Action For Adrenal Disease

 

Watch online Saturday April 16 at 1:00 PM eastern at https://plus.google.com/events/cpjbd8celcbfgngp8und662s198?hl=en

Secondary Adrenal Insufficiency and Addison's Disease can be deadly, mostly because of the lack of education and awareness. We have lost too many and need to be proactive in preventing unnecessary deaths! Join us as we educate on what these diseases are, how easily they can become deadly and preventive measures we can all take to help this community. Brought to you by the National Adrenal Disease Foundation, with speakers who have personal experience with these diseases.

Our program will include:

Senior Administrator Nichole Klute Rushton••• of the Addison’s Disease Support Group (https://www.facebook.com/groups/addisons.support/) on Facebook, will speak in detail about the unfortunate adrenal insufficient patients who have tragically passed, reminding us that the danger of loss of life is a reality for every person with adrenal insufficiency who doesn’t receive the vital hormones they need

Administrator Debby Hunter ••• of the Living With Addison's Disease Support on Facebook (https://www.facebook.com/groups/LivingWithAddisonsDisease/) who will give us tips on how we can approach our local emergency facilities and hospitals with information about adrenal insufficiency and its care in a crisis situation. She will also share her own personal experience with going through an adrenal crisis.

Deputy Sheriff Chris Spires••• who will speak on life as the husband of an Addison’s disease patient, and share with us how the law enforcement community views adrenal insufficient patients

Melanie Wong ••• National Adrenal Disease Foundation (http://www.NADF.us) Executive Director, who will speak about the recent tragic losses, and the vital importance of reminding the medical community about adrenal insufficiency, as well as NADF’s latest project to get NADF Adrenal Crisis Care posters displayed in every emergency room facility in the United States.

Adrenal Insufficiency Patients Require More Education on Adrenal Crisis

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/

New Jersey Ambulances Carrying Solu-Cortef

The New Jersey Department of Health passed a waiver in October of last year that allows ambulances to carry Solu- Cortef, for the purposes of treating an adrenal crisis. As a result, New Jersey ambulances can be better prepared to treat adrenal insufficiency.

This news was brought to NADF by Karen Fountain of the CARES Foundation, who has been helping push state health directors to accept protocols to help treat adrenal insufficient patients during an emergency.

Adrenal insufficient people in New Jersey should contact their local EMS to make them aware of the waiver, and encourage them to carry Solu-Cortef in their ambulances.

The hope is that other states, and eventually the entire country and beyond, will start having their ambulances carry the needed medication to treat adrenal crisis.

http://www.nadf.us

An Adrenal Crisis Survey

This Survey is to gather information for the Adrenal Insufficiency Awareness Organization's grant project to create educational materials for ER personnel. 

The survey is for those of you who have been to the ER during an impending or full blown Adrenal Crisis. (you may fill it out for a child or yourself) 

Your help is appreciated! 

At the end of the survey you will have the chance to enter for a chance to win an Adrenal Insufficiency Awareness Pin.

https://www.surveymonkey.com/s/ERcare

Adrenal Insufficiency - Female Patient found Unconscious with Syringe Nearby

Crew’s misdiagnosis of drug overdose could have cost patient her life

Kimberly Doran | From the October 2012 Issue |

A call comes in to 9-1-1 dispatch. “Help” is all that’s spoken before the operator hears the phone hit the floor. The 9-1-1 dispatcher calls back only to get a busy signal. Police and EMS are dispatched for a well-being call.

On arrival, the front door is found to be slightly ajar. The crew knocks, but there’s no reply. They find a young woman  lying on the floor, naked, and in a pool of vomit. A syringe with an unknown substance is on the ground nearby. Suspecting a drug overdose, the EMS crew begins treating the patient for this condition. The patient is unconscious with emesis around her head and face. Her vital signs are blood pressure 60/45, heart rate of 130 bpm and respiratory rate of 10.

The patient shows no signs of waking. The crew clears the airway and administers oxygen. An IV is established, and the patient is readied for transport. As the crew leaves the scene, one of the medics turns to shut the door and sees a vial under a chair. He retrieves it and notes that the label says Solu-Cortef (a glucocorticoid). He bags it for the emergency department (ED). Following his instinct, he looks around the area for medications and finds two bottles. One is labeled dexamethosone and the other is labeled fludrocortisone. He takes his findings and rushes out the door into the awaiting ambulance. During transport, the patient continues to deteriorate.

The medic administers 0.5 mg of narcan and a 500mL bolus of normal saline with no response. He radios ahead to let the hospital know that they’re en route. Now questioning the original diagnosis of drug overdose, he reports the medications he found on the scene in hopes it will help the receiving physician determine the cause of the patient’s condition.

Arrival at the ED
On arrival to the ED, the medic hands over the loaded syringe containing 2mL of unidentified solution, as well as the empty vial of Solu-Cortef and the bottles of dexamethosone and fludrocortisones.

As the crew arrives at the hospital, the ED physician meets the crew and informs them that he’s familiar with the medications. He says they're all used for people who have various forms of adrenal insufficiency (AI). The symptoms seen in this patient coincide with life-threatening adrenal crisis. The physician administers 100 mg of Solu-Cortef via IV and within minutes, the patient rouses. In 30 minutes, she can explain what happened in the desperate moments before her crisis.

Adrenal Insufficiency
Adrenal Insufficiency (AI) is a life-threatening in which the body is unable to produce enough cortisol to sustain life. In other words, their adrenal cortex is “asleep.” People suffering from AI take daily cortisol/glucocorticoid steroid replacement because whatever adrenal function is depleted. These patients are glucocorticoid dependent. In times of injury, dehydration, illness or surgery, they require an injection of Solu-Cortef. Solu-Cortef contains both glucocorticoid and mineralocorticoid properties, helping the body to compensate during a stress event.

The adrenal medulla (inside of the adrenal gland) secretes epinephrine and norepinephrine. The adrenal cortex (outer layer of the adrenal gland) secretes cortisol and aldosterone. Cortisol, a glucocorticoid, is often called the “stress” hormone. One of cortisol's functions is elevating blood glucose levels in times of stress. It also functions as a mediator for several inflammatory pathways.

Absence of cortisol can result in hypotension, hypoglycemia and death. Aldosterone, a mineralocorticoid, is responsible for the regulation of sodium and water. Absence of aldosterone can result in hypotension and electrolyte imbalance. AI in the prehospital setting may be difficult to recognize in the absence of a good history, including medications, to point providers in the cause of the problem. Two life-threatening conditions associated with AI include hypotension and hypoglycemia.

If not managed, these two conditions are life threatening. Prehospital treatment should include management of the patient’s airway, vascular access and fluid resuscitation. If blood glucose levels are low, the patient should receive dextrose per local protocol. It’s important to complete a thorough physical assessment and obtain a complete patient history before treating patients with this condition. Providers may confuse patients having an adrenal crisis with drug overdose patients because of their similar symptoms. Although AI is rare, it should still be considered as a potential diagnosis.

