AACE Position Statement: Coronavirus (COVID-19) and People with Adrenal Insufficiency and Cushing’s Syndrome

Posted on March 31, 2020 by MaryO

With the novel COVID-19 virus continuing to spread, it is crucial to adhere to the advice from experts and the Centers for Disease Control and Prevention (CDC) to help reduce risk of infection for individuals and the population at large. This is particularly important for people with adrenal insufficiency and people with uncontrolled Cushing’s Syndrome.

Studies have reported that individuals with adrenal insufficiency have an increased rate of respiratory infection-related deaths, possibly due to impaired immune function. As such, people with adrenal insufficiency should observe the following recommendations:

  • Maintain social distancing to reduce the risk of contracting COVID-19
  • Continue taking medications as prescribed
  • Ensure appropriate supplies for oral and injectable steroids at home, ideally a 90-day preparation
    • In the case of hydrocortisone shortages, ask your pharmacist and physician about replacement with different strengths of hydrocortisone tablets that might be available. Hydrocortisone (or brand name Cortef) tablets have 5 mg, 10 mg or 20 mg strength
  • In cases of acute illness, increase the hydrocortisone dose per instructions and call the physician’s office for more details
    • Follow sick day rules for increasing oral glucocorticoids or injectables per your physician’s recommendations
      • In general, patients should double their usual glucocorticoid dose in times of acute illness
      • In case of inability to take oral glucocorticoids, contact your physician for alternative medicines and regimens
  • If experiencing fever, cough, shortness of breath or other symptoms, call both the COVID-19 hotline (check your state government website for contact information) and your primary care physician or endocrinologist
  • Monitor symptoms and contact your physician immediately following signs of illness
  • Acquire a medical alert bracelet/necklace in case of an emergency

Individuals with uncontrolled Cushing’s Syndrome of any origin are at higher risk of infection in general. Although information on people with Cushing’s Syndrome and COVID-19 is scarce, given the rarity of the condition, those with Cushing’s Syndrome should strictly adhere to CDC recommendations:

  • Maintain social distancing to reduce the risk of contracting COVID-19
  • If experiencing fever, cough, shortness of breath or other symptoms, call both the COVID-19 hotline (check your state government website for contact information) and your primary care physician or endocrinologist

In addition, people with either condition should continue to follow the general guidelines at these times:

  • Stay home as much as possible to reduce your risk of being exposed
    • When you do go out in public, avoid crowds and limit close contact with others
    • Avoid non-essential travel
  • Wash your hands with soap and water regularly, for at least 20 seconds, especially before eating or drinking and after using the restroom and blowing your nose, coughing or sneezing
  • If soap and water are not readily available, use an alcohol-based sanitizer with at least 60% alcohol
  • Cover your nose and mouth when coughing or sneezing with a tissue or a flexed elbow, then throw the tissue in the trash
  • Avoid touching your eyes, mouth or nose when possible

From https://www.aace.com/recent-news-and-updates/aace-position-statement-coronavirus-covid-19-and-people-adrenal

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Metoclopramide Can Mask Adrenal Insufficiency After Gland Removal in BMAH Patients

Posted on May 8, 2019 by MaryO

Metoclopramide, a gastrointestinal medicine, can increase cortisol levels after unilateral adrenalectomy — the surgical removal of one adrenal gland — and conceal adrenal insufficiency in bilateral macronodular adrenal hyperplasia (BMAH) patients, a case report suggests.

The study, “Retention of aberrant cortisol secretion in a patient with bilateral macronodular adrenal hyperplasia after unilateral adrenalectomy,” was published in Therapeutics and Clinical Risk Management.

BMAH is a subtype of adrenal Cushing’s syndrome, characterized by the formation of nodules and enlargement of both adrenal glands.

In this condition, the production of cortisol does not depend on adrenocorticotropic hormone (ACTH) stimulation, as usually is the case. Instead, cortisol production is triggered by a variety of stimuli, such as maintaining an upright posture, eating mixed meals — those that contain fats, proteins, and carbohydrates — or exposure to certain substances.

A possible treatment for this condition is unilateral adrenalectomy. However, after the procedure, some patients cannot produce adequate amounts of cortisol. That makes it important for clinicians to closely monitor the changes in cortisol levels after surgery.

Metoclopramide, a medicine that alleviates gastrointestinal symptoms and is often used during the postoperative period, has been reported to increase the cortisol levels of BMAH patients. However, the effects of metoclopramide on BMAH patients who underwent unilateral adrenalectomy are not clear.

