In October 2002, Congress passed the Benign Brain Tumor Cancer Registries Amendment Act, which will force hospitals, clinics and doctors to report pituitary tumor incidence rates in the data collection of cancer registries. The problem remains diagnosis. No report of incidence rates is possible without it.

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– Why aren't pituitary tumors/disease common knowledge?

There are four main reasons:

Pituitary tumors/disease present a vast array of symptoms, and it's often the symptoms that get treated, not the disease. As a result, pituitary patients can spend years being misdiagnosed as their tumors grow. People with undetected pituitary tumors can die of heart attacks, hypothyroidism, adrenal insufficiency or water balance problems, all of which can mask the main cause: a pituitary tumor.

Dollars spent. As a result, we have failed to answer the most important question: Why are pituitary tumors so common?

There is a lack of education within the medical community and among the general public.

The insurance industry hasn't caught on to the untold billions of dollars that could be saved through early diagnosis and treatment. Once it becomes clear that it's in everyone's best interest, the word will spread.

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– How are pituitary tumors treated?

It depends on the type of tumor and how far it has invaded into the brain, as well as the patient's age and overall health. Three kinds of treatment are used: surgery (removing the tumor during an operation), radiation therapy (using high-dose x-rays/proton beams to kill tumor cells) and drug therapy to shrink and sometimes eradicate the tumor. Drugs can also block the pituitary gland from making too much hormone. A Transphenoidal operation can remove the tumor through a cut in the nasal passage, leaving no external scar. (Sometimes a surgeon prefers to go through an incision under the upper lip instead of the nose, also with no external scarring.) A craniotomy removes the tumor through a cut in the front of the skull; this is rarely done for large invasive tumors.

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– Is pituitary tumor surgery risky?

Surgery is a common treatment, and it is almost always successful IF performed by a skilled and specialized neurosurgeon. The smaller the tumor, the greater the chance the surgery will be successful. Large tumors that grow upward (into the nerves that mediate vision) or laterally (into the cavernous sinuses that contain the major arteries to the brain-the carotid arteries) increase the risk of surgery and are generally associated with a lower probability of cure. Early diagnosis and treatment is the key.

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– Is there a long recovery process from pituitary tumor surgery?

In most cases, no. Patients often report immediate relief from symptoms after Transphenoidal Hypophysectomy and little pain. Some leave the hospital the same day.

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– How effective is radiation therapy?

Radiation therapy uses high-energy x-rays to kill cancer cells and abnormal pituitary cells and shrink tumors. Radiation for pituitary tumors these days almost always comes from a radiation source outside the body (external radiation therapy). Radiation therapy may be used if medication/surgery fails to control the tumor. There are several different types of radiation therapy.

Standard External Beam Radiotherapy uses a radiation source that is nonselective and radiates all cells in the path of the beam; in almost all cases other portions of the brain in the area of the pituitary are in the path of the radiation beam.
Proton Beam Treatment employs a specific type of radiation in which "protons", a form of radioactivity, are directed specifically to the pituitary gland. The advantage of this technique is less damage to tissues surrounding the pituitary gland.

Gamma Knife Irradiation combines standard external beam radiotherapy with a technique that focuses the radiation through many different ports. This treatment tends to do less damage to tissues adjacent to the pituitary gland. Some clinics are using proton beam therapy; laser-guided MR imaged stereotactic intrasellar tumor localization can be used for selected patients with untreated pituitary tumors, incomplete surgical resection, or tumor recurrence.

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– Are all pituitary tumors created equal?

No. There are more than a dozen very different disorders that result from pituitary tumors and disease. For example, if a tumor forms in an ACTH secreting pituitary cell, it could result in the overproduction of cortisol (Cushing's Disease) or the underproduction of cortisol (adrenal insufficiency, often referred to as Secondary Addison's Disease). Cushing's is a condition characterized by excessive fat accumulation in central parts of the body (obesity, including a rounded or fat-filled face), diabetes, hypertension, a low serum potassium, thinning and bruising of skin, and osteoporosis. Symptoms of adrenal insufficiency include dehydration, low blood pressure and sodium level, and excessive weight loss. Primary Addison's Disease is caused when the adrenal glands fail to work on their own, i.e. fail to respond to directions from the pituitary and hypothalamus.