Authors’ note: Parts of the above case are taken from a true story. However, the difference is that there was no syringe on the floor, no vial under the chair and no one found the medications. The patient was treated with charcoal and diagnosed as a drug-overdose patient. She likely would have died, but her mother charged into the ED and expressed the need for Solu-Cortef. Security was called, but luckily someone listened, researched and called the patient’s treating physician. The patient was treated and released. 

From Journal of Emergency Medical Services

Stand Up and Be Counted!

For all Cushies, diagnosed or not, friends and family - add your name  and whatever info you want to share to this map.  The directions are similar to those for AI, below.

Cushing's MemberMap

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If you have Adrenal Insufficiency, a friend of mine from Adrenal Insufficiency United has a similar map.  Please add your info to her map, as well.

Adrenal Insufficiency Map

Directions for the AI map:

LET’S FIND EACH OTHER! (please read the instructions)

Use ONE MARKER PER PERSON WITH AI (to ensure an accurate representation do not add yourself as a parent or family member) However, If you have lost a loved one to AI, feel free to add them just indicate it in the description box.

After navigating to the map

CLICK “ADD” on top right of map

ENTRY NAME: Initials, first name, City...any of these are fine..or just leave it blank and it will say anonymous

LOCATION: if you don’t want your address known just “click on a map location”

I put my daughter in a park near our home :o) zoom in or out to find your location.

DESCRIPTION: Age of affected, or any other description you’d like

PHOTO: not required

MARKER Addison’s is the default so make sure you look at the whole list. If you have more than one condition which causes your or your childs’ AI, just pick the one you feel is the most relevant and then feel free to add the others in the Description box.

Please do not use ADRENAL INSUFFICIENCY UNITED’S marker. Right now it’s for our main office, but we’ll add more locations/contacts in the future.

CLICK SUBMIT

After you submit, write down the url link for future edits. Then click your marker, and then again click the BLUE initials, city, or anonymous...this will open up a more detailed window to add DOB and Diagnosis info plus anything else you like. Just remember if you put any personal email it will be able to be viewed by anyone.

If you mess up don’t fret, just contact me, as the admin of the map I can fix your entry.

 

Adrenal Crisis

This is a "knol" that I wrote in 2008.  It was accepted by the Open Journal of Medicine.  

Now that "knols" are being discontinued, the Open Journal moved this to their site but all of the images were lost in the move.  Also, the name of one of their authors was added to my knol.  

I'm posting this here to keep the information as it was, not as it has morphed into.

Addison’s_Disease.pdf Download this file

 

What is the best long-term management strategy for patients with primary adrenal insufficiency?

Authors: Quinkler, Marcus¹; Hahner, Stefanie²

Source: Clinical Endocrinology, Volume 76, Number 1, 1 January 2012 , pp. 21-25(5)

Publisher: Wiley-Blackwell

Abstract Summary

Primary adrenal insufficiency is treated with glucocorticoid and mineralocorticoid replacement therapy. Recent data revealed that health-related quality of life in adrenal insufficiency is impaired in many patients and that patients with adrenal insufficiency are also threatened by an increased mortality and morbidity. This may be caused by inadequate glucocortiocid therapy and adrenal crisis. Therefore, the optimization of hormone replacement therapy remains one of the most challenging tasks in endocrinology because it is largely based on clinical grounds because of the lack of objective assessment tools.

This article provides answers to the important daily clinical questions, such as correct dose finding, dose adaptation in special situations, e g, pregnancy, improvement of quality of life and measures for protection from adrenal crisis. Other important aspects discussed are side effects of glucocortiocid replacement therapy and interactions with other drugs.

Document Type: Research article

DOI: http://dx.doi.org/10.1111/j.1365-2265.2011.04103.x

Affiliations: 1: Clinical Endocrinology, Charité Campus Mitte, Charité University Medicine Berlin, Berlin 2: Department of Internal Medicine I, University Hospital, University of Wuerzburg, Wuerzburg, Germany

Publication date: 2012-01-01

From http://www.ingentaconnect.com/content/bsc/cend/2012/00000076/00000001/art00005

(Addison's Disease) Coma man defies odds to write book

By Lisa Smyth

 

A Northern Ireland man has defied medical predictions and overcome the effects of a rare condition that left him in a coma for three months.

Jonathan Fisher is a survivor of an Addisonian crisis which affected him so badly doctors feared he would never recover.

His mother ignored medical advice to switch off his life-support system as doctors believed he was brain dead.

However, convinced he could recover after noticing that he was reacting to her with the occasional slight movement of his little finger she refused to give up on her son.

Now — against all the odds — he has made a remarkable recovery and has regained many of the skills lost as a result of his condition.

Addison’s disease is a rare disorder of the adrenal glands. It affects the production of two hormones — cortisol and aldosterone — which help to regulate blood pressure.

If left untreated, the amount of steroid hormones in the body will gradually fall and the symptoms of Addison’s disease will get progressively worse.

Eventually, this will cause an adrenal crisis — when the symptoms become very severe and blood pressure drops to a dangerously low level. An adrenal crisis can be fatal if it is not treated immediately.

Jonathan can now get around Lisburn in his electric wheelchair and has even managed to complete his first book, August Always — a triumph as he has considerable speech impairment and great difficulty using his hands to operate a keyboard.

The process has been long and laborious but Jonathan said he was determined to share his experience with others.

“There are moments in life that define us, like birth and death,” he said.

“Along the journey there will be crises of passion, of love, of faith and desire, but none so devastating as an Addisonian crisis. I am Jonathan Fisher, a survivor.

“August Always is my memoir. I believe in the incredible. I dream of a better future.”

Background

Addison’s disease is a rare disorder of the adrenal glands which are located on top of the kidneys. The condition affects the production of two hormones. Cortisol, which helps to regulate blood pressure, maintaining blood glucose and heart function. And aldosterone which also helps regulate blood pressure. Addison’s sufferers must get treatment if their blood pressure falls as it can be fatal.

From http://www.belfasttelegraph.co.uk/news/health/coma-man-defies-odds-to-write-book-16076458.html

DuoCort Pharma's Orphan Drug Plenadren® Granted European Marketing Authorization for Adrenal Insufficiency

HELSINGBORG, Sweden and EXTON, Pa., Nov. 7, 2011 /PRNewswire/ -- The Swedish specialty pharma company, DuoCort Pharma, announced today that the European Commission has granted a European Marketing Authorisation for Plenadren®(hydrocortisone, modified release tablet), an orphan drug for treatment of adrenal insufficiency in adults,  bringing these patients their first pharmaceutical innovation in over 50 years.

