Low Cortisol or an Adrenal Crisis – Learning the difference

Some are very sick and believe it can be treated at home, others are low on cortisol and believe they urgently need Emergency Department treatments. It can be very hard to tell the difference at times.

Many with Addison’s Disease, especially in the early years after diagnosis, don’t have a clear understanding of what an Adrenal Crisis is­.

Some are very sick and believe it can be treated at home, others are low on cortisol and believe they urgently need Emergency Department treatments.  It can be very hard to tell the difference at times.

Then we have the Dr’s view, based on books, not on experience, which is, don’t know so won’t treat.

THE FACTS:

  1. Low Cortisol WILL lead to an Adrenal Crisis, if not dealt with;
  2. Adrenal Crisis DOES need Emergency Medical Treatment;
  3. Adrenal Crisis WILL lead to Death (or worse) if not treated urgently and correctly;
  4. Dr’s DO cause Adrenal Crisis’ because they don’t know, or don’t listen to their patients.

I am guessing the first thing you are asking is…

View original post 2,110 more words

Living with an Addisonian

(Low Cortisol or an Adrenal Crisis – PDF of this blog.)

Many with Addison’s Disease, especially in the early years after diagnosis, don’t have a clear understanding of what an Adrenal Crisis is­.

Some are very sick and believe it can be treated at home, others are low on cortisol and believe they urgently need Emergency Department treatments.  It can be very hard to tell the difference at times.

Then we have the Dr’s view, based on books, not on experience, which is, don’t know so won’t treat.

THE FACTS:

  1. Low Cortisol WILL lead to an Adrenal Crisis, if not dealt with;
  2. Adrenal Crisis DOES need Emergency Medical Treatment;
  3. Adrenal Crisis WILL lead to Death (or worse) if not treated urgently and correctly;
  4. Dr’s DO cause Adrenal Crisis’ because they don’t know, or don’t listen to their patients.

I am guessing the first thing you are asking is…

View original post 2,110 more words

‘Adrenal Fatigue’ Not Always Used Accurately

Dear Dr. Roach: I had apoplexy, a ruptured pituitary tumor, developed panhypopituitarism, then adrenal insufficiency. I am doing fairly well with cortisol replacement, thyroid supplement and oral diabetic medicine.

My problem is exhaustion that comes on very easily. I have other ailments to blame, too — chronic pain from fibromyalgia and tendinitis. I am 67. I am still able to work. Is adrenal fatigue a real issue, and if so, what can be done about it? — S.M.

Answer: The term “adrenal fatigue” is increasingly used, and not always correctly — or, at least, it is used in cases where it’s not clear if that is actually the case. But let me start by discussing what has happened to you. Pituitary apoplexy is bleeding into the pituitary gland, usually into a pituitary tumor, as in your case. This may cause severe headaches and vision changes, and often it prevents the pituitary from making the many important hormones that control the endocrine glands and regulate the body.

For example, without TSH from the pituitary gland, the thyroid won’t release thyroid hormone, and importantly, the adrenal gland can’t make cortisol without the influence of ACTH from the pituitary.

Rather than trying to replace TSH, ACTH and the other pituitary hormones, it is easier to directly replace the hormones made by the adrenal, thyroid and gonads. That’s why you are taking cortisol and thyroid hormone, and why younger women take estrogen and men testosterone. Although there is nothing wrong with your thyroid and adrenal glands, they simply won’t work unless stimulated.

Inadequate adrenal function from any cause leads to profound fatigue, and in the presence of severe stress, such as surgery or major infection, the body’s need for cortisol increases dramatically. Unless enough adrenal hormone is given in response, the result can be an immediate life-threatening condition called an Addisonian crisis.

Readers may email questions to ToYourGoodHealth@med.cornell.edu.

From http://www.vnews.com/To-Your-Good-Health–Adrenal-Fatigue–not-Always-Used-Accurately-1802516

Adrenal Insufficiency Patients Require More Education on Adrenal Crisis

adrenalcrisispathway

Greater efforts to educate patients with adrenal insufficiency and their families about prevention of adrenal crisis may be necessary, according to data presented at the American Association of Clinical Endocrinologists (AACE) 24th Annual Scientific & Clinical Congress.

