NADF NEWS® Q & A – 2022
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On this page you will find questions and answers from the quarterly published NADF News.
Written by Paul Margulies, MD, FACE, FACP, Medical Director, NADF. Clinical Associate Professor of Medicine, Zucker School of Medicine at Hofstra/Northwell. |
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Q&A from June 2022
estion: I had both adrenal glands surgically removed in September 2020 and now have adrenal insufficiency, which is treated with hydrocortisone and fludrocortisone. In October 2021, I learned that I had sudden onset cirrhosis of the liver, which no one can seem to explain. I’ve never used illegal drugs, have no history of hepatitis, and drink very rarely (1-2 drinks a few times a year before my diagnosis. I don’t drink at all now). Could the steroids possibly have anything to do with it?
Answer: Replacement steroids used to manage adrenal insufficiency do not cause any type of liver disease. The most common cause of cirrhosis is not alcohol. The most common causes are fatty liver and non-alcoholic steatohepatitis or NASH. Your doctor will need to do a thorough evaluation, including ruling out old infection from viral hepatitis. Imaging of the liver may include a new technique that measures the amount of fibrosis. A liver biopsy may be needed. Management should be with a gastroenterologist who sees a lot of liver disease.
Question: I’m supposed to have an aggressive preparation for an upcoming colonoscopy and endoscopy. Is it safe to alter potassium and electrolytes for someone with Primary Adrenal Insufficiency (PAD?)
Answer: The prep itself is safe for adrenal insufficiency. I recommend plenty of clear fruit juices like apple or white grape juice rather that Gatorade. The procedure itself is not stressful, but the anesthetist or anesthesiologist must know about the adrenal insufficiency and be prepared to give iv hydrocortisone only if the procedure goes much longer than expected or if there is a complication.
Question: I was diagnosed with CREST syndrome (2 positive ANA years apart plus other tests) and was being treated about 5 years before I was diagnosed with Addison’s and put on daily steroids. My ANA became negative, and I was taken off treatment. I’m still symptomatic but I can’t get a rheumatologist to see me. Could my daily steroids for Addison’s be affecting my ANA tests?
Answer: CREST is a complex rheumatology disorder that affects multiple organs. The ANA test is mostly used to diagnose and monitor systemic lupus, which is not part of CREST. However, the ANA is often abnormal in other disorders and is commonly seen in healthy women. The negative ANA at this time would not be the result of the use of maintenance dose glucocorticoids for the Addison’s disease. Having a previous diagnosis of CREST, it is very important to see a rheumatologist now to sort out if you really have it and to provide guidance on managing the residual symptoms.
Question: Has there ever been a case study or case of a primary adrenal insufficiency patient not being accurately diagnosed with cancer, specifically, colon, because of steroid dependency and the absence of weight loss?
Answer: I am not aware of any actual study or case of this sort. Cancer screening can be difficult with coexisting medical conditions in general. Certainly, in the case of colon cancer, weight loss should not be considered as an accurate sign. Screening with colonoscopy to find early cancer should be performed regularly. The objective is to find suspicious lesions before they cause any signs or symptoms.
Question: I’ve had secondary AI for 10 years and feel confident in my ability to manage it on a daily basis but having watched my husband prep for his first colonoscopy recently, I feel some trepidation. After all, isn’t diarrhea one of the primary triggers of adrenal crisis? Colonoscopy preparation results in extreme diarrhea for several hours! I’d appreciate any guidance you can offer on what I need to discuss with my endocrinologist and my surgeon, and how I can limit the risk to my health during the preparation.
Answer: Despite the concern about the diarrhea, colonoscopy is not a cause for concern in people with primary or secondary adrenal insufficiency. During the prep day when medications are given to help with the clean out and clear liquids are necessary, if appropriate fluids are consumed there will be no need for extra doses of hydrocortisone. I do recommend using clear fruit juices like apple juice and white grape juice because they add needed calories. The procedure itself is also not stressful. Light anesthesia is used, not general anesthesia. Pre-surgical stress dosing of hydrocortisone is not needed. I would simply consider an extra dose of 10 mg orally before going to the procedure only if you feel very anxious. It is important to inform the physician doing the colonoscopy and the anesthesiologist about the adrenal insufficiency, so they are prepared to give IV hydrocortisone if the procedure is prolonged or there are any complications.