Developed by DuoCort Pharma, Plenadren® is a dual release hydrocortisone replacement therapy designed to better mimic the normal physiological cortisol profile in order to improve outcomes for patients suffering from adrenal insufficiency. Plenadren® is given as an oral tablet once daily.  It has an outer layer releasing hydrocortisone immediately and an inner core releasing the rest of the drug more slowly during the day.

Although glucocorticoid hormone replacement therapy for adrenal insufficiency has been available for decades, studies have recorded complications and comorbidities including premature death, impaired quality of life, increased risk of cardiovascular diseases, and decreased bone mineral density in treated patients, most likely because it is difficult to match the natural secretion pattern of cortisol.  

Maria Forss, CEO of DuoCort Pharma, said: "The marketing authorization for Plenadren® in Europe is an important step towards addressing the unmet needs of these patients."    

The approval of Plenadren® follows the positive opinion adopted by the Committee for Medicinal Products for Human Use (CHMP) in July 2011. Plenadren® is now approved for marketing in all countries of the European Union (EU) as well as in the European Economic Area (EEA), namely Iceland, Norway and Lichtenstein.  

Professor Gudmundur Johannsson of the Department of Endocrinology, Sahlgrenska University Hospital, Gothenburg, Sweden, and Chief Medical Officer of DuoCort Pharma, said: "Plenadren® offers a welcome new treatment option to help patients suffering from adrenal insufficiency. Plenadren® can improve therapy for many of the almost 200,000 patients in Europe who suffer from this disease and who need life-long cortisol replacement therapy for their survival."

On October 26, 2011, ViroPharma Incorporated (NASDAQ: VPHM) signed a definitive agreement to acquire DuoCort Pharma AB.  The companies expect to complete the acquisition in November 2011. On closing, ViroPharma will pay an upfront closing cost of 220 million Swedish kroner (SEK) or $33 million in US dollars (USD).  Additionally, there are contingent milestone payments of up to 860 million SEK or $130 million USD associated with manufacturing, sales thresholds and territory expansion.  

About Adrenal insufficiency

Adrenal insufficiency (cortisol deficiency) is a rare, life-threatening disease that affects patients in their active years. To survive, patients suffering from this disease need lifelong replacement therapy with hydrocortisone. Treatment of adrenal insufficiency involves replacing, or substituting, the hormones that the patient's own adrenal glands are not producing. Cortisol is replaced using hydrocortisone, the synthetic form of cortisol.

About Plenadren® (hydrocortisone, modified release tablet)

Plenadren is the first true innovation in over 50 years in the treatment of adrenal insufficiency.

Hypersensitivity to the active substance of Plenadren or to any of the excipients may occur.  During acute adrenal insufficiency, parenteral administration of hydrocortisone in high doses, together with physiological sodium chloride solution for injection, must be given.  Use of Plenadren with potent CYP 3A4 inducers and inhibitors may merit an adjustment of hydrocortisone dosage.  High (supra-physiological) dosages of cortisone can cause elevation of blood pressure, salt and water retention, and increased excretion of potassium.  Long-term treatment with higher than physiological hydrocortisone doses can lead to clinical features resembling Cushing's syndrome with increased adiposity, abdominal obesity, hypertension and diabetes, and thus result in an increased risk of cardiovascular morbidity and mortality. All glucocorticoids increase calcium excretion and reduce the bone remodeling rate.  Patients with adrenal insufficiency on long term glucocorticoid replacement therapy have been found to have reduced bone mineral density.  Psychiatric adverse events may occur with systemic glucocorticoids.

The most common adverse reactions observed in clinical studies have been fatigue, gastroenteritis, upper respiratory tract infection, sedation, vertigo and dry eyes. 

About ViroPharma Incorporated

ViroPharma Incorporated is an international biopharmaceutical company committed to developing and commercializing novel solutions for physician specialists to address unmet medical needs of patients living with diseases that have few if any clinical therapeutic options, including C1 esterase inhibitor deficiency, treatment of seizures in children and adolescents, and C. difficile infection (CDI).  Our goal is to provide rewarding careers to employees, to create new standards of care in the way serious diseases are treated, and to build international partnerships with the patients, advocates, and health care professionals we serve.  ViroPharma's commercial products address diseases including hereditary angioedema (HAE), seizures in children and adolescents, and CDI; for full U.S. prescribing information on our products, please download the package inserts athttp://www.viropharma.com/Products.aspx; the prescribing information for other countries can be found atwww.viropharma.com. 

ViroPharma routinely posts information, including press releases, which may be important to investors in the investor relations and media sections of our company's web site, www.viropharma.com. The company encourages investors to consult these sections for more information on ViroPharma and our business.

About DuoCort Pharma

DuoCort Pharma is a drug development company focused on improving glucocorticoid therapy. The company has its origins among researchers at the Sahlgrenska Academy at Gothenburg University  and at Uppsala University in Sweden. DuoCort Pharma has developed Plenadren®, an improved glucocorticoid replacement therapy for patients with adrenal insufficiency, which is a rare disease. DuoCort Pharma has orphan drug designations in EU, Switzerland and the USA for Plenadren®. Plenadren® is a once daily, dual-release hydrocortisone oral tablet. It has an outer layer that releases the drug immediately and an inner core that releases the drug over the day. The tablets come in both 5 mg and 20 mg strengths. For more information please visit www.duocort.com.

DuoCort Pharma is a project company of the life science incubator PULS. For more information visit www.pulsinvest.se.  

Disclosure Notice

Certain statements in this press release contain forward-looking statements that involve a number of risks and uncertainties. Forward-looking statements provide our current expectations or forecasts of future events, including statements about the benefits of the business combination transaction involving ViroPharma and DuoCort Pharma, including, among others, future financial and operating results, enhanced revenues, ViroPharma's plans, objectives, expectations and intentions and other statements that are not historical facts. The following factors, among others, could cause actual results to differ from those set forth in the forward-looking statements: the ability to achieve the other conditions to closing on the proposed schedule; the risk that the business will not be integrated successfully; the risk that revenues following the acquisition will be lower than expected, including the successful commercialization of Plenadren; potential for disruption from the transaction making it more difficult to maintain relationships with manufacturers, employees or other suppliers; competition and its effect on pricing, spending, third-party relationships and revenues; our ability to achieve favorable pricing for Plenadren from European regulatory authorities; the risk that the safety and/or efficacy results of existing clinical trials for Plenadren will not be consistent with the results of additional clinical studies, including the required registry study, or with commercial usage; market acceptance of Plenadren; and our inability to maintain the orphan drug status associated with Plenadren. These factors, and other factors, including, but not limited to those described in our annual report on Form 10-K for the year ended December 31, 2010 and quarterly reports on Form 10-Q filed with the Securities and Exchange Commission, could cause future results to differ materially from the expectations expressed in this press release. The forward-looking statements contained in this press release are made as of the date hereof and may become outdated over time. ViroPharma does not assume any responsibility for updating any forward-looking statements. These forward looking statements should not be relied upon as representing our assessments as of any date subsequent to the date of this press release.