Additionally, the researchers, who looked at patients treated for adrenal insufficiency, found that many are not being adequately trained or equipped to deal with an adrenal crisis.

“These patients can crash and we are not doing enough to help prevent problems,” study investigator Nitika Malhotra, MD, endocrinologist in Lansing, Michigan, said. “We did this study because we think this is a big problem.”

Malhotra, who presented the study findings at the meeting, explained that patients with adrenal insufficiency are at risk for developing adrenal crisis, and it is now estimated that 8% of patients with adrenal insufficiency are hospitalized for adrenal crisis each year.

The problem, according to Malhotra, is that far too many patients are failing to receive crises prevention education. Moreover, they are not receiving emergency glucocorticoid kits.

“All of the families need to be taught and that is not happening,” Malhotra said in an interview with Endocrinology Advisor. “It will reduce the morbidity and mortality and the hospitalization, and it may improve the quality of life of patients too.”

For their study, Malhotra and her colleagues collected data from patients with adrenal insufficiency who were seen at a single institution between March 2009 and March 2014.

The investigators conducted a retrospective chart review and examined age, gender, causes of adrenal insufficiency, glucocorticoid dose, and monitoring for hyponatremia and hyperkalemia. They also looked at postural blood pressure, crises prevention education for glucocorticoid dose adjustments during stress, and whether patients had a Medic Alert ID or a parenteral glucocorticoid kit.

The researchers identified 85 patients (29 males and 56 females) with adrenal insufficiency. Of these patients, 33 patients had primary adrenal insufficiency (38.8%) and 52 had secondary adrenal insufficiency (61.2%). The mean age of the patients was 55.8 years.

Among the 85 patients, 23 (27%) had postural blood pressures checked — five of whom were positive (21.7%). Seventy-seven patients (90.6%) were monitored for electrolytes, and 41 patients (48.2%) were on steroid doses above 20 mg per day.

However, the researchers found that only 57 patients (67.1%) had received steroid dose adjustment instructions. In addition, only 29 patients (34.1%) had a Medic Alert ID, and only 17 patients (20%) were setup with emergency parenteral glucocorticoid kits.

Even though this study has many inherent limitations, Malhotra said, it appears that the preventive strategies for adrenal crisis in patients with adrenal insufficiency are not being consistently followed.

Patient education is paramount for achieving a successful prevention strategy for adrenal crisis, and endocrinologists have a responsibility to make sure that all patients with adrenal insufficiency have a Medic Alert ID and access to emergency glucocorticoid kits, according to Malhotra.

Furthermore, she said families should receive adequate education about parenteral steroid administration and steroid dose adjustments in stressful situations.

At her institution, Malhotra said, endocrinologists are introducing an automated electronic alert in their electronic medical records to determine if this electronic prompt will improve adherence.

Reference

  1. Malhotra N et al. Abstract #102. Presented at: American Association of Clinical Endocrinologists (AACE) 24th Annual Scientific & Clinical Congress; May 13-17, 2015; Nashville, Tenn.

 

From http://www.endocrinologyadvisor.com/aace-2015/adrenal-crisis-in-adrenal-insufficiency/article/415123/

BLA Instead of Second Pituitary Surgery

One of the problems that can arise with a BLA (bilateral adrenalectomy) instead of a repeat pituitary surgery for Cushing’s recurrence is Adrenal Insufficiency.  Another is Nelson’s Syndrome.

Nelson’s syndrome is a rare disorder that occurs in some patients with Cushing’s disease patients as a result of removing both adrenal glands. In Nelson’s syndrome, the pituitary tumor continues to grow and release the hormone ACTH.

This invasive tumor enlarges, often causing visual loss, pituitary failure and headaches. One key characteristic of Nelson’s disease is dark skin pigmentation, resulting from the skin pigment cells responding to the release of ACTH.


AnchorNelson’s Syndrome: Physiology

Nelson’s syndrome can develop as a result of a specific treatment (bilateral adrenalecomy) for the pituitary disease called Cushing’s disease. The harmful effects of Cushing’s disease are due to the excessive amount of the hormone cortisol produced by the adrenal glands.

To treat Cushing’s disease, your doctor may recommend removing the adrenal glands, during a procedure called a bilateral adrenalectomy. The procedure will stop cortisol production and provide relief. However, the procedure does not treat the actual tumor. Rapid growth of the pituitary tumor can occur.