If the tumor forms in a growth hormone producing cell, it can overproduce growth hormone. Tumors that form from growth hormone producing cells cause two different clinical pictures. If they occur in children before the growth plates in long bones have closed, excessive growth hormone will cause gigantism. (Do you recall the movie "My Giant" with Billy Crystal and the basketball player?). If the growth hormone excess occurs during adulthood there is excessive enlargement of the hands, feet, and jaw, as well as soft tissue swelling of many tissues (acromegaly). Acromegaly is associated with an increased probability of developing diabetes mellitus, heart attack, hypertension, and certain types of cancer including malignancy of the colon. Most commonly the facial changes develop subtly and may not be noticed by the patient or his/her family.

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– What happens after a tumor is removed/treated?

There may be permanent loss of some or all pituitary hormones, an imbalance that can be treated with Hormone Replacement Therapy, which has been inaccurately associated with only one group of patients: post-menopausal women. HRT can replace thyroid, growth, testosterone, or adrenal hormones when those made by the pituitary to stimulate the endocrine glands are no longer produced. It can be lifesaving therapy for the many millions of patients who need to replace hormones they no longer make.

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– How do I get diagnosed?

Anyone suffering from a constellation of complaints/clinical findings suspicious for pituitary disease should get blood and urinary hormone levels checked and, if indicated, a brain MRI, keeping in mind that microadenomas may not show up on the x-rays. Combinations of three or more of the following may suggest the possibility of a pituitary tumor: sexual dysfunction, depression, galactorrhea, infertility, growth problems, osteoporosis, obesity (specifically central), severe vision problems, easy bruising, aching joints, carpel tunnel syndrome, disrupted menses, early menopause, muscle weakness, fatigue.

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– Where do I go to get tested?

It is important to go to a hospital with a Neuroendocrine Unit or Pituitary Testing Facility, preferably both. (This may necessitate plane travel, but it would be worth every penny.) Pituitary blood tests can be very complicated and must be performed by specially trained technicians who can conduct dynamic hormone testing and precisely-timed blood sampling, administer testing agents, and have special expertise in measuring pituitary hormones. Tests that require special attention include ACTH (Cortrosyn) stimulation, bilateral inferior petrosal sinus ACTH sampling, Insulin tolerance, growth hormone stimulation, TRH stimulation, GnRH stimulation, Oral glucose tolerance, and GHRH/arginine testing for growth hormone reserve.

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– Where do I go to get treated?

Ideally you want to be seen by a pituitary endocrinologist associated with a neuroendocrine or pituitary unit with a pituitary testing facility. The interpretation of complex blood tests can result in misdiagnoses. This can be associated with microadenomas (very small tumors) that do not show up on an MRI of the brain. It often takes a real expert to get the right answers and avoid pitfalls.

SIMPLY THE BEST: Pituitary Center brings life-changing treatment to thousands

I searched on the internet for three words: Best Pituitary Surgeon. This article appeared.

Dr. Edward Laws is not my neurosurgeon. My neurosurgeon trained directly with Dr. Laws. Dr. Laws is widely considered to be the best pituitary surgeon in the world. He has done more pituitary surgeries than anyone else. Like over 4,500.

My neurosurgeon was the chief neurosurgery resident under Dr. Laws during his tenure at the University of Virginia. They would do 6 pituitary surgeries a day together.

In surgery, it is all about who you train with. I have complete faith in my doctor. I really felt positive about my surgeon choice before researching Laws again. Now, I admit to feeling even better!

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Simply the Best
Pituitary Center brings life-changing treatment to thousands


Photo by Andrew Shurtleff
Dr. Edward Laws prepares to remove a pituitary tumor from a patient. These tumors account for 20 percent of all primary brain tumors, and Laws is internationally recognized as the top pituitary surgeon.

By Fariss Samarrai

Dr. Edward Laws stands on a wooden pedestal as he extracts a brain tumor through a woman’s right nostril. His resident, Dr. Adam Kanter, and a team of operating room specialists, have prepared the patient for the procedure by first anesthetizing her, and then, by sending long and slim instruments up the woman’s nostril, they have removed a thin wafer of bone, allowing direct access through the air sinuses to the pituitary gland at the base of the brain.

Laws’ job is to remove a tumor that has grown on the pituitary by carefully scraping loose and periodically removing fragments of the mass of bad cells while being careful to leave the healthy tissue of the pituitary and the brain intact. He does this while looking at the inside of the patient’s head through a binocular endoscope with a micro-camera attached, allowing everyone in the OR to observe the procedure on a video monitor.