 

SOURCE DuoCort Pharma

From http://www.prnewswire.com/news-releases/duocort-pharmas-orphan-drug-plenadren-granted-european-marketing-authorization-for-adrenal-insufficiency-133348658.html

Addison's Blog Alerts ~ February 2, 2011

Addison's Disease (Primary Adrenal Insufficiency) | NurseXchange
By isl30fvi3w
Addison's Disease is the hyposecretion of adrenocortical hormones. Addisonian Crisis - can be precipitated by stress, fatal if not treated. SIGNS AND.
NurseXchange - http://nursexchange.com/


Anyone else have Addison's Disease? – 4HealthAnswers.com
By michele
Anyone else have Addison's Disease? Asked By: michele; Category: Addison's Disease. Answer this Question : You must be logged in to post an answer. Signup Here, it takes 5 seconds :). Other Questions ...
4HealthAnswers.com - http://4healthanswers.com/

Symptoms of Addison’s disease

How to tell if your patient is having an acute crisis.

Fred W. Wurster III, AAS, NREMT-P | From the January 2011 Issue and Evolution in Resuscitation Issue

It’s 8:30 on a Tuesday morning, and your crew is responding to an unresponsive 18-year-old male patient. You and your partner are well-seasoned paramedics who have responded to thousands of calls in your careers. Your mobile data computer (MDC) provides further information from the 9-1-1-center that states, “patient is a known diabetic and the patient’s father is attempting to administer oral glucose.”

On arrival, you’re greeted by the patient’s stepmother. She says, “I think it’s his blood sugar.” You’re directed to the third floor of an old farmhouse. After climbing two sets of stairs, you find a male patient partially clothed, lying on the floor and moaning.

As you approach, his father states, “I tried to give him his oral glucose, but I don’t know how much I got him.”

The patient’s room is disheveled with no obvious threats to EMS, and it appears as though the patient may have been out of control or trashing around prior to your arrival. He continues to moan incomprehensibly.

When approached, he moans louder and becomes agitated. You also note that he’s partially covered in feces. After numerous attempts of trying to perform an assessment, the patient becomes more agitated, and you request assistance from the police department.

Patient Assessment
Although no immediate threats are noted, you maintain a safe distance from the patient until the police arrive. His father is able to calm him, so you can make contact. However, the patient still doesn’t communicate with you when questioned during your assessment, and he continues to moan.

The patient can be non-forcefully rolled over onto his back, and you’re able to obtain a baseline set of vital signs, including blood glucose level. The initial set of vital signs are as follows: heart rate is 86, blood pressure is 88/60, respiratory rate is 16, and non-labored, blood glucose level is 113 mg/dL.

His skin is warm, slightly pale and dry, and you note no visible trauma, bleeding or other abnormalities. While questioning the father further, he reports that his son may have overdosed on something because he found a blue liquid-like substance in a coffee cup next to the patient’s bed. The father says he’s unaware of the patient having any history of alcohol or substance abuse, although he believes the patient may occasionally use marijuana and does smoke cigarettes.

On further assessment, you notice a tattoo on the patient’s left forearm. In addition to the patient’s name, the tattoo reads “insulin dependent diabetic, Addison’s disease, no allergies.”

The crew comments about the tattoo having his medical information, and the father reports that because the patient has had many problems with his diabetes while out in public, he feels it was appropriate that the information be tattooed on his son’s arm.

Transport
The patient again becomes agitated and aggressive, attempting to bite your partner. With the assistance of the police department, he’s secured in a Reeves stretcher, and you begin to carry him down the stairs. He continues to thrash around, almost uncontrollably, and he almost slides out of the stretcher several times.

Your protocols don’t allow the use of Ativan without verbal orders from a physician, so you make contact with the medical command physician at the hospital to obtain orders to administer it in an attempt to chemically subdue the patient. A report is relayed to the medical command physician, and he authorizes your request to administer 1 milligram of Ativan and orders an additional 1 milligram if needed.

The patient continues to be uncontrollable, and you have two failed attempts at IV access because of the patient’s thrashing around.

You then attempt to administer the Ativan intranasally, and the patient fights you. After several attempts, you decide to switch to the intramuscular route and are successful. After a minute or so, the patient calms down and is resting on the stretcher. At the patient’s father’s request, you transport the patient to a hospital that isn’t the closest.

Prior to departure, the patient’s father says he’s only seen his son act like this one other time he can remember, which was when he was diagnosed with Addison’s. During transport, your continued assessment reveals no major trauma, other than some abrasions on the patient’s shoulders and knees. You also note that he’s extremely thin, but he appears to be healthy and has a bronze hue to his skin.

The patient remains non-verbal and doesn’t respond appropriately when questioned. He appears to drift in and out of consciousness, but he maintains a patent airway.

The patients’ vital signs are reassessed with no changes noted, and an ECG is obtained with no abnormalities or ectopy present. The rhythm is sinus tachycardia at a rate of 118 beats per minute. You repeat a blood glucose test, which reveals a reading of 126mg/dL.

You’re able to successfully establish IV access with an 18-gauge needle and administer a 250 mL bolus of normal saline solution followed by titration to KVO. You also attempt to administer oxygen via numerous devices, but he fights off all efforts.

Arrival
On arrival at the emergency department (ED), you ask your partner what he knows about Addison’s disease. Neither of you knows anything more than it causes renal insufficiency and that most people who have Addison’s also suffer from insulin-dependent diabetes. Your differential diagnosis is still leading to you think this patient may be suffering from some type of overdose or adverse reaction to an unknown substance.

You transfer the patient to the awaiting nursing staff and attending ED physician. After reporting your findings, assessment, scene and patient presentation, while you’re cleaning up your equipment, the ED physician comes over and asks whether you know that you saved the patient’s life. You and your partner look at each other
in amazement.

You ask him what’s wrong with the patient, and he replies that it’s an acute Addisonian crisis, explaining that the patient was at a critical level and could have potentially suffered irreversible effects. He said it would be several hours before they knew the outcome and that says he’s only seen one case like this in his 17 years of practicing medicine.

You wish the family well and clear the hospital. As you return to the station, you and your partner discuss the dynamics of the call.

Although you are both seasoned paramedics with lots of experience, you agree that you’ve never seen anything like this.

Addison’s Disease
After returning to the station, you contact your medical director and ask for some insight into the disease, its process and how to treat and recognize it should you cross paths with it again. He tells you that Addison’s disease is a rare disorder of the endocrine system caused by a lack of the steroid hormones that are produced by the adrenal glands.

Treatment is exactly what you did: monitor the patient, keep them safe and rapidly transport them to a hospital for definitive care. The disease initially presents subtly and without any real prodrome or awareness until the majority of symptoms present.