Answer: Replacement steroids used to manage adrenal insufficiency do not cause any type of liver disease. The most common cause of cirrhosis is not alcohol. The most common causes are fatty liver and non-alcoholic steatohepatitis or NASH. Your doctor will need to do a thorough evaluation, including ruling out old infection from viral hepatitis. Imaging of the liver may include a new technique that measures the amount of fibrosis. A liver biopsy may be needed. Management should be with a gastroenterologist who sees a lot of liver disease.
Question: I’m supposed to have an aggressive preparation for an upcoming colonoscopy and endoscopy. Is it safe to alter potassium and electrolytes for someone with Primary Adrenal Insufficiency (PAD?)
Answer: The prep itself is safe for adrenal insufficiency. I recommend plenty of clear fruit juices like apple or white grape juice rather that Gatorade. The procedure itself is not stressful, but the anesthetist or anesthesiologist must know about the adrenal insufficiency and be prepared to give iv hydrocortisone only if the procedure goes much longer than expected or if there is a complication.
Question: I was diagnosed with CREST syndrome (2 positive ANA years apart plus other tests) and was being treated about 5 years before I was diagnosed with Addison’s and put on daily steroids. My ANA became negative, and I was taken off treatment. I’m still symptomatic but I can’t get a rheumatologist to see me. Could my daily steroids for Addison’s be affecting my ANA tests?
Answer: CREST is a complex rheumatology disorder that affects multiple organs. The ANA test is mostly used to diagnose and monitor systemic lupus, which is not part of CREST. However, the ANA is often abnormal in other disorders and is commonly seen in healthy women. The negative ANA at this time would not be the result of the use of maintenance dose glucocorticoids for the Addison’s disease. Having a previous diagnosis of CREST, it is very important to see a rheumatologist now to sort out if you really have it and to provide guidance on managing the residual symptoms.
Question: Has there ever been a case study or case of a primary adrenal insufficiency patient not being accurately diagnosed with cancer, specifically, colon, because of steroid dependency and the absence of weight loss?
Answer: I am not aware of any actual study or case of this sort. Cancer screening can be difficult with coexisting medical conditions in general. Certainly, in the case of colon cancer, weight loss should not be considered as an accurate sign. Screening with colonoscopy to find early cancer should be performed regularly. The objective is to find suspicious lesions before they cause any signs or symptoms.
Question: I’ve had secondary AI for 10 years and feel confident in my ability to manage it on a daily basis but having watched my husband prep for his first colonoscopy recently, I feel some trepidation. After all, isn’t diarrhea one of the primary triggers of adrenal crisis? Colonoscopy preparation results in extreme diarrhea for several hours! I’d appreciate any guidance you can offer on what I need to discuss with my endocrinologist and my surgeon, and how I can limit the risk to my health during the preparation.
Answer: Despite the concern about the diarrhea, colonoscopy is not a cause for concern in people with primary or secondary adrenal insufficiency. During the prep day when medications are given to help with the clean out and clear liquids are necessary, if appropriate fluids are consumed there will be no need for extra doses of hydrocortisone. I do recommend using clear fruit juices like apple juice and white grape juice because they add needed calories. The procedure itself is also not stressful. Light anesthesia is used, not general anesthesia. Pre-surgical stress dosing of hydrocortisone is not needed. I would simply consider an extra dose of 10 mg orally before going to the procedure only if you feel very anxious. It is important to inform the physician doing the colonoscopy and the anesthesiologist about the adrenal insufficiency, so they are prepared to give IV hydrocortisone if the procedure is prolonged or there are any complications.
Q&A from March 2022
Question: How does the endocrinologist know how much fludrocortisone to start an Addison’s patient on when diagnosed? What tests and/or symptoms does the doctor and patient monitor over time to determine if different dosing needed? Is there any time that a patient would need to take more fludrocortisone for a singular event (e.g., sweating more)?
Answer: Fludrocortisone is the medication that replaces the hormone aldosterone, the mineralocorticoid hormone. This hormone tells the kidneys to absorb sodium and excrete potassium. This helps to maintain blood volume and blood pressure. In untreated Addison’s disease, the body loses sodium and retains potassium, so blood pressure tends to be low, contributing to lightheadedness and fainting. In prescribing fludrocortisone, the endocrinologist will often start with an average dose of 0.1 mg per day and then adjust from there. Adjustments are based on clinical response, including blood pressure, drop in blood pressure on standing, symptoms like lightheadedness, and laboratory tests like serum potassium, sodium and BUN. One of the most useful tests of adequacy of fludrocortisone dosing is the plasma renin. This measures the kidney response to blood volume. If it is high, more fludrocortisone is needed. If it is low, and blood pressure is elevated, it would be appropriate to lower the dose. Keep in mind that there is also some mineralocorticoid activity in the hydrocortisone. Fludrocortisone has a long duration of action in the body, so sometimes low doses like 1/2 tablet every 2 or 3 days can be used. With that long duration and slow metabolism, it is not useful to add more for acute events or illnesses. It is better to add more hydrocortisone, salt and fluids for acute events that may include sweating and fluid loss.
