Lower health-related quality of life observed in patients with Addison’s disease, Cushing’s syndrome

Patients with hypothalamic-pituitary-adrenal axis dysregulations report health-related quality of life that is far lower than that of the general population, according to findings of a prospective study.

“In most centers, both patients with adrenal deficiency and patients with Cushing’s syndrome are managed by the same team,” Charlotte DeBucy, of the Center for Rare Adrenal Diseases at Cochin Hospital in Paris, and colleagues wrote. “Despite the usual perception that both types of diseases alter quality of life, few studies have similarly investigated the impact of cortisol dysregulations on [health-related quality of life]. Such studies are important, however, to identify meaningful differences that would be important to consider to improve management and outcome.”

De Bucy and colleagues analyzed data from 343 patients with Addison’s disease or Cushing’s syndrome followed in routine practice at a single center in France between September 2007 and April 2014 (78% women; mean age, 48 years; mean length of time since diagnosis, 7.8 years; 61% married). All participants completed the short-form health survey (SF-36), a survey of health-related quality-of-life measures and the 12-item general health questionnaire (GHQ-12), a measure of psychological well-being or distress. Questionnaires were completed at baseline and at 6, 12, 24 and 36 months. Patients with Cushing’s syndrome were also assessed for cortisol status at baseline and at follow-up evaluations.

Within the cohort, 206 had Cushing’s syndrome of pituitary origin, 91 had Cushing’s syndrome of adrenal origin and 46 patients had Addison’s disease; 16% were included in the study before any treatment was initiated.

Researchers found that mean standard deviation scores for psychological and physical dimensions of the SF-36 were “well below” those of the general population, but diagnosis, cortisol status and time since treatment initiation all influenced individual scores. Cushing’s syndrome of pituitary origin was associated with worse health-related quality of life, especially for physical functioning, social functioning and mental health. In Cushing’s syndrome, health-related quality of life was generally worse during periods of hypercortisolism, but scores for these patients were lower than those of patients with Addison’s disease even during periods of hypocortisolism or eucortisolism, according to the researchers.

“The differences were particularly large for physical functioning and role-physical subscales,” the researchers wrote.

They also found that mental health scores for patients with Cushing’s syndrome decreased during periods of hypocortisolism, whereas other adrenal conditions were associated with higher mental health scores.

More than half of patients, regardless of diagnosis and cortisol status, had psychological distress requiring attention, according to the GHQ-12 survey.

“Our findings are important for clinical practice,” the researchers wrote. “The consequences of cortisol dysregulation on [health-related quality of life] should be considered in the management of adrenal insufficiency and even more (in) Cushing’s syndrome patients, and these consequences can be long term, affecting apparently cured patients. Early information on these consequences might be helpful for patients who often perceive a poor quality of life as the result of inadequate disease control or treatment. Even if this possibility exists, knowing that adrenal diseases have long-lasting effects on [health-related quality of life] may be helpful for patients to cope with them.” – by Regina Schaffer

Disclosure: L’association Surrénales supported this study. The researchers report no relevant financial disclosures.

From http://www.healio.com/endocrinology/adrenal/news/in-the-journals/%7B842655ce-e710-4476-a3c2-2909b06434ed%7D/lower-health-related-quality-of-life-observed-in-patients-with-addisons-disease-cushings-syndrome

History of Cortisone’s Discovery

It was Christmas Day in 1914 when the Mayo Clinic chemist Edward C. Kendall, PhD, first succeeded in isolating pure crystalline thyroxin using 6,500 pounds of hog thyroid glands, a success that would set him on the course for making one of the greatest discoveries in medicine in the last century.

His pivotal discovery, according to William F. Young, Jr., MD, MSc, chair of the division of endocrinology, diabetes, metabolism and nutrition at the Mayo Clinic College of Medicine, would lead Kendall, a self-described “hormone hunter,” to conduct adrenal experiments that would eventually change the course of medicine in ways he couldn’t have imagined. Kendall and his team’s discovery of cortisone would lead not only to a breakthrough treatment, Young said, but a Nobel Prize and international acclaim.