The most common symptoms include fatigue, lightheadedness, headache, muscle weakness and pain, fever, weight loss, nausea, vomiting, diarrhea, excessive sweating, sudden changes in mood and personality, excessive craving for salty foods and a bronzed or tanned skin color. Large populations of people with Addison’s disease also suffer from Type I diabetes and require insulin injections.

An acute Addisonian crisis presents when someone previously diagnosed with Addison’s suffers from an infection or traumatic injury or if they suddenly stop taking their glucocorticoids. This may also be how the onset of Addison’s is diagnosed for the first time in some patients.

Symptoms of an acute crisis include severe vomiting and diarrhea resulting in dehydration, hypotension, syncopal episodes leading to unconsciousness, confusion, psychosis, slurred speech, agitation, combativeness, hypoglycemia and seizures.

If untreated or improperly treated, an acute Addisonian crisis can be fatal. These patients require prehospital administration of steroids, such as Hydrocortisone, Prednisone, or Solu-Cortef to replace the missing cortisol.

When you identify someone with a past history of Addison’s who is presenting as this patient did, it’s paramount to protect the patient and rapidly transport them to a hospital for treatment. Remembering the signs and symptoms of Addison’s may make the difference between life and death for these patients especially when they’re in an acute crisis state.

This patient was admitted to the local hospital, was subsequently transferred to a pediatric specialty hospital and was discharged after two weeks.

He had been complaining of cold and flu-like symptoms for a few days prior to our encounter, which may have triggered the acute crisis. JEMS

This article originally appeared in January 2011 JEMS as
“Crisis Averted: Recognize the symptoms of Addison’s disease before they escalate.”

From http://www.jems.com/article/addison-s-disease/do-you-think-i-should-send-my

Adrenal Crisis Tips

From Ellen, on the Cushing's Help Message Boards

I have a very good friend who has had more adrenal crises than anyone care to count (more than 20). She has tried hard to teach me some important things for the day I should ever have a crisis. Among them is the reality that (as before surgery) success of your care depends on YOU getting everything prepared for the worst as best you can. We can no more depend on the ER staff than any other doc out there who isn't a specialist in pituitary medicine. You all have already done much of preparing by having your medic alert bracelets on, your injectable Cortef (bring it with you in case they don't have it there) and your letter from Dr. F. But that isn't enough much of the time as you have painfully discovered.

1) Prevention is the key. You, Mary, are SO overdoing it, I don't know what to say. You shouldn't even be leaving your house right now, let alone taking on the care of small children. You need a good talking to, missy. Perhaps you can choose to do ONE easy task a day but overall-you should be bored out of your gourd sitting on your tuckus. The more you do, the more you risk events like this. The hardest part is understanding that recovery is not a linear improvement every day. You are going to have weeks or maybe months where you can do no more than you did the first week after surgery. This recovery takes a long time when surgery works. Each tiny task you accomplish depletes you in an additive way. It might not have seemed much at the time to unload the dishwasher but you better believe it counts when you add in each additional task you want to accomplish.

2) Everything is additive. It isn't just what you did today but also what you did the last three, four or five days. You may have felt good the first day but each successive day my guess is you could feel your body pushing a bit. I find I say things like, "If I could just get this ONE more job done, then I will rest" before I am off to the next job. Before I know it, it is too late. Think hard about the twinges you feel the days before this happened this time around, when you were tired. How do you feel in the evenings after a day of activity? Those are the signs to look for and treat early the next time. You are having to listen to your body in a whole new way. Learn your earliest signs.

3) Take more Cortef when you first get those twinges above--the days before a crisis might strike.

4) Everyone in your household needs to be trained to give you Cortef. Teach them that confusion on your part indicates a crisis coming on-if you aren't making sense they need to understand that YOU are not able to help yourself. In most cases the oral cortef will keep you out of the ER if someone else makes sure you take it. Don't hesitate, don't let yourself talk them out of helping you-just take it-it is better to err high than low with your history of crises right now.

5) Knowing that in spite of all of this you need ER care potentially, consider calling the liason in person and talking real-time about your needs for future visits-explaining how quickly things become life threatening. They need to have something about your history in the computer already-a copy of that letter from Dr. F plus their own notes that it is on the up and up along with the note to please page your doctor. Give them a recipe to follow that they have pre-approved and it will help greatly. Most ER doctors will never see an adrenal crisis patient in their ER. Doctors have limits on their abilities just as everyone else does. I can read English really well but if you handed me a book written by someone in 1610, I would likely take longer to get through it because I am not as familiar with the format...the 'wherefore art thous' are English but they sure slow you down. That is what happens in the ER to Addisonians...doctors eventually get there but it takes longer because it is unfamiliar. Help them out by giving them the Cliff-Notes before you ever get there.

5A) Also insist that for now they order and keep Solu-Cortef on the shelves for you. Many (most?) hospitals do NOT have it stocked, as my friend discovered over and over again when she went to the ER. It took hours for them to track some down and give it to her. In the meantime, she was getting sicker and sicker. She finally asked the liason to help be certain they had it for her, ready to go. Now, they have it ready for her, they know her and they know what to do. She is often out of there in about 3 hours.

6) If you are vomiting/collapsed clearly in serious trouble, by the time you head off to the ER, call 911/ambulance so you get taken in and cared for without waiting. Your life is at risk by that stage and you need immediate care. It is justified and potentially life-saving.

7) Always have a trained advocate with you, have several back ups in your life. My friend has her husband but she also has me ready to go-have the hospital list several people to call ahead of time in case you arrive on your own and they can't reach your primary person. Your advocates need to know what to do independently of you. They need to know what to say and how to push the ER staff to get things done on your behalf. We, as patients struggling with Cushing's, are used to having to push but most people are very intimidated by medical personnel and often hang back, figuring they must know what to do. Make sure they understand this just isn't the case sometimes and that your life may depend on what they say or get the staff to do. It is critical they contact your endocrinologist-have your advocate INSIST they do this. If they won't, have your advocate page the doctor for you. Be sure you go over a plan with your advocate periodically or answer their questions about what may happen. I went over much of my plan with my husband prior to my surgery but discovered that within days of surgery, he had forgotten most of what I said. He really wanted to help but just hadn't taken in the medical stuff because it was overwhelming and scary. Keep going over it until they are comfortable.

I really hope these are the last ER visits for you all. I have agonized each time my friend goes into another crisis. I know that in spite of everything you do (or don't do) to prevent a crisis, they still happen. Hopefully the next time around, everything will be in place for you all to have a smooth experience.

So, sit down, turn on that television and get comfy girls. TAKE IT EASY!!

