NADF News® Q & A – 2009On 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 March, 2009
Question: I have had Addison’s disease since 1969, and I am 73 years old. I have always been active. This year, it is harder to do things and had a lot of muscle and joint pain, which I take pain meds for. My question: Does age ever require an increase in steroids? I take 25 mg. cortisone acetate in the am and 12.5 mg pm. I also take 0.1 mg fludrocortisone acetate and 0.1 mg Synthroid. Thank you.
Answer: There is no general tendency to need a higher dose of replacement glucocorticoid with aging. The aches and pain are more likely the normal symptoms of arthritis rather than adrenal symptoms. Discuss the symptoms with your doctor. You might benefit from a consultation with a rheumatologist.
Question: Recently my Solu-Cortef injections expired. Per NADF’s e-mail of July 6, 2007, dexamethasone sodium phosphate was recommended as a substitute for Solu-Cortef. Last week I got my prescription renewed with dexamethasone, because it doesn’t need complicated mixing like Solu-Cortef. However, last evening I was going through old NADF newsletters. In a 2003 Q&A to Dr. Margulies, he replied that dexamethasone is not a great substitute for hydrocortisone because: "it contains no mineralcorticoid activity (the only reason to take an injection of steroids); it would not provide enough stimulation to blood pressure.” Should I get a new prescription for Solu-Cortef? Is there any injectable solution that is good for an Addison’s emergency that does not need mixing? This is a very difficult process for lay persons. Thanks for all the years of NADF and it’s support!
Answer: I haven’t changed my mind about dexamethasone. For emergencies, Solu-Cortef and/or the other brand of hydrocortisone are superior because it contains mineralocorticoid and well as glucocorticoid activity and therefore helps to maintain blood pressure.
Question: My 20 year old son is a member of NADF because he has Addison’s. His endo of 11 years died, and he has a new one now. He is 165 pounds, and she recommends he injects 200 mg of Solu-Cortef if there is an emergency. His previous endo rec 100 mg. What is the dosage used by adult Addisonians? I fear that if he uses too much, it will be detrimental instead of helpful. I would appreciate any help you can give us with this question.
Answer: A stress dose of 100 mg of hydrocortisone (Solu-Cortef is hydrocortisone) is adequate for emergencies while waiting for transportation to a health care facility. Keep in mind that the average human produces about 20 to 35 mg of cortisol per day. When stressed, we will produce more. The typical suggested stress dose of 100 mg every 8 hours IV or IM given in hospitals for medical emergencies or surgery is purposely an exaggeration of the normal physiologic response, but is generally safe. Although there is no significant long term ill effect of giving an acute dose of 200 mg in an emergency, it is much more than necessary, and might temporarily raise blood sugar and lower potassium.
Answer: There is no general tendency to need a higher dose of replacement glucocorticoid with aging. The aches and pain are more likely the normal symptoms of arthritis rather than adrenal symptoms. Discuss the symptoms with your doctor. You might benefit from a consultation with a rheumatologist.
Question: Recently my Solu-Cortef injections expired. Per NADF’s e-mail of July 6, 2007, dexamethasone sodium phosphate was recommended as a substitute for Solu-Cortef. Last week I got my prescription renewed with dexamethasone, because it doesn’t need complicated mixing like Solu-Cortef. However, last evening I was going through old NADF newsletters. In a 2003 Q&A to Dr. Margulies, he replied that dexamethasone is not a great substitute for hydrocortisone because: "it contains no mineralcorticoid activity (the only reason to take an injection of steroids); it would not provide enough stimulation to blood pressure.” Should I get a new prescription for Solu-Cortef? Is there any injectable solution that is good for an Addison’s emergency that does not need mixing? This is a very difficult process for lay persons. Thanks for all the years of NADF and it’s support!
Answer: I haven’t changed my mind about dexamethasone. For emergencies, Solu-Cortef and/or the other brand of hydrocortisone are superior because it contains mineralocorticoid and well as glucocorticoid activity and therefore helps to maintain blood pressure.
Question: My 20 year old son is a member of NADF because he has Addison’s. His endo of 11 years died, and he has a new one now. He is 165 pounds, and she recommends he injects 200 mg of Solu-Cortef if there is an emergency. His previous endo rec 100 mg. What is the dosage used by adult Addisonians? I fear that if he uses too much, it will be detrimental instead of helpful. I would appreciate any help you can give us with this question.