Researchers in Japan described the case of a 61-year-old postmenopausal woman whose levels of cortisol remained high after surgery due to metoclopramide ingestion.

The patient was first examined because she had experienced high blood pressure, abnormal lipid levels in the blood, and osteoporosis for ten years. She also was pre-obese.

She was given medication to control blood pressure with no results. The lab tests showed high serum cortisol and undetectable levels of ACTH, suggesting adrenal Cushing’s syndrome.

Patients who have increased cortisol levels, but low levels of ACTH, often have poor communication between the hypothalamus, the pituitary, and the adrenal glands. These three glands — together known as the HPA axis — control the levels of cortisol in healthy people.

Imaging of the adrenal glands revealed they were both enlarged and presented nodules. The patient’s cortisol levels peaked after taking metoclopramide, and her serum cortisol varied significantly during the day while ACTH remained undetectable. These results led to the BMAH diagnosis.

The doctors performed unilateral adrenalectomy to control cortisol levels. The surgery was successful, and the doctors reduced the dose of glucocorticoid replacement therapy on day 6.

Eight days after the surgery, however, the patient showed decreased levels of fasting serum cortisol, which indicated adrenal insufficiency — when the adrenal glands are unable to produce enough cortisol.

The doctors noticed that metoclopramide was causing an increase in serum cortisol levels, which made them appear normal and masked the adrenal insufficiency.

They stopped metoclopramide treatment and started replacement therapy (hydrocortisone) to control the adrenal insufficiency. The patient was discharged 10 days after the surgery.

The serum cortisol levels were monitored on days 72 and 109 after surgery, and they remained lower than average. Therefore she could not stop hydrocortisone treatment.

The levels of ACTH remained undetectable, suggesting that the communication between the HPA axis had not been restored.

“Habitual use of metoclopramide might suppress the hypothalamus and pituitary via negative feedback due to cortisol excess, and lead to a delayed recovery of the HPA axis,” the researchers said.

Meanwhile, the patient’s weight decreased, and high blood pressure was controlled.

“Detailed surveillance of aberrant cortisol secretion responses on a challenge with exogenous stimuli […] is clinically important in BMAH patients,” the study concluded. “Caution is thus required for assessing the actual status of the HPA axis.”

From https://cushingsdiseasenews.com/2019/05/07/metoclopramide-conceals-adrenal-insufficiency-after-gland-removal-bmah-patients-case-report/

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Health Alert: Adrenal Crisis Causes Death in Some People Who Were Treated with hGH

Posted on July 28, 2018 by MaryO

Doctors conducting the follow-up study of individuals treated with hGH looked at causes of death among recipients and found some disturbing news. Many more people have died from a treatable condition called adrenal crisis than from CJD (MaryO’Note: Creutzfeldt-Jakob Disease). This risk does not affect every recipient. It can affect those who lack other hormones in addition to growth hormone. Please read on to find out if this risk applies to you. Death from adrenal crisis can be prevented.

Adrenal crisis is a serious condition that can cause death in people who lack the pituitary hormone ACTH. ACTH is responsible for regulating the adrenal gland. Often, people are unaware that they lack this hormone and therefore do not know about their risk of adrenal crisis.

Most people who were treated with hGH did not make enough of their own growth hormone. Some of them lacked growth hormone because they had birth defects, tumors or other diseases that cause the pituitary gland to malfunction or shut down. People with those problems frequently lack other key hormones made by the pituitary gland, such as ACTH, which directs the adrenal gland to make cortisol, a hormone necessary for life. Having too little cortisol can be fatal if not properly treated.

Treatment with hGH does not cause adrenal crisis, but because a number of people lacking growth hormone also lack ACTH, adrenal crisis has occurred in some people who were treated with hGH. In earlier updates we have talked about how adrenal crisis can be prevented, but people continue to die from adrenal crisis, which is brought on by lack of cortisol. These deaths can be prevented. Please talk to your doctor about whether you are at risk for adrenal crisis.