A Systematic Review and Meta-Analysis of Randomized Placebo-Controlled Trials of DHEA Treatment Effects on Quality of Life in Women with Adrenal Insufficiency

Aziz A. Alkatib, Mihaela Cosma, Mohamed B. Elamin, Dana Erickson, Brian A. Swiglo, Patricia J. Erwin, and Victor M. Montori*

Department of Medicine (A.A.A.), Knowledge and Encounter Research Unit (A.A.A., M.C., M.B.E., D.E., B.A.S., P.J.E., V.M.M.), Mayo Clinic Libraries (P.J.E.), and Division of Endocrinology, Diabetes, Metabolism, and Nutrition (M.C., D.E., B.A.S., V.M.M.), Mayo Clinic, Rochester, Minnesota 55905

* To whom correspondence should be addressed. E-mail: montori.victor@mayo.edu.

 

Context: Women with primary or secondary adrenal insufficiency report a decreased health-related quality of life (HRQOL) despite traditional adrenal replacement therapy. Dehydroepiandrosterone (DHEA) has been studied as an agent to improve HRQOL in these patients.

 

Objective: We sought to conduct a systematic review and meta-analysis of randomized controlled trials of DHEA effects on HRQOL in women with adrenal insufficiency.

Data Sources: We searched electronic databases (MEDLINE, EMBASE, Cochrane CENTRAL, Web of Science, CINAHL, and PsycInfo) and reference lists of eligible studies through July 2008.

 

Study Selection: Eligible trials randomly assigned women with primary or secondary adrenal insufficiency to either DHEA or control and measured the effect of treatment on HRQOL.

 

Data Extraction: Reviewers working independently and in duplicate assessed the methodological quality of trials and collected data on patient characteristics, interventions, and outcomes.

 

Data Synthesis: We found 10 eligible trials that measured HRQOL and depression, anxiety, and sexual function. Random-effects meta-analysis showed a small improvement in HRQOL in women treated with DHEA compared with placebo [effect size of 0.21; 95% confidence interval, 0.08 to 0.33; inconsistency (I2) = 32%]. There was a small beneficial effect of DHEA on depression; effects on anxiety and sexual well-being were also small and not statistically significant.

 

Conclusions: DHEA may improve, in a small and perhaps trivial manner, HRQOL and depression in women with adrenal insufficiency. There was no significant effect of DHEA on anxiety and sexual well-being. The evidence appears insufficient to support the routine use of DHEA in women with adrenal insufficiency.

 

From http://jcem.endojournals.org/cgi/content/abstract/jc.2009-0672v1

Adrenal Crisis

A Bhattacharyya, J Macdonald, AA LAkhdar.

The adrenal cortex normally produces three principal steroid hormones: the glucocorticoid cortisol, the mineralocorticoid adosterone, and a small quantity of sex steroids. In primary adrenocortical insufficiency, there is a deficiency of both cortisol and aldosterone with characteristic clinical and laboratory findings.

 

In contrast, with a pituitary disorder there is isolated hypocortisolism, because its production is dependant on pituitary adrenocortrophic hormone (ACTH), whereas aldosterone production is controlled by extracellular fluid volume, rennin and serum potassium. Acute adrenocortical crisis is an absolute medical emergency and its presentation is not always typical.

 

We describe three recent cases of acute adrenocortical crisis in our hospital who presented in three different ways in three different wards.

 

International Journal of clinical Practice 2001;55:141-4.

 

From http://www.diabetesendocrinology.in/2010/11/06/adrenal-crisis/

About the Adrenal Glands

Adapted from cushings-help.com and The Merck Manual

Adrenal Gland Disorders

The body has two adrenal glands, one near the top of each kidney. The inner part (medulla) of the adrenal glands secretes hormones such as adrenaline (epinephrine) that affect blood pressure, heart rate, sweating, and other activities also regulated by the sympathetic nervous system. The outer part (cortex) secretes many different hormones, including corticosteroids (cortisone-like hormones), androgens (male hormones), and mineralocorticoids, which control blood pressure and the levels of salt and potassium in the body.

The adrenal glands are part of a complex system that produces interacting hormones. The hypothalamus produces corticotropin-releasing hormone, triggering the pituitary gland to secrete corticotropin, which regulates the production of corticosteroids by the adrenal glands. Adrenal glands may stop functioning when either the pituitary or hypothalamus fails to produce sufficient amounts of the appropriate hormones. Underproduction or overproduction of any adrenal hormones can lead to serious illness.

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Underactive Adrenal Glands

Addison's disease (adrenocortical insufficiency) results when underactive adrenal glands produce insufficient amounts of corticosteroids.

 

Addison's disease affects about 4 out of every 100,000 people. The disease can strike at any age and affects males and females about equally. In 30 percent of people with Addison's disease, the adrenal glands are destroyed by a cancer, amyloidosis, an infection such as tuberculosis, or another identifiable disease. In the other 70 percent, the cause isn't known for certain, but scientists strongly suspect the adrenal glands are destroyed by an autoimmune reaction.

 

The adrenal glands are also suppressed in people who take corticosteroids such as prednisone. Ordinarily, the dose of corticosteroids is tapered slowly before the drug is stopped completely. When corticosteroids are stopped suddenly after being taken for a month or more, the adrenal glands may be unable to produce corticosteroids in sufficient amounts for several weeks or even months, depending on the dose of corticosteroids and the duration of treatment. Certain other drugs, such as ketoconazole taken to treat fungal infections, can also block the natural production of corticosteroids, resulting in a deficiency.

Corticosteroid deficiency can lead to many problems. For example, when corticosteroids are lacking, the body excretes large amounts of sodium and retains potassium, leading to low levels of sodium and high levels of potassium in the blood. The kidneys aren't able to concentrate urine, so when a person with a corticosteroid deficiency drinks too much water or loses too much sodium, the blood level of sodium falls. Inability to concentrate urine ultimately causes the person to urinate excessively and become dehydrated. Severe dehydration and a low sodium level reduce blood volume and can culminate in shock.

corticosteroid deficiency also leads to an extreme sensitivity to insulin, a hormone normally present in the blood, so that the blood sugar levels may fall dangerously low. The deficiency prevents the body from manufacturing carbohydrates from protein, fighting infections, or healing wounds very well. Muscles weaken, and even the heart can become weak and unable to pump blood adequately.

 

To compensate for a deficiency of corticosteroids, the pituitary gland produces more corticotropin, the hormone that normally stimulates the adrenal glands. Since corticotropin also affects melanin production, people with Addison's disease often develop a dark pigmentation of the skin and the lining of the mouth. The excessive pigmentation usually occurs in patches. Even people with dark skin can develop excessive pigmentation, although the change may be hard to recognize. Excessive pigmentation doesn't occur when adrenal insufficiency is caused by pituitary or hypothalamus insufficiency, conditions in which the basic problem is a deficiency of corticotropin.