Answer: A stress dose of 100 mg of hydrocortisone (Solu-Cortef is hydrocortisone) is adequate for emergencies while waiting for transportation to a health care facility. Keep in mind that the average human produces about 20 to 35 mg of cortisol per day. When stressed, we will produce more. The typical suggested stress dose of 100 mg every 8 hours IV or IM given in hospitals for medical emergencies or surgery is purposely an exaggeration of the normal physiologic response, but is generally safe. Although there is no significant long term ill effect of giving an acute dose of 200 mg in an emergency, it is much more than necessary, and might temporarily raise blood sugar and lower potassium.
Q&A from June, 2009
Question: NADF recently forwarded the COAST news #175 Summer 2009. On pages 6 & 7 are articles citing information from Autoimmunity Research Foundation/Professor Trevor Marshall. I looked at the website cited in the article, www.autoimmunityresearch.org and the companion website www.marshallprotocol.com. I had come across these websites before and had discounted the theories and this antibiotic "protocol” as probably poor medicine. Marshall’s theory of cause may have some base, but his treatments sound so contrary to current medical advice regarding antibiotic use. I have not seen any other research done about his theory of autoimmunity cause. There are other websites indicating that this Marshall Protocol may actually cause Addison’s (and other diseases). One of these is www.lassesen.com/cfids/MarshallProtocolRisks.htm. I would like to know Dr. Margulies' opinion of Marshall’s theories and protocol. Since the two articles in the COAST news were so prominent, could we see Dr. Margulies views in the NADF News?
Answer: I had not read this article before. It presents some interesting theories, but I am bothered by the conclusions it makes. Low vitamin D levels are being found in a large percentage of the population, not just people with osteoporosis. Low vitamin D levels have been associated with a higher risk of death from all causes – including heart disease and cancer. The attempt to link replacement of vitamin D with the incidence of autoimmune diseases is illogical. Higher doses of vitamin D are only very recently being suggested, and most of the population is seriously undertreated. I recommend that most individuals should have a baseline 25OH vitamin D level measured. If it is low, I suggest replacement with vitamin D3. I see no reason not to recommend this to people with autoimmune diseases.
Question: One interesting thing that came out of a support group meeting was that you shouldn’t mix antacids with hydrocortisone. An attendee said that as soon as she stopped Prilosec, she felt so much better. Another has not been doing well at all, and she takes Nexium every day. Her skin has become darker and darker lately. I thought I’d pass that on.
Answer: Proton pump inhibitors are well known to affect absorption of calcium, digoxin and ketoconizole. I could not find any study of steroid absorption, but I suspect there may be individuals who may have this side effect. I would suggest that each person who seems to have this side effect should try going off the drug, possibly try a different drug in the same category (they have different chemistries) or try to switch to a H2 blocker for antacid therapy (drugs like Pepcid and Zantac).
Question: At the support group meeting, a woman said that her doctor pre-fills syringes for her with the dexamethasone, and that they’re good for up to a year. I’ve been told that once they’re in syringes, that they go bad quickly. Another member said that her doctor pre-fills her syringes also.
Answer: I am not comfortable with the idea of prefilled syringes, mainly because of the risk of contamination. The prefilled syringe is not totally air-tight and bacteria might get in there.
Question: I am a member of NADF because I’ve had Addison’s for the last 7 years and I am hoping you can help me because I have not gotten a reply from my endocrinologist. I will be having an angiogram, and I need to know how to prepare for this. Is there anything special that my cardiologist needs to do before doing the angiogram? Thank you for your prompt reply and assistance on this matter.
Answer: An angiogram is not as stressful as surgery, so IV hydrocortisone is usually not needed. I would still suggest contacting your own endocrinologist, but I can recommend taking a double dose of your usual morning hydrocortisone before going for the angiogram. Remind the cardiologist about your condition and have him contact your endocrinologist if there are any post-procedure issues that might necessitate giving you extra doses of hydrocortisone.