He pulls out another fragment of the tumor.

“These tumors have the texture of tofu,” he says, though the tissue he is extracting is bloody. He mentions in passing that he used to tell people that pituitary tumors have the texture of tapioca, but that many people don’t seem to know what that is anymore.

Laws removes a final fragment of the tumor, and his work is done for now. The procedure is called trans-sphenoidal surgery — basically access-through-the-nose surgery. By day’s end Laws will have removed pituitary tumors from six patients, likely making the lives of these people immeasurably better.

Laws is director of the Pituitary Center at U.Va. He has removed pituitary tumors from more than 4,300 patients, more than any other doctor in the world. Most of these surgeries have been performed at the U.Va. center he helped found in 1972. Laws is internationally recognized as the top pituitary tumor surgeon. Medical residents from around the world come to U.Va. to train under him.

Illustration by Craig Luce

Pituitary Tumors
Where they are and how they are removed
The pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances. Pituitary tumors are reached by passing long and slim surgical instruments through the air sinuses to the diseased gland.


The procedure, like all planned operations, is methodical, calm, controlled, predictable, with precautions in place for possible problems or emergencies. The operation is so un-invasive, it more resembles routine dental work than major surgery. Yet, Laws and his colleagues are working at the very edge of the brain. There is little room here for error.

In past years, pituitary surgery was more dramatic, and intensive, for both the surgeon and the patient. It involved removing a portion of the skull and pushing the brain aside to reach the pituitary. The recovery period was long, and there was much greater risk for damage and complications. Laws and others invented and refined the current technique — passage through the nose — which rarely involves direct contact with the brain, even as they operate within its midst.

The pituitary gland is located at the base of the brain near the spinal cord, in the lower center of the head. This gland, normally the size of a pea, is the master gland that controls the production of all hormones. The pituitary controls growth, sexual maturation, fertility, metabolism, stress response and fluid balances.

For reasons not well understood, the pituitary is subject to the slow growth of tumors. These tumors are the third-most common type of primary brain tumor, accounting for 20 percent of all brain tumors. Though these tumors are almost always benign, they aren’t friendly. They can cause the pituitary gland to produce excessive or insufficient amounts of hormones, leading to a vast array of unpleasant symptoms, including severe and chronic headaches, infertility, sexual dysfunction, fatigue, weight gain, depression, apathy, sleep disorders, memory loss, and in some cases, loss of vision. Pituitary disorders also are the cause of Cushing’s syndrome, which results in obesity, “moonlike” features of the face, excess body hair and thin brittle bones.

Pituitary surgeries are discussed prior to and after each operation.

Because the symptoms of pituitary disorders are so varied, it can take years to arrive at a correct diagnosis,” Laws said. “Patients often think that their health problems are attributed to just getting older.”

It takes a brain scan in the form of magnetic resonance imaging to confirm the presence of a pituitary tumor. Sometimes the tumor is three times the size of the gland itself, putting pressure on the brain and optic nerve, resulting in headaches and vision loss. Small tumors often can be treated with hormones and drugs. Radiation may shrink the tumor or inhibit its growth. If the tumor continues to grow, and symptoms worsen, surgery may be necessary.

Laws discovered early, at the beginning of his medical training at Johns Hopkins University, that he was interested in neurosurgery. His fascination with the brain has continued throughout his 40-year career as a surgeon and brain tumor researcher.
“How beautiful the brain is,” he said recently. “I’m amazed with what happens with the human brain, and I love dealing with it in an intimate fashion.” He describes the brain as “one of the greatest of God’s creations.”

But he’s happy to fix that creation when something goes wrong.

“We can restore vision,” he said of pituitary surgery. “We can restore fertility and reverse body changes. This is enormously satisfying.”

These tumors are identified using magnetic resonance imaging.

One of the first patients Laws treated with the through-the-nose surgical method was a 21-year-old man named Harvey Gartner. The surgery was performed in 1969 at The Johns Hopkins Hospital where Laws was a neurosurgery resident. Gartner had been growing rapidly since he was 6 months old. His bones were elongated, and he suffered from arthritis and other ailments.

“Those were tough years,” Gartner said of his youth.

The operation took 12 hours to complete, and all of the pituitary gland was removed, as was standard at the time. Gartner, now 56, is on lifetime hormone replacement therapy, but his health is generally good.

“I would have died,” if not for the surgery, he said.