Symptoms

Soon after developing Addison's disease, a person feels weak, tired, and dizzy when standing up after sitting or lying down. The skin becomes dark; this darkness may seem like tanning, but it appears on both sun-exposed and nonexposed areas. Black freckles may develop over the forehead, face, and shoulders; a bluish-black discoloration may develop around the nipples, lips, mouth, rectum, scrotum, or vagina. Most people lose weight, become dehydrated, have no appetite, and develop muscle aches, nausea, vomiting, and diarrhea. Many become unable to tolerate cold. Unless the disease is severe, symptoms tend to become apparent only during times of stress.

 

If the disease isn't treated, severe abdominal pains, profound weakness, extremely low blood pressure, kidney failure, and shock may occur, especially if the body is subjected to stress such as an injury, surgery, or severe infection. Death may quickly follow.

Diagnosis

Because the symptoms may start slowly and be subtle, and because no single laboratory test is definitive, doctors often don't suspect Addison's disease at the outset. Sometimes a major stress, such as an accident, operation, or serious illness, makes the symptoms more obvious and precipitates a crisis.

 

Blood tests may show a lack of corticosteroids, especially cortisol, as well as low sodium and high potassium levels. Measures of kidney function, such as tests for blood urea nitrogen and creatinine, usually indicate that the kidneys aren't working well. Corticosteroid levels, usually tested after an injection of corticotropin (a challenge test), can help the doctor distinguish adrenal gland insufficiency from pituitary gland insufficiency. When it is the latter, an injection of corticotropin-releasing hormone reveals whether the cause of the problem is hypothalamus insufficiency.

Treatment

Regardless of the cause, Addison's disease can be life-threatening and must be treated first with corticosteroids. Usually treatment can be started with prednisone taken orally. However, people who are severely ill may be given cortisol intravenously at first and then prednisone tablets. Most people with Addison's disease also need to take 1 or 2 tablets of fludrocortisone every day to help restore the body's normal excretion of sodium and potassium. Fludrocortisone can eventually be reduced or discontinued in some people; however, they will need to take prednisone every day for the rest of their lives. Larger doses of prednisone may be needed when the body is stressed, especially from an illness. Although treatment must be continued for life, the outlook for a normal life span is excellent.

---

Overactive Adrenal Glands

The adrenal glands can produce too much of one or more hormones. Changes in the adrenal glands themselves or overstimulation by the pituitary gland may be the cause. The symptoms and treatment depend on which hormones--androgenic steroids, corticosteroids, or aldosterone--are being overproduced.

Overproduction of Androgenic Steroids

Overproduction of androgenic steroids (testosterone and similar hormones) is a condition that leads to virilization, the development of exaggerated masculine characteristics in either men or women.

 

Mild overproduction of androgens is common but may lead only to increased hair growth (hirsutism). True virilizing disease is rare, affecting only about 1 or 2 of every 100,000 women. The incidence of virilizing disease in men is almost impossible to guess.

Symptoms

Signs of virilization include hairiness of the face and body, baldness, acne, deepening of the voice, and increased muscularity. In women, the uterus shrinks, the clitoris enlarges, the breasts become smaller, and normal menstruation stops. Both men and women may experience an increased sex drive.

Diagnosis

The combination of body changes makes virilization relatively easy for a doctor to recognize. A test can determine the level of androgenic steroids in the urine. If the level is high, the dexamethasone suppression test can help determine whether the problem is a cancer, a noncancerous tumor (adenoma), or an enlargement of the hormone-producing portions of the adrenal cortex (adrenal hyperplasia). With this test, the corticosteroid dexamethasone is given orally. If the problem is adrenal hyperplasia, dexamethasone prevents the adrenal glands from producing androgenic steroids. If the problem is an adenoma or cancer of the adrenal glands, dexamethasone reduces androgenic steroid production only partially or not at all. The doctor may also order a computed tomography (CT) or magnetic resonance imaging (MRI) scan to obtain a view of the adrenal glands.

Treatment

Androgen-producing adenomas and adrenal cancers are usually treated by surgically removing the adrenal gland. For adrenal hyperplasia, small amounts of corticosteroids such as dexamethasone generally reduce the production of androgenic steroids, but these drugs may also cause symptoms of Cushing's syndrome if too large a dose is given.

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Overproduction of Corticosteroids

Overexposure to corticosteroids, whether from overproduction by the adrenal glands or from administration of excessive amounts by a doctor, results in Cushing's syndrome.

An abnormality in the pituitary gland, such as a tumor, can cause the pituitary to produce large amounts of corticotropin, the hormone that controls the adrenal glands. Pituitary tumors that overproduce corticotropin occur in about 6 in every 1 million people. Small-cell carcinoma in the lung and some other tumors outside the pituitary gland can produce corticotropin as well (a condition called ectopic corticotropin syndrome). This is the most common cause of excessive adrenal cortical function, found in at least 10 percent of people with small-cell carcinoma in the lung, a common type of tumor.

Sometimes the adrenal gland produces excessive corticosteroids even when corticotropin levels are low, usually when a benign tumor (adenoma) has developed in the adrenal gland. Benign tumors of the adrenal cortex are extremely common; half of all people have them by the age of 70. Only a small fraction of these benign tumors are active; the incidence of adenomas causing disease is about 2 in every 1 million people. Cancerous tumors of the adrenal cortex are equally common, but cancers causing endocrine disease are quite rare.

Symptoms

Because corticosteroids alter the amount and distribution of body fat, a person with Cushing's syndrome usually has a large, round face (moon face). Excessive fat develops throughout the torso and may be particularly noticeable at the top of the back (buffalo hump). Fingers, hands, and feet are usually slender in proportion to the thickened trunk. Muscles lose their bulk, leading to weakness. The skin becomes thin, bruises easily, and heals poorly when bruised or cut. Purple streaks that look like stretch marks may develop over the abdomen.

 

High corticosteroid levels over time raise the blood pressure, weaken bones (osteoporosis), and diminish resistance to infections. The risk of developing kidney stones and diabetes is increased, and mental disturbances, including depression and hallucinations, may occur. Women with Cushing's syndrome usually have an irregular menstrual cycle. Children who have the condition grow slowly and remain short. In some people, the adrenal glands also produce large amounts of androgenic steroids, leading to increased facial and body hair, balding, and an increased sex drive.

Diagnosis

Doctors who suspect Cushing's syndrome after observing the symptoms measure the blood level of cortisol, the main corticosteroid hormone. Normally, cortisol levels are high in the morning and decrease during the day. In people who have Cushing's syndrome, cortisol levels are very high in the morning and don't decrease late in the day as would be expected. Measuring cortisol in the urine can be useful because tests performed a few hours apart can indicate how much cortisol has been produced in that time.