Answer: I had not read this article before. It presents some interesting theories, but I am bothered by the conclusions it makes. Low vitamin D levels are being found in a large percentage of the population, not just people with osteoporosis. Low vitamin D levels have been associated with a higher risk of death from all causes – including heart disease and cancer. The attempt to link replacement of vitamin D with the incidence of autoimmune diseases is illogical. Higher doses of vitamin D are only very recently being suggested, and most of the population is seriously undertreated. I recommend that most individuals should have a baseline 25OH vitamin D level measured. If it is low, I suggest replacement with vitamin D3. I see no reason not to recommend this to people with autoimmune diseases.
Question: One interesting thing that came out of a support group meeting was that you shouldn’t mix antacids with hydrocortisone. An attendee said that as soon as she stopped Prilosec, she felt so much better. Another has not been doing well at all, and she takes Nexium every day. Her skin has become darker and darker lately. I thought I’d pass that on.
Answer: Proton pump inhibitors are well known to affect absorption of calcium, digoxin and ketoconizole. I could not find any study of steroid absorption, but I suspect there may be individuals who may have this side effect. I would suggest that each person who seems to have this side effect should try going off the drug, possibly try a different drug in the same category (they have different chemistries) or try to switch to a H2 blocker for antacid therapy (drugs like Pepcid and Zantac).
Question: At the support group meeting, a woman said that her doctor pre-fills syringes for her with the dexamethasone, and that they’re good for up to a year. I’ve been told that once they’re in syringes, that they go bad quickly. Another member said that her doctor pre-fills her syringes also.
Answer: I am not comfortable with the idea of prefilled syringes, mainly because of the risk of contamination. The prefilled syringe is not totally air-tight and bacteria might get in there.
Question: I am a member of NADF because I’ve had Addison’s for the last 7 years and I am hoping you can help me because I have not gotten a reply from my endocrinologist. I will be having an angiogram, and I need to know how to prepare for this. Is there anything special that my cardiologist needs to do before doing the angiogram? Thank you for your prompt reply and assistance on this matter.
Answer: An angiogram is not as stressful as surgery, so IV hydrocortisone is usually not needed. I would still suggest contacting your own endocrinologist, but I can recommend taking a double dose of your usual morning hydrocortisone before going for the angiogram. Remind the cardiologist about your condition and have him contact your endocrinologist if there are any post-procedure issues that might necessitate giving you extra doses of hydrocortisone.
Q&A from September, 2009
Question: My wife was diagnosed with Addison’s disease in 1975. She has been cautioned NEVER to donate blood. Her doctor emphatically stated that the amount of blood given in a 'normal’ donation (a pint if memory serves me correctly) could result in her death! While that opinion is just that – an opinion – certainly she will not go against the advice of her doctor. I also realize that her general physical condition is unique, and that such a warning was given with due consideration to the uniqueness of her own condition. My only purpose in writing this is to add a note of caution for any Addisonian considering blood donation.
Answer: They (Plasma Services Group) are obviously aware of the Addison’s disease diagnosis for any donors. From their website it indicates that they take the plasma by a process called plasmaphoresis. This keeps the red cells in the patient and is therefore safer than taking whole blood. If performed slowly with adequate hydration and monitoring, it is safe for Addisonians who are well controlled and asymptomatic.
Question: I have heard that no one with autoimmune disease should EVER submit to live vaccine, because live vaccines stir up cytokines, and very often cause antibodies to go on a rampage.
Answer: There is no clear evidence that the live virus immunization is unsafe for Addisonians. However, the injection has fewer annoying side effects, so I suggest the killed virus vaccine for Addisonians rather than the live virus.
Question: Thank you for this info, but I have an additional question. Is Squalene safe? It is not FDA approved and was in the anthrax vaccine given to our soldiers during the Gulf War. This adjuvant is feared by many in Europe, and this is one of the reasons people over there don’t want the vaccine. It is in the injectable. According to info on the internet, it can bring on more immune disorders even in healthy people. So if Addisonians are more susceptible to immune disorders, can this be harmful to them?
Answer: There is no Squalene in the H1N1 flu vaccine. It is made in eggs and contains no adjuvants.
Question: Hello, I am a 64-year-old woman who has had Addison’s disease for 36 years. I am very unsure about getting the H1N1 vaccine. I have read that this virus may have been around before and that older people may have already been exposed and have an immunity to it. Would there be any harmful affects to getting the injection if you already have an immunity? I have also been told that if you have certain other conditions (I have fibromyalgia), that the vaccine could worsen those conditions.