In an interview prior to presenting the Clark T. Swain Memorial History of Endocrinology Lecture at ENDO 2017, Young said that understanding the history behind such a monumental discovery can help endocrinologists see how hormone research has evolved, and provides insight into how to make advances in basic science and improve patient care. In preparing to tell Kendall’s story, Young completed archival research at Mayo and uncovered information that has not previously been published, he said.

“The cortisone story originated at Mayo Clinic, where I have been on staff for 33 years,” Young told Endocrine Today. “Although much of this story is not new information, it is not familiar to the current generations of endocrine scientists and clinical endocrinologists. It is a story of discovery science, clinical intuition, persistence, team science, patient volunteerism and sacrifice, hopes, and dreams.”

‘A big oak tree’

When Kendall first took on the project of preparing better adrenal extracts to potentially treat Addison’s disease in 1930, he was already thinking bigger, Young said.

“He once said, ‘I want to grow a great big oak tree … I am not interested in a bunch of blackberry bushes,’” Young said.

During his experiments at Mayo Clinic, the cost of bovine adrenals rose from 0.20 cents a pound to $3 per pound, equivalent to $42 per pound today. In 1934, Kendall struck a deal with Parke Davis Co., were he would extract “adrenalin” at no cost for the company if it would, in turn, deliver to him 600 pounds of bovine adrenals each week, Young said. He would then use the adrenal cortex for his studies.

In addition, Kendall struck a side deal with Wilson Labs, Young said, for an additional 300 pounds of bovine adrenals per week, to produce a cortical extract for them. He would in turn use the adrenal medullas to boost his production of adrenalin for the Park Davis deal.

“From 1934 to 1949, virtually all of the adrenaline used in North America was manufactured at Mayo Clinic in the small town of in Rochester, Minnesota,” Young said. “This lab ran 24 hours a day, in three shifts. By 1949, over 150 tons of adrenal glands had been processed at Mayo Clinic … $12.4 million in research supply dollars.”

A new discovery

In 1934, Kendall recognized through his work that the adrenal cortex produced more than one hormone, Young said. Over the next year, Kendall’s group isolated five crystalline compounds, naming them compounds “A” through “E” based on their order of identification. Compound “E” — what would later be named cortisone — was found to be biologically active, Young said.

Interest in synthesizing the active hormone from the adrenal cortex grew as part of the American war effort in the 1940s, Young said, and the U.S. National Research Council made it a priority. By 1948, 9,000 mg of “compound E” had been synthesized for clinical study; 2,000 mg were given to each of three investigators at Mayo Clinic for studies in patients with Addison’s disease and the remaining 3,000 mg were saved for future study.

In 1948, a patient known as H.G., a 28-year-old women with progressive inflammatory arthritis, presented to the clinic, Young said. After an unsuccessful treatment with the Swedish hepatoxin lactophenin — a therapy used at the time that induced jaundice in some patients, leading to remission — her physician, Philip Hench, went to Kendall for help. Kendall agreed to give Hench some of the remaining 3,000 mg of “compound E,” if Hench could convince Merck to grant permission.

The clinicians did get permission, and H.G. began treatment. Within days, Young said, the improvement was remarkable. Reading from the original, handwritten notes of Hench and his colleagues in rheumatology, , Young detailed the patient’s progress:

“Rolled over and turned off the radio with ease for the first time in weeks,” the notes said from “day 3.” “No more trembling of knees when moving.”

The clinicians were so amazed, Young said, that they filmed H.G’s progress. Young, who obtained the original films from the Mayo Clinic archives, showed footage of a crippled H.G. struggling to stand, only to be walking normally.

“They started taking videos because they realized no one would believe them,” Young said as the video played. “That they actually had something that could affect, up until this point, a crippling disorder.”

Hench came up with the acronym “cortisone,” adapted from corticosterone.

The discovery became international news. In December 1950, Kendall, Hench along with Tadeus Reichstein, received the 1950 Nobel Prize in Physiology and Medicine — just 27 months after H.G. received her first dose of “compound E.”

The future of corticosteroids

Today, Young said, corticosteroids are used for their anti-inflammatory and immunosuppressive properties across the field of medicine. Natural and synthetic glucocorticoids are used to treat a wide variety of non-adrenal diseases, from allergies, to gastrointestinal disorders and infectious diseases.