Gartner is now a computer programmer and businessman in Jacksonville, Fla. He has remained in contact with Laws, and the pediatrician who cared for him throughout his life, Dr. Robert Blizzard, a U.Va. professor emeritus of pediatric endocrinology. At the time of Gartner’s surgery, Hopkins was the top pituitary center in the United States. That title has long since shifted to U.Va. as a result of Laws, Blizzard and others coming here to establish their interdisciplinary center.

Today, surgeons rarely need to remove the entire pituitary gland. Instead, when surgery is necessary, they remove only the tumor attached to the gland. The entire procedure, including prepping the patient for surgery and the post-operative period, can be accomplished in a couple of hours. The patient is able to return home in two or three days and can resume normal activities in two to four weeks.

Within a few days after surgery, the patient’s severe headaches will have diminished. Vision will be restored within a day in cases where the tumor had pressed against the optic nerve. Body changes, such as weight loss and normalization of facial features, will occur in about six months. Throughout the patient’s life, teams of endocrinologists and other specialists will measure the patient’s hormone levels and develop and refine treatment plans. Laws said some patients might not even need hormone replacement therapy.

“I don’t think anybody does this work better than we do at U.Va.,” Laws said recently between surgeries. “We’re improving our techniques and reducing the recurrence of tumors after surgery.”

At this moment he is called back into the operating room. Another patient is prepped and waiting.

PITUITARY SURGERY: Post-Op FAQs

After surgery FAQs, from Cushing's Help and Support

I've finally been diagnosed and am going to have a transsphenoidal. What can I expect post-surgery?

AFTER YOUR TRANSSPHENOIDAL SURGERY

After your physicians have determined that it is reasonably safe to discharge you from the hospital following transsphenoidal pituitary surgery there are a number of important situations that may arise. Most people feel well after discharge. However, you should be aware of these possible problems, just in case. The following general guidelines are provided to promote your health and safety.
Headache, facial, and sinus pain are not uncommon following pituitary surgery. As you may have noted, the pain and discomfort typically improve on a daily basis following surgery. If you should experience a worsening of your pain or discomfort, please contact your neurosurgeon immediately.

Worsening headache, fever, chills, yellowish green nasal discharge, and neck stiffness may all signify an infectious process complicating your surgery. You should notify either your neurosurgeon, endocrinologist, or primary physician immediately should any of these symptoms and signs develop.

Persistent bloody, clear watery, or yellowish green nasal discharge should prompt an immediate call to one of your physicians.

Development of abnormalities in your vision should prompt an urgent call to your neurosurgeon, neuroopthalmologist, or any other one of your physicians.

Chest pain or discomfort, shortness of breath, swelling of one or both of your legs, and passage of dark black tarry stools may represent medical complications in patients who undergo surgery of any type. Contact your physicians should any of these symptoms or signs occur.

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 or one of your other physicians immediately. 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. Thus, if these symptoms develop, contact your endocrinologist or one of your other physicians immediately.

You may or may not have been prescribed hormones at the time of discharge. If so, you should take these medications, without interruption, as prescribed by your physician. Adjustments in your glucocorticoid hormone dosage may be required. Please consult the instructions for patients with adrenal insufficiency for general recommendations. You may be asked to withhold your dose of glucocorticoid replacement at the time of your first postoperative follow-up visit. Contact your endocrinologist for advice on this matter if specific instructions have not been provided. The instructions for patients with hypothyroidism on thyroxine replacement may be consulted for advice regarding thyroid hormone medication. You should consult the instructions for patients with diabetes insipidus treated with vasopressin if you have been diagnosed with diabetes insipidus or suspect that you may have developed the disorder. Above all, contact your physicians if you have any questions whatsoever about any one of your medications.

In general, the first postoperative follow-up visit will be scheduled to occur four weeks after surgery. If problems develop prior to that time, you will be asked to return to the office for evaluation. Subsequent follow-up is tailored to the individual needs of each patient and in part depends upon the diagnosis, presence of residual disease, likelihood of recurrent disease, extent and type of hormonal disorders, and other complications of pituitary disease.

In most cases, lifelong follow-up is necessary. You should ensure that you receive appropriate follow-up by physicians knowledgeable regarding the diagnosis and management of pituitary disorders.

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Once again, MaryO has done a wonderful job organizing the Cushing's Help and Support website to help us Cushies access info when we need it. Thanks, Mary!

Adrenal Insufficiency & Cortisol Withdrawal

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.