Answer: If the H1N1 flu vaccine is available to you; there is no reason you should not take it. Even if you had been exposed to the virus in the past, there is no harm in taking the vaccine now. There is no contraindication except allergy to eggs.
Answer: They (Plasma Services Group) are obviously aware of the Addison’s disease diagnosis for any donors. From their website it indicates that they take the plasma by a process called plasmaphoresis. This keeps the red cells in the patient and is therefore safer than taking whole blood. If performed slowly with adequate hydration and monitoring, it is safe for Addisonians who are well controlled and asymptomatic.
Question: I have heard that no one with autoimmune disease should EVER submit to live vaccine, because live vaccines stir up cytokines, and very often cause antibodies to go on a rampage.
Answer: There is no clear evidence that the live virus immunization is unsafe for Addisonians. However, the injection has fewer annoying side effects, so I suggest the killed virus vaccine for Addisonians rather than the live virus.
Question: Thank you for this info, but I have an additional question. Is Squalene safe? It is not FDA approved and was in the anthrax vaccine given to our soldiers during the Gulf War. This adjuvant is feared by many in Europe, and this is one of the reasons people over there don’t want the vaccine. It is in the injectable. According to info on the internet, it can bring on more immune disorders even in healthy people. So if Addisonians are more susceptible to immune disorders, can this be harmful to them?
Answer: There is no Squalene in the H1N1 flu vaccine. It is made in eggs and contains no adjuvants.
Question: Hello, I am a 64-year-old woman who has had Addison’s disease for 36 years. I am very unsure about getting the H1N1 vaccine. I have read that this virus may have been around before and that older people may have already been exposed and have an immunity to it. Would there be any harmful affects to getting the injection if you already have an immunity? I have also been told that if you have certain other conditions (I have fibromyalgia), that the vaccine could worsen those conditions.
Answer: If the H1N1 flu vaccine is available to you; there is no reason you should not take it. Even if you had been exposed to the virus in the past, there is no harm in taking the vaccine now. There is no contraindication except allergy to eggs.
Q&A from December, 2009
Question: You have been helpful in the past while we try to help my dad. He was diagnosed with Addison's a couple of years ago. They are having a horrible time regulating his blood pressure. It constantly fluctuates from extreme lows to too high. He also suffers from extreme confusion and repetition. They took him for a second opinion, and this doctor doesn’t think he has Addison’s because he does not have any pigment discoloration, but they don’t know what it is. My mother is constantly trying to regulate his steroids and BP medicines. She is worn out. I had not seen him since his diagnosis. They arrived for a visit a couple of days ago. I was stunned to see the changes in him. When I got home from work he was totally confused and kept repeating the same story over and over (about 15 times in 30 minutes). This confusion had started around 3 pm more so after he took his medicine. He took hydrocortisone, sodium. We sat down to eat dinner and he took his medicine (multivitamin, calcium with vitamin D, Aggrenox, vitamin B and Simvastatin). Very shortly after dinner he was fine. He had a normal conversation and was engaged with us. It was the most unbelievable thing. Everyone thinks he has dementia, but dementia doesn’t go away after taking medicine. He has days the confusion is worse and might last all day. Are you aware of this type of problem with Addison’s patients? Thank you for any input you can provide.
Answer: From the pattern of improvement after eating, one must consider hypoglycemia as a cause of the confusion. True dementia would not respond like that. Also, the fluctuating blood pressure suggests an element of essential hypertension which is now complicated by the coexisting (presumptive) Addison’s disease. This is a situation where his doctor should arrange for home blood pressure as well as home blood glucose monitoring, to sort out what really happens. It is important to avoid diuretics for the blood pressure. Often a combination of low dose fludrocortisone with a drug like Norvasc can balance the blood pressure. If hypoglycemia is documented, an adjustment of the hydrocortisone regimen and a change in diet would be necessary.
Question: A member would like to know if it is typical to lose bone and muscle mass with Addison’s. He is 73 and he has told me several times that he has lost all his bones and muscles.