The important story of patient H.G. — and the scientific journey of Kendall and his colleagues — still resonates, Young said.

“My hope is that this story will remind us of our endocrine heritage and give us an opportunity to recognize the unlimited potential for discovery, research and clinical investigation that is taking place in research laboratories and clinical endocrine centers across the globe,” Young said in an interview. “In the current environment in the U.S., where federal research funds are being cut back, it is important to recall where the major advances in research and public health have come from.”

“There are many other messages in the presentation,” Young said. “For example, the importance of ‘team science’— a phrase only recently coined — has been in place for decades. It is team science that has led to many of the major advances in medicine, including the therapeutic use of corticosteroids.” – by Regina Schaffer

Reference:

Young WF. A Chemist, a Patient and the 1950 Nobel Prize in Physiology and Medicine: The Stories Behind the Stories on Cortisone. Presented at: The Endocrine Society Annual Meeting; April 1-4, 2017; Orlando, Fla.

Disclosures: Young reports no relevant financial disclosures.

 

From http://www.healio.com/endocrinology/adrenal/news/online/%7Bd8d71bcc-a981-418e-9d41-af4b2dcaa48f%7D/history-of-cortisones-discovery-offers-lessons-in-team-science-persistence

8 Things You Should Know About Addison’s Disease

adrenal-insufficiency

 

Cortisol gets a bad rap these days. (Guilty!) Yes, this hormone surges when you’re stressed. And yes, chronic stress is bad news for your health. But while too much cortisol can lead to all sorts of stress-related side effects, too little cortisol is equally debilitating.

Just ask someone with Addison’s disease. If you suffer from this condition, your adrenal glands fail to make adequate amounts of cortisol, says Betul Hatipoglu, MD, an endocrinologist at Cleveland Clinic.

Cortisol plays a role in regulating your blood pressure, heart function, digestion, and a lot else, Hatipoglu explains. So if your adrenal glands poop out and your cortisol levels plummet, a lot can go wrong. (In as little as 30 days, you can be a whole lot slimmer, way more energetic, and so much healthier just by following the simple, groundbreaking plan in The Thyroid Cure!)

Here’s what you need to know about this condition—starting with its craziest symptom.

It can make your teeth appear whiter.

Hatipoglu once met with a patient who was suffering from fatigue, belly pain, and mild weight loss. “Her doctors thought she was depressed,” Hatipoglu recalls. Toward the end of their appointment, Hatipoglu noticed the woman’s teeth looked very white. She realized they looked white because the woman’s skin was tan. “I asked her if she’d been on vacation, and she said she hadn’t been in the sun, and that’s when I knew,” Hatipoglu says. Some Addison’s-related hormone shifts can make the skin appear darker, almost like a tan. “Addison’s is the only disease I know of that can cause darkening of the skin,” she says.

Its (other) symptoms are popular ones.

 Along with darker skin, other symptoms of Addison’s include nausea, mild-to-severe abdominal or bone pain, weight loss, a lack of energy, forgetfulness, and low blood pressure, Hatipoglu says. Of course, those same symptoms are linked to many other health issues, from thyroid disease to cancer. “It’s very easy to confuse with other disorders, so many people see a lot of doctors before finally receiving a proper diagnosis,” she says. (One exception: For young women who develop Addison’s disease, loss of body hair is a warning sign, Hatipoglu adds.)
It’s rare.
Doctors also miss or misinterpret the symptoms of Addison’s disease because it’s very uncommon. “I’m not sure if it’s quite one in a million, but it’s very rare,” Hatipoglu says. “It makes sense that many doctors don’t think of it when examining a patient with these symptoms.”
It’s often confused with adrenal insufficiency.

A lot of online resources mention Addison’s disease and adrenal insufficiency as though they were two names for the same condition. They’re not the same, Hatipoglu says. While a thyroid issue or some other hormone-related imbalance could mess with your adrenal function, Addison’s disease refers to an autoimmune disorder in which your body attacks and destroys your adrenal glands.

That destruction can happen quickly.