In about 15-25 percent of patients who had a bilateral adrenalectomy, Nelson’ syndrome develops within one to four years.


Darkening of Skin Color - Nelson's Syndrome SymptomAnchor

Nelson’s Syndrome: Symptoms

The most obvious symptom of Nelson’s syndrome is the darkening of the skin color (hyperpigmentation).

Macroadenomas

Macroadenomas are large pituitary tumors. Large tumors can compress surrounding structures, primarily the normal pituitary gland and optic (visual) pathways, causing symptoms. The symptoms that result from the compression are independent of the effects of excess growth hormone secretion.
This may result in vision problems:

  • Vision loss. This occurs when macroadenomas grow upward into the brain cavity, compressing the optic chiasm.
  • A loss of the outer peripheral vision, called a bitemporal hemianopsia Bitemporal Hemianopsia - Symptom of Nelson's Syndrome
    • When severe, a patient can only see what is directly in front of them.
    • Many patients do not become aware of their visual loss until it is quite severe.
  • Other visual problems can include:
    • Loss of visual acuity (blurry vision), especially if the macroadenoma grows forward and compresses an optic nerve.
    • Colors not perceived as bright as usual

Pituitary Failure or Hypopituitarism

Increased compression of the normal gland can cause hormone insufficiency, called hypopituitarism. The symptoms depend upon which hormone is involved.


AnchorNelson’s Syndrome: Diagnosis

Most patients with Nelson’s syndrome have undergone a bilateral adrenalectomy for the treatment of Cushing’s disease

Diagnostic testing includes:

  • Hormone testing. Typically, the blood ACTH levels are very elevated. Learn more about hormone testing at the UCLA Pituitary Tumor Program.
  • MRI imaging. Magnetic resonance imaging (MRI) scan of the pituitary gland can detect the presence of an adenoma, a pituitary tumor.

AnchorNelson’s Syndrome: Treatment Options

Surgery for Nelson's Syndrome

Treating Nelson’s syndrome effectively requires an experienced team of experts. Specialists at the UCLA’s Pituitary Tumor Program have years of experience managing the complex coordination and care for treatment of Nelsons’ syndrome.

Treatment options include:

AnchorSurgery for Nelson’s Syndrome

Surgical removal of the pituitary adenoma is the ideal treatment; however, it is not always possible. Surgical removal requires advanced surgical approaches, including delicate procedures involving the cavernous sinus.

If surgery is required, typically the best procedure is through a nasal approach. Our neurosurgeons who specialize in pituitary tumor surgery are experts in the minimally invasive expanded endoscopic endonasal technique. This procedure removes the tumor while minimizing complications, hospital time and discomfort. This advanced technique requires specialized training and equipment.

Very large tumors that extend into the brain cavity may require opening the skull (craniotomy) to access the tumor. Our surgeons are also experts in the minimally invasive “key-hole” craniotomy, utilizing a small incision hidden in the eyebrow.

AnchorRadiation Therapy for Nelson’s Syndrome

Radiation Therapy for Nelson's SyndromeRadiation therapy can be effective in controlling the growth of the tumor. However, if you received radiation therapy in the past, additional radiation may not be safe.

Our Pituitary Tumor Program offers the latest in radiation therapy, including stereotactic radiosurgery. This approach delivers a highly focused dose of radiation to the tumor while leaving the surrounding brain structures unharmed (with the exception of the normal pituitary gland).

One consequence of radiation treatment is that it can cause delayed pituitary failure. This typically occurs several years after treatment, and continued long-term follow-up with an endocrinologist is important. You may require hormone replacement therapy.

Medical Therapy for Nelson’s Syndrome

Medication for Nelson's SyndromeMedical therapies for the treatment of Nelson’s syndrome are currently limited, but include:

  • Somatostatin-analogs (SSAs). These medications are typically used to treat acromegaly. A small number of Nelson’s syndrome patients may respond.
  • Cabergoline. This medication is typically used to treat prolactinomas; you may require a very high dose.
  • Temozolomide. This is a type of chemotherapy used to treat primary brain tumors called glioblastoma.

If you require medication to treat Nelson’s syndrome, our endocrinologists will monitor you closely.