Question: Can Addison’s disease cause neuropathy? Like in your hands and feet?
Answer: Addison’s disease does not cause neuropathy. However, there are two rare diseases of the nervous system that are associated with Addison’s disease. They are caused by an inherited enzyme disorder that causes injury to the nerves in the brain and spinal cord. When it presents in infancy, it is called adrenoleukodystrophy. If it occurs in an adult, it is milder and is called adrenomyeloneuropathy.
Question: Does DHEA affect salt balance? I’ve experienced cramping feet since starting. Is hair loss an effect of taking DHEA?
Answer: DHEA is a mild androgen (male hormone) made in the adrenal glands. It is low in Addison’s disease along with cortisol and aldosterone. Replacement with low doses of DHEA, usually 25 mg for women, 50 mg for men has been found to improve sense of well-being. This is a vague benefit and is hard to study. I usually suggest a trial of it after the doses of hydrocortisone and fludrocortisone are stabilized. Some people feel a benefit, some don’t. Since it is not a medical necessity, I give my patients the option. DHEA does not have any significant mineralocorticoid activity, so it should not cause fluid retention or cramping. Since it is a mild androgen, it can contribute to hair loss, acne and facial hair growth. That is why a lower dose is used for women. However, if any of these dermatologic side effects occur even with a low dose, I advise that it be stopped.
Question: Should individuals with Adrenal Insufficiency take potassium? I was always told not to.
Answer: Primary adrenal insufficiency causes an elevation in serum potassium due to the deficiency of aldosterone. Since this hormone is not deficient in secondary adrenal insufficiency, potassium levels are usually normal in that condition. People with PAI or Addison’s disease should not take potassium supplements since it would add to the tendency for high levels from stress or inadequate hydrocortisone or fludrocortisone dosage. Generally, when replacement doses are adequate and stress levels are normal, there is no need to eliminate high potassium foods, but it is not advisable to purposely add high potassium foods.
Question: It appears to me that my daughter stays sicker longer with colds and that kind of thing. Is it true that Addison’s patients’ take longer to recover from common colds and the like?
Answer: We know from recent studies that people with Addison’s disease have an increased susceptibility to viral infections because of the effect of maintenance glucocorticoids on the immune mechanism that fights viral infections. However, there is no literature on the duration of viral symptoms. I suspect this is an individual phenomenon. From talking to my patients who do not have adrenal disease, I find a lot of variability in their response to viral infections. Remember that those with Addison’s should add extra hydrocortisone when they do have an infection and maintain the extra dose until they feel better. This can reduce the severity of symptoms.
Question: Can I fast with Addison’s disease?
Answer: Yes, if the hydrocortisone and fludrocortisone doses are taken on time, a short fast of a few hours would be harmless. Prolonged fasts or greater than one day might cause hypoglycemia, which would be risky.
Question: I was taking too much Florinef (.2 mg), and now my blood pressure is too high. I’ve reduced it by one fourth. How long before I can expect my BP to come down?
Answer: Fludrocortisone has a very long duration of action. Changes in the dosage take days to a few weeks to reach a new stable effect. Lowering the dose to reduce blood pressure may take 2 to 4 weeks to stabilize.
Question: Is it safe to take elderberry and hydrocortisone? Not necessarily at the same time but, in the same day. Is it safe to take elderberry daily while being steroid dependent?
Answer: I have no experience with any of my patients taking elderberry. It has been promoted for its immune support, but there is not much scientific evidence of a real benefit. It is probably safe for most people, but I am concerned about one of its properties - it is a mild diuretic. That may make it questionable for people with primary adrenal insufficiency since it may reduce sodium and blood pressure. If an individual does try it, monitor for side effects, including dizziness and nausea. If any side effects occur, stop it.
Question: I was diagnosed with autoimmune primary adrenal insufficiency in March 2021. I’m quite stable on a replacement dose of hydrocortisone and fludrocortisone, despite occasional episodes of nausea, dizziness and fatigue. How often is it recommended for someone with Addison’s to see an endocrinologist for follow-up? And what additional testing is recommended at follow-up? Specifically, I’m interested to hear about recommendations for follow-up testing of cortisol, aldosterone, ACTH, adrenal antibodies, curve test, etc., and whether this is useful. I’ve been told that now that I have a diagnosis, no additional testing is necessary other than checking my electrolytes levels. I guess I was just thinking this might be helpful to see if there was any improvement in cortisol production once on a replacement dose.