While it takes months or even years for some Addison’s sufferers to lose all hormone production in their adrenal glands, for others the disease can knock out those organs very rapidly—in a matter of days, Hatipoglu says. “That’s very uncommon,” she adds. But compared to other less-severe adrenal issues, the symptoms of Addison’s tend to present more dramatically, she explains. That means a sufferer is likely to experience several of the symptoms mentioned above, and those symptoms will continue to grow worse as time passes.

Anybody can get it.

Addison’s is not picky. It can strike at any age, regardless of your sex or ethnicity, Hatipoglu says. While there’s some evidence that genetics may play a role—if other people in your family have the disease or some other endocrine disorder, that may increase your risk—there’s really no way to predict who will develop the disease, she adds.

Screening for Addison’s is pretty simple.

If your doctor suspects Addison’s, he or she will conduct a blood test to check for your levels of cortisol and another hormone called ACTH. “Usually the results of that screening are very clear,” Hatipoglu says. If they’re not, some follow-up tests can determine for sure if you have the condition.

There are effective treatments.

Those treatments involve taking oral hormone supplements.  In extreme cases, if the patient’s body does not properly absorb those supplements, injections may be necessary, Hatipoglu explains. “But patients live a normal life,” she adds. “It’s a treatable disease, and the treatments are effective.”

From http://www.prevention.com/health/addisons-disease-symptoms

Past News Items: My 37-year-old daughter has Addison’s disease.

old-news

 

Because, sometimes Old News is still valid!

From Tuesday, September 16, 2008

DEAR DR. DONOHUE: My 37-year-old daughter has Addison’s disease. Many doctors saw her when she was hospitalized a year ago. She had to go back to the hospital because of stomach upset, back pain and dehydration. Her skin has darkened. She was told she would be fine after she started steroids. This hasn’t happened. She is constantly sick. Do you have any good news? — L.K.

ANSWER: With Addison’s disease, the adrenal glands have stopped producing their many hormones. Those hormones include cortisone and aldosterone. Cortisone gives us energy, combats inflammation and figures into many of the body’s most important functions. Aldosterone is essential for blood pressure maintenance. Without adrenal gland hormones, the skin darkens, especially the elbow skin and the creases in the hands.

Treatment is straightforward: Replace the missing hormones. Maybe the dosage of her hormone medicines needs revision. If she’s hasn’t shortly turned the corner, she should get a second opinion from an endocrinologist, a specialist in this kind of illness.

From http://www.kilgorenewsherald.com/news/2008/0916/advice/009.html

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Addison’s Disease vs Cushing’s Syndrome Nursing

Cushing’s and Addison’s Disease. An endocrine NCLEX review on how to differentiate between Cushing’s Syndrome/Disease vs Addison’s and Addisonian Crisis. In this video, I will discuss the pathophysiology, signs & symptoms, and nursing interventions for these endocrine disorders of the adrenal cortex and pituitary glands.

 

Addison’s Disease and Cushing’s Syndrome/Disease review notes for nursing school and NCLEX exam. In nursing school and for the NCLEX exam, you will need to know how to provide care to a patient with either Addison’s Disease or Cushing’s.

However, many students get these two endocrine disorders confused, but these review notes will help you differentiate between them.

These NCLEX review notes will cover:

  • Signs and Symptoms of Addison’s Disease vs Cushing’s
  • Causes of Addison’s Disease and Cushing’s
  • Nursing Management of Addison’s Disease and Cushing’s

After reviewing these notes, don’t forget to take the Addison’s Disease vs Cushing’s Quiz.

Addison’s Disease vs Cushing’s

Major Players in these endocrine disorders:

  • Adrenal Cortex
  • Steroid Hormones
    • Corticosteroids (specifically Aldosterone (mineralocorticoid) & Cortisol (glucocorticoid)

Role of Adrenal Cortex: releases steroid hormones and sex hormones

Role of Aldosterone: regulates blood pressure through renin-angiotensin-aldosterone system, helps retain sodium and secretes potassium (balances sodium and potassium levels).

Role of Cortisol: “STRESS Hormone” helps the body deal with stress such as illness or injury, increases blood glucose though glucose metabolism, break downs fats, proteins, and carbs, regulates electrolytes.