Answer: One of the major problems in the treatment of Addison’s disease is to balance the necessary replacement steroid dose for good health and well-being against the negative effects of excess steroid use. In excess, glucocorticoid hormones can cause muscle wasting and weakness as well as osteoporosis or bone thinning, which can increase the risk of fracture. Therefore, these effects are not due to the Addison’s disease itself, but from the treatment. That is one of the reasons it is important to adjust the steroid dosage to the lowest dose that keeps the patient comfortable and free of adrenal insufficiency symptoms. At the same time, it is vital to maintain good nutrition, with adequate amounts of protein, calcium and vitamin D. When osteoporosis is found, additional treatment with bisphosphonate therapy (Fosamax, Actonel or Boniva) can be useful.
Question: I was diagnosed with Addison’s disease in 1993 at the age of 46 years. It was decided in about 1998 that I should have a bone density scan done. As a result of that test, I was prescribed Fosamax (70 mg) once a week and have been on it since (also because of the steroids prescribed to treat the Addison’s). I read somewhere that perhaps the benefits of Fosamax diminish after 10 years, and could in fact become a harmful thing. Can you enlighten me on the subject? Thank you!
Answer: Fosamax and the other bisphosphonate drugs, Actonel and Boniva, are very effective in improving bone density and reducing the risk of fractures in people with osteoporosis and in people at increased risk of developing osteoporosis because they take high dose steroids or drugs like aromatase inhibitors for breast cancer. It is important to note that many doctors have prescribed these drugs incorrectly for lesser degrees of bone loss, such as osteopenia. Osteopenia is a mild bone loss that does not produce the significant risk of fracture seen in osteoporosis. It is very important for each person who has a bone density study to go over the degree of bone loss to clarify if it is osteopenia or osteoporosis. If it is osteoporosis, the bisphosphonate drugs can be used, and follow-up bone density measurements should be performed about every 2 years. Most of the time there is gradual improvement and it often plateaus at 5 to 10 years. Very often there is enough improvement that the previous oseoporosis has reverted to osteopenia. When that is seen, or if the person has been on the drug for 5 to 10 years and the effect has reached a plateau, the drug should be stopped. It is also important to note that the replacement doses of steroids used for Addison’s disease do not necessarily mean that bisphosphonate treatment is needed. I would use it only if there is evidence of osteoporosis, or if aromatase inhibitors are also needed.
Question: Would you please address in a newsletter and at any future medical conferences an issue that physicians need to alert their patients too. I recently had a cholecystectomy. Just prior to the procedure I reminded the anesthesiologist that I couldn’t have Etomidate due to my adrenal suppression. He made no comment, just looked at me; I could tell by the look on his face that he seemed unaware of the correlation between Etomidate and adrenal suppression. A recent article dealing with this topic PubMed abstract J Trauma 2008 Sep:65(3):573-9. Adrenal suppression following a single dose of Etomidate for rapid sequence induction: a prospective randomized study. I’m not trying to be a "know it all”. I just wanted to wake up alive and educate and/or remind the anesthesiologist about Etomidate. That night after surgery my BP was 70/50 and the next am 88/53. I don’t know if he gave me Etomidate or that I just didn’t dose myself with enough dexamethasone prior to the procedure and the next day. (P.S. the surgeon didn’t know about how much steroid dosing either.) Thanks.
Answer: I do not know how often Etomidate is actually used as an anesthesia adjunct, but it is well established that it can suppress adrenocortical function and lower cortisol levels for several hours. In fact, it has been used to treat adrenal cancer, but is not very useful long term because it must be given IV and causes sedation. It is very important to note that it would not affect an Addisonian taking steroids because it suppresses adrenal production, not cortisol metabolism. Therefore, it would not have produced any effect in the person with Addison’s disease getting a steroid prep for surgery. It could, however, affect a person with undiagnosed Addison’s or a person with borderline secondary adrenal insufficiency who it not given a steroid prep.
Answer: From the pattern of improvement after eating, one must consider hypoglycemia as a cause of the confusion. True dementia would not respond like that. Also, the fluctuating blood pressure suggests an element of essential hypertension which is now complicated by the coexisting (presumptive) Addison’s disease. This is a situation where his doctor should arrange for home blood pressure as well as home blood glucose monitoring, to sort out what really happens. It is important to avoid diuretics for the blood pressure. Often a combination of low dose fludrocortisone with a drug like Norvasc can balance the blood pressure. If hypoglycemia is documented, an adjustment of the hydrocortisone regimen and a change in diet would be necessary.