Answer: Once a diagnosis of autoimmune adrenal insufficiency is made and replacement hydrocortisone and fludrocortisone is prescribed, I recommend frequent follow up every 2 to 3 months until you are comfortable that the dosages of both medications are optimal. It is important to have face to face meetings with the endocrinologist. The doctor should look for signs of persistent adrenal insufficiency as well as overtreatment - on the physical examination as well as detailed questioning about symptoms, including fatigue, dizziness, nausea, and salt craving. Blood studies should include electrolytes, but also plasma renin to help establish the appropriate dose of fludrocortisone. The dose of hydrocortisone should be the lowest dose that prevents signs and symptoms of adrenal insufficiency. Once there is stability, I suggest face to face meetings every 6 months. There should be a physical exam and discussion of adrenal symptoms and intercurrent other medical history. There should be a discussion of management of any acute medical events and whether appropriate steroids were given. There is no benefit to repeating blood tests for cortisol, ACTH, aldosterone or adrenal antibodies. Since I suggest using the lowest replacement dose of hydrocortisone that prevents signs and symptoms, if there really is some recovery of adrenal reserve (seen in a minority of patients), it will be apparent by allowing a low dose of glucocorticoid. I do not suggest a routine repeat of the entire diagnostic work-up because it has a very low yield of useful information. I do recommend routine re-testing for other autoimmune endocrine conditions, especially thyroid disease and vitamin B12 deficiency. Make sure your endocrinologist is advised about any new medical diagnosis and treatment from other doctors. Make sure you are familiar with appropriate emergency measures for acute illness and injuries.
Answer: Fludrocortisone is the medication that replaces the hormone aldosterone, the mineralocorticoid hormone. This hormone tells the kidneys to absorb sodium and excrete potassium. This helps to maintain blood volume and blood pressure. In untreated Addison’s disease, the body loses sodium and retains potassium, so blood pressure tends to be low, contributing to lightheadedness and fainting. In prescribing fludrocortisone, the endocrinologist will often start with an average dose of 0.1 mg per day and then adjust from there. Adjustments are based on clinical response, including blood pressure, drop in blood pressure on standing, symptoms like lightheadedness, and laboratory tests like serum potassium, sodium and BUN. One of the most useful tests of adequacy of fludrocortisone dosing is the plasma renin. This measures the kidney response to blood volume. If it is high, more fludrocortisone is needed. If it is low, and blood pressure is elevated, it would be appropriate to lower the dose. Keep in mind that there is also some mineralocorticoid activity in the hydrocortisone. Fludrocortisone has a long duration of action in the body, so sometimes low doses like 1/2 tablet every 2 or 3 days can be used. With that long duration and slow metabolism, it is not useful to add more for acute events or illnesses. It is better to add more hydrocortisone, salt and fluids for acute events that may include sweating and fluid loss.
Question: Can Addison’s disease cause neuropathy? Like in your hands and feet?
Answer: Addison’s disease does not cause neuropathy. However, there are two rare diseases of the nervous system that are associated with Addison’s disease. They are caused by an inherited enzyme disorder that causes injury to the nerves in the brain and spinal cord. When it presents in infancy, it is called adrenoleukodystrophy. If it occurs in an adult, it is milder and is called adrenomyeloneuropathy.
Question: Does DHEA affect salt balance? I’ve experienced cramping feet since starting. Is hair loss an effect of taking DHEA?
Answer: DHEA is a mild androgen (male hormone) made in the adrenal glands. It is low in Addison’s disease along with cortisol and aldosterone. Replacement with low doses of DHEA, usually 25 mg for women, 50 mg for men has been found to improve sense of well-being. This is a vague benefit and is hard to study. I usually suggest a trial of it after the doses of hydrocortisone and fludrocortisone are stabilized. Some people feel a benefit, some don’t. Since it is not a medical necessity, I give my patients the option. DHEA does not have any significant mineralocorticoid activity, so it should not cause fluid retention or cramping. Since it is a mild androgen, it can contribute to hair loss, acne and facial hair growth. That is why a lower dose is used for women. However, if any of these dermatologic side effects occur even with a low dose, I advise that it be stopped.
Question: Should individuals with Adrenal Insufficiency take potassium? I was always told not to.