Cushing’s (Syndrome & Disease)

Cushing’s: hyper-secretion of CORTISOL (watch video for clever ways to remember this)

Cushing’s Syndrome vs Cushing’s Disease

Cushing’s Syndrome: caused by an outside cause or medical treatment such as glucocorticoid therapy

Cushing’s Disease: caused from an inside source due to the pituitary gland producing too much ACTH (Adrenocorticotropic hormone) which causes the adrenal cortex to release too much cortisol.

Signs & Symptoms of Cushing’s

Remember the mnemonic: “STRESSED” (remember there is too much of the STRESS hormone CORTISOL)

Skin fragile

Truncal obesity with small arms

Rounded face (appears like moon), Reproductive issues amennorhea and ED in male(due to adrenal cortex’s role in secreting sex hormones)

Ecchymosis, Elevated blood pressure

Striae on the extremities and abdomen (Purplish)

Sugar extremely high (hyperglycemia)

Excessive body hair especially in women…and Hirsutism (women starting to have male characteristics), Electrolytes imbalance: hypokalemia

Dorsocervical fat pad (Buffalo hump), Depression

Causes of Cushing’s

  • Glucocorticoid drug therapy ex: Prednisone
  • Body causing it: due to tumors and cancer on the *pituitary glands or adrenal cortex, or genetic predisposition

Nursing Management for Cushing’s Syndrome

  • Prep patient for Hypophysectomy to remove the pituitary tumor
  • Prep patient for Adrenalectomy:
    • If this is done educate pt about cortisol replacement therapy after surgery
  • Risk for infection and skin breakdown
  • Monitor electrolytes blood sugar, potassium, sodium, and calcium levels

Addison’s Disease

Addison’s: Hyposecretion of Aldosterone & Cortisol (watch the video for a clever way on how to remember this and not get it confused with Cushing’s)

Signs & Symptoms of Addison’s Disease

Remember the phrase: “Low STEROID Hormones” (remember you have low production of aldosterone & cortisol which are STEROID hormones)

Sodium & Sugar low (due to low levels of cortisol which is responsible for retention sodium and increases blood glucose), Salt cravings

Tired and muscle weakness

Electrolyte imbalance of high Potassium and high Calcium

Reproductive changes…irregular menstrual cycle and ED in men

lOw blood pressure (at risk for vascular collapse)….aldosterone plays a role in regulating BP

Increased pigmentation of the skin (hyperpigmentation of the skin)

Diarrhea and nausea, Depression

Causes of Addison’s Disease

  • Autoimmune due to the adrenal cortex becoming damaged due to the body attacking itself:
    • Tuberculosis/infections
    • Cancer
    • Hemorrhaging of the adrenal cortex due to a trauma

Nursing Management of Addison’s Disease

  • Watching glucose and K+ level
  • Administer medications to replace the low hormone levels of cortisol and aldosterone
  • For replacing cortisol:
    • ex: Prednisone, Hydrocortisone
      • Education: Patient needs to report if they are having stress such as illness, surgery, or extra stress in life ( will need to increase dosage), take medication exactly as prescribed….don’t stop abruptly without consulting with MD.
  • For replacing aldosterone:
    • ex: Fludrocortisone aka Florinef
      • Education: consume enough salt..may need extra salt
  • Wearing a medical alert bracelet
  • Eat diet high in proteins and carbs, and make sure to consume enough sodium
  • Avoid illnesses, stress, strenuous exercise

Watch for Addisonian Crisis

This develops when Addison’s Disease isn’t treated.

In addisonian crisis, the patient has extremely LOW CORTISOL levels (life threatening).

Remember the 5 S’s

  1. Sudden pain in stomach, back, and legs
  2. Syncope (going unconscious)
  3. Shock
  4. Super low blood pressure
  5. Severe vomiting, diarrhea and headache
  • NEED IV Cortisol STAT:
    • Solu-Cortef and IV fluids (D5NS to keep blood sugar and sodium levels good and fluid status)
  • Watch for risk for infection, neuro status (confusion, agitation), electrolyte levels (sodium and potassium, glucose)

Addison’s vs Cushing’s Quiz

 

From http://www.registerednursern.com/addisons-disease-vs-cushings-review-notes-for-nclex/

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