Question: A member would like to know if it is typical to lose bone and muscle mass with Addison’s. He is 73 and he has told me several times that he has lost all his bones and muscles.
Answer: One of the major problems in the treatment of Addison’s disease is to balance the necessary replacement steroid dose for good health and well-being against the negative effects of excess steroid use. In excess, glucocorticoid hormones can cause muscle wasting and weakness as well as osteoporosis or bone thinning, which can increase the risk of fracture. Therefore, these effects are not due to the Addison’s disease itself, but from the treatment. That is one of the reasons it is important to adjust the steroid dosage to the lowest dose that keeps the patient comfortable and free of adrenal insufficiency symptoms. At the same time, it is vital to maintain good nutrition, with adequate amounts of protein, calcium and vitamin D. When osteoporosis is found, additional treatment with bisphosphonate therapy (Fosamax, Actonel or Boniva) can be useful.
Question: I was diagnosed with Addison’s disease in 1993 at the age of 46 years. It was decided in about 1998 that I should have a bone density scan done. As a result of that test, I was prescribed Fosamax (70 mg) once a week and have been on it since (also because of the steroids prescribed to treat the Addison’s). I read somewhere that perhaps the benefits of Fosamax diminish after 10 years, and could in fact become a harmful thing. Can you enlighten me on the subject? Thank you!
Answer: Fosamax and the other bisphosphonate drugs, Actonel and Boniva, are very effective in improving bone density and reducing the risk of fractures in people with osteoporosis and in people at increased risk of developing osteoporosis because they take high dose steroids or drugs like aromatase inhibitors for breast cancer. It is important to note that many doctors have prescribed these drugs incorrectly for lesser degrees of bone loss, such as osteopenia. Osteopenia is a mild bone loss that does not produce the significant risk of fracture seen in osteoporosis. It is very important for each person who has a bone density study to go over the degree of bone loss to clarify if it is osteopenia or osteoporosis. If it is osteoporosis, the bisphosphonate drugs can be used, and follow-up bone density measurements should be performed about every 2 years. Most of the time there is gradual improvement and it often plateaus at 5 to 10 years. Very often there is enough improvement that the previous oseoporosis has reverted to osteopenia. When that is seen, or if the person has been on the drug for 5 to 10 years and the effect has reached a plateau, the drug should be stopped. It is also important to note that the replacement doses of steroids used for Addison’s disease do not necessarily mean that bisphosphonate treatment is needed. I would use it only if there is evidence of osteoporosis, or if aromatase inhibitors are also needed.
Question: Would you please address in a newsletter and at any future medical conferences an issue that physicians need to alert their patients too. I recently had a cholecystectomy. Just prior to the procedure I reminded the anesthesiologist that I couldn’t have Etomidate due to my adrenal suppression. He made no comment, just looked at me; I could tell by the look on his face that he seemed unaware of the correlation between Etomidate and adrenal suppression. A recent article dealing with this topic PubMed abstract J Trauma 2008 Sep:65(3):573-9. Adrenal suppression following a single dose of Etomidate for rapid sequence induction: a prospective randomized study. I’m not trying to be a "know it all”. I just wanted to wake up alive and educate and/or remind the anesthesiologist about Etomidate. That night after surgery my BP was 70/50 and the next am 88/53. I don’t know if he gave me Etomidate or that I just didn’t dose myself with enough dexamethasone prior to the procedure and the next day. (P.S. the surgeon didn’t know about how much steroid dosing either.) Thanks.
Answer: I do not know how often Etomidate is actually used as an anesthesia adjunct, but it is well established that it can suppress adrenocortical function and lower cortisol levels for several hours. In fact, it has been used to treat adrenal cancer, but is not very useful long term because it must be given IV and causes sedation. It is very important to note that it would not affect an Addisonian taking steroids because it suppresses adrenal production, not cortisol metabolism. Therefore, it would not have produced any effect in the person with Addison’s disease getting a steroid prep for surgery. It could, however, affect a person with undiagnosed Addison’s or a person with borderline secondary adrenal insufficiency who it not given a steroid prep.