  • Why should people treated with hGH know about adrenal crisis? Among the people who received hGH, those who had birth defects, tumors, and other diseases affecting the brain lacked hGH and often, other hormones made by the pituitary gland. A shortage of the hormones that regulate the adrenal glands can cause many health problems. It can also lead to death from adrenal crisis. This tragedy can be prevented.
  • What are adrenal hormones? The pituitary gland makes many hormones, including growth hormone and ACTH, a hormone which signals the adrenal glands to make cortisol, a hormone needed for life. If the adrenal gland doesn’t make enough cortisol, replacement medications must be taken. The most common medicines used for cortisol replacement are:
    • Hydrocortisone
    • Prednisone
    • Dexamethasone
  • What is adrenal crisis? Adrenal hormones are needed for life. The system that pumps blood through the body cannot work during times of physical stress, such as illness or injury, if there is a severe lack of cortisol (or its replacement). People who lack cortisol must take their cortisol replacement medication on a regular basis, and when they are sick or injured, they must take extra cortisol replacement to prevent adrenal crisis. When there is not enough cortisol, adrenal crisis can occur and may rapidly lead to death.
  • What are the symptoms of lack of adrenal hormones? If you don’t have enough cortisol or its replacement, you may have some of these problems:
    • feeling weak
    • feeling tired all the time
    • feeling sick to your stomach
    • vomiting
    • no appetite
    • weight loss

    When someone with adrenal gland problems has weakness, nausea, or vomiting, that person needs immediate emergency treatment to prevent adrenal crisis and possible death.

  • Why are adrenal hormones so important? Cortisol (or its replacement) helps the body respond to stress from infection, injury, or surgery. The normal adrenal gland responds to serious illness by making up to 10 times more cortisol than it usually makes. It automatically makes as much as the body needs. If you are taking a cortisol replacement drug because your body cannot make these hormones, you must increase the cortisol replacement drugs during times of illness, injury, or surgery. Some people make enough cortisol for times when they feel well, but not enough to meet greater needs when they are ill or injured. Those people might not need cortisol replacement every day but may need to take cortisol replacement medication when their body is under stress. Adrenal crisis is extremely serious and can cause death if not treated promptly. Discuss this problem with your doctor to help decide whether you need more medication or other treatment to protect your health.
  • How is adrenal crisis treated? People with adrenal crisis need immediate treatment. Any delay can cause death. When people with adrenal crisis are vomiting or unconscious and cannot take medicine, the hormones can be given as an injection. Getting an injection of adrenal hormones can save your life if you are in adrenal crisis. If you lack the ability to make cortisol naturally, you should carry a medical ID card and wear a Medic-Alert bracelet to tell emergency workers that you lack adrenal hormones and need treatment. This precaution can save your life if you are sick or injured.
  • How can I prevent adrenal crisis?
    • If you are always tired, feel weak, and have lost weight, ask your doctor if you might have a shortage of adrenal hormones.
    • If you take hydrocortisone, prednisone, or dexamethasone, learn how to increase the dose when you become ill.
    • If you are very ill, especially if you are vomiting and cannot take pills, seek emergency medical care immediately. Make sure you have a hydrocortisone injection with you at all times, and make sure that you and those around you (in case you’re not conscious) know how and when to administer the injection.
    • Carry a medical ID card and wear a bracelet telling emergency workers that you have adrenal insufficiency and need cortisol. This way, they can treat you right away if you are injured.

Remember: Some people who lacked growth hormone may also lack cortisol, a hormone necessary for life. Lack of cortisol can cause adrenal crisis, a preventable condition that can cause death if treated improperly. Deaths from adrenal crisis can be prevented if patients and their families recognize the condition and are careful to treat it right away. Adrenal crisis is a medical emergency. Know the symptoms and how to adjust your medication when you are ill. Taking these precautions can save your life.

From https://www.niddk.nih.gov/health-information/endocrine-diseases/national-hormone-pituitary-program/health-alert-adrenal-crisis-causes-death-people-treated-hgh

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Steroid Medication for Nasal Obstruction in Infants May Cause Cushing’s Syndrome

Posted on March 10, 2018 by MaryO

Intranasal steroid drops used to treat nasal obstruction may cause Cushing’s syndrome and adrenal insufficiency in infants, a case study of two patients suggests.

The study, “Iatrogenic Cushing’s syndrome and adrenal insufficiency in infants on intranasal dexamethasone drops for nasal obstruction – Case series and literature review,” was published in the International Journal of Pediatric Otorhinolaryngology.

Children with nasal obstruction may have severe delays in development and can face life-threatening complications later in life such as obstructive sleep apnea and cardiopulmonary problems.

While intranasal steroid drops have become increasingly popular as a substitute for surgery, they can have adverse effects. In addition to suppressing the immune system and changing metabolism, high levels of corticosteroids in the blood may cause Cushing’s syndrome.