Answer: Primary adrenal insufficiency causes an elevation in serum potassium due to the deficiency of aldosterone. Since this hormone is not deficient in secondary adrenal insufficiency, potassium levels are usually normal in that condition. People with PAI or Addison’s disease should not take potassium supplements since it would add to the tendency for high levels from stress or inadequate hydrocortisone or fludrocortisone dosage. Generally, when replacement doses are adequate and stress levels are normal, there is no need to eliminate high potassium foods, but it is not advisable to purposely add high potassium foods.
Question: It appears to me that my daughter stays sicker longer with colds and that kind of thing. Is it true that Addison’s patients’ take longer to recover from common colds and the like?
Answer: We know from recent studies that people with Addison’s disease have an increased susceptibility to viral infections because of the effect of maintenance glucocorticoids on the immune mechanism that fights viral infections. However, there is no literature on the duration of viral symptoms. I suspect this is an individual phenomenon. From talking to my patients who do not have adrenal disease, I find a lot of variability in their response to viral infections. Remember that those with Addison’s should add extra hydrocortisone when they do have an infection and maintain the extra dose until they feel better. This can reduce the severity of symptoms.
Question: Can I fast with Addison’s disease?
Answer: Yes, if the hydrocortisone and fludrocortisone doses are taken on time, a short fast of a few hours would be harmless. Prolonged fasts or greater than one day might cause hypoglycemia, which would be risky.
Question: I was taking too much Florinef (.2 mg), and now my blood pressure is too high. I’ve reduced it by one fourth. How long before I can expect my BP to come down?
Answer: Fludrocortisone has a very long duration of action. Changes in the dosage take days to a few weeks to reach a new stable effect. Lowering the dose to reduce blood pressure may take 2 to 4 weeks to stabilize.
Question: Is it safe to take elderberry and hydrocortisone? Not necessarily at the same time but, in the same day. Is it safe to take elderberry daily while being steroid dependent?
Answer: I have no experience with any of my patients taking elderberry. It has been promoted for its immune support, but there is not much scientific evidence of a real benefit. It is probably safe for most people, but I am concerned about one of its properties - it is a mild diuretic. That may make it questionable for people with primary adrenal insufficiency since it may reduce sodium and blood pressure. If an individual does try it, monitor for side effects, including dizziness and nausea. If any side effects occur, stop it.
Question: I was diagnosed with autoimmune primary adrenal insufficiency in March 2021. I’m quite stable on a replacement dose of hydrocortisone and fludrocortisone, despite occasional episodes of nausea, dizziness and fatigue. How often is it recommended for someone with Addison’s to see an endocrinologist for follow-up? And what additional testing is recommended at follow-up? Specifically, I’m interested to hear about recommendations for follow-up testing of cortisol, aldosterone, ACTH, adrenal antibodies, curve test, etc., and whether this is useful. I’ve been told that now that I have a diagnosis, no additional testing is necessary other than checking my electrolytes levels. I guess I was just thinking this might be helpful to see if there was any improvement in cortisol production once on a replacement dose.
Answer: Once a diagnosis of autoimmune adrenal insufficiency is made and replacement hydrocortisone and fludrocortisone is prescribed, I recommend frequent follow up every 2 to 3 months until you are comfortable that the dosages of both medications are optimal. It is important to have face to face meetings with the endocrinologist. The doctor should look for signs of persistent adrenal insufficiency as well as overtreatment - on the physical examination as well as detailed questioning about symptoms, including fatigue, dizziness, nausea, and salt craving. Blood studies should include electrolytes, but also plasma renin to help establish the appropriate dose of fludrocortisone. The dose of hydrocortisone should be the lowest dose that prevents signs and symptoms of adrenal insufficiency. Once there is stability, I suggest face to face meetings every 6 months. There should be a physical exam and discussion of adrenal symptoms and intercurrent other medical history. There should be a discussion of management of any acute medical events and whether appropriate steroids were given. There is no benefit to repeating blood tests for cortisol, ACTH, aldosterone or adrenal antibodies. Since I suggest using the lowest replacement dose of hydrocortisone that prevents signs and symptoms, if there really is some recovery of adrenal reserve (seen in a minority of patients), it will be apparent by allowing a low dose of glucocorticoid. I do not suggest a routine repeat of the entire diagnostic work-up because it has a very low yield of useful information. I do recommend routine re-testing for other autoimmune endocrine conditions, especially thyroid disease and vitamin B12 deficiency. Make sure your endocrinologist is advised about any new medical diagnosis and treatment from other doctors. Make sure you are familiar with appropriate emergency measures for acute illness and injuries.