From http://pituitary.ucla.edu/body.cfm?id=53

 

Myth: “Each Person Requires the Same Dose of Steroid in Order to Survive…

Myth: “Each person requires the same dose of steroid in order to survive with Secondary or Primary Adrenal Insufficiency”

myth-busted

Fact: In simple terms, Adrenal Insufficiency occurs when the body does not have enough cortisol in it. You see, cortisol is life sustaining and we actually do need cortisol to survive. You have probably seen the commercials about “getting rid of extra belly fat” by lowering your cortisol. These advertisements make it hard for people to actually understand the importance of the function of cortisol.

After a Cushing’s patient has surgery, he/she goes from having very high levels of cortisol to no cortisol at all. For pituitary patients, the pituitary, in theory, should start working eventually again and cause the adrenal glands to produce enough cortisol. However, in many cases; the pituitary gland does not resume normal functioning and leaves a person adrenally insufficient. The first year after pit surgery is spent trying to get that hormone to regulate on its own normally again. For a patient who has had a Bilateral Adrenalectomy (BLA), where both adrenal glands are removed as a last resort to “cure” Cushing’s; his/her body will not produce cortisol at all for his/her life. This causes Primary Adrenal Insufficiency.

All Cushing’s patients spend time after surgery adjusting medications and weaning slowly from steroid (cortisol) to get the body to a maintenance dose, which is the dose that a “normal” body produces. This process can be a very long one. Once on maintenance, a patient’s job is not over. He/She has to learn what situations require even more cortisol. You see, cortisol is the stress hormone and also known as the Fight or Flight hormone. Its function is to help a person respond effectively to stress and cortisol helps the body compensate for both physical and emotional stress. So, when faced with a stressor, the body will produce 10X the baseline levels in order to compensate. When a person can not produce adequate amounts of cortisol to compensate, we call that Adrenal Insufficiency. If it gets to the point of an “Adrenal Crisis”, this means that the body can no longer deal and will go into shock unless introduced to extremely high levels of cortisol, usually administered through an emergency shot of steroid.

There are ways to help prevent a crisis, by taking more steroid than the maintenance dose during times of stress. This can be anything from going to a family function (good stress counts too) to fighting an infection or illness. Acute stressors such as getting into a car accident or sometimes even having a really bad fight require more cortisol as well.

It was once believed that everyone responded to every stressor in the exact same way. So, there are general guidelines about how much more cortisol to introduce to the body during certain stressors. For instance, during infection, a patient should take 2-3X the maintenance dose of steroid (cortisol). Also, even the maintenance dose was considered the same for everyone. Now a days, most doctors will say that 20 mg of Hydrocortisone (Steroid/Cortisol) is the appropriate maintenance dose for EVERYONE. Now, we know that neither is necessarily true. Although the required maintenance dose is about the same for everyone; some patients require less and some require more. I have friends who will go into an adrenal crisis if they take LESS than 30 mg of daily steroid. On the other hand, 30 mg may be way too much for some and those folks may even require LESS daily steroid, like 15 mg. Also, I want to stress (no pun intended) that different stressors affect different people differently. For some, for instance, an acute scare may not affect them. However, for others, receiving bad news or being in shock WILL put their bodies into crisis. That person must then figure out how much additional steroid is needed.

Each situation is different and each time may be different. Depending on the stressor, a person may need just a little more cortisol or a lot. Every person must, therefore, learn their own bodies when dealing with Adrenal Insufficiency. This is VERY important! I learned this the hard way. As a Clinical Psychologist; I assumed that my “coping skills” would be enough to prevent a stressor from putting me into crisis. That was FAR from the truth! I have learned that I can not necessarily prevent my body’s physiological response to stress. People often ask me, “BUT you are a psychologist! Shouldn’t you be able to deal with stress?!!!!” What they don’t realize is that my BODY is the one that has to do the job of compensating. Since my body can not produce cortisol at all, my job is to pay close attention to it so that I can take enough steroid to respond to any given situation. We all have to do that. We all have to learn our own bodies. This is vitally important and will save our lives!

To those we have lost in our community to Adrenal Insufficiency after treatment of Cushing’s, Rest in Peace my friends! Your legacies live on forever!

~ By Karen Ternier Thames

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