Researchers at Weill Cornell Medical College presented two cases of adrenal gland insufficiency and Cushing’s syndrome caused by intranasal dexamethasone drops. Dexamethasone is a type of corticosteroid medication.

First, they described the case of a 3-month-old boy who was taken to the hospital following a life-threatening episode at home after feeding. A physical evaluation revealed nasal congestion with no additional anatomic abnormalities.

Treatment with nasal dexamethasone drops three times a day improved his breathing. While the dosage was later decreased to three drops once daily, a congestion episode led the mother to increase the dose back to the initial recommendation.

After seven weeks of treatment, the boy was noted to have facial puffiness, leading to an endocrine evaluation that revealed low cortisol levels. The dose was eventually reduced, and the boy’s cortisol levels returned to normal after several months.

The second case was a 6-week-old boy with a history of chronic congestion and difficulty feeding. He had severe nasal obstruction and required intubation due to respiratory distress. A nasal exam revealed damaged mucosa with severe nasal cavity narrowing, and he began treatment with three ciprofloxacin-dexamethasone drops three times a day.

After two and a half weeks of treatment, the boy’s cortisol levels were considerably low, and adrenal insufficiency was diagnosed. The treatment dose was reduced in an attempt to improve cortisol levels, but nasal obstruction symptoms continued.

The child then underwent surgery to resolve his nasal obstruction, and the treatment with steroid drops was discontinued. While his cortisol levels subsequently improved, they continued to be low, suggesting that he may have a hormone-related disease.

Despite the benefits of steroid-based nasal drops, small infants are more sensitive to steroid compounds. In addition, nasal drops are more easily absorbed than nasal sprays, suggesting that infants taking these medications should be better controlled for side effects.

“Patients started on this therapy must be closely monitored in a multi-disciplinary fashion to ensure patient safety and optimal symptom resolution,” the researchers suggested.

From https://cushingsdiseasenews.com/2018/03/09/cushing-syndrome-infants-can-be-caused-by-steroid-based-nasal-drops-study-suggests/

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A 12-year-old boy died from Addison’s disease after the chance of lifesaving treatment was ‘missed’

Posted on September 30, 2017 by MaryO

The death of a 12-year-old boy who was suffering from undiagnosed Addison’s disease was preventable, an inquest heard.

Ryan Lee Morse had been unwell from July 2012, with his parents noticing his skin darkening and him becoming lethargic and losing weight.

His condition worsened over the following months and he died during the early hours of December 8, 2012.

During the time he was unwell, Ryan’s mother, Carol Ann Morse, took him to Abernant Surgery in Abertillery several times.

She said: “Ryan was rarely ill as a child. In June 2012, which was towards the end of Ryan’s first comprehensive school year, I noticed his skin colour changing.

“His skin seemed to be getting darker.”

She said his joint areas, including elbows and knees, were getting darker. Under his eyes, it looked as if he had not slept for a month. I don’t suppose it worried me at the time because it was gradual.”

A post mortem was held on December 12 by Dr E. J. Lazda, a consultant pathologist at University Hospital of Wales in Cardiff who concluded that Ryan died as a result of Addison’s disease.

An inquest into Ryan’s death was held at Newport Coroner’s Court on Thursday.

Dr Yvette Cloette, a consultant paediatrician since 2004, was called during the early hours of December 8, 2012, by a registrar where she was told the details of Ryan’s death.

She said: “Ryan’s parents told me he had been unwell since July.

“It was thought Ryan had been particularly unwell since the Thursday before he passed away. He had to be collected from school that day. On (the) Friday morning, she said he hallucinated. His temperature did settle that afternoon but then he had diarrhoea.

“As his mum was cleaning him, she noticed his genitalia were black.

“I then examined Ryan. At this time I formed the opinion that Ryan may have had Addison’s disease. I didn’t share this with the family at the time because I didn’t have enough evidence.

“I believe that Ryan’s death was preventable. Addison’s is a disease which, once recognised, can be treated.”

She said it was easier to put things together retrospectively, as opposed to when treating an acute illness as a GP.

David Bowen, senior coroner for Gwent, paid tribute to Ryan’s family during the hearing.

“Before summing up, I think it’s right that I pay tribute to the dignity that has been shown by Mrs Morse and her family.

“It can’t have been easy for them to rehear events that took place over five years ago.

“Please accept my belated condolences.”

Mr Bowen told the inquest that Ryan had been fit and well up until July 2012.