 

If the cortisol levels are high, the doctor may recommend a dexamethasone suppression test. The test is based on the ability of dexamethasone to suppress the pituitary gland, thereby reducing adrenal gland stimulation. First a urine sample is tested for cortisol. Then dexamethasone is given, and cortisol levels are measured in another sample of urine. If the Cushing's syndrome is caused by pituitary stimulation, the level of cortisol will fall; if the Cushing's syndrome is caused by stimulation from a nonpituitary source of corticotropin or an adrenal tumor, the urinary cortisol level will remain high.

 

Results of a dexamethasone suppression test may not be clear-cut. Other laboratory tests may be needed to help determine the precise cause of the syndrome. These tests may be followed by a computed tomography (CT) or magnetic resonance imaging (MRI) scan of the pituitary or adrenal glands and by a chest x-ray or CT scan of the lungs.

Treatment

Treatment is directed at the pituitary or adrenal gland depending on the source of the problem. Surgery or radiation therapy may be needed to remove or destroy a pituitary tumor. Adenomas of the adrenal gland can often be removed surgically. Both adrenal glands may have to be removed if these treatments aren't effective or if no tumor is present. Any person who has had both adrenal glands removed, and many people who have had part of their adrenal glands removed, must take corticosteroids for life.

 

Some 5 to 10 percent of the people who have both adrenal adrenal glands removed develop Nelson's syndrome. In this condition, the pituitary gland enlarges, producing large amounts of corticotropin and other hormones such as beta-melanocyte-stimulating hormone, which darkens the skin. If necessary, Nelson's syndrome can be treated with radiation or surgical removal of the pituitary gland.

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Overproduction of Aldosterone

Overproduction of aldosterone (hyperaldosteronism) by the adrenal glands is a condition that affects the blood levels of sodium, potassium, bicarbonate, and chloride, leading to high blood pressure, weakness, and, rarely, periods of paralysis.

 

Aldosterone, a hormone produced and secreted by the adrenal glands, signals the kidney to excrete less sodium and more potassium. Aldosterone production is regulated partly by corticotropin in the pituitary and partly by a control mechanism in the kidneys (the renin-angiotensin-aldosterone system).  Renin, an enzyme produced in the kidneys, controls the activation of the hormone angiotensin, which stimulates the adrenal glands to produce aldosterone.

 

Hyperaldosteronism can be caused by a tumor (usually noncancerous) in the adrenal gland (a condition called Conn's syndrome). Sometimes hyperaldosteronism is a response to certain diseases. For example, the adrenal glands secrete large amounts of aldosterone if the blood pressure is very high or if the artery that carries blood to the kidneys is narrowed.

Symptoms

High levels of aldosterone can lead to low levels of potassium, causing weakness, tingling, muscle spasms, and paralysis. The nervous system may not function properly. Some people become extremely thirsty and urinate frequently, and some experience personality changes.

Symptoms of hyperaldosteronism are also associated with eating licorice, which contains a chemical very similar to aldosterone. In rare cases, people who eat a great deal of candy with real licorice flavoring may develop all the symptoms of hyperaldosteronism.

Diagnosis and Treatment

A doctor who suspects that high blood pressure or related symptoms are caused by hyperaldosteronism may measure the sodium and potassium levels in the blood. The doctor may also measure aldosterone levels and, if they're high, may prescribe spironolactone, a drug that blocks the action of aldosterone, to see if the levels return to normal. Other tests generally aren't needed.

 

When too much aldosterone is being produced, doctors examine the adrenal glands for an adenoma or cancer. While a computed tomography (CT) or magnetic resonance imaging (MRI) scan can be helpful, exploratory surgery is often necessary. If a growth is found, it can usually be removed. When a simple adenoma is removed, blood pressure returns to normal and other symptoms disappear about 70 percent of the time. If no tumor is found and the entire gland is overactive, partial removal of the adrenal glands may not control high blood pressure and complete removal will produce adrenal insufficiency, requiring treatment for the rest of the person's life. However, spironolactone can usually control the symptoms, and drugs for high blood pressure are readily available. Rarely do both adrenal glands have to be removed.

 

More:

Addison’s Disease Knol at http://knol.google.com/k/addison-s-disease#

Addison's Disease (Adrenal insufficiency): Caused by low levels of cortisonelike hormones produced by the adrenal glands. The cause is usually unknown, but may be a complications of tuberculosis, cancer, pituitary disease of cortisone drugs.

Addison's Disease
This Topic on the Message Boards.

 

Adrenal: Pertaining to one or both of these endocrine glands located on top of the kidneys. These glands secrete many hormones, including epinephrine (adrenaline), norepinephrine, and the corticosteroid, and play an important part in the body's endocrine system.

The adrenal is made up of an outer wall (the cortex) and an inner portion (the medulla).

A closer look at the adrenal glands, including an illustration.

Adrenal cortex: Outer layer of the adrenal gland, it secretes various hormones including cortisone, estrogen, testosterone, cortisol, androgen, aldosterone and progesterone.

 

Adrenalectomy: Surgical removal of the adrenal glands is a final measure for halting excess cortisol production This is used only when all other measures fail in individuals with pituitary tumors.

 

Adrenal gland tumor: A benign tumor, or adenoma, that usually results in the excess production of adrenal gland hormones.

 

Sometimes, an abnormality of the adrenal glands, most often an adrenal tumor, causes Cushing's syndrome. The average age of onset is about 40 years. Most of these cases involve non-cancerous tumors of adrenal tissue, called adrenal adenomas, which release excess cortisol into the blood. adrenal glands. In Primary Pigmented Micronodular Adrenal Disease and the familial Carney's complex, surgical removal of the adrenal glands is required.

 

Adrenal insufficiency: See Addison's Disease. Adrenal insufficiency is a life threatening chronic illness. An active and vigorous lifestyle with normal life expectancy is possible as long as the prescribed medications are taken regularly and adjusted when indicated. As with most chronic diseases, adrenal insufficiency demands that the patients take responsibility and develop self-management skills and techniques.

Read an article on Adrenal insufficiency

Adrenal medulla: Middle part of the adrenal gland, it secretes epinephrine (adrenalin) and norepinephrine.

 

Adrenocortical carcinomas, or adrenal cancers: These are the least common cause of Cushing's syndrome. Cancer cells secrete excess levels of several adrenal cortical hormones, including cortisol and adrenal androgens. Adrenocortical carcinomas usually cause very high hormone levels and rapid development of symptoms.

 

Adrenocortical hyperplasia: Increase in the number of cells of the adrenal cortex. It secretes cortisol, androgens and aldosterone. Increased production of any or all of these hormones may result in a variety of disorders, such as Cushing's syndrome and hypertension.

 

Adrenocorticotropic-hormone deficiency: Not enough ACTH is produced by the pituitary gland.

 

Adrenoleukodystrophy: Disturbance in brain substance caused by abnormal function of the adrenal gland.

 

Aldosterone: Adrenal hormone that affects the body's handling of sodium, chloride, and potassium.

Read an article on Aldosterone testing