“However at about that time, his parents began to notice a gradual change in his skin and a fluctuation in his general health.”

He had been diagnosed with a viral infection and prescribed Paracetamol, he said.

Over the next six to eight weeks, he did not improve.

Mr Bowen said: “Consequently, his mother took him back to the doctor. The GP was more concerned about the rash, it seems to me, than any of the other symptoms.

“He prescribed tablets and cream for that condition.”

Mr Bowen said that during October and November 2012, “Ryan’s health became much more of a concern for his parents.”

He suffered from headaches, pains in his legs, and occasional episodes of projectile vomiting.

On November 7, Mrs Morse took Ryan back to the GP surgery, where she described symptoms to Dr Rudling, who took samples of blood.

On November 21, they returned to receive the blood test results.

The results revealed a “slightly lower than normal” white blood cell count. The inquest heard Ryan was told he was still suffering from a viral infection that had been diagnosed some months earlier.

Mr Bowen said: “It appears that about this time, there was an outbreak of Norovirus or vomiting and sickness in the area that may have confused the diagnosis.”

Mrs Morse said: “I’d been told to bring Ryan back in January so I thought I would just get Christmas out of the way and take him back. I’d been a carer for 9-10 years but my job didn’t give me any insight into what Ryan had.”

On November 29, 2012, Ryan returned to school, but around a week later on December 6 he was so ill that his mum had to collect him early.

The following day, on December 7, Mrs Morse rang Abernant Surgery saying she needed to speak to a doctor.

Between 8.50am and 8.55am, she received a call from Dr Lyndsey Elizabeth Thomas.

Mrs Morse said: “She asked if he’d been given Paracetamol and I explained he wouldn’t take it. She asked what his temperature was like.

“I’d said Ryan was awake (that morning) and talking rubbish.”

The inquest heard Mrs Morse was asked to take Ryan to the surgery, but she said she was unable to.

“She then told me to give Ryan some dissolvable Paracetamol and see how it goes until dinner. She said fetch him up if you need to.”

Dr Lyndsey Elizabeth Thomas said her contact with Ryan was limited to a single telephone conversation with his mother on December 7.

She said: “I considered whether Ryan needed to be seen or admitted to hospital.

“I clearly recall explaining that if she had any concerns or if Ryan’s delirium or temperature didn’t improve in two hours, he would need to be seen, I would be able to go and visit him at the end of the morning surgery if necessary.”

Mrs Morse said she later noticed that her son’s genitals were black.

She rang the surgery and was put her through to Dr Rudling.

Mrs Morse said: “She said ‘it’s all to do with his hormones’. Phone Monday and we’ll fit him in. At this point I didn’t know what to think.

“I was thinking I’ll take him in on Monday and see what they say. There was no more temperature, no more sickness and no more diarrhoea.”

The inquest heard Dr Joanne Louise Rudling, who qualified in 1993, joined Abernant in August 2011.

She said her first contact with Ryan was in November 2012.

On December 7, Dr Rudling said the receptionist took a call from Ryan’s mother while she was in reception.

Dr Rudling said: “I decided to speak to Ryan’s mother in reception there and then.

“She also asked if this could be age related, I said it could be but I would have to examine him first.

“The impression I got was Ryan was improving. His mother was concerned about the darkening of his genitalia.”

Ryan’s father said goodnight around 10.15pm and went to bed. At around 11.10pm Mrs Morse could see Ryan had fallen asleep, and went to sleep herself at around 11.30pm.

She said: “I woke up and saw it was 4.10am and then I looked at Ryan and looking at his chest could see he wasn’t breathing.

“I started to do chest compressions, dialled 999, continued chest compressions until the paramedics arrived. They took over. They told me Ryan had died.”

Mr Bowen said: “This is a rare but natural disease, one which apparently GPs will not normally encounter.

“Unfortunately, neither doctor nor parents thought it necessary to refer Ryan to hospital, where the true nature of his illness may have been diagnosed.”

Recording a narrative conclusion, Mr Bowen said Ryan died of natural causes.

He said: “The opportunity to administer life-saving treatment was missed.”

Speaking after the inquest, Ryan’s sister Christina Morse said: “First of all I would like to thank everyone involved with Ryan and Ryan’s case.

“Today, after five long years, the coroner has come to the conclusion that Ryan’s death was due to natural causes and that Ryan’s death was preventable.”

From http://www.walesonline.co.uk/news/wales-news/boy-died-addisons-disease-after-13